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骨髓纤维化和全血细胞减少自发缓解,随后发生伴有髓外肿块的急性髓系白血病。

Spontaneous resolution of myelofibrosis and pancytopenia followed by the development of acute myeloid leukemia with an extramedullary mass.

作者信息

Inoue S, Limsuwan A, McQueen R

机构信息

Department of Pediatrics, Hurley Medical Center, Flint, Michigan 48503, USA.

出版信息

J Pediatr Hematol Oncol. 1998 May-Jun;20(3):268-70. doi: 10.1097/00043426-199805000-00016.

DOI:10.1097/00043426-199805000-00016
PMID:9628442
Abstract

PURPOSE

Spontaneous resolution of myelofibrosis is extremely rare. A patient with myelofibrosis and pancytopenia that spontaneously resolved with subsequent development of acute myelomonocytic leukemia and an extramedullary mass of the ovary is described.

PATIENT AND METHODS

A 2-year-old girl had severe pancytopenia and myelofibrosis without signs of myeloid metaplasia. The patient was transfused with packed red blood cells and platelets and monitored.

RESULTS

The pancytopenia and myelofibrosis resolved spontaneously within 1 month after initial presentation and her blood counts normalized. The patient developed a chloroma of the ovary and acute myelomonocytic leukemia 13 months later and eventually died from disease.

CONCLUSIONS

This patient initially had acute myelofibrosis which was most likely secondary to occult acute myelomonocytic leukemia. The transfusions may have been a contributing factor in the spontaneous remission.

摘要

目的

骨髓纤维化的自发缓解极为罕见。本文描述了一名患有骨髓纤维化和全血细胞减少症的患者,该病症自发缓解,随后发展为急性粒单核细胞白血病,并出现卵巢髓外肿块。

患者及方法

一名2岁女孩患有严重的全血细胞减少症和骨髓纤维化,无髓样化生迹象。该患者接受了浓缩红细胞和血小板输注并进行监测。

结果

全血细胞减少症和骨髓纤维化在初次就诊后1个月内自发缓解,她的血细胞计数恢复正常。13个月后,该患者出现卵巢绿色瘤和急性粒单核细胞白血病,最终因病死亡。

结论

该患者最初患有急性骨髓纤维化,很可能继发于隐匿性急性粒单核细胞白血病。输血可能是自发缓解的一个促成因素。

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