Abstract
An autopsy case of malignant histiocytosis (MH) in a 16 y.o. male is reported. He was acutely ill at admission, complaining of high fever, diarrhea and generalized skin symptoms. Hepatosplenomegaly and an enlarged lymph node were noticed. Bone marrow aspirate showed increased numbers of histiocytoid cells with various degree of atypism. They had vacuoles and azurophilic granules, but rarely showed erythrophagocytosis. Biopsied lymph node microscopically showed proliferation of atypical cells with the distinct appearance of histiocyte. Immunohistochemically, they had positive marker stain for lysozyme, α1-antitrypsin and anti-macrophage. A diagnosis of MH was made. Genotypic analysis by Southern method showed germline TCR and JH genes. Ultrastructurally, mature tumor cells were rich in organallae and contained 10nm filaments. The patient died after development of marked pancytopenia. Total duration of illness was 4.5 months. Autopsy revealed systemic involvement of lymph nodes, bone marrow, liver, spleen and lungs by diffuse proliferation of atypical histiocytoid cells. They showed a diversity of cellular atypism, including mature and immature histiocytes, and pleomorphic and multinucleated giant cells. The present case is characterised clinically by prominent skin desquamation and progressive course, and pathologically by proliferation of a diversity of neoplastic histiocytoid and its precursor cells.
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