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

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== Overview ==
== Overview ==
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare type of T-cell [[non-Hodgkin lymphoma]] (NHL).


==Classification==
==Classification==
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== Pathophysiology==
== Pathophysiology==
Subcutaneous panniculitis-like T-cell lymphoma is a cytotoxic T-cell lymphoma, which preferentially infiltrates subcutaneous tissue, primarily involving the extremities and trunk. It is composed of atypical lymphoid cells of varying size, often with marked [[fat necrosis]] and [[karyorrhexis]].
=== Associated Conditions===
=== Associated Conditions===


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== Screening ==
== 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=uspreventive> Recommendations. U.S Preventive Services Task Force.  Accessed on March 8, 2016</ref>
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>
== Natural History, Complications and Prognosis==
== Natural History, Complications and Prognosis<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>==
 
* With subcutaneous panniculitis-like T-cell lymphoma, nodules form under the skin in the subcutaneous tissue (subcutaneous nodules).
* Usually, the nodules occur in the legs or trunk of the body. 
* 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]])
* 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 ==  
== Diagnosis ==  
===Staging===
===Staging===

Revision as of 16:53, 8 March 2016

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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]

Synonyms and keywords:: SPTCL; SPTCL-AB; SPTCL-GD

Overview

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare type of T-cell non-Hodgkin lymphoma (NHL).


Classification

Subcutaneous panniculitis-like T-cell lymphoma may be classified into either alpha/beta subtype (SPTCL-AB) or gamma/delta subtype (SPTCL-GD).

Subcutaneous panniculitis-like T-cell lymphoma classification[1]
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. It is composed of atypical lymphoid cells of varying size, often with marked fat necrosis and karyorrhexis.

Associated Conditions

Microscopic Pathology

Causes

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

Differentiating type page name here from other Diseases

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

Epidemiology and Demographics

Age

The incidence of subcutaneous panniculitis-like T-cell lymphoma increases with age; the median age at diagnosis is 35 years.

Gender

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

Risk Factors

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

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.[2]

Natural History, Complications and Prognosis[1]

  • With subcutaneous panniculitis-like T-cell lymphoma, nodules form under the skin in the subcutaneous tissue (subcutaneous nodules).
  • Usually, the nodules occur in the legs or trunk of the body.
  • 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

Staging

Staging for subcutaneous panniculitis-like T-cell lymphoma is provided in the following table:[3]

Revised staging system for primary nodal lymphomas (Lugano classification)
Stage Involvement Extranodal (E) status
Limited
Stage I One node or a group of adjacent nodes Single extranodal lesions without nodal involvement
Stage II Two or more nodal groups on the same side of the diaphragm Stage I or II by nodal extent with limited contiguous extranodal involvement
Stage II bulky II as above with "bulky" disease Not applicable
Advanced
Stage III Nodes on both sides of the diaphragm; nodes above the diaphragm with spleen involvement Not applicable
Stage IV Additional noncontiguous extralymphatic involvement Not applicable

Symptoms

Symptoms of the subcutaneous panniculitis-like T-cell lymphoma include:

Physical Examination

Vitals

Skin

  • Subcutaneous nodules

HEENT

Thorax

Abdomen

Extremities

Laboratory Findings

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

Biopsy

Lymph node or bone marrow 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

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[1]
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

  1. 1.0 1.1 1.2 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
  2. Recommendations. U.S Preventive Services Task Force. Accessed on March 8, 2016
  3. Cheson, Bruce D.; Fisher, Richard I.; Barrington, Sally F.; Cavalli, Franco; Schwartz, Lawrence H.; Zucca, Emanuele; Lister, T. Andrew; Alliance, Australasian Leukaemia and Lymphoma Group; Eastern Cooperative Oncology Group; European Mantle Cell Lymphoma Consortium; Italian Lymphoma Foundation; European Organisation for Research; Treatment of Cancer/Dutch Hemato-Oncology Group; Grupo Español de Médula Ósea; German High-Grade Lymphoma Study Group; German Hodgkin's Study Group; Japanese Lymphorra Study Group; Lymphoma Study Association; NCIC Clinical Trials Group; Nordic Lymphoma Study Group; Southwest Oncology Group; United Kingdom National Cancer Research Institute (2014-09-20). "Recommendations for initial evaluation, staging, and response assessment of Hodgkin and non-Hodgkin lymphoma: the Lugano classification". Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology. 32 (27): 3059–3068. doi:10.1200/JCO.2013.54.8800. ISSN 1527-7755. PMID 25113753.