Papuloerythroderma heralding recurrence of an aggressive T-cell Leukemia

Abstract

Background: T-cell prolymphocytic leukemia (T-PLL) is a rare and aggressive malignancy. Diagnosis is achieved by proper interpretation of the morphology, immunophenotype, and cytogenetics obtained. It is highly responsive to the single agent alemtuzumab and providing treatment once diagnosed dramatically improves survival. Case Discussion: A 49-year-old Filipino female, diagnosed with T-PLL, achieved morphologic remission after six cycles of cyclophosphamide, doxorubicin, vincristine, and prednisone. After three months, she presented with a diffuse and generalized erythematous papulonodular cutaneous eruption with a markedly elevated white blood cell count. The peripheral blood smear was distinctive for prolymphocytes seen as large mononuclear cells with slightly condensed chromatin with one to two very prominent nucleoli. Flow cytometry showed bright CD5, CD7, and moderately bright CD3, CD5, cCD3, CD2. Immunohistochemistry of the bone marrow core was TdT negative. Punch biopsies of the skin revealed a malignant dermal lymphoid proliferation with IHC markers positive for T-cell lymphoid infiltration. The findings were consistent with relapsed disease. Her white blood cell count continued to rise despite treatment and she expired due to hyperleukocytosis. Conclusion: T-PLL is an aggressive malignancy with a historic median overall survival of seven months with conventional chemotherapy and up to two years with the advent of alemtuzumab therapy. Skin lesions may be seen in up to 25% of cases and of the reported cutaneous manifestations, erythroderma is rare. We report a rare case of T-PLL recurrence heralded by a diffuse and generalized erythematous papulonodular cutaneous eruption.

Keywords

• T-cell leukemia
• Prolymphocytic leukemia
• Papuloerythroderma,
• Cutaneous manifestations