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慢性特发性骨髓纤维化。一种可逆性疾病?

Chronic idiopathic myelofibrosis. A reversible disease?

作者信息

Kelemen E, Krasznai G, Endes P, Szinay G

出版信息

Acta Haematol. 1977;57(3):171-7. doi: 10.1159/000207878.

Abstract

A patient with chronic idiopathic myelofibrosis was subjected to splenectomy 1 year after diagnosis. As a clinically unexpected finding, lymph node biopsy suggested the presence of non-Hodgkin lymphoma. The patient was subjected to intensive combined cytostatic therapy. In the following months, signs and symptoms of myelofibrosis regressed remarkably. The patient died 31 months after splenectomy in massive gastrointestinal bleeding. At post-mortem, myelofibrosis could not be detected in three bone marrow areas and a regular, fat-containing, hypercellular marrow was present. The nature of the previous lymph noede pathology was reconsidered, and angioimmunoblastic lymphadenopathy was diagnosed.

摘要

一名慢性特发性骨髓纤维化患者在诊断后1年接受了脾切除术。作为一个临床意外发现,淋巴结活检提示存在非霍奇金淋巴瘤。该患者接受了强化联合细胞抑制治疗。在接下来的几个月里,骨髓纤维化的体征和症状显著消退。患者在脾切除术后31个月死于大量胃肠道出血。尸检时,在三个骨髓区域未检测到骨髓纤维化,而是存在一个规则的、含脂肪的、细胞增多的骨髓。重新考虑了先前淋巴结病变的性质,诊断为血管免疫母细胞性淋巴结病。

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