Hairy cell leukemia classification: Difference between revisions

Jump to navigation Jump to search
VisualWikitext
No edit summary
No edit summary
 
(27 intermediate revisions by 5 users not shown)
Line 1: Line 1:
__NOTOC__
__NOTOC__
{{Hairy cell leukemia}}
{{Hairy cell leukemia}}
{{CMG}}
{{CMG}}; {{AE}} {{HL}}, {{VKG}}; {{GRR}} {{Nat}}


==Overview==
==Overview==
Two variants have been described:  Hairy cell leukemia-variant[http://ajcp.metapress.com/link.asp?id=8qytyq1clqmhq9cl], which usually is diagnosed in older men (median age above 70), and a Japanese variant.  The non-Japanese variant is more difficult to treat than either 'classic' HCL or the Japanese variant HCL.
* [[Hairy cell leukemia]] may be classified into two sub-types which include [[hairy cell leukemia]] (classic) and [[hairy cell leukemia]] (variant).


==Classification==
==Classification==
 
* [[Hairy cell leukemia]] may be classified into two sub-types which include:<ref name="MatutesWotherspoon2003">{{cite journal|last1=Matutes|first1=E|last2=Wotherspoon|first2=A|last3=Catovsky|first3=D|title=The variant form of hairy-cell leukaemia|journal=Best Practice & Research Clinical Haematology|volume=16|issue=1|year=2003|pages=41–56|issn=15216926|doi=10.1016/S1521-6926(02)00086-5}}</ref><ref name="AronsSuntum2009">{{cite journal|last1=Arons|first1=E.|last2=Suntum|first2=T.|last3=Stetler-Stevenson|first3=M.|last4=Kreitman|first4=R. J.|title=VH4-34+ hairy cell leukemia, a new variant with poor prognosis despite standard therapy|journal=Blood|volume=114|issue=21|year=2009|pages=4687–4695|issn=0006-4971|doi=10.1182/blood-2009-01-201731}}</ref><ref name="pmid20558005">{{cite journal |vauthors=Robak T |title=Hairy-cell leukemia variant: recent view on diagnosis, biology and treatment |journal=Cancer Treat. Rev. |volume=37 |issue=1 |pages=3–10 |date=February 2011 |pmid=20558005 |doi=10.1016/j.ctrv.2010.05.003 |url=}}</ref><ref name="RobakMatutes2015">{{cite journal|last1=Robak|first1=T.|last2=Matutes|first2=E.|last3=Catovsky|first3=D.|last4=Zinzani|first4=P. L.|last5=Buske|first5=C.|title=Hairy cell leukaemia: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up|journal=Annals of Oncology|volume=26|issue=suppl 5|year=2015|pages=v100–v107|issn=0923-7534|doi=10.1093/annonc/mdv200}}</ref>
===Hairy cell leukemia-variant===
:* [[Hairy cell leukemia]] ('''classic''')
 
:* [[Hairy cell leukemia]] ('''variant''')
'''Hairy cell leukemia-variant''', or HCL-V, is usually described as a prolymphocytic variant of hairy cell leukemia.<ref>{{cite web |url=http://bloodjournal.hematologylibrary.org/cgi/content/abstract/76/1/157 |title=A variant form of hairy cell leukemia resistant to alpha-interferon: clinical and phenotypic characteristics of 17 patients -- Sainati et al. 76 (1): 157 -- Blood |accessdate=2007-09-10 |format= |work=}}</ref> It was first formally described in 1980 by a paper from the University of Cambridge's Hayhoe lab.<ref name="pmid7206776">{{cite journal |author=Cawley JC, Burns GF, Hayhoe FG |title=A chronic lymphoproliferative disorder with distinctive features: a distinct variant of hairy-cell leukaemia |journal=Leuk. Res. |volume=4 |issue=6 |pages=547-59 |year=1980 |pmid=7206776 |doi=}}</ref> About 10% of HCL patients have this variant form of the disease, representing about 60-75 new HCL-V patients each year in the U.S.  While classic HCL primarily affects men, HCL-V is somewhat more evenly divided between males and females.<ref name="pmid11243388">{{cite journal |author=Matutes E, Wotherspoon A, Brito-Babapulle V, Catovsky D |title=The natural history and clinico-pathological features of the variant form of hairy cell leukemia |journal=Leukemia |volume=15 |issue=1 |pages=184-6 |year=2001 |pmid=11243388 |doi=}}</ref>
* [[Hairy cell leukemia]] (variant) is still considered a provisional entity at this time, as the [[World Health Organization|WHO]] is unable to recognize it as distinct [[Disease|disease]] due to insufficient evidence.<ref name="pmid21300984">{{cite journal| author=Campo E, Swerdlow SH, Harris NL, Pileri S, Stein H, Jaffe ES| title=The 2008 WHO classification of lymphoid neoplasms and beyond: evolving concepts and practical applications. | journal=Blood | year= 2011 | volume= 117 | issue= 19 | pages= 5019-32 | pmid=21300984 | doi=10.1182/blood-2011-01-293050 | pmc=PMC3109529 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21300984  }} </ref>
 
* The chart below illustrates characteristic findings associated with each sub-type:<ref name="pmid16990106">{{cite journal| author=Matutes E| title=Immunophenotyping and differential diagnosis of hairy cell leukemia. | journal=Hematol Oncol Clin North Am | year= 2006 | volume= 20 | issue= 5 | pages= 1051-63 | pmid=16990106 | doi=10.1016/j.hoc.2006.06.012 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16990106  }} </ref>
Similar to B-PLL in [[Chronic Lymphocytic Leukemia]], HCL-V is a more aggressive disease which is harder to treat successfully than classic HCL. Many treatment approaches, such as Interferon-alpha, CHOP and common alkylating agents like cyclophosphamide provide very little benefit.<ref name="pmid11243388"> </ref> Pentostatin and cladribine provide some benefit to many HCL-V patients, but with shorter remissions and lower response rates compared to classic HCL.  More than half of patients respond partially to splenectomy.<ref name="pmid11243388"> </ref>
{{familytree/start |summary=Molecular classification criteria}}
 
{{familytree | | | | | | | | Y01 | | | | | | | | | Y01=<div style="width: 20em; padding:0.5em;">'''Hairy cell leukemia classification'''</div>}}
In terms of B cell development, the prolymphocytes are less developed than lymphocyte cells or plasma cells, but are still more developed than their lymphoblastic precursors.
{{familytree | | | | | | |,|-|^|-|.| | | | | | }}
 
{{familytree | | | | | | A02 | | A03 | A02=<div style="width: 20em; padding:0.5em;">'''Hairy cell leukemia'''</div>|A03=<div style="width: 20em; padding:0.5em;">'''Hairy cell leukemia variant'''</div>}}
HCL-V differs from classic HCL principally in these respects:
{{familytree|boxstyle= border-top: 0px; | | | | | | A02 | | A03 |  A02=<div style="width: 20em; padding:0.5em;text-align:left">
* High white blood cell counts, sometimes in excess of 100,000 cells per microliter;
* '''Occurs in approximately 95% of the cases'''<br>
* More aggressive course of disease that requires more frequent treatment;
* '''The male to female ratio is approximately 4 to 1'''<br>
* Cells with an unusually large nucleolus for their size;
* '''The median age at diagnosis is 55 years'''<br>
* Little excess fibronectin (which is produced by classic hairy cells<ref>{{cite web |url=http://bloodjournal.hematologylibrary.org/cgi/content/abstract/83/2/497 |title=The bone marrow fibrosis of hairy-cell leukemia is caused by the synthesis and assembly of a fibronectin matrix by the hairy cells -- Burthem and Cawley 83 (2): 497 -- Blood |accessdate=2007-09-10 |format= |work=}}</ref>) to interfere with bone marrow biopsies; and
* '''Less than 10% [[lymphocytosis]] is observed'''<br>
* Low or no expression of CD25 (also called the Interleukin-2 [IL-2] receptor alpha chain or p55) on cell surfaces.<ref>{{cite web |url=http://bloodjournal.hematologylibrary.org/cgi/content/abstract/82/2/528 |title=Phenotypic analysis of hairy cell leukemia: "variant" cases express the interleukin-2 receptor beta chain, but not the alpha chain (CD25) -- de Totero et al. 82 (2): 528 -- Blood |accessdate=2007-09-10 |format= |work=}}</ref>
* '''Decreased level of [[monocyte]] on peripheral blood smear'''<br>
 
* '''Decreased [[hemoglobin]] level in approximately 85% of the patients'''<br>
The lack of CD25, which is part of the receptor for a key immunoregulating hormone, may explain why HCL-V cases are normally resistant to treatment by immune system hormones.<ref>{{cite web |url=http://bloodjournal.hematologylibrary.org/cgi/content/abstract/76/1/157 |title=A variant form of hairy cell leukemia resistant to alpha-interferon: clinical and phenotypic characteristics of 17 patients -- Sainati et al. 76 (1): 157 -- Blood |accessdate=2007-09-10 |format= |work=}}</ref>
* '''[[Thrombocytopenia]] is present in approximately 80% of the patients'''<br>
 
* '''[[CD103]] +ve, [[CD11c]] +ve, and [[CD25]] +ve on immunophenotype'''<br>
HCL-V, which has a high proportion of hairy cells without a functional p53 tumor suppressor gene, is somewhat more likely to transform into a higher-grade disease, with Daniel Catovsky suggesting a transformation rate of 5% in the U.K., which is similar to the Ricther's transformation rate for SLVL and CLL.<ref name="pmid11243388"> </ref>  Among HCL-V patients, the most aggressive cases normally have the least amount of p53 gene activity.<ref name="pmid10576509">{{cite journal |author=Vallianatou K, Brito-Babapulle V, Matutes E, Atkinson S, Catovsky D |title=p53 gene deletion and trisomy 12 in hairy cell leukemia and its variant |journal=Leuk. Res. |volume=23 |issue=11 |pages=1041-5 |year=1999 |pmid=10576509 |doi=}}</ref> Hairy cells without the p53 gene tend, over time, to displace the less aggressive p53+ hairy cells.
* '''DBA 44 +ve, [[Cyclin D1]] +ve, and [[Annexin A1]] +ve on immunohistochemistry'''<br>
 
* '''Presence of [[BRAF]] V600E mutation on cytogenetic tests'''
===Hairy cell leukemia-Japanese variant===
</div>|A03=<div style="width: 20em; padding:0.5em;text-align:left">
 
* '''Occurs in approximately 5% of the cases'''<br>
'''Hairy cell leukemia-Japanese variant''' or HCL-J. There is also a Japanese variant, which is more easily treated.
* '''The male to female ratio is approximately 2 to 1'''<br>
* '''The median age at diagnosis is greater than 70 years'''<br>
* '''Greater than 90% [[lymphocytosis]] is observed'''<br>
* '''Normal level of [[monocyte]] on peripheral blood smear'''<br>
* '''Normal [[hemoglobin]] level in most of the patients'''<br>
* '''Normal [[platelets]] count in most of the patients'''<br>
* '''[[CD103]] +ve, [[CD11c]] +ve, and [[CD25]] -ve on immunophenotype'''<br>
* '''DBA 44 +ve, [[Cyclin D1]] +ve, and [[Annexin A1]] -ve on immunohistochemistry'''<br>
* '''Presence of [[BRAF]] wild type mutation on cytogenetic tests'''</div>}}
{{familytree/end}}
<br>
<br>


==References==
==References==
Line 40: Line 51:
{{WikiDoc Help Menu}}
{{WikiDoc Help Menu}}
{{WikiDoc Sources}}
{{WikiDoc Sources}}
[[Category:Up-To-Date]]
[[Category:Oncology]]
[[Category:Medicine]]
[[Category:Hematology]]
[[Category:Immunology]]

Latest revision as of 17:20, 8 April 2019

Hairy cell leukemia Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Hairy cell leukemia from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

Staging

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

Chest X Ray

CT

MRI

Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Hairy cell leukemia classification On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Hairy cell leukemia classification

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Hairy cell leukemia classification

CDC on Hairy cell leukemia classification

Hairy cell leukemia classification in the news

Blogs on Hairy cell leukemia classification

Directions to Hospitals Treating Hairy cell leukemia

Risk calculators and risk factors for Hairy cell leukemia classification

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Haytham Allaham, M.D. [2], Vamsikrishna Gunnam M.B.B.S [3]; Grammar Reviewer: Natalie Harpenau, B.S.[4]

Overview

Classification

  • Hairy cell leukemia (variant) is still considered a provisional entity at this time, as the WHO is unable to recognize it as distinct disease due to insufficient evidence.[5]
  • The chart below illustrates characteristic findings associated with each sub-type:[6]
 
 
 
 
 
 
 
Hairy cell leukemia classification
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Hairy cell leukemia
 
Hairy cell leukemia variant
 
 
 
 
 
  • Occurs in approximately 95% of the cases
  • The male to female ratio is approximately 4 to 1
  • The median age at diagnosis is 55 years
  • Less than 10% lymphocytosis is observed
  • Decreased level of monocyte on peripheral blood smear
  • Decreased hemoglobin level in approximately 85% of the patients
  • Thrombocytopenia is present in approximately 80% of the patients
  • CD103 +ve, CD11c +ve, and CD25 +ve on immunophenotype
  • DBA 44 +ve, Cyclin D1 +ve, and Annexin A1 +ve on immunohistochemistry
  • Presence of BRAF V600E mutation on cytogenetic tests
 
  • Occurs in approximately 5% of the cases
  • The male to female ratio is approximately 2 to 1
  • The median age at diagnosis is greater than 70 years
  • Greater than 90% lymphocytosis is observed
  • Normal level of monocyte on peripheral blood smear
  • Normal hemoglobin level in most of the patients
  • Normal platelets count in most of the patients
  • CD103 +ve, CD11c +ve, and CD25 -ve on immunophenotype
  • DBA 44 +ve, Cyclin D1 +ve, and Annexin A1 -ve on immunohistochemistry
  • Presence of BRAF wild type mutation on cytogenetic tests



References

  1. ↑ Matutes, E; Wotherspoon, A; Catovsky, D (2003). "The variant form of hairy-cell leukaemia". Best Practice & Research Clinical Haematology. 16 (1): 41–56. doi:10.1016/S1521-6926(02)00086-5. ISSN 1521-6926.
  2. ↑ Arons, E.; Suntum, T.; Stetler-Stevenson, M.; Kreitman, R. J. (2009). "VH4-34+ hairy cell leukemia, a new variant with poor prognosis despite standard therapy". Blood. 114 (21): 4687–4695. doi:10.1182/blood-2009-01-201731. ISSN 0006-4971.
  3. ↑ Robak T (February 2011). "Hairy-cell leukemia variant: recent view on diagnosis, biology and treatment". Cancer Treat. Rev. 37 (1): 3–10. doi:10.1016/j.ctrv.2010.05.003. PMID 20558005.
  4. ↑ Robak, T.; Matutes, E.; Catovsky, D.; Zinzani, P. L.; Buske, C. (2015). "Hairy cell leukaemia: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up". Annals of Oncology. 26 (suppl 5): v100–v107. doi:10.1093/annonc/mdv200. ISSN 0923-7534.
  5. ↑ Campo E, Swerdlow SH, Harris NL, Pileri S, Stein H, Jaffe ES (2011). "The 2008 WHO classification of lymphoid neoplasms and beyond: evolving concepts and practical applications". Blood. 117 (19): 5019–32. doi:10.1182/blood-2011-01-293050. PMC 3109529. PMID 21300984.
  6. ↑ Matutes E (2006). "Immunophenotyping and differential diagnosis of hairy cell leukemia". Hematol Oncol Clin North Am. 20 (5): 1051–63. doi:10.1016/j.hoc.2006.06.012. PMID 16990106.


Template:WikiDoc Sources

Retrieved from "https://www.wikidoc.org/index.php?title=Hairy_cell_leukemia_classification&oldid=1561886"