Conditions / Immune
subcutaneous panniculitis-like T-cell lymphoma
info · Immune · ICD-10: C86.3
A primary cutaneous T-cell non-Hodgkin lymphoma characterized by infiltration of subcutaneous tissue by neoplastic alpha-beta cytotoxic T cells mimicking panniculitis. Homozygous or compound heterozygous mutation in the HAVCR2 gene may contribute to developmen
A primary cutaneous T-cell non-Hodgkin lymphoma characterized by infiltration of subcutaneous tissue by neoplastic alpha-beta cytotoxic T cells mimicking panniculitis. Homozygous or compound heterozygous mutation in the HAVCR2 gene may contribute to development of this disease, with some evidence of incomplete penetrance.
Signs and symptoms
- Panniculitis
- Hypertriglyceridemia
- Hypofibrinogenemia
- Increased circulating ferritin concentration
- Fever
- Autoimmunity
- Facial edema
- Hemophagocytosis
- Anemia
- Pancytopenia
Also known as: SPTCL