Subcutaneous Panniculitis-Like T-Cell Lymphoma (SPTCL) with alpha-beta T-Cell Receptors (TCR) is usually an indolent condition that responds well to high-dose systemic corticosteroid therapy.


Clinical features:

(1) B-symptoms (fever, chills, night sweats, weight loss) may be absent or mild

(2) recurrent nonulcerating subcutaneous nodules or plaques

(3) cytopenias may be absent or mild

(4) variable presence of an autoimmune disorder

(5) some patients develop the hemophagocytic syndrome (HPS), which is associated with a worse prognosis

(6) absence of lymphoma in lymph nodes or other extracutaneous sites


Pathologic features:

(1) medium to large lymphocytes with nuclear pleomorphism infiltrate the subcutaneous tissue (panniculitis)

(2) patchy necrosis is present

(3) apoptosis is mild to moderate


Immunoperoxidase staining:

(1) CD3 positive and CD4 negative

(2) CD8 positive

(3) CD30, CD56 and TCR-delta negative


Differential diagnosis:

(1) cutaneous T-cell lymphoma with gamma-delta T-cell receptors

(2) other forms of panniculitis


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