Subcutaneous panniculitis-like T-cell lymphoma

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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; 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. Subcutaneous panniculitis-like T-cell lymphoma may be classified into either alpha/beta subtype (SPTCL-AB) or gamma/delta subtype (SPTCL-GD). On microscopic histopathological analysis, atypical lymphoid cells, fat necrosis, and karyorrhexis are characteristic findings of subcutaneous panniculitis-like T-cell lymphoma. Subcutaneous panniculitis-like T-cell lymphoma commonly affects young adults (35 years). Females are more commonly affected with subcutaneous panniculitis-like T-cell lymphoma than males. 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 subcutaneous panniculitis-like T-cell lymphoma include fever, weight loss, night sweats, and painless swellings on extremities and trunk. Subcutaneous nodule biopsy is diagnostic of subcutaneous panniculitis-like T-cell lymphoma. The predominant therapy for subcutaneous panniculitis-like T-cell lymphoma is chemotherapy. Adjunctive radiotherapy and stem cell transplant may be required.[1][2]

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[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 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

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]

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.[4]

Natural History, Complications and Prognosis

  • With subcutaneous panniculitis-like T-cell lymphoma, nodules form under the skin in the subcutaneous tissue (subcutaneous nodules).[2][1]
  • 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

Symptoms

Symptoms of the subcutaneous panniculitis-like T-cell lymphoma include:[1]

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. Recommendations. U.S Preventive Services Task Force. http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=Subcutaneous+panniculitis-like+T-cell+lymphoma. Accessed on March 8, 2016

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