[Multicentric histiocytosis with hematological involvement].

INTRODUCTION

The aim of this work was to present a case of multicentric 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 spleen 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 to the diagnosis of multicentric histiocytosis. Bone marrow aspiration was normal. Pancytopenia then developed with bone marrow hypoplasia without infiltration. Corticosteroids then cyclophosphamide were uneffective for either the skin disease or the hematologic disorder. The patient developed severe buccal aphthosis which responded well to thalidomide. This treatment reduced the size and the number of skin nodules. Finally, renal failure of unknown origin was observed.

DISCUSSION

Electron microscopy is essential for positive and differential diagnosis of atypical multicentric histiocytosis. Hematological disorders associated with multicentric histiocytosis may either be specific or totally independent.