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).

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

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)	Included in the cutaneous T-cell lymphoma group. More aggressive type. Hemophagocytic syndrome is most often associated with SPTCL-GD and has a less favourable prognosis.

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.

Microscopic Pathology

• 

  Biopsy of soft tissue : lymphoid infiltrate with pattern resembling lobular and septal panniculitis

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:

• Benign panniculitis
• Cutaneous T-cell lymphoma
• Extranodal NK-T-cell lymphoma

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.

Natural History, Complications and Prognosis

• 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:

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:

• 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

• Cervical lymphadenopathy

Thorax

• Thoracic masses suggestive of central lymphadenopathy

Abdomen

• Abdominal masses suggestive of central lymphadenopathy
• Hepatosplenomegaly

Extremities

• Peripheral lymphadenopathy

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
• Immunophenotyping

• Positive: BetaF1; CD8; CD30; CLA; T-cell intracellular antigen (T1A1); and perforin
• Negative: CD3; CD5; and CD56

Biopsy

Lymph node or subcutaneous nodule 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

Therapy	Description
Chemotherapy	Combination therapy : CHOP – Cyclophosphamide AND Doxorubicin AND Vincristine AND Prednisone
Radiation therapy	External beam radiation therapy may be used to treat the lymph nodes.
Stem cell transplant	May be used in some people with subcutaneous panniculitis-like T-cell lymphoma when their lymphoma recurs after treatment.