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Abstract
Two cases of peripheral T-cell lymphoma with primarily subcutaneous involvement and clinically presenting as panniculitis were reported in two children. One child developed florid hemophagocytic syndrome and was treated by combination chemotherapy but died 14 months later of disseminated fungal infection. The other child had mild systemic symptoms but no histological evidence of hemophagocytosis. Despite a more florid lymphomatous infiltrate, he attained a complete remission after a short course of prednisone and remains symptom-free at 3 years. Our report confirms that subcutaneous T-cell lymphoma is a distinct clinicopathological entity and highlights the two modes of clinical presentation. The development of florid hemophagocytic syndrome indicates a bad prognosis. This entity must be distinguished from other causes of panniculitis and immunophenotypic analysis of the atypical cells is essential in the diagnosis. Combination chemotherapy is the treatment of choice, but in patients who pursue an indolent course the administration of aggressive chemotherapy may be deferred.