Track: Case Reports

Topic: Lymphoproliferative Disorders

Abstract:

A 63-year-old male presented with a six-week history of a slowly progressive, pruritic rash affecting his trunk and proximal extremities. A few months prior, he had complaints of dark urine and left upper abdominal pain. Initial work-up revealed elevated liver enzymes, lymphocytosis, splenomegaly, a pancreatic mass, and hypermetabolic lymphadenopathy in multiple regions. The rash began as pruritic, pinpoint, non-blanching erythematous macules in the axillae, which subsequently spread over weeks. Skin biopsy revealed atypical lymphocytic infiltrate with moderate cytologic atypia and hyperchromatism. There were scattered mitotic figures and dermal hemorrhage. No epidermotropism was identified. The atypical cells were CD3, CD4, CD5, and CD7 positive. Lymph node fine needle aspiration and peripheral blood flow cytometry revealed a clonal population of T-cells positive for CD2, CD3, CD4, CD5, CD7, CD43, and GATA3 and negative for CD25. Clonal T-cell receptor gamma and beta gene rearrangement assays were positive on the lymph node tissue and TCR gamma was positive on the skin tissue. Bone marrow biopsy results confirmed a CD52 negative T-cell prolymphocytic leukemia (T-PLL) with 17p- aberration. Immunohistochemical staining of the skin biopsy confirmed these markers and cutaneous infiltration of T-PLL. Fluorescence in situ hybridization for TCL1 rearrangement was positive at 84%. The patient is currently undergoing treatment with fludarabine/mitoxantrone/cyclophosphamide, with plans for allogeneic hematopoietic stem cell transplantation. This case illustrates T-cell prolymphocytic leukemia with cutaneous infiltration. T-PLL involves the skin in roughly 27% of cases and is an important differential for dermatologists and dermatopathologists to consider.

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Nakhle Saba, MD