MULTICENTRIC RETICULOHISTIOCYTOSIS WITH H EMATOLOGIC INVOLVEMENT

Introduction. The aim of this work was to present a case of multicentr ic histiocytosis with haematologic involvement. Case report. A 68-year -old man presented with poor general health and a nodular eruption of the skin and larynx. On clinical examination there was an enlarged spl een and laboratory results revealed an inflammatory syndrome, platelet count 60,000 and myelemia with 10 p. 100 immature elements in a white cell count of 14,000. Pathology and ultrastructure examinations led t o the diagnosis of multicentric histiocytosis. Bone marrow aspiration was normal. Pancytopenia then developed with bone marrow hypoplasia wi thout infiltration. Corticosteroids then cyclophosphamide were uneffec tive for either the skin disease or the hematologic disorder. The pati ent developed severe buccal aphthosis which responded well to thalidom ide. This treatment reduced the size and the number of skin nodules. F inally, renal failure of unknown origin was observed. Discussion. Elec tron microscopy is essential for positive and differential diagnosis o f atypical multicentric histiocytosis. Hematological disorders associa ted with multicentric histiocytosis may either be specific or totally independent.