Premium
Malignant histiocytosis associated with autoimmune thrombocytopenia
Author(s) -
Ohno Tatsuharu,
Sugiyama Tateo,
Furukawa Hiroo,
Ishibashi Takafumi,
Okuma Minoru
Publication year - 1994
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.2830450310
Subject(s) - autoantibody , pathology , bone marrow , histiocytosis , spleen , medicine , malignant histiocytosis , immunohistochemistry , platelet , lymphoma , langerhans cell histiocytosis , infiltration (hvac) , monocyte , antibody , immunology , histiocyte , physics , disease , thermodynamics
We describe a patient with malignant histlocytosis whose serum contained an autoantlbody against platelet protein. The patient was admitted because of nasal bleeding and high‐grade fever. The clinical course was fulminantly progressive and terminated with cerebral hemorrhage. Bone marrow aspirate showed the proliferation of large atypical cells, some of which exhibited phagocytic activity. At postmortem examination, there was diffuse infiltration of these atypical cells In the liver, spleen, kidney, and bone marrow. Morphological, cytochemlcal, immunohistochemlcal, and genotypic characteristics suggested that the proliferating cells were derived from the monocyte‐rnacrophage system. Western blot analysis revealed the presence of autoantibody against an ∼88 kd molecule of platelet proteins. Although the relationship of cause and effect remains to be clarified, this autoantibody appeared to have stimulated thrombophagocytosls of the neoplastic cells. © 1994 Wiley‐Liss, Inc.