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; SPTCL-AB; SPTCL-GD

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.

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.

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

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

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.