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RISS 인기검색어
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- 저자 : 이근석(K . S . Lee) (검색결과 1 건)
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이종태(J . T . Lee),이근석(K . S . Lee),김인호(I . H . Kim),방영주(Y . J . Bang),박선양(S . Y . Park),김병국(B . K . Kim),김노경(N . N . Kim),허대석(D . S . Heo) 대한내과학회 1996 대한내과학회지 Vol.51 No.4
N/A Objectives: Malignant histiocytosis is a rare, rapidly fatal disorder, characterized by systemic proliferation of abnormal histiocytes. Most patients present with fever, organomegaly, and pancytopenia, and die within a few months. Although malignant histiocytosis represents diagnostically an important histiocytic disorder, its pathogenesis, natural history, response to therapy are not well known. In Korea, there are few reports on malignant histiocytosis. The purpose of this investigation is to assess the clinical spectrum natural history, laboratory findings, histopathologic findings response to therapy of malignant histijiocytos is diagnosed at Seoul national university hospital. Methods: The clinical records and histologic materials from 29 cases of malignant histiocytosis diagnosed at Seoul national university hospital between 1985 and 1994 have been reviewed. The criteria for the histopathologic diagnosis of malignant histiocytosis were the presence of large, abnormal malignant histiocytes with frequent mistosis to be distinguished from reactive histiocytic proliferation. All histologic materials were fixed in formalin, embedded in paraffin, sectioned, and stained with hematoxylin and eosin. Bone marrow aspirates were stained with Jener-Giemsa. of 29 patients, 20 patients were treated with combination chemotherapy; CHOP in 8 patients, COPBLAM in 3 patients, BVP in 7 patients, C-MOPP in 1 patient, Pdplusvincristine in 1 patient. Results: The median age was 32 years, with a 2.2:1 male to female prepondance. Major physical findings included temperature elevation (93%), hepatomegaly (90%), splenomegaly (86%), jaundice (5%). Common laboratory findings were anemia (86%), thrombocytopenia (90%), splenomegaly (86%), jaundice (45%). Common laboratory findings were anemia (86%), thrombocytopenia (90%), leukopenia (72%), hypocalcemia (97%), abnormal liver function test (100%). Histologic features observed in bone marrow were increased histiocytes, atypical immature histiocytic infiltration with hemophagocytosis. Histologic features observed in lymph node were destruction of normal structure with infiltration and proliferation of immature histiocytes. Prominent features observed in liver were sinusoidal histiocytic infiltration. The response to treatment was encouraging. One patient achieved a completed remission (5%), 5 patients achieved a partial remission (25%), and overall response rates were 30%. Conclusion: The distinctive clinical and histologic findings warrant recognition and separation of malignant histiocytosis from other histiocytic and hematopoietic disorders and long-term survival is possible in some patients if treated early and aggressively with combination chemotherapy.
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