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一名慢性粒单核细胞白血病患者的致命性高白细胞综合征

Fatal hyperleukocytic syndrome in a patient with chronic myelomonocytic leukemia.

作者信息

Aul C, Gattermann N, Germing U, Südhoff T, Hollmig K A, Heyll A

机构信息

Department of Internal Medicine, Heinrich Heine University, Düsseldorf, Germany.

出版信息

Leuk Res. 1997 Mar;21(3):249-53. doi: 10.1016/s0145-2126(96)00117-8.

DOI:10.1016/s0145-2126(96)00117-8
PMID:9111170
Abstract

A 53-year-old male patient was admitted to our hospital with painful splenomegaly. He was diagnosed as having chronic myelomonocytic leukemia (CMML) with leukocytosis, monocytosis, increased lysozyme concentrations in serum und urine, and lack of the Philadelphia chromosome. The clinical course of the disease was characterized by rapidly rising leukocyte counts, cutaneous infiltrates, respiratory insufficiency and neurological symptoms. Excessive hyperleukocytosis with a significant increase in monocytic cells led to microcirculatory obstruction, vascular endothelial damage and organ malfunction. This complication could not be prevented by low-dose chemotherapy with cytosine arabinoside. The patient finally died from pulmonary and cerebral hyperleukocytic syndrome.

摘要

一名53岁男性患者因脾脏肿大伴疼痛入住我院。他被诊断为慢性粒单核细胞白血病(CMML),伴有白细胞增多、单核细胞增多、血清和尿液中溶菌酶浓度升高,且无费城染色体。该疾病的临床病程特点为白细胞计数迅速上升、皮肤浸润、呼吸功能不全和神经症状。单核细胞显著增多导致的过高白细胞血症引起微循环阻塞、血管内皮损伤和器官功能障碍。低剂量阿糖胞苷化疗无法预防这一并发症。患者最终死于肺部和脑部高白细胞血症综合征。

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Clinical management of CMML-State of the art.慢性粒单核细胞白血病的临床管理——最新进展
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