Image Description
Prognosis of subcutaneous panniculitis-like T-cell lymphoma (SPTCL): It is an indolent lesion with excellent prognosis. It remains confined to the subcutaneous tissues throughout its clinical course. The 5-yr disease-specific survival rate is 80-90%. If the patients develop hemophagocytic syndrome, the prognosis worsens and the 5-yr survival rate drops to around 50%.
The first line treatment consists of immunomodulation therapy with steroids or cyclosporin A. Patients who develop hemophagocytic syndrome require systemic chemotherapy.
This high magnification image of SPTCL shows small to medium-sized pleomorphic lymphoid cells with hyperchromatic nuclei surrounding individual adipocytes.
About the Disease
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare cytotoxic T-cell lymphoma involving subcutaneous fat. It is positive for alpha-beta receptor (Beta F1+). Cases that express gamma-delta are classified as cutaneous gamma-delta T-cell lymphoma. SPTCL is seen over a wide age range (median age 36 years). Patients develop solitary or multiple, firm, erythematous/violaceous, deep-seated plaques or nodules without ulceration, usually on extremities or trunk. About 50% of patients have B symptoms (fever, malaise, fatigue, and weight loss). SPTCL is associated with immune dysregulation. Many patients have history of systemic lupus erythematosus (20% of cases) or have received immunomodulation or immunosuppressive therapy. Lupus erythematosus panniculitis (LEP) and SPTCL share clinical and morphologic features. The neoplastic cells are pleomorphic, mostly small to medium-sized with occasional large cells localized to the subcutaneous fat. They have hyperchromatic nuclei, scant cytoplasm, and surround individual adipocytes. Immunophenotype: Positive markers: CD3, CD8, Beta F1, TIA1, granzyme B, perforin; Negative markers: CD4, CD56, gamma-delta T-cell receptor, CD30, EBV. T-cell receptor genes are clonally rearranged. SPTCL is an indolent lesion with favorable prognosis. It remains confined to the skin throughout its clinical course. The 5-yr disease-specific survival rate is 80-90%. Prognosis worsens if hemophagocytic syndrome develops. References:Calonje, E et al (2020). McKee's Pathology of the Skin with Clinical Correlations - 5th Ed. Elsevier. Jaffe, E. S. et al (2017). Hematopathology - Second Edition. Philadelphia, PA. Elsevier. Revised 4th Edition of WHO Classification of Tumors of Hematopoietic & Lymphoid Tissues, 2017.