A tumor of alpha/beta CD8+ cytotoxic T-cells with almost
exclusive involvement of the subcutaneous tissues. Cell size ranges from small, to medium-sized or
This lymphoma affects a wide age range. Patients develop
subcutaneous nodules most frequently on legs, trunk, arms, face. Initially this disease follows an
indolent course, but some patients develop a usually fatal hemophagocytic syndrome, manifesting as
pancytopenia and/or coagulopathy. Sometimes subcutaneous nodules are accompanied by fever and malaise.
Hepatosplenomegaly can be present but lymphadenopathy is usually absent.
Lobular panniculitis that appears deceptively benign.
Polymorphous infiltrate centered on fat with rimming of fat cells by atypical cells. Karyorrhexis
results in so-called bean bag cells. Many cases show angioinvasion/ angiodestruction and are associated
with fat necrosis. Hemophagocytosis may be present.
A histiocytic/ granulomatous component is usually present and may be the predominating feature. +/-
CD8+, (sometimes coexpression of CD8+ and CD4+), CD4-,
TIA-1+, CD3+, CD56-. BF1+
Cloncally rearranged T-cell receptor, but no
specific chromosomal abnormalities. EBV negative.
Protracted clinical course, often with spontaneous temporary
remissions. Some patients develop a terminal hemophagocytic syndrome after prolonged disease duration.
lupus panniculitis, cytophagic histiocytic
Initial description of SPLTL included cases with a gamma/delta
T-cell phenotype. These cases are often CD4-, and CD8- double negative and co-express CD56. These cases
pursue an aggressive clinical course and are excluded from the SPLTL category in the WHO-EORTC. They are
grouped either with extranodal NK/T cell lymphoma, nasal type or gamma/delta T-cell lymphoma.
- Subcutaneous panniculitic T-cell lymphoma is a tumor of cytotoxic T lymphocytes. Hum Pathol. 1998 Apr;29(4):397-403.
- Marzano AV, Berti E, Paulli M, Caputo R. Cytophagic histiocytic panniculitis and subcutaneous panniculitis-like T-cell lymphoma: report of 7 cases. Arch Dermatol. 2000 Jul;136(7):889-96.
- Salhany KE, Macon WR, Choi JK, Elenitsas R, Lessin SR, Felgar RE, Wilson DM, Przybylski GK, Lister J, Wasik MA, Swerdlow SH. Subcutaneous panniculitis-like T-cell lymphoma: clinicopathologic, immunophenotypic, and genotypic analysis of alpha/beta and gamma/delta subtypes. Am J Surg Pathol. 1998 Jul;22(7):881-93.
- Massone C, Chott A, Metze D, Kerl K, Citarella L, Vale E, Kerl H, Cerroni L. Subcutaneous, blastic natural killer (NK), NK/T-cell and other cytotoxic lymphomas of the skin. Am J Surg Pathol 2004;28:719-735.
- El Shabrawi-Caelen L, Cerroni L, Kerl H. The clinicopathologic spectrum of cytotoxic lymphomas of the skin. Semin Cutan Med Surg 2000 Jun;19(2):118-123.
- Santucci M et al. Cytotoxic/ Natural killer cell cutaneous lymphomas. Cancer 2003,97:610-627.