Abstract
Malignant histiocytosis is characterised by a systemic, neoplastic proliferation of histiocytes and their precursors. The clinical and pathologic expressions are quite variable among children with this entity and there is no uniform conceptual classification.
In this paper, a 12 y.o. boy was reported to have malignant histiocytosis with unusual clinical picture such as unhealing granuloma and hypergammaglobulinemia. The patient was admitted with remittent fever of one month's duration, swollen tender left leg, and a necrotic and ulcerative granulation in the soft palate. The laboratory examinations on admission showed leucopenia and raised serum IgG and IgE. There was no lymphadenopathy or hepatosplenomegaly. The biopsy of the palatal lesion mainly consisted of granulomatous tissues with necrosis. The infiltration of atypical histiocytes was observed around the necrosis in small amounts. However the repeated biopsies of the palatal lesion and left leg revealed negative for atypical histiocytes. The granulomatous lesions were progressive, and were resistant to antibiotic therapy, and the patient subsequently developed hepatosplenomegaly. Malignant histiocytosis was seriously suspected. VEMP chemotherapy and irradiation were instituted to cause a temporary regression of granuloma but the patient died after one month. Atypical histiocytes were demonstrated in the peripheral and the bone marrow blood smears at the late stage and the diagnosis of malignant histiocytosis was confirmed by postmortem needle biopsy of the spleen.
