Subcutaneous panniculitis-like T-cell lymphoma: Difference between revisions

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'''For patient information, click [[Insert page name here (patient information)|Insert page name here]]'''
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{{CMG}}; {{AE}} {{AS}}
{{CMG}}; {{AE}} {{AS}}; <bdi>Sindhuja Palle</bdi>


{{SK}}  
{{SK}} SPTCL; Subcutaneous panniculitis-like T-cell lymphoma alpha/beta subtype; Subcutaneous panniculitis-like T-cell lymphoma gamma/delta subtype; SPTCL-AB; SPTCL-GD


== Overview ==
== Overview ==
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare type of T-cell [[non-Hodgkin lymphoma]] (NHL). Subcutaneous panniculitis-like T-cell lymphoma is a cytotoxic T-cell lymphoma, which preferentially infiltrates subcutaneous tissue, primarily involving the extremities and trunk. According to the World Health Organization-European Organization for Research and Treatment of Cancer, SPTCL is classified under Primary Cutaneous T-Cell Lymphomas and expresses the TCRαβ phenotype (SPTCL-AB). TCRγδ phenotypes are now a separate entity from SPTCL, known as the Cutaneous γδ T-cell Lymphoma. On microscopic histopathological analysis, atypical lymphoid cells, [[fat necrosis]],and [[karyorrhexis]] are characteristic findings of subcutaneous panniculitis-like T-cell lymphoma. Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary and the 5-year survival rate of patients with subcutaneous panniculitis-like T-cell lymphoma is approximately 80%. Symptoms of the SPTCL include [[fever]], [[weight loss]], [[night sweats]], and painless swellings on extremities and trunk. Subcutaneous nodule biopsy is diagnostic of SPTCL. The predominant therapy for SPTCL is [[chemotherapy]]. Adjunctive [[radiotherapy]] and [[stem cell transplant]] may be required.<ref name="seer.cancer.gov">Subcutaneous panniculitis-like T-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52df/. Accessed on March 08, 2016 </ref><ref name="canadiancancer">Subcutaneous panniculitis-like T-cell lymphoma. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/subcutaneous-panniculitis-like-t-cell-lymphoma/?region=nb. Accessed on March 8, 2016 </ref>
==Classification==
Subcutaneous panniculitis-like T-cell lymphoma is classified under Primary cutaneous lymphomas and expresses the TCRαβ phenotype.<ref name="canadiancancer" />
{| style="border: 0px; font-size: 90%; margin: 3px;" align="center"
|+
! style="background: #4479BA;; color:#FFF;" | Name
! style="background: #4479BA;; color:#FFF;" | Description
|-
| style="text-align: center; padding: 5px 5px; background: #F5F5F5;" | Alpha/Beta subtype (SPTCL-AB)
| style="padding: 5px 5px; background: #F5F5F5;" |
* Now considered the only type of subcutaneous panniculitis-like T cell lymphoma
* Slow-growing (indolent) disease with a favourable prognosis.
|-
| style="text-align: center; padding: 5px 5px; background: #F5F5F5;" | Gamma/Delta subtype (SPTCL-GD)
| style="padding: 5px 5px; background: #F5F5F5;" |
* Included in the [[cutaneous T-cell lymphoma group]]. 
* More aggressive type.
* [[Hemophagocytic syndrome]] is most often associated with SPTCL-GD and has a less favourable prognosis.
|}


== Pathophysiology==
== Pathophysiology==
=== Associated Conditions===
Subcutaneous panniculitis-like T-cell lymphoma is a cytotoxic T-cell lymphoma, which preferentially infiltrates subcutaneous tissue, primarily involving the extremities and trunk.<ref name="seer.cancer.gov">Subcutaneous panniculitis-like T-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52df/. Accessed on March 08, 2016 </ref>
 
===Microscopic Pathology===
===Microscopic Pathology===
On microscopic histopathological analysis, atypical lymphoid cells, [[fat necrosis]], and [[karyorrhexis]] are characteristic findings of subcutaneous panniculitis-like T-cell lymphoma.<ref name="seer.cancer.gov">Subcutaneous panniculitis-like T-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52df/. Accessed on March 08, 2016 </ref>
[[File:Subcutaneous panniculitis-like T-cell lymphoma biopsy 1 .jpg|thumb|200x200px|center]]
[[File:Subcutaneous panniculitis-like T-cell lymphoma biospy 3.jpg|center|thumb|200x200px]]


==Causes==
==Causes==
There are no established causes for subcutaneous panniculitis-like T-cell lymphoma.
There are no established causes for subcutaneous panniculitis-like T-cell lymphoma.
==Differentiating type page name here from other Diseases==
==Differentiating SPTCL from other Diseases==
Subcutaneous panniculitis-like T-cell lymphoma must be differentiated from other diseases such as:
Subcutaneous panniculitis-like T-cell lymphoma must be differentiated from other diseases such as: <ref name="pmid191959752">{{cite journal| author=Parveen Z, Thompson K| title=Subcutaneous panniculitis-like T-cell lymphoma: redefinition of diagnostic criteria in the recent World Health Organization-European Organization for Research and Treatment of Cancer classification for cutaneous lymphomas. | journal=Arch Pathol Lab Med | year= 2009 | volume= 133 | issue= 2 | pages= 303-8 | pmid=19195975 | doi=10.1043/1543-2165-133.2.303 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19195975  }}</ref>
* Benign [[panniculitis]]
 
* [[Cutaneous T-cell lymphoma]]
* Benign [[panniculitis]]<small>
* [[Extra nodal NK-T cell lymphoma]]
*[[Extranodal NK-T-cell lymphoma]]
*[[Lupus erythematosus panniculitis]]
*[[Primary cutaneous CD56+ Natural Killer-like T-Cell lymphoma]]
*[[Primary cutaneous anaplastic large cell lymphoma]]
*[[Mycosis Fungoides]]
*[[Blastic plasmacytoid dendritic cell neoplasm]]
*[[Primary cutaneous lymphomas]]
 
==Differentiating Blastic NK cell Lymphoma from other Diseases==
'''<small>ABBREVIATIONS'''
 
'''N/A''': Not available, '''NL''': Normal, '''FISH''': Fluorescence in situ hybridization, '''PCR''': Polymerase chain reaction, '''LDH''': Lactate dehydrogenase, '''PUD''': Peptic ulcer disease, '''EPO''': Erythropoietin, '''LFTs''': Liver function tests, '''RFTs''': Renal function tests, '''LAP''': Leukocyte alkaline phosphatase, '''LAD''': Leukocyte alkaline dehydrgenase, '''WBCs''': White blood cells. </small>
<small>
{| class="wikitable"
! colspan="2" rowspan="4" style="background:#4479BA; color: #FFFFFF;" align="center" + |Myeloproliferative neoplasms (MPN)
! colspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" + |Clinical manifestations
! colspan="11" style="background:#4479BA; color: #FFFFFF;" align="center" + |Diagnosis
! rowspan="4" style="background:#4479BA; color: #FFFFFF;" align="center" + |Other features
|-
! rowspan="3" style="background:#4479BA; color: #FFFFFF;" align="center" + |Symptoms
! rowspan="3" style="background:#4479BA; color: #FFFFFF;" align="center" + |Physical examination
! colspan="9" style="background:#4479BA; color: #FFFFFF;" align="center" + |CBC & Peripheral smear
! rowspan="3" style="background:#4479BA; color: #FFFFFF;" align="center" + |Bone marrow biopsy
! rowspan="3" style="background:#4479BA; color: #FFFFFF;" align="center" + |Other investigations
|-
! colspan="7" style="background:#4479BA; color: #FFFFFF;" align="center" + |WBCs
! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" + |Hb
! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" + |Plat-<br>elets
|-
! style="background:#4479BA; color: #FFFFFF;" align="center" + |<small>Leuko-cytes</small>
! style="background:#4479BA; color: #FFFFFF;" align="center" + |<small>Blasts</small>
! style="background:#4479BA; color: #FFFFFF;" align="center" + |<small>Left<br>shift</small>
! style="background:#4479BA; color: #FFFFFF;" align="center" + |<small>Baso-<br>phils</small>
! style="background:#4479BA; color: #FFFFFF;" align="center" + |<small>Eosino-<br>phils</small>
! style="background:#4479BA; color: #FFFFFF;" align="center" + |<small>Mono-<br>cytes</small>
! style="background:#4479BA; color: #FFFFFF;" align="center" + |<small>Others</small>
|-
| colspan="2" |[[Chronic myeloid leukemia]]<br>([[CML]]), [[BCR/ABL|BCR-ABL1]]+<ref name="pmid9012696">{{cite journal |vauthors=Savage DG, Szydlo RM, Goldman JM |title=Clinical features at diagnosis in 430 patients with chronic myeloid leukaemia seen at a referral centre over a 16-year period |journal=Br. J. Haematol. |volume=96 |issue=1 |pages=111–6 |date=January 1997 |pmid=9012696 |doi= |url=}}</ref><ref name="pmid26434969">{{cite journal |vauthors=Thompson PA, Kantarjian HM, Cortes JE |title=Diagnosis and Treatment of Chronic Myeloid Leukemia in 2015 |journal=Mayo Clin. Proc. |volume=90 |issue=10 |pages=1440–54 |date=October 2015 |pmid=26434969 |pmc=5656269 |doi=10.1016/j.mayocp.2015.08.010 |url=}}</ref>
|
* <small>Asymptomatic</small>
* <small>Constitutional</small>
* <small>[[Hyperviscosity]]  and/or [[anemia]] related</small>
* <small>[[Bleeding]]</small>
* <small>[[Infection]]</small>
|
* <small>[[Splenomegaly]]<br>(46–76%)</small>
* <small>[[Purpura]]</small>
* <small>[[Anemia]] related</small>
* <small>[[Priapism]]</small>
|↑
|<small><2%</small>
| +
|<small>↑</small>
|<small>↑</small>
|<small>↑</small>
|<small>N/A</small>
|<small>[[Anemia|↓]]</small>
|<small>NL</small>
|
* <small>Hypercellurarity with ↑ [[granuloscytosis]] and ↓ [[erythrocytosis]]</small>
 
* <small>[[Fibrosis]]</small>
|
* <small>[[FISH]] for t(9;22)(q34;q11.2)</small>
* <small>[[Reverse transcriptase]] quantitative [[PCR]] (RQ-PCR) for BCR-ABL</small>
|
* <small>[[Granulocytic]] [[dysplasia]] is minimal/absent</small>
* <small>May present with [[blast]] crisis</small>
* <small>Absolute [[leukocytosis]]</small>  <small>(median of 100,000/µL)</small>
* <small>Classic [[myelocyte]] bulge</small>
* <small>[[thrombocytopenia]] indicates advanced stage</small>
|-
| colspan="2" |[[Chronic neutrophilic leukemia]] ([[CNL]])<ref name="pmid29440636">{{cite journal |vauthors=Szuber N, Tefferi A |title=Chronic neutrophilic leukemia: new science and new diagnostic criteria |journal=Blood Cancer J |volume=8 |issue=2 |pages=19 |date=February 2018 |pmid=29440636 |pmc=5811432 |doi=10.1038/s41408-018-0049-8 |url=}}</ref><ref name="pmid28028025">{{cite journal |vauthors=Maxson JE, Tyner JW |title=Genomics of chronic neutrophilic leukemia |journal=Blood |volume=129 |issue=6 |pages=715–722 |date=February 2017 |pmid=28028025 |pmc=5301820 |doi=10.1182/blood-2016-10-695981 |url=}}</ref><ref name="pmid26366092">{{cite journal |vauthors=Menezes J, Cigudosa JC |title=Chronic neutrophilic leukemia: a clinical perspective |journal=Onco Targets Ther |volume=8 |issue= |pages=2383–90 |date=2015 |pmid=26366092 |pmc=4562747 |doi=10.2147/OTT.S49688 |url=}}</ref>
|
* <small>Asymptomatic</small>
* <small>Constitutional symptoms</small>
* <small>[[Bleeding]]</small>
* <small>[[Infection]]</small>
|
* <small>[[Splenomegaly]]</small>
* <small>[[Heptomegaly]]</small>
* <small>[[Purpura]]</small>
* <small>[[Anemia]] related</small>
|↑
|<small>Minimal</small>
| +
|<small>NL</small>
|<small>NL</small>
|<small>NL</small>
|
* <small>↑  [[LDH]]</small>
* <small>↑ [[B12]] levels</small>
|<small>[[Anemia|↓]]</small>
|<small>[[Thrombocytopenia|↓]]</small>
|
* <small>Uniforme and intense hypercellularity with minimal to none [[fibrosis]]</small>
* <small>[[Neutrophil]] toxic granulations and [[Dohle bodies]]</small>
|
* <small>[[FISH]]</small>
* <small>Imaging for [[hepatosplenomegaly]]</small>
|
* <small>Associationed with [[polycythemia vera]] and [[plasma cell disorders]]</small>
* <small>[[Leukocytosis]] with<br>[[chronic]] [[neutrophilia]]</small>
|-
| colspan="2" |[[Polycythemia vera]]<br>([[PV]])<ref name="pmid29194068">{{cite journal |vauthors=Vannucchi AM, Guglielmelli P, Tefferi A |title=Polycythemia vera and essential thrombocythemia: algorithmic approach |journal=Curr. Opin. Hematol. |volume=25 |issue=2 |pages=112–119 |date=March 2018 |pmid=29194068 |doi=10.1097/MOH.0000000000000402 |url=}}</ref><ref name="pmid30252337">{{cite journal |vauthors=Pillai AA, Babiker HM |title= |journal= |volume= |issue= |pages= |date= |pmid=30252337 |doi= |url=}}</ref><ref name="pmid30281843">{{cite journal |vauthors=Tefferi A, Barbui T |title=Polycythemia vera and essential thrombocythemia: 2019 update on diagnosis, risk-stratification and management |journal=Am. J. Hematol. |volume=94 |issue=1 |pages=133–143 |date=January 2019 |pmid=30281843 |doi=10.1002/ajh.25303 |url=}}</ref><ref name="pmid28028026">{{cite journal |vauthors=Rumi E, Cazzola M |title=Diagnosis, risk stratification, and response evaluation in classical myeloproliferative neoplasms |journal=Blood |volume=129 |issue=6 |pages=680–692 |date=February 2017 |pmid=28028026 |pmc=5335805 |doi=10.1182/blood-2016-10-695957 |url=}}</ref>
|
* <small>Constitutional</small>
 
*  <small>[[Thromboembolism]]<br>and [[bleeding]]</small>
 
* <small>[[Pruritus]] after<br>a warm bath</small>
* <small>[[PUD]] related</small>
|
* <small>Facial ruddiness</small>
* <small>Related to underlying cause</small>
* <small>[[Splenomegaly]]</small>
* <small>[[Renal]] [[bruit]]</small>
|<small>NL or ↑</small>
|<small>None</small>
| -
|<small>↑ or ↓</small>
|<small>NL or ↑</small>
|<small>NL</small>
|
* <small>↓ Serum [[ferritin]]</small>
* <small>↓ [[Folate]] levels</small>
* <small>↑↑ [[B12]] levels</small>
|<small>↑↑</small>
 
|<small>NL</small>
|
* <small>Hypercellularity for age with tri-lineage growth</small>
 
* <small>[[Myelofibrosis]] (in up to 20% of patients)</small>
|
* <small>[[Radioisotope]] studies</small>
* <small>[[Serum]] [[EPO]] levels</small>
* <small>[[LFTs]]</small>
* <small>[[RFTs]]</small>
* <small>[[Imaging]] studies</small>
|
* <small>May transform into [[myelofibrosis]] or [[leukemia]]</small>
|-
| colspan="2" |[[Primary myelofibrosis]] ([[PMF]])<ref name="pmid26891375">{{cite journal |vauthors=Cervantes F, Correa JG, Hernandez-Boluda JC |title=Alleviating anemia and thrombocytopenia in myelofibrosis patients |journal=Expert Rev Hematol |volume=9 |issue=5 |pages=489–96 |date=May 2016 |pmid=26891375 |doi=10.1586/17474086.2016.1154452 |url=}}</ref><ref>{{cite book | last = Hoffman | first = Ronald | title = Hematology : basic principles and practice | publisher = Elsevier | location = Philadelphia, PA | year = 2018 | isbn = 9780323357623 }}</ref><ref name="pmid16919893">{{cite journal |vauthors=Michiels JJ, Bernema Z, Van Bockstaele D, De Raeve H, Schroyens W |title=Current diagnostic criteria for the chronic myeloproliferative disorders (MPD) essential thrombocythemia (ET), polycythemia vera (PV) and chronic idiopathic myelofibrosis (CIMF) |journal=Pathol. Biol. |volume=55 |issue=2 |pages=92–104 |date=March 2007 |pmid=16919893 |doi=10.1016/j.patbio.2006.06.002 |url=}}</ref><ref>{{cite book | last = Hoffman | first = Ronald | title = Hematology : basic principles and practice | publisher = Elsevier | location = Philadelphia, PA | year = 2018 | isbn = 9780323357623 }}</ref>
|
* <small>Constitutional</small>
* <small>[[Anemia]] related</small>
* <small>[[Bleeding]]</small>
* <small>[[Infection]]</small>
* <small>[[Abdominal]] [[Pain]]</small>
|
* <small>[[Hepatosplenomegaly]]</small>
* <small>[[Petechiae]] & [[ecchymoses]]</small>
* <small>Abdominal distension</small>
* <small>[[Lymphadenopathy]]</small>
|↓
|<small>[[Erythroblasts]]</small>
| -
|<small>Absent</small>
|<small>NL</small>
|<small>NL</small>
|
* <small>↑ [[LAP]]</small>
* <small>↑ [[LAD]]</small>
* ↑ <small>[[Uric acid]]</small>
* <small>↑ [[B12]] levels</small>
|[[Anemia|<small>↓</small>]]
|<small>[[Thrombocytopenia|↓]]</small>
|
* <small>Variable with [[fibrosis]] or hypercellularity</small>
|
* <small>JAK2 mutation</small>
* <small>[[CALR]] [[mutation]]</small>
* <small>[[MPL]] [[mutation]]</small>
|
* <small>[[Bone marrow]] aspiration shows a dry tap</small>
* <small>Variable with [[leukocytosis]] or [[leukopenia]]</small>
|-
| colspan="2" |[[Essential thrombocythemia]] ([[ET]])<ref name="pmidhttp://dx.doi.org/10.1182/blood-2007-04-083501">{{cite journal| author=Schmoldt A, Benthe HF, Haberland G| title=Digitoxin metabolism by rat liver microsomes. | journal=Biochem Pharmacol | year= 1975 | volume= 24 | issue= 17 | pages= 1639-41 | pmid=http://dx.doi.org/10.1182/blood-2007-04-083501 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10  }} </ref><ref>{{Cite journal
| author = [[Daniel A. Arber]], [[Attilio Orazi]], [[Robert Hasserjian]], [[Jurgen Thiele]], [[Michael J. Borowitz]], [[Michelle M. Le Beau]], [[Clara D. Bloomfield]], [[Mario Cazzola]] & [[James W. Vardiman]]
| title = The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia
| journal = [[Blood]]
| volume = 127
| issue = 20
| pages = 2391–2405
| year = 2016
| month = May
| doi = 10.1182/blood-2016-03-643544
| pmid = 27069254
}}</ref><ref>{{Cite journal
| author = [[A. Tefferi]], [[R. Fonseca]], [[D. L. Pereira]] & [[H. C. Hoagland]]
| title = A long-term retrospective study of young women with essential thrombocythemia
| journal = [[Mayo Clinic proceedings]]
| volume = 76
| issue = 1
| pages = 22–28
| year = 2001
| month = January
| doi = 10.4065/76.1.22
| pmid = 11155408
}}</ref>
|
* <small>Headache</small>
* <small>[[Dizziness]]</small>
* <small>[[Visual]] disturbances</small>
* <small>[[Priapism]]</small>
* <small>Acute [[chest pain]]</small>
|
* <small>[[Splenomegaly]]</small>
* <small>[[Skin]] [[bruises]]</small>
|
<small>NL or ↑</small>
|
<small>None</small>
| -
|
<small>↓ or absent</small>
|
<small>NL</small>
|
<small>NL</small>
|
* <small>N/A</small>
|
[[Anemia|<small>↓</small>]]
|
<small>↑↑</small>
|
* <small>Normal/Hypercellular</small>
|
* <small>[[JAK2]] [[mutation]]</small>
* <small>[[CALR]] [[mutation]]</small>
* <small>[[MPL]] [[mutation]]</small>
|
* <small>[[Thrombosis]]</small>
* <small>[[Hemorrhage]]</small>
* <small>[[Pregnancy]] loss</small>
|-
| colspan="2" |[[Chronic eosinophilic leukemia]],<br>not otherwise specified<br>(NOS)<ref name="pmid27512192">{{cite journal |vauthors=Vidyadharan S, Joseph B, Nair SP |title=Chronic Eosinophilic Leukemia Presenting Predominantly with Cutaneous Manifestations |journal=Indian J Dermatol |volume=61 |issue=4 |pages=437–9 |date=2016 |pmid=27512192 |pmc=4966405 |doi=10.4103/0019-5154.185716 |url=}}</ref><ref name="pmid29892549">{{cite journal |vauthors=Hofmans M, Delie A, Vandepoele K, Van Roy N, Van der Meulen J, Philippé J, Moors I |title=A case of chronic eosinophilic leukemia with secondary transformation to acute myeloid leukemia |journal=Leuk Res Rep |volume=9 |issue= |pages=45–47 |date=2018 |pmid=29892549 |pmc=5993353 |doi=10.1016/j.lrr.2018.04.001 |url=}}</ref><ref name="pmid23662039">{{cite journal |vauthors=Yamada Y, Rothenberg ME, Cancelas JA |title=Current concepts on the pathogenesis of the hypereosinophilic syndrome/chronic eosinophilic leukemia |journal=Transl Oncogenomics |volume=1 |issue= |pages=53–63 |date=2006 |pmid=23662039 |pmc=3642145 |doi= |url=}}</ref><ref name="pmid27722133">{{cite journal |vauthors=Kim TH, Gu HJ, Lee WI, Lee J, Yoon HJ, Park TS |title=Chronic eosinophilic leukemia with FIP1L1-PDGFRA rearrangement |journal=Blood Res |volume=51 |issue=3 |pages=204–206 |date=September 2016 |pmid=27722133 |doi=10.5045/br.2016.51.3.204 |url=}}</ref>
|
* <small>Constitutional</small>
* <small>[[Rash]]</small>
* <small>[[Rhinitis]]</small>
* <small>[[Gastritis]]</small>
* <small>[[Thromboembolism]]<br>related</small>
|
* <small>[[Hypertension]]</small>
* <small>[[Eczema]], [[mucosal]] [[ulcers]], [[erythema]]</small>
* <small>[[Angioedema]]</small>
 
* <small>[[Ataxia]]</small>
* <small>[[Anemia]]</small>
* <small>[[Lymphadenopathy]]</small>
* <small>[[Hepatosplenomegaly]]</small>
|<small>[[Leukocytosis|↑]]</small>
|<small>Present</small>
| +
|<small>↑</small>
|<small>↑↑</small>
|<small>↑</small>
|
* <small>↑ [[B12]] levels</small>
* ↑ <small>[[LDH]]</small>
|<small>[[Anemia|↓]]</small>
|<small>[[Thrombocytopenia|↓]]</small>
|
* <small>Hypercelluar with  ↑ [[eosinophilic]] precursors, ↑ [[eosinophils]], and atypical [[mononuclear cells]]</small>
|
* <small>[[FISH]]</small>
* <small>Cytogenetic analysis of purified [[eosinophils]] and [[X-chromosome]] inactivation analysis</small>
|
* <small>[[Heart failure]]</small> <small>[[Lung fibrosis]]</small>
* <small>[[Encephalopathy]]</small>
* <small>[[Erythema annulare centrifugam]]</small>
|-
| colspan="2" |[[Myeloproliferative neoplasm|MPN]],<br>unclassifiable
|<small>
*Similar to other<br>[[Myeloproliferative neoplasm|myeloproliferative neoplasms]]</small>
|<small>
*Similar to other<br>[[Myeloproliferative neoplasm|myeloproliferative neoplasms]]</small>
|<small>[[Leukocytosis|↑]]</small>
|<small>Variable</small>
|<small>↑ or ↓</small>
|<small>↑ or ↓</small>
|<small>↑ or ↓</small>
|
* <small>May resemble other<br>[[myeloproliferative neoplasms]]</small>
|<small>↓</small>
|<small>↑</small>
|
* <small>↑ [[megakaryocyte]] proliferation with variable hypercellularity in [[granulocytic]] or [[erythrocytic]] cell lines</small>
|
* <small>N/A</small>
|<small>
*Similar to other [[myeloprolifeartive neoplasms]] but do not fulfil the criteria to be classified to a specific type</small>
|-
| colspan="2" |[[Mastocytosis]]<ref name="pmid24262698">{{cite journal |vauthors=Carter MC, Metcalfe DD, Komarow HD |title=Mastocytosis |journal=Immunol Allergy Clin North Am |volume=34 |issue=1 |pages=181–96 |date=February 2014 |pmid=24262698 |pmc=3863935 |doi=10.1016/j.iac.2013.09.001 |url=}}</ref><ref name="pmid29494109">{{cite journal |vauthors=Macri A, Cook C |title= |journal= |volume= |issue= |pages= |date= |pmid=29494109 |doi= |url=}}</ref><ref name="pmid25031064">{{cite journal |vauthors=Lladó AC, Mihon CE, Silva M, Galzerano A |title=Systemic mastocytosis - a diagnostic challenge |journal=Rev Bras Hematol Hemoter |volume=36 |issue=3 |pages=226–9 |date=2014 |pmid=25031064 |pmc=4109736 |doi=10.1016/j.bjhh.2014.03.003 |url=}}</ref><ref name="pmid28031180">{{cite journal |vauthors=Valent P, Akin C, Metcalfe DD |title=Mastocytosis: 2016 updated WHO classification and novel emerging treatment concepts |journal=Blood |volume=129 |issue=11 |pages=1420–1427 |date=March 2017 |pmid=28031180 |pmc=5356454 |doi=10.1182/blood-2016-09-731893 |url=}}</ref>
|
* <small>Constitutional</small>
 
* <small>[[Pruritus]] & [[Flushing]]</small>
* <small>[[Urticaria]] & [[Blisters]]</small>
 
* <small>[[Hypotension]] & [[PUD]]</small>
* <small>[[Bleeding]]</small>
* <small>[[Bronchoconstriction]]</small>
 
|
* <small>[[Mastocytosis exanthema]]</small>
* <small>[[Blistering]]</small>
* <small>[[Swelling]]</small>
* <small>[[Lymphadenopathy]]</small>
* <small>[[Bleeding]]</small>
* <small>[[Fibrosis]]</small>
|<small>[[Leukocytosis|↑]]</small>
|<small>None</small>
| -
|<small>NL</small>
|<small>[[Eosinophilia|↑]]</small>
|<small>NL</small>
|
* <small>↑ [[Alkaline phosphatase]]</small>
* <small>↑ [[LDH]]</small>
|<small>[[Anemia|↓]]</small>
|<small>↓ or ↑</small>
|
* <small>Multifocal dense infiltrates of [[mast cells]] with atypical [[morphology]] in >25 %</small>
|
* <small>Cytogenetic analysis for [[c-KIT]] [[receptor]] [[mutations]]</small>
* <small>[[Serum]] [[tryptase]] levels</small>
* <small>24-hour urine test for N-methyl [[histamine]] and 11-beta-[[prostaglandine]]</small>
|
* <small>Skin most commonly involved</small>
* <small>Susceptibility to [[anaphylaxix]]</small>
* <small>[[Osteoporosis]]</small>
|-
| colspan="2" |[[Myeloid]]/[[lymphoid]] [[neoplasms]]<br>with [[eosinophilia]] and rearrangement<br>of [[PDGFR|PDGFRA]], [[PDGFRB]], or [[FGFR1]],<br>or with [[PCM1]]-[[JAK2]]<ref name="KumarChen2015">{{cite journal|last1=Kumar|first1=Kirthi R.|last2=Chen|first2=Weina|last3=Koduru|first3=Prasad R.|last4=Luu|first4=Hung S.|title=Myeloid and Lymphoid Neoplasm With Abnormalities of FGFR1 Presenting With Trilineage Blasts and RUNX1 Rearrangement|journal=American Journal of Clinical Pathology|volume=143|issue=5|year=2015|pages=738–748|issn=1943-7722|doi=10.1309/AJCPUD6W1JLQQMNA}}</ref><ref>{{Cite journal
| author = [[Paolo Strati]], [[Guilin Tang]], [[Dzifa Y. Duose]], [[Saradhi Mallampati]], [[Rajyalakshmi Luthra]], [[Keyur P. Patel]], [[Mohammad Hussaini]], [[Abu-Sayeef Mirza]], [[Rami S. Komrokji]], [[Stephen Oh]], [[John Mascarenhas]], [[Vesna Najfeld]], [[Vivek Subbiah]], [[Hagop Kantarjian]], [[Guillermo Garcia-Manero]], [[Srdan Verstovsek]] & [[Naval Daver]]
| title = Myeloid/lymphoid neoplasms with FGFR1 rearrangement
| journal = [[Leukemia & lymphoma]]
| volume = 59
| issue = 7
| pages = 1672–1676
| year = 2018
| month = July
| doi = 10.1080/10428194.2017.1397663
| pmid = 29119847
}}</ref><ref>{{Cite journal
| author = [[Ximena Montenegro-Garreaud]], [[Roberto N. Miranda]], [[Alexandra Reynolds]], [[Guilin Tang]], [[Sa A. Wang]], [[Mariko Yabe]], [[Wei Wang]], [[Lianghua Fang]], [[Carlos E. Bueso-Ramos]], [[Pei Lin]], [[L. Jeffrey Medeiros]] & [[Xinyan Lu]]
| title = Myeloproliferative neoplasms with t(8;22)(p11.2;q11.2)/BCR-FGFR1: a meta-analysis of 20 cases shows cytogenetic progression with B-lymphoid blast phase
| journal = [[Human pathology]]
| volume = 65
| pages = 147–156
| year = 2017
| month = July
| doi = 10.1016/j.humpath.2017.05.008
| pmid = 28551329
}}</ref><ref>{{Cite journal
| author = [[Paola Villafuerte-Gutierrez]], [[Montserrat Lopez Rubio]], [[Pilar Herrera]] & [[Eva Arranz]]
| title = A Case of Myeloproliferative Neoplasm with BCR-FGFR1 Rearrangement: Favorable Outcome after Haploidentical Allogeneic Transplantation
| journal = [[Case reports in hematology]]
| volume = 2018
| pages = 5724960
| year = 2018
| month =
| doi = 10.1155/2018/5724960
| pmid = 30647980
}}</ref>
|
* <small>Asymptomatic</small>
* <small>Constitutional</small>
* <small>[[Rash]]</small>
* <small>[[Cough]] & breathlessness</small>
* <small>[[Peripheral neuropathy]]/<br>[[encephalopathy]]</small>
|
* <small>[[Fever]]</small>
* <small>[[Lymphadenopathy]]</small>
| ↑
| <small>NL</small>
| -
| <small>NL</small>
| <small>[[Eosinophilia|↑]]</small>
|<small>[[Monocytosis|↑]]</small>
|
* <small>None</small>
| <small>NL</small>
| <small>[[Thrombocytopenia|↓]]</small>
|
* <small>[[Myeloid]] expansion with [[eosinophilia]]</small>
|
* <small>[[FISH]] shows t(8;13) and t(8;22)</small>
|
* <small>May present or evolve into [[acute myeloid]] or [[lymphoblastic leukemia]]</small>
* <small>[[Leukocytosis]] (30 - 59 × 10<sup>9</sup>/L</small>
|-
| colspan="2" |B-lymphoblastic leukemia/lymphoma<ref name="pmid30107017">{{cite journal |vauthors=Kamiya-Matsuoka C, Garciarena P, Amin HM, Tremont-Lukats IW, de Groot JF |title=B lymphoblastic leukemia/lymphoma presenting as seventh cranial nerve palsy |journal=Neurol Clin Pract |volume=3 |issue=6 |pages=532–534 |date=December 2013 |pmid=30107017 |pmc=6082360 |doi=10.1212/CPJ.0b013e3182a78ef0 |url=}}</ref><ref name="pmid29029550">{{cite journal |vauthors=Zhang X, Rastogi P, Shah B, Zhang L |title=B lymphoblastic leukemia/lymphoma: new insights into genetics, molecular aberrations, subclassification and targeted therapy |journal=Oncotarget |volume=8 |issue=39 |pages=66728–66741 |date=September 2017 |pmid=29029550 |pmc=5630450 |doi=10.18632/oncotarget.19271 |url=}}</ref>
|
* <small>Constitutional</small>
* <small>[[Anemia]] related</small>
* <small>[[Bleeding]]</small>
* <small>[[Infection]]</small>
* <small>[[Bone pain]]</small>
|
* <small>[[Pallor]]</small>
* <small>[[Petechiae]]</small>
* <small>[[Organomegaly]]</small>
* <small>[[Lymphadenopathy]]</small>
|<small>NL or ↑</small>
|<small>>25%</small>
|<small>N/A</small>
|<small>↑ or ↓</small>
|<small>↑ or ↓</small>
|<small>↑ or ↓</small>
|
* <small>[[Auer rod|Auer bodies]]</small>
|[[Anemia|<small>↓</small>]]
|<small>[[Thrombocytopenia|↓]]</small>
|
* <small>Hypercellular with [[blast]] infilteration</small><small>with or without [[myelodysplasia]]</small>
|
* <small>[[Cytogenetic analysis]]</small>
* <small>[[Flow cytometry]]</small>
 
* <small>[[FISH]]</small>
|
* <small>May present as [[Extramedullary myeloid tumor|extramedullary]] disease ([[Extramedullary Myeloid Cell Tumor|Myeloid sarcoma]])</small>
|-
| colspan="2" |[[Myelodysplastic syndrome|Myelodysplastic syndromes]]<br>([[MDS]])<ref name="pmid24300826">{{cite journal |vauthors=Germing U, Kobbe G, Haas R, Gattermann N |title=Myelodysplastic syndromes: diagnosis, prognosis, and treatment |journal=Dtsch Arztebl Int |volume=110 |issue=46 |pages=783–90 |date=November 2013 |pmid=24300826 |pmc=3855821 |doi=10.3238/arztebl.2013.0783 |url=}}</ref><ref name="pmid26769228">{{cite journal |vauthors=Gangat N, Patnaik MM, Tefferi A |title=Myelodysplastic syndromes: Contemporary review and how we treat |journal=Am. J. Hematol. |volume=91 |issue=1 |pages=76–89 |date=January 2016 |pmid=26769228 |doi=10.1002/ajh.24253 |url=}}</ref>
|
* <small>Constitutional</small>
* <small>[[Anemia]] related</small>
* <small>[[Bleeding]]</small>
* <small>[[Infection]]</small>
|
* <small>[[Pallor]]</small>
* <small>[[Petechiae]]</small>
* <small>[[Organomegaly]]</small>
|[[Leukopenia|<small>↓</small>]]
|<small>Variable</small>
| -
|<small>↓</small>
|<small>↓</small>
|<small>↓</small>
|
* <small>Macro-ovalocytes</small>
* <small>Basophilic stippling</small>
* [[Howell-Jolly body|<small>Howell-Jolly body</small>]]
|[[Anemia|<small>↓</small>]]
|<small>[[Thrombocytopenia|↓]]</small>
|
* <small>Hypercellular</small><small>/ normocellular [[bone marrow]] with [[Dysplastic change|dysplastic]] changes</small>
|
* <small>[[Cytogenetic analysis]]</small>
* <small>[[Flow cytometry]]</small>
|
* <small>[[Leukemia]] transformation</small>
* <small>Acquired pseudo-Pelger-Huët anomaly</small>
|-
| colspan="2" |[[Acute myeloid leukemia]] ([[AML]])<br>and related [[neoplasms]]<ref name="pmid3864727">{{cite journal |vauthors=Islam A, Catovsky D, Goldman JM, Galton DA |title=Bone marrow biopsy changes in acute myeloid leukaemia. I: Observations before chemotherapy |journal=Histopathology |volume=9 |issue=9 |pages=939–57 |date=September 1985 |pmid=3864727 |doi= |url=}}</ref><ref name="pmid17587881">{{cite journal |vauthors=Orazi A |title=Histopathology in the diagnosis and classification of acute myeloid leukemia, myelodysplastic syndromes, and myelodysplastic/myeloproliferative diseases |journal=Pathobiology |volume=74 |issue=2 |pages=97–114 |date=2007 |pmid=17587881 |doi=10.1159/000101709 |url=}}</ref>
|
* <small>Constitutional</small>
* <small>[[Anemia]] related</small>
* [[Bleeding|<small>Bleeding</small>]]
* [[Bone pain|<small>Bone pain</small>]]
* [[Joint pain|<small>Joint pain</small>]]
* [[Infections|<small>Infections</small>]]
|
* <small>[[Infection]] related</small>


== Epidemiology and Demographics ==
* [[Pallor|<small>Pallor</small>]]
===Age===
* [[Chloroma|<small>Leukemia cutis</small>]]
The incidence of subcutaneous panniculitis-like T-cell lymphoma increases with age; the median age at diagnosis is 35 years.
* <small>[[Bruising]] & [[Petechia|petechiae]]</small>
===Gender===
* <small>[[Lymphadenopathy]]</small>
Females are more commonly affected with subcutaneous panniculitis-like T-cell lymphoma than males.
 
== Risk Factors ==
* [[Hepatomegaly|<small>Hepatosplenomegaly</small>]]
There are no established risk factors for subcutaneous panniculitis-like T-cell lymphoma.
|<small>NL or ↑</small>
== Screening ==
|<small>↑</small>
According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for subcutaneous panniculitis-like T-cell lymphoma.<ref name=uspreventive> Recommendations. U.S Preventive Services Task Force. Accessed on March 8, 2016</ref>
|<small>N/A</small>
== Natural History, Complications and Prognosis==
|<small>↑ or ↓</small>
|<small>↑ or ↓</small>
|<small>↑ or ↓</small>
|
* <small>↑ [[Potassium]]</small> 
* <small>↑ [[Uric acid]]</small>
* <small>↑ [[Phosphorus]]</small>
* <small>↓ [[Calcium]]</small>
* <small>↑ [[LDH]]</small>
|[[Anemia|<small>↓</small>]]
|<small>[[Thrombocytopenia|↓]]</small>
|
* <small>Increased  immature[[myeloid]] [[cells]]</small>
<small>with [[Dysplastic change|dysplasia]]</small>     
|
* <small>[[Cytogenetic analysis]]</small>
* <small>[[Flow cytometry]]</small>
* <small>[[FISH]]</small>
|
* <small>Common in [[Down syndrome]]</small>
|-
| colspan="2" |[[Blastic plasmacytoid dendritic cell neoplasm|Blastic plasmacytoid<br>dendritic cell neoplasm]]<ref>{{Cite journal
| author = [[F. Julia]], [[T. Petrella]], [[M. Beylot-Barry]], [[M. Bagot]], [[D. Lipsker]], [[L. Machet]], [[P. Joly]], [[O. Dereure]], [[M. Wetterwald]], [[M. d'Incan]], [[F. Grange]], [[J. Cornillon]], [[G. Tertian]], [[E. Maubec]], [[P. Saiag]], [[S. Barete]], [[I. Templier]], [[F. Aubin]] & [[S. Dalle]]
| title = Blastic plasmacytoid dendritic cell neoplasm: clinical features in 90 patients
| journal = [[The British journal of dermatology]]
| volume = 169
| issue = 3
| pages = 579–586
| year = 2013
| month = September
| doi = 10.1111/bjd.12412
| pmid = 23646868
}}</ref><ref>{{Cite journal
| author = [[Livio Pagano]], [[Caterina Giovanna Valentini]], [[Alessandro Pulsoni]], [[Simona Fisogni]], [[Paola Carluccio]], [[Francesco Mannelli]], [[Monia Lunghi]], [[Gianmatteo Pica]], [[Francesco Onida]], [[Chiara Cattaneo]], [[Pier Paolo Piccaluga]], [[Eros Di Bona]], [[Elisabetta Todisco]], [[Pellegrino Musto]], [[Antonio Spadea]], [[Alfonso D'Arco]], [[Stefano Pileri]], [[Giuseppe Leone]], [[Sergio Amadori]] & [[Fabio Facchetti]]
| title = Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: an Italian multicenter study
| journal = [[Haematologica]]
| volume = 98
| issue = 2
| pages = 239–246
| year = 2013
| month = February
| doi = 10.3324/haematol.2012.072645
| pmid = 23065521
}}</ref><ref>{{Cite journal
| author = [[Joseph D. Khoury]]
| title = Blastic Plasmacytoid Dendritic Cell Neoplasm
| journal = [[Current hematologic malignancy reports]]
| volume = 13
| issue = 6
| pages = 477–483
| year = 2018
| month = December
| doi = 10.1007/s11899-018-0489-z
| pmid = 30350260
}}</ref><ref>{{Cite journal
| author = [[Shinichiro Sukegawa]], [[Mamiko Sakata-Yanagimoto]], [[Ryota Matsuoka]], [[Haruka Momose]], [[Yusuke Kiyoki]], [[Masayuki Noguchi]], [[Naoya Nakamura]], [[Rei Watanabe]], [[Manabu Fujimoto]], [[Yasuhisa Yokoyama]], [[Hidekazu Nishikii]], [[Takayasu Kato]], [[Manabu Kusakabe]], [[Naoki Kurita]], [[Naoshi Obara]], [[Yuichi Hasegawa]] & [[Shigeru Chiba]]
| title = &#91;Blastic plasmacytoid dendritic cell neoplasm accompanied by chronic myelomonocytic leukemia successfully treated with azacitidine&#93;
| journal = <nowiki>[[[Rinsho ketsueki] The Japanese journal of clinical hematology]]</nowiki>
| volume = 59
| issue = 12
| pages = 2567–2573
| year = 2018
| month =  
| doi = 10.11406/rinketsu.59.2567
| pmid = 30626790
}}</ref>
|
* <small>[[Cutaneous]] symptoms (brown/purple nodular lesions) on [[face]], [[scalp]], [[lower limb]] & [[trunk]]</small>
|
* <small>Brown/violaceous [[bruise]] like lesions</small>
* <small>[[Lymphadenopathy]]</small>
* <small>[[Splenomegaly]]</small>
| <small>NL</small>
| <small>↑</small>
|
|<small>NL</small>
| <small>NL</small>
| <small>NL</small>
|
* <small>[[Neutropenia]]</small>
| [[Anemia|<small>↓</small>]]
| <small>[[Thrombocytopenia|↓]]</small>
|
* <small>[[Malignant|Malignant cells]]</small>
|
* <small>[[Immunohistochemistry]] or [[flow cytometry]] for [[CD4]] & [[CD56]]</small>
|
* <small>TdT expression positive</small>
* <small>May develop [[chronic myelomonocytic leukemia]] (CMML)</small>
|-
| rowspan="4" |[[Myelodysplastic]]<br>/[[Myeloproliferative neoplasm|myeloproliferative<br>neoplasms]] (MDS/MPN)
|<small>[[Chronic myelomonocytic leukemia]] (CMML)</small><ref name="pmid27185207">{{cite journal |vauthors=Patnaik MM, Tefferi A |title=Chronic myelomonocytic leukemia: 2016 update on diagnosis, risk stratification, and management |journal=Am. J. Hematol. |volume=91 |issue=6 |pages=631–42 |date=June 2016 |pmid=27185207 |doi=10.1002/ajh.24396 |url=}}</ref>
: <ref name="pmid22615103">{{cite journal |vauthors=Parikh SA, Tefferi A |title=Chronic myelomonocytic leukemia: 2012 update on diagnosis, risk stratification, and management |journal=Am. J. Hematol. |volume=87 |issue=6 |pages=610–9 |date=June 2012 |pmid=22615103 |doi=10.1002/ajh.23203 |url=}}</ref><ref name="pmid25869097">{{cite journal |vauthors=Benton CB, Nazha A, Pemmaraju N, Garcia-Manero G |title=Chronic myelomonocytic leukemia: Forefront of the field in 2015 |journal=Crit. Rev. Oncol. Hematol. |volume=95 |issue=2 |pages=222–42 |date=August 2015 |pmid=25869097 |pmc=4859155 |doi=10.1016/j.critrevonc.2015.03.002 |url=}}</ref>
|
* <small>Constitutional</small>
* <small>[[Anemia]] related</small>
* <small>[[Bleeding]]</small>
* <small>[[Infections]]</small>
* <small>[[Bone]] [[pain]]</small>
* <small>[[Leukemia Cutis]]</small>
|
* <small>[[Organomegaly]]</small>
* <small>[[Bruising]]</small>
|↑
| <small>< 20%</small>
|
|<small>NL</small>
|<small>[[Eosinophilia|↑]]</small>
|↑↑
 
|
* <small>↑ [[LDH]]</small>
 
|<small>[[Anemia|↓]]</small>
|<small>[[Thrombocytopenia|↓]]</small>
|
* <small>[[Myelodysplastic]] and [[myeloproliferative]] feature</small>
|
* <small>[[Cytogenetic analysis]]</small>
* <small>[[Flow cytometry]]</small>
|
* <small>Overlapping of both, [[MDS]] and [[MPN]]</small>
* <small>Absolute [[monocytosis]] > 1 × 10<sup>9</sup>/L (defining feature)</small>
 
* <small>MD-CMML:</small><small>[[WBC]] ≤ 13 × 10<sup>9</sup>/L (FAB)</small>


== Diagnosis ==
* <small> MP-CMML:</small><small>[[WBC]] > 13 × 10<sup>9</sup>/L</small> <small>(FAB)</small>
===Staging===
Staging for subcutaneous panniculitis-like T-cell lymphoma is provided in the following table:<ref>{{Cite journal| doi = 10.1200/JCO.2013.54.8800| issn = 1527-7755| volume = 32| issue = 27| pages = 3059–3068| last1 = Cheson| first1 = Bruce D.| last2 = Fisher| first2 = Richard I.| last3 = Barrington| first3 = Sally F.| last4 = Cavalli| first4 = Franco| last5 = Schwartz| first5 = Lawrence H.| last6 = Zucca| first6 = Emanuele| last7 = Lister| first7 = T. Andrew| last8 = Alliance, Australasian Leukaemia and Lymphoma Group| last9 = Eastern Cooperative Oncology Group| last10 = European Mantle Cell Lymphoma Consortium| last11 = Italian Lymphoma Foundation| last12 = European Organisation for Research| last13 = Treatment of Cancer/Dutch Hemato-Oncology Group| last14 = Grupo Español de Médula Ósea| last15 = German High-Grade Lymphoma Study Group| last16 = German Hodgkin's Study Group| last17 = Japanese Lymphorra Study Group| last18 = Lymphoma Study Association| last19 = NCIC Clinical Trials Group| last20 = Nordic Lymphoma Study Group| last21 = Southwest Oncology Group| last22 = United Kingdom National Cancer Research Institute| title = Recommendations for initial evaluation, staging, and response assessment of Hodgkin and non-Hodgkin lymphoma: the Lugano classification| journal = Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology| date = 2014-09-20| pmid = 25113753}}</ref>


{| style="border: 0px; font-size: 90%; margin: 3px;" align=center
|+ '''Revised staging system for primary nodal lymphomas (Lugano classification)'''
! style="background: #4479BA; color:#FFF;" | Stage
! style="background: #4479BA; color:#FFF;" | Involvement
! style="background: #4479BA; color:#FFF;" | Extranodal (E) status
|-
|-
| style="padding: 5px 5px; background: #DCDCDC;" colspan=3 | '''Limited'''
|<small>[[Atypical chronic myeloid leukemia]] (aCML), [[BCR/ABL|BCR-ABL]]1-</small><ref name="pmid26637732">{{cite journal |vauthors=Dao KH, Tyner JW |title=What's different about atypical CML and chronic neutrophilic leukemia? |journal=Hematology Am Soc Hematol Educ Program |volume=2015 |issue= |pages=264–71 |date=2015 |pmid=26637732 |pmc=5266507 |doi=10.1182/asheducation-2015.1.264 |url=}}</ref><ref name="pmid22289493">{{cite journal |vauthors=Muramatsu H, Makishima H, Maciejewski JP |title=Chronic myelomonocytic leukemia and atypical chronic myeloid leukemia: novel pathogenetic lesions |journal=Semin. Oncol. |volume=39 |issue=1 |pages=67–73 |date=February 2012 |pmid=22289493 |pmc=3523950 |doi=10.1053/j.seminoncol.2011.11.004 |url=}}</ref>
|<small>
*Asymptomatic</small>
* Constitutional
* [[Hyperviscosity|Hyperviscosity]]  and/or [[anemia]] related
* [[Bleeding|Bleeding]]
* [[Infection|Infection]]
|<small>
* [[Splenomegaly]]</small>  (46–76%)
* [[Purpura]]
* [[Anemia]] related
* [[Priapism]]
|↑
|<small><20%</small>
| +
|<small><2% of WBCs</small>
|<small>N/A</small>
|<small>N/A</small>
|
* <small>N/A</small>
|<small>[[Anemia|↓]]</small>
|<small>[[Thrombocytopenia|↓]]</small>
|
* <small>Granulocytic [[hyperplasia]] with prominent [[dysplasia]]</small>
|
* <small>[[Cytogenetic analysis]]</small>
* <small>[[Flow cytometry]]</small>
|
* <small>Granulocytic [[dysplasia]] is prominent</small>
* <small>Absence of ''[[BCR/ABL|BCR-ABL]]'' or ''PDGFRA'', [[PDGFRB|''PDGFRB'',]] or ''[[FGFR1]]'' rearrangements</small>
* <small>[[WBC]] > 13 × 10<sup>9</sup>/L</small>
|-
|-
| style="padding: 5px 5px; background: #F5F5F5;" | Stage I
|<small>[[Juvenile myelomonocytic leukemia (patient information)|Juvenile myelomonocytic leukemia (]]JMML)</small><ref name="pmid9226148">{{cite journal |vauthors=Aricò M, Biondi A, Pui CH |title=Juvenile myelomonocytic leukemia |journal=Blood |volume=90 |issue=2 |pages=479–88 |date=July 1997 |pmid=9226148 |doi= |url=}}</ref><ref name="pmid80255132">{{cite journal |vauthors=Hasle H |title=Myelodysplastic syndromes in childhood--classification, epidemiology, and treatment |journal=Leuk. Lymphoma |volume=13 |issue=1-2 |pages=11–26 |date=March 1994 |pmid=8025513 |doi=10.3109/10428199409051647 |url=}}</ref>
| style="padding: 5px 5px; background: #F5F5F5;" | One node or a group of adjacent nodes
|
| style="padding: 5px 5px; background: #F5F5F5;" | Single extranodal lesions without nodal involvement
* <small>[[Infections]]</small>
* <small>[[Anemia]] related</small>
|
* <small>[[Hepatosplenomegaly]]</small>
* <small>[[Lymphadenopathy]]</small>
* <small>[[Rash]]</small>
|<small>[[Leukocytosis|↑]]</small>
|<small>↑</small>
|<small>N/A</small>
|<small>N/A</small>
|<small>N/A</small>
|<small>[[Monocytosis|↑]]</small>
|
* <small>↓ [[Serum]] [[Iron]]</small>
* <small>↑ [[Vitamin B12|B12]] levels</small>
|<small>[[Anemia|↓]]</small>
|<small>[[Thrombocytopenia|↓]]</small>
|
* <small>Hypercelluar with  ↑ [[myeloid cells]] in stages of maturation</small>
|
* <small>[[Cytogenetic analysis]]</small>
* <small>[[Flow cytometry]]</small>
|
* <small>Polyclonal [[hypergammaglobulinemia]]</small>
|-
|-
| style="padding: 5px 5px; background: #F5F5F5;" | Stage II
|<small>[[MDS]]/[[MPN]] with ring sideroblasts and [[thrombocytosis]] (MDS/MPN-RS-T)</small><ref name="pmid28188970">{{cite journal |vauthors=Patnaik MM, Tefferi A |title=Refractory anemia with ring sideroblasts (RARS) and RARS with thrombocytosis (RARS-T): 2017 update on diagnosis, risk-stratification, and management |journal=Am. J. Hematol. |volume=92 |issue=3 |pages=297–310 |date=March 2017 |pmid=28188970 |doi=10.1002/ajh.24637 |url=}}</ref><ref name="pmid30186759">{{cite journal |vauthors=Alshaban A, Padilla O, Philipovskiy A, Corral J, McAlice M, Gaur S |title=Lenalidomide induced durable remission in a patient with MDS/MPN-with ring sideroblasts and thrombocytosis with associated 5q- syndrome |journal=Leuk Res Rep |volume=10 |issue= |pages=37–40 |date=2018 |pmid=30186759 |doi=10.1016/j.lrr.2018.08.001 |url=}}</ref><ref name="pmid30524760">{{cite journal |vauthors=Bouchla A, Papageorgiou SG, Tsakiraki Z, Glezou E, Pavlidis G, Stavroulaki G, Bazani E, Foukas P, Pappa V |title=Plasmablastic Lymphoma in an Immunocompetent Patient with MDS/MPN with Ring Sideroblasts and Thrombocytosis-A Case Report |journal=Case Rep Hematol |volume=2018 |issue= |pages=2525070 |date=2018 |pmid=30524760 |pmc=6247723 |doi=10.1155/2018/2525070 |url=}}</ref>
| style="padding: 5px 5px; background: #F5F5F5;" | Two or more nodal groups on the same side of the diaphragm
|
| style="padding: 5px 5px; background: #F5F5F5;" | Stage I or II by nodal extent with limited contiguous extranodal involvement
* <small>Constitutional</small>
* <small>[[Anemia]] related</small>
* <small>[[Thrombosis]]</small>
 
|
* <small>Variable</small>
|<small>NL or ↑</small>
|<small>NL</small>
| -
|<small>NL</small>
|<small>N/A</small>
|<small>N/A</small>
|
* ↑ <small>[[Serum]] [[Iron]]</small>
|<small>[[Anemia|↓]]</small>
|<small>[[Thrombocytosis|↑]]</small>
|
* <small>Hypercellularity with [[dyserythropoiesis]] and increased [[megakaryocytes]]</small>
|
* <small>[[Cytogenetic analysis]]</small>
* <small>[[Flow cytometry]]</small>
|
* <small>Large atypical [[megakaryocytes]]</small>
 
* <small>Ringed [[sideroblasts]]</small>
* <small>[[SF3B1]] [[mutation]]</small>
|-
|-
| style="padding: 5px 5px; background: #F5F5F5;" | Stage II bulky
| rowspan="3" |T cell leukemia/<br>lymphoma
| style="padding: 5px 5px; background: #F5F5F5;" | II as above with "bulky" disease
*
| style="padding: 5px 5px; background: #F5F5F5;" | Not applicable
*
|<small>Subcutaneous panniculitis-like T-cell lymphoma/<br>lymphoma</small><ref name="pmid26276771">{{cite journal |vauthors=You MJ, Medeiros LJ, Hsi ED |title=T-lymphoblastic leukemia/lymphoma |journal=Am. J. Clin. Pathol. |volume=144 |issue=3 |pages=411–22 |date=September 2015 |pmid=26276771 |doi=10.1309/AJCPMF03LVSBLHPJ |url=}}</ref><ref name="pmid19284608">{{cite journal |vauthors=Patel KJ, Latif SU, de Calaca WM |title=An unusual presentation of precursor T cell lymphoblastic leukemia/lymphoma with cholestatic jaundice: case report |journal=J Hematol Oncol |volume=2 |issue= |pages=12 |date=March 2009 |pmid=19284608 |pmc=2663564 |doi=10.1186/1756-8722-2-12 |url=}}</ref><ref name="pmid24822133">{{cite journal |vauthors=Elreda L, Sandhu M, Sun X, Bekele W, Cohen AJ, Shah M |title=T-cell lymphoblastic leukemia/lymphoma: relapse 16 years after first remission |journal=Case Rep Hematol |volume=2014 |issue= |pages=359158 |date=2014 |pmid=24822133 |pmc=4005062 |doi=10.1155/2014/359158 |url=}}</ref>
|
* <small>Constitutional</small>
* <small>[[Anemia]] Related</small>
* <small>[[Bleeding]]</small>
 
|
* <small>[[Lymphadenopathy]]</small>
|<small>↑</small>
|<small>>25% [[Blast|blasts]] ([[Leukemia]])</small>
 
<small><25% [[Blast|blasts]] ([[Lymphoma]])</small>
|<small>↑ or ↓</small>
|<small>↑ or ↓</small>
|<small>↑ or ↓</small>
|
* ↑ <small>[[LDH]]</small>
* <small>Positive for TdT</small>
|<small>↓</small>
|<small>↓</small>
|
* <small>Hypercelluarity with increased [[T cells]] precursors</small>
|
* <small>[[Cytogenetic analysis]]</small><small>[[Flow cytometry]]</small>
* <small>[[FISH]]</small>
|
* <small>May involve [[skin]].</small>
|-
|-
| style="padding: 5px 5px; background: #DCDCDC;" colspan=3 | '''Advanced'''
|<small>T-lymphoblastic leukemia/<br>lymphoma</small><ref name="pmid26276771">{{cite journal |vauthors=You MJ, Medeiros LJ, Hsi ED |title=T-lymphoblastic leukemia/lymphoma |journal=Am. J. Clin. Pathol. |volume=144 |issue=3 |pages=411–22 |date=September 2015 |pmid=26276771 |doi=10.1309/AJCPMF03LVSBLHPJ |url=}}</ref><ref name="pmid19284608">{{cite journal |vauthors=Patel KJ, Latif SU, de Calaca WM |title=An unusual presentation of precursor T cell lymphoblastic leukemia/lymphoma with cholestatic jaundice: case report |journal=J Hematol Oncol |volume=2 |issue= |pages=12 |date=March 2009 |pmid=19284608 |pmc=2663564 |doi=10.1186/1756-8722-2-12 |url=}}</ref><ref name="pmid24822133">{{cite journal |vauthors=Elreda L, Sandhu M, Sun X, Bekele W, Cohen AJ, Shah M |title=T-cell lymphoblastic leukemia/lymphoma: relapse 16 years after first remission |journal=Case Rep Hematol |volume=2014 |issue= |pages=359158 |date=2014 |pmid=24822133 |pmc=4005062 |doi=10.1155/2014/359158 |url=}}</ref>
|
* <small>Constitutional</small>
* <small>[[Anemia]] Related</small>
* <small>[[Bleeding]]</small>
* <small>[[Superior vena cava syndrome]]</small>
|
* <small>[[Lymphadenopathy]]</small>
* <small>[[Mediastinal mass]]</small>
*  <small>[[Pleural effusions]]</small>
* <small>[[Trachea|Tracheal]] obstruction</small>
* <small>[[Pericardial effusions]]</small>
|<small>↑</small>
|<small>>25% [[Blast|blasts]] ([[Leukemia]])</small>
 
<small><25% [[Blast|blasts]] ([[Lymphoma]])</small>
|<small>↑ or ↓</small>
|<small>↑ or ↓</small>
|<small>↑ or ↓</small>
|
* ↑ <small>[[LDH]]</small>
* <small>Positive for TdT</small>
|<small>↓</small>
|<small>↓</small>
|
* <small>Hypercelluarity with increased [[T cells]] precursors</small>
|
* <small>[[Cytogenetic analysis]]</small><small>[[Flow cytometry]]</small>
* <small>[[FISH]]</small>
|
* <small>May involve [[brain]], [[skin]], and [[testes]].</small>
|-
|-
| style="padding: 5px 5px; background: #F5F5F5;" | Stage III
|<small>Provisional entity: Natural killer (NK) cell lymphoblastic leukemia/lymph</small><ref name="pmid28868017">{{cite journal |vauthors=Sedick Q, Alotaibi S, Alshieban S, Naheet KB, Elyamany G |title=Natural Killer Cell Lymphoblastic Leukaemia/Lymphoma: Case Report and Review of the Recent Literature |journal=Case Rep Oncol |volume=10 |issue=2 |pages=588–595 |date=2017 |pmid=28868017 |doi=10.1159/000477843 |url=}}</ref>
| style="padding: 5px 5px; background: #F5F5F5;" | Nodes on both sides of the diaphragm; nodes above the diaphragm with spleen involvement
|
| style="padding: 5px 5px; background: #F5F5F5;" | Not applicable
* <small>Constitutional</small>
* <small>[[Anemia]] Related</small>
* <small>[[Bleeding]]</small>
* <small>[[Superior vena cava syndrome]]</small>
|
* <small>[[Lymphadenopathy]]</small>
* <small>[[Mediastinal mass]]</small>
*  <small>[[Pleural effusions]]</small>
* <small>[[Trachea|Tracheal]] obstruction</small>
* <small>[[Pericardial effusions]]</small>
|<small>↑</small>
|<small>↑</small>
|<small>↑ or ↓</small>
|<small>↑ or ↓</small>
|<small>↑ or ↓</small>
|
* ↑ <small>[[LDH]]</small>
|<small>↓</small>
|<small>↓</small>
|
* <small>N/A</small>
|
* <small>[[Cytogenetic analysis]]</small>
 
* <small>[[FISH]]</small>
* <small>[[Flow cytometry]]</small>
|
* <small>Similar to [[T-cell]] lymphoblastic [[leukemia]] but may have more aggressive clinical course.</small>
* <small>[[Diagnosis]] is usually based on presence of [[CD56]] expression, and  [[T-cell]]-associated markers such as [[CD2]] and [[CD7]].</small>
*  <small>[[B cell|B-cell]] markers are absent.</small>
|-
|-
| style="padding: 5px 5px; background: #F5F5F5;" | Stage IV
|<small>Provisional entity: Early T-cell precursor lymphoblastic leukemia</small><ref name="pmid26747249">{{cite journal |vauthors=Jain N, Lamb AV, O'Brien S, Ravandi F, Konopleva M, Jabbour E, Zuo Z, Jorgensen J, Lin P, Pierce S, Thomas D, Rytting M, Borthakur G, Kadia T, Cortes J, Kantarjian HM, Khoury JD |title=Early T-cell precursor acute lymphoblastic leukemia/lymphoma (ETP-ALL/LBL) in adolescents and adults: a high-risk subtype |journal=Blood |volume=127 |issue=15 |pages=1863–9 |date=April 2016 |pmid=26747249 |pmc=4915808 |doi=10.1182/blood-2015-08-661702 |url=}}</ref><ref name="pmid23695450">{{cite journal |vauthors=Haydu JE, Ferrando AA |title=Early T-cell precursor acute lymphoblastic leukaemia |journal=Curr. Opin. Hematol. |volume=20 |issue=4 |pages=369–73 |date=July 2013 |pmid=23695450 |pmc=3886681 |doi=10.1097/MOH.0b013e3283623c61 |url=}}</ref>
| style="padding: 5px 5px; background: #F5F5F5;" | Additional noncontiguous extralymphatic involvement
|
| style="padding: 5px 5px; background: #F5F5F5;" | Not applicable
* <small>Constitutional</small>
* <small>[[Anemia]] Related</small>
* <small>[[Bleeding]]</small>
* <small>[[Superior vena cava syndrome]]</small>
|
* <small>[[Lymphadenopathy]]</small>
* <small>[[Mediastinal mass]]</small>
*  <small>[[Pleural effusions]]</small>
* <small>[[Trachea|Tracheal]] obstruction</small>
* <small>[[Pericardial effusions]]</small>
|<small>↑</small>
|<small>↑</small>
|<small>↑ or ↓</small>
|<small>↑ or ↓</small>
|<small>↑ or ↓</small>
|
* ↑ <small>[[LDH]]</small>
|<small>↓</small>
|<small>↓</small>
|
* <small>Hypercelluarity with increased [[T cells]] precursors</small>
|
* <small>[[Cytogenetic analysis]]</small>
* <small>[[FISH]]</small>
* <small>[[Flow cytometry]]</small>
|
* <small>Similar to [[T cell|T-cell]] lymphoblastic [[leukemia]] but is more aggressive clinically and cell are characterized by [[Flow cytometry|cytometry]] as [[CD1a]]<sup>−</sup>, [[CD8]]<sup>−</sup>, [[CD5]]<sup>−</sup> (dim), and positivity for 1 or more stem cell or [[myeloid]] [[antigens]].</small>
* <small>[[Gene expression]] indicates more immature [[cells]] as compared to other subtypes of [[T cell|T-cell]] [[neoplasms]].</small>
|}
|}


== Epidemiology and Demographics ==
===Age===
Subcutaneous panniculitis-like T-cell lymphoma commonly affects young adults (35 years).<ref name="seer.cancer.gov">Subcutaneous panniculitis-like T-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52df/. Accessed on March 08, 2016 </ref>
===Gender===
Females are more commonly affected with subcutaneous panniculitis-like T-cell lymphoma than males.<ref name="seer.cancer.gov">Subcutaneous panniculitis-like T-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52df/. Accessed on March 08, 2016 </ref><ref name="pmid191959752" />
The female to male ratio is 2:1.
== Risk Factors ==
There are no established risk factors for subcutaneous panniculitis-like T-cell lymphoma however, many patients have an associated autoimmune disorder at diagnosis.
== Screening ==
According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for subcutaneous panniculitis-like T-cell lymphoma. <ref name="pmid191959752" />
== Natural History, Complications and Prognosis==
* Painless nodules form under the skin in the subcutaneous tissue (subcutaneous nodules).  <ref name="pmid191959752" /><ref name="canadiancancer">Subcutaneous panniculitis-like T-cell lymphoma. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/subcutaneous-panniculitis-like-t-cell-lymphoma/?region=nb. Accessed on March 8, 2016 </ref><ref name="seer.cancer.gov">Subcutaneous panniculitis-like T-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52df/. Accessed on March 08, 2016 </ref>
* Skin nodules may demonstrate regression with [[lipoatrophy]] at the site of the lesion.
* New nodules can appear at different sites over the course of the disease.
* The nodules commonly occur in the extremities or trunk. Other areas include: face, neck and back. 
* Skin nodules range in size from 0.5 cm to several centimeters in diameter. Larger nodules may become necrotic, ulceration is rare.
* The infiltrate involves the fat lobules, usually sparing the septa. The overlying dermis and epidermis are typically uninvolved. Dissemination to lymph nodes and other organs is rare.
* People with this type of lymphoma can also develop a very serious condition where certain parts of the immune system are activated (hemophagocytic syndrome).
* Hemophagocytic syndrome causes:
:* [[Fever]]
:* Enlarged liver and spleen ([[hepatosplenomegaly]])
:* A lower number of red blood cells, white blood cells and platelets in the blood ([[pancytopenia]])
:* [[Failure to thrive]]
:* [[Maculopapular rash]]
:* CNS symptoms
:* [[Lymphadenopathy]]
:* [[Coagulopathy]]
:* Abnormal [[Liver function tests|LFT]]<nowiki/>s
:* High serum [[Ferritin]]
* Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary and the 5-year survival rate of patients with subcutaneous panniculitis-like T-cell lymphoma is approximately 80%
== Diagnosis ==
=== Symptoms ===
=== Symptoms ===
Symptoms of the subcutaneous panniculitis-like T-cell lymphoma include:
Symptoms of SPTCL include:<ref name="seer.cancer.gov">Subcutaneous panniculitis-like T-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52df/. Accessed on March 08, 2016 </ref> <ref name="pmid191959752" />
* [[Fever]]
* [[Fever]]
* [[Chills]]
* [[Weight loss]]
* [[Weight loss]]
* [[Night sweats]]
* [[Night sweats]]
* Painless swelling in the neck, axilla, groin, thorax, and abdomen
* [[Myalgias]]
* Painless swellings on extremities and trunk
=== Physical Examination ===
=== Physical Examination ===
====Vitals====
====Vitals====
Line 80: Line 920:
====Skin====
====Skin====
* Subcutaneous nodules
* Subcutaneous nodules
====HEENT====
'''Abdomen'''
* [[Lymphadenopathy|Cervical lymphadenopathy]]
====Thorax====
* Thoracic masses suggestive of [[Lymphadenopathy|central lymphadenopathy]]
====Abdomen====
* [[Abdominal mass]]es suggestive of [[Lymphadenopathy|central lymphadenopathy]]
* [[Hepatosplenomegaly]]
* [[Hepatosplenomegaly]]
====Extremities====
* [[Lymphadenopathy|Peripheral lymphadenopathy]]
===Laboratory Findings ===  
===Laboratory Findings ===  
Laboratory tests for subcutaneous panniculitis-like T-cell lymphoma include:
Laboratory tests for subcutaneous panniculitis-like T-cell lymphoma include:<ref name="seer.cancer.gov">Subcutaneous panniculitis-like T-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52df/. Accessed on March 08, 2016 </ref>
* [[Complete blood count]] (CBC): cytopenia
* [[Complete blood count]] (CBC): cytopenia
* Blood chemistry studies: Increased [[liver function tests]]  
* Blood chemistry studies: Increased [[liver function tests]]  
* Cytogenetic analysis
* Cytogenetic analysis
* [[Flow cytometry]]
* [[Flow cytometry]]
* [[Immunophenotyping]]
:* Positive: BetaF1; [[CD8]]; [[CD30]]; [[CLA]]; T-cell intracellular antigen (T1A1); and [[perforin]]
:* Negative: [[CD3]]; [[CD5]]; and [[CD56]]
====Biopsy====
====Biopsy====
Lymph node or bone marrow biopsy is diagnostic of subcutaneous panniculitis-like T-cell lymphoma.
Subcutaneous nodule biopsy is diagnostic of subcutaneous panniculitis-like T-cell lymphoma.
====CT ====
[[CT]] scan may be helpful in the diagnosis of subcutaneous panniculitis-like T-cell lymphoma.
==== MRI ====
[[MRI]] scan may be helpful in the diagnosis of subcutaneous panniculitis-like T-cell lymphoma.
==== Other Imaging Findings ====
==== Other Imaging Findings ====
[[PET]] scan may be helpful in the diagnosis of subcutaneous panniculitis-like T-cell lymphoma.
[[CT]], [[MRI]], and [[PET]] scan may be helpful in the diagnosis of subcutaneous panniculitis-like T-cell lymphoma.


==Treatment==
==Treatment==
Line 108: Line 940:
===Medical Therapy===
===Medical Therapy===


{| style="border: 0px; font-size: 90%; margin: 3px;" align=center
{| style="border: 0px; font-size: 90%; margin: 3px;" align="center"
|+ '''Treatment of subcutaneous panniculitis-like T-cell lymphoma<ref name= canadiancancer>Subcutaneous panniculitis-like T-cell lymphoma. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/subcutaneous-panniculitis-like-t-cell-lymphoma/?region=nb. Accessed on March 8, 2016 </ref>
|+ '''Treatment of subcutaneous panniculitis-like T-cell lymphoma<ref name="canadiancancer">Subcutaneous panniculitis-like T-cell lymphoma. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/subcutaneous-panniculitis-like-t-cell-lymphoma/?region=nb. Accessed on March 8, 2016 </ref>'''
! style="background: #4479BA; color:#FFF;" | Therapy  
! style="background: #4479BA; color:#FFF;" | Therapy  
! style="background: #4479BA; color:#FFF;" | Description  
! style="background: #4479BA; color:#FFF;" | Description  
Line 120: Line 952:
| style="padding: 5px 5px; background: #DCDCDC;" | [[Radiation therapy]]
| style="padding: 5px 5px; background: #DCDCDC;" | [[Radiation therapy]]
| style="padding: 5px 5px; background: #F5F5F5;" |  
| style="padding: 5px 5px; background: #F5F5F5;" |  
* [[External beam radiation therapy]] may be used to treat the lymph nodes.
* [[External beam radiation therapy]] may be used.
|-
|-
| style="padding: 5px 5px; background: #DCDCDC;" | [[Stem cell transplant]]
| style="padding: 5px 5px; background: #DCDCDC;" | [[Stem cell transplant]]
Line 130: Line 962:
{{Reflist|2}}
{{Reflist|2}}


[[Category:Disease]]
[[Category:Disease]]
[[Category:Oncology]]
 
[[Category:Up-To-Date]]
[[Category:Oncology]]
[[Category:Oncology]]
[[Category:Medicine]]<small>[[Category:Hematology]]

Latest revision as of 01:07, 24 October 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [2]; Sindhuja Palle

Synonyms and keywords: SPTCL; Subcutaneous panniculitis-like T-cell lymphoma alpha/beta subtype; Subcutaneous panniculitis-like T-cell lymphoma gamma/delta subtype; SPTCL-AB; SPTCL-GD

Overview

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare type of T-cell non-Hodgkin lymphoma (NHL). Subcutaneous panniculitis-like T-cell lymphoma is a cytotoxic T-cell lymphoma, which preferentially infiltrates subcutaneous tissue, primarily involving the extremities and trunk. According to the World Health Organization-European Organization for Research and Treatment of Cancer, SPTCL is classified under Primary Cutaneous T-Cell Lymphomas and expresses the TCRαβ phenotype (SPTCL-AB). TCRγδ phenotypes are now a separate entity from SPTCL, known as the Cutaneous γδ T-cell Lymphoma. On microscopic histopathological analysis, atypical lymphoid cells, fat necrosis,and karyorrhexis are characteristic findings of subcutaneous panniculitis-like T-cell lymphoma. Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary and the 5-year survival rate of patients with subcutaneous panniculitis-like T-cell lymphoma is approximately 80%. Symptoms of the SPTCL include fever, weight loss, night sweats, and painless swellings on extremities and trunk. Subcutaneous nodule biopsy is diagnostic of SPTCL. The predominant therapy for SPTCL is chemotherapy. Adjunctive radiotherapy and stem cell transplant may be required.[1][2]

Classification

Subcutaneous panniculitis-like T-cell lymphoma is classified under Primary cutaneous lymphomas and expresses the TCRαβ phenotype.[2]

Name Description
Alpha/Beta subtype (SPTCL-AB)
  • Now considered the only type of subcutaneous panniculitis-like T cell lymphoma
  • Slow-growing (indolent) disease with a favourable prognosis.
Gamma/Delta subtype (SPTCL-GD)

Pathophysiology

Subcutaneous panniculitis-like T-cell lymphoma is a cytotoxic T-cell lymphoma, which preferentially infiltrates subcutaneous tissue, primarily involving the extremities and trunk.[1]

Microscopic Pathology

On microscopic histopathological analysis, atypical lymphoid cells, fat necrosis, and karyorrhexis are characteristic findings of subcutaneous panniculitis-like T-cell lymphoma.[1]

Causes

There are no established causes for subcutaneous panniculitis-like T-cell lymphoma.

Differentiating SPTCL from other Diseases

Subcutaneous panniculitis-like T-cell lymphoma must be differentiated from other diseases such as: [3]

Differentiating Blastic NK cell Lymphoma from other Diseases

ABBREVIATIONS

N/A: Not available, NL: Normal, FISH: Fluorescence in situ hybridization, PCR: Polymerase chain reaction, LDH: Lactate dehydrogenase, PUD: Peptic ulcer disease, EPO: Erythropoietin, LFTs: Liver function tests, RFTs: Renal function tests, LAP: Leukocyte alkaline phosphatase, LAD: Leukocyte alkaline dehydrgenase, WBCs: White blood cells.

Myeloproliferative neoplasms (MPN) Clinical manifestations Diagnosis Other features
Symptoms Physical examination CBC & Peripheral smear Bone marrow biopsy Other investigations
WBCs Hb Plat-
elets
Leuko-cytes Blasts Left
shift
Baso-
phils
Eosino-
phils
Mono-
cytes
Others
Chronic myeloid leukemia
(CML), BCR-ABL1+[4][5]
<2% + N/A NL
Chronic neutrophilic leukemia (CNL)[6][7][8] Minimal + NL NL NL
Polycythemia vera
(PV)[9][10][11][12]
  • Constitutional
NL or ↑ None - ↑ or ↓ NL or ↑ NL ↑↑ NL
  • Hypercellularity for age with tri-lineage growth
Primary myelofibrosis (PMF)[13][14][15][16] Erythroblasts - Absent NL NL
  • Variable with fibrosis or hypercellularity
Essential thrombocythemia (ET)[17][18][19]

NL or ↑

None

-

↓ or absent

NL

NL

  • N/A

↑↑

  • Normal/Hypercellular
Chronic eosinophilic leukemia,
not otherwise specified
(NOS)[20][21][22][23]
Present + ↑↑
MPN,
unclassifiable
Variable ± ↑ or ↓ ↑ or ↓ ↑ or ↓
  • N/A
Mastocytosis[24][25][26][27]
  • Constitutional
None - NL NL ↓ or ↑
Myeloid/lymphoid neoplasms
with eosinophilia and rearrangement
of PDGFRA, PDGFRB, or FGFR1,
or with PCM1-JAK2[28][29][30][31]
NL - NL
  • None
NL
  • FISH shows t(8;13) and t(8;22)
B-lymphoblastic leukemia/lymphoma[32][33] NL or ↑ >25% N/A ↑ or ↓ ↑ or ↓ ↑ or ↓
Myelodysplastic syndromes
(MDS)[34][35]
Variable -
  • Leukemia transformation
  • Acquired pseudo-Pelger-Huët anomaly
Acute myeloid leukemia (AML)
and related neoplasms[36][37]
NL or ↑ N/A ↑ or ↓ ↑ or ↓ ↑ or ↓

with dysplasia

Blastic plasmacytoid
dendritic cell neoplasm
[38][39][40][41]
NL NL NL NL
Myelodysplastic
/myeloproliferative
neoplasms
(MDS/MPN)
Chronic myelomonocytic leukemia (CMML)[42]
[43][44]
< 20% NL ↑↑
  • Overlapping of both, MDS and MPN
  • Absolute monocytosis > 1 × 109/L (defining feature)
  • MD-CMML:WBC ≤ 13 × 109/L (FAB)
  •  MP-CMML:WBC > 13 × 109/L (FAB)
Atypical chronic myeloid leukemia (aCML), BCR-ABL1-[45][46] <20% + <2% of WBCs N/A N/A
  • N/A
Juvenile myelomonocytic leukemia (JMML)[47][48] N/A N/A N/A
MDS/MPN with ring sideroblasts and thrombocytosis (MDS/MPN-RS-T)[49][50][51]
  • Variable
NL or ↑ NL - NL N/A N/A
T cell leukemia/
lymphoma
Subcutaneous panniculitis-like T-cell lymphoma/
lymphoma
[52][53][54]
>25% blasts (Leukemia)

<25% blasts (Lymphoma)

± ↑ or ↓ ↑ or ↓ ↑ or ↓
  • LDH
  • Positive for TdT
  • Hypercelluarity with increased T cells precursors
T-lymphoblastic leukemia/
lymphoma
[52][53][54]
>25% blasts (Leukemia)

<25% blasts (Lymphoma)

± ↑ or ↓ ↑ or ↓ ↑ or ↓
  • LDH
  • Positive for TdT
  • Hypercelluarity with increased T cells precursors
Provisional entity: Natural killer (NK) cell lymphoblastic leukemia/lymph[55] ± ↑ or ↓ ↑ or ↓ ↑ or ↓
  • N/A
Provisional entity: Early T-cell precursor lymphoblastic leukemia[56][57] ± ↑ or ↓ ↑ or ↓ ↑ or ↓
  • Hypercelluarity with increased T cells precursors

Epidemiology and Demographics

Age

Subcutaneous panniculitis-like T-cell lymphoma commonly affects young adults (35 years).[1]

Gender

Females are more commonly affected with subcutaneous panniculitis-like T-cell lymphoma than males.[1][3]

The female to male ratio is 2:1.

Risk Factors

There are no established risk factors for subcutaneous panniculitis-like T-cell lymphoma however, many patients have an associated autoimmune disorder at diagnosis.

Screening

According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for subcutaneous panniculitis-like T-cell lymphoma. [3]

Natural History, Complications and Prognosis

  • Painless nodules form under the skin in the subcutaneous tissue (subcutaneous nodules). [3][2][1]
  • Skin nodules may demonstrate regression with lipoatrophy at the site of the lesion.
  • New nodules can appear at different sites over the course of the disease.
  • The nodules commonly occur in the extremities or trunk. Other areas include: face, neck and back.
  • Skin nodules range in size from 0.5 cm to several centimeters in diameter. Larger nodules may become necrotic, ulceration is rare.
  • The infiltrate involves the fat lobules, usually sparing the septa. The overlying dermis and epidermis are typically uninvolved. Dissemination to lymph nodes and other organs is rare.
  • People with this type of lymphoma can also develop a very serious condition where certain parts of the immune system are activated (hemophagocytic syndrome).
  • Hemophagocytic syndrome causes:
  • Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary and the 5-year survival rate of patients with subcutaneous panniculitis-like T-cell lymphoma is approximately 80%

Diagnosis

Symptoms

Symptoms of SPTCL include:[1] [3]

Physical Examination

Vitals

Skin

  • Subcutaneous nodules

Abdomen

Laboratory Findings

Laboratory tests for subcutaneous panniculitis-like T-cell lymphoma include:[1]

Biopsy

Subcutaneous nodule biopsy is diagnostic of subcutaneous panniculitis-like T-cell lymphoma.

Other Imaging Findings

CT, MRI, and PET scan may be helpful in the diagnosis of subcutaneous panniculitis-like T-cell lymphoma.

Treatment

Medical Therapy

Treatment of subcutaneous panniculitis-like T-cell lymphoma[2]
Therapy Description
Chemotherapy
  • Combination therapy :
Radiation therapy
Stem cell transplant
  • May be used in some people with subcutaneous panniculitis-like T-cell lymphoma when their lymphoma recurs after treatment.

References

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  2. 2.0 2.1 2.2 2.3 Subcutaneous panniculitis-like T-cell lymphoma. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/subcutaneous-panniculitis-like-t-cell-lymphoma/?region=nb. Accessed on March 8, 2016
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