Subcutaneous panniculitis-like T-cell lymphoma in a 25-year-old male patient with sickle cell disease

Abstract Sickle cell disease (SCD) is a hereditary blood disorder that often has multiple comorbidities. Patients occasionally develop malignant neoplasms, but the risk of lymphoma in SCD is currently unknown. Here, we report a unique case of subcutaneous panniculitis-like T-cell lymphoma (SPTCL) in a 25-year-old male patient with SCD. The patient suffered from episodes of sickling crisis since his initial SCD diagnosis and had been treated with supportive care. Hydroxyurea was added at the age of 23 years old. Two years later, he presented with right cheek swelling, and the biopsy showed a lymphohistiocytic infiltrate within adipose tissue resembling lobular panniculitis. Immunohistochemistry demonstrated CD8/β-F1-positive T-cells around the fat vacuoles, with a high proliferative index. The histopathologic features suggested a diagnosis of SPTCL. A subsequent TCR β gene rearrangement analysis detected a clonal amplicon, confirming the diagnosis. Because of the lack of systemic symptoms, the patient received conservative therapy with prednisone and responded well with resolution of his right cheek swelling within one month. To the best of our knowledge, this is the first reported case of SPTCL associated with SCD. The proposed lymphomagenesis in the setting of SCD is also discussed.