Subcutaneous panniculitis-like T-cell lymphoma: Difference between revisions
No edit summary |
No edit summary |
||
| Line 9: | Line 9: | ||
== Overview == | == Overview == | ||
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare type of T-cell [[non-Hodgkin lymphoma]] (NHL). | |||
==Classification== | ==Classification== | ||
| Line 31: | Line 34: | ||
== 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=== | ||
| Line 52: | Line 56: | ||
== 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
For patient information, click Insert page name here
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).
| Name | Description |
|---|---|
| Alpha/Beta subtype (SPTCL-AB) |
|
| 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:
- 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
Staging
Staging for subcutaneous panniculitis-like T-cell lymphoma is provided in the following table:[3]
| 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:
- Fever
- Weight loss
- Night sweats
- Painless swelling in the neck, axilla, groin, thorax, and abdomen
Physical Examination
Vitals
- Fever is often present
Skin
- Subcutaneous nodules
HEENT
Thorax
- Thoracic masses suggestive of central lymphadenopathy
Abdomen
- Abdominal masses suggestive of central lymphadenopathy
- Hepatosplenomegaly
Extremities
Laboratory Findings
Laboratory tests for subcutaneous panniculitis-like T-cell lymphoma include:
- Complete blood count (CBC): cytopenia
- Blood chemistry studies: Increased liver function tests
- Cytogenetic analysis
- Flow cytometry
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
| Therapy | Description |
|---|---|
| Chemotherapy |
|
| Radiation therapy |
|
| Stem cell transplant |
|
References
- ↑ 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
- ↑ Recommendations. U.S Preventive Services Task Force. Accessed on March 8, 2016
- ↑ 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.