[Peripheral T-cell lymphoma associated with hemophagocytic syndrome: a recently identified entity. Clinico-pathologic and immunohistochemical study of 2 cases]
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A Saragoni1, B Falini, L Medri
1Servizio di anatomia e istologia patologica, Ospedale Morgagni-Pierantoni di Forlì.
Abstract:
The authors report on two examples of peripheral T-cell lymphoma associated with hemophagocytic syndrome. The latter represents a recently described entity, which is characterized by pancytopenia, hepatosplenomegaly, fever and rapid downhill clinical course. Microscopic examination of different tissue samples shows bone-marrow, liver and spleen infiltration by small-medium sized lymphoid elements, which display peripheral T-cell phenotype and are associated with an exuberant hyperplasia of hemophagocytosing, benign-looking histiocytes. The clinical and pathological findings, along with the main differential diagnoses, are discussed.