Subcutaneous panniculitis-like T-cell lymphoma

Jump to: navigation, search

WikiDoc Resources for Subcutaneous panniculitis-like T-cell lymphoma

Articles

Most recent articles on Subcutaneous panniculitis-like T-cell lymphoma

Most cited articles on Subcutaneous panniculitis-like T-cell lymphoma

Review articles on Subcutaneous panniculitis-like T-cell lymphoma

Articles on Subcutaneous panniculitis-like T-cell lymphoma in N Eng J Med, Lancet, BMJ

Media

Powerpoint slides on Subcutaneous panniculitis-like T-cell lymphoma

Images of Subcutaneous panniculitis-like T-cell lymphoma

Photos of Subcutaneous panniculitis-like T-cell lymphoma

Podcasts & MP3s on Subcutaneous panniculitis-like T-cell lymphoma

Videos on Subcutaneous panniculitis-like T-cell lymphoma

Evidence Based Medicine

Cochrane Collaboration on Subcutaneous panniculitis-like T-cell lymphoma

Bandolier on Subcutaneous panniculitis-like T-cell lymphoma

TRIP on Subcutaneous panniculitis-like T-cell lymphoma

Clinical Trials

Ongoing Trials on Subcutaneous panniculitis-like T-cell lymphoma at Clinical Trials.gov

Trial results on Subcutaneous panniculitis-like T-cell lymphoma

Clinical Trials on Subcutaneous panniculitis-like T-cell lymphoma at Google

Guidelines / Policies / Govt

US National Guidelines Clearinghouse on Subcutaneous panniculitis-like T-cell lymphoma

NICE Guidance on Subcutaneous panniculitis-like T-cell lymphoma

NHS PRODIGY Guidance

FDA on Subcutaneous panniculitis-like T-cell lymphoma

CDC on Subcutaneous panniculitis-like T-cell lymphoma

Books

Books on Subcutaneous panniculitis-like T-cell lymphoma

News

Subcutaneous panniculitis-like T-cell lymphoma in the news

Be alerted to news on Subcutaneous panniculitis-like T-cell lymphoma

News trends on Subcutaneous panniculitis-like T-cell lymphoma

Commentary

Blogs on Subcutaneous panniculitis-like T-cell lymphoma

Definitions

Definitions of Subcutaneous panniculitis-like T-cell lymphoma

Patient Resources / Community

Patient resources on Subcutaneous panniculitis-like T-cell lymphoma

Discussion groups on Subcutaneous panniculitis-like T-cell lymphoma

Patient Handouts on Subcutaneous panniculitis-like T-cell lymphoma

Directions to Hospitals Treating Subcutaneous panniculitis-like T-cell lymphoma

Risk calculators and risk factors for Subcutaneous panniculitis-like T-cell lymphoma

Healthcare Provider Resources

Symptoms of Subcutaneous panniculitis-like T-cell lymphoma

Causes & Risk Factors for Subcutaneous panniculitis-like T-cell lymphoma

Diagnostic studies for Subcutaneous panniculitis-like T-cell lymphoma

Treatment of Subcutaneous panniculitis-like T-cell lymphoma

Continuing Medical Education (CME)

CME Programs on Subcutaneous panniculitis-like T-cell lymphoma

International

Subcutaneous panniculitis-like T-cell lymphoma en Espanol

Subcutaneous panniculitis-like T-cell lymphoma en Francais

Business

Subcutaneous panniculitis-like T-cell lymphoma in the Marketplace

Patents on Subcutaneous panniculitis-like T-cell lymphoma

Experimental / Informatics

List of terms related to Subcutaneous panniculitis-like T-cell lymphoma

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; 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: [4]

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][4]

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. [4]

Natural History, Complications and Prognosis

  • Painless nodules form under the skin in the subcutaneous tissue (subcutaneous nodules). [4][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] [4]

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

  1. 1.0 1.1 1.2 1.3 1.4 1.5 1.6 1.7 Subcutaneous panniculitis-like T-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52df/. Accessed on March 08, 2016
  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
  3. 3.0 3.1 3.2 3.3 3.4 3.5 3.6 An Illustrative Case of Subcutaneous Panniculitis-Like T-Cell Lymphoma. Hindawi Publishing Corporation. http://www.hindawi.com/journals/jsc/2011/824528/. Accessed on March 08, 2016
  4. 4.0 4.1 4.2 4.3 4.4 Parveen Z, Thompson K (2009). "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.". Arch Pathol Lab Med. 133 (2): 303–8. PMID 19195975. doi:10.1043/1543-2165-133.2.303. 

Linked-in.jpg