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[伴有严重血小板增多症的巨核细胞假性髓细胞化生]

[Megakaryocytic pseudomyelosis with severe thrombocytosis].

作者信息

Krug K, Rohrberg R, Wessel H

出版信息

Z Gesamte Inn Med. 1981 Oct 1;36(19):734-7.

PMID:6171109
Abstract

A 66-year-old female patient complained of loss of body weight and fatigue. The clinical examination revealed a thrombocytosis with a maximum count of 3.200 . 10(9) platelets and a leukocytosis with maximally 25 . 10(9) white cells in the peripheral blood. The bone marrow showed a large increase of megakaryocytes. Under the diagnosis of megakaryocytic myelosis a chemotherapy with 186 mg busulfan was performed. In the course of this treatment the clinical picture of a sepsis occurred which could not be controlled by antibiotics. The patient died four months after her admission to the clinic. The essential findings in autopsy were a caseous tuberculosis of the lymph nodes with haematogenic generalization which appeared as a septic tuberculosa gravissima ("typhobacillosis" Landouzy). The bone marrow was atrophic. Spleen liver and lymph nodes were without evidence for a myeloproliferative disorder. Thus, the initial diagnosis had to be changed to a megakaryocytic pseudomyelosis with massive thrombocytosis as a reaction to the tuberculous infection. The differential diagnosis of megakaryocytic myelosis, other disorders of the myeloproliferative syndrome, and the reactive thrombocytosis are discussed.

摘要

一名66岁女性患者主诉体重减轻和疲劳。临床检查发现血小板增多症,外周血中血小板最高计数为3200×10⁹,白细胞增多症,白细胞最高计数为25×10⁹。骨髓显示巨核细胞大量增加。在诊断为巨核细胞骨髓增殖症后,进行了186毫克白消安的化疗。在治疗过程中出现了败血症的临床表现,抗生素无法控制。患者入院四个月后死亡。尸检的主要发现是淋巴结干酪样结核伴血行播散,表现为严重的脓毒症性结核(兰杜齐的“伤寒杆菌病”)。骨髓萎缩。脾脏、肝脏和淋巴结无骨髓增殖性疾病证据。因此,最初的诊断不得不改为巨核细胞假性骨髓增殖症伴大量血小板增多,作为对结核感染的反应。文中讨论了巨核细胞骨髓增殖症、骨髓增殖综合征的其他疾病以及反应性血小板增多症的鉴别诊断。

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