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[日本骨髓衰竭诊断检查调查:日本特发性骨髓衰竭综合征国家研究小组报告]

[Survey on examinations for diagnosis of bone marrow failure in Japan: a report from the Japanese National Research Group on Idiopathic Bone Marrow Failure Syndromes].

作者信息

Araseki Kayano, Matsuda Akira, Tohyama Kaoru, Ishikawa Takayuki, Kawabata Hiroshi, Miyazaki Yasushi, Nakao Shinji, Tomonaga Masao, Takaori-Kondo Akifumi, Kurokawa Mineo, Omine Mitsuhiro, Ozawa Keiya

机构信息

Division of Hematology, Department of Internal Medicine, Faculty of Medicine, Saitama Medical University.

出版信息

Rinsho Ketsueki. 2012 Jul;53(7):691-7.

PMID:22975771
Abstract

Using a registration sheet of a prospective registration system for aplastic anemia (AA)/myelodysplastic syndromes (MDS), by the National Research Group on Idiopathic Bone Marrow Failure Syndromes, Japan, we carried out a survey on examinations for diagnosis of bone marrow failure. Bone marrow trephine biopsy was performed in 66 of 105 cases (63%) [Original diagnosis: AA 51 cases (80%), MDS 12 (32%), undiagnosable 3 (75%)]. Bone marrow aspiration was performed in all cases, and aspiration was performed at least twice in 36 cases (34%). The first-line anatomic site for bone marrow aspiration was the posterior iliac crest (62%). Cytogenetic examination was performed in 93%. The concordance rate between the original and the central review diagnosis was 93% among the studied cases: AA, Idiopathic cytopenia of undetermined significance (ICUS) and MDS in total. Flow cytometry analysis to detect paroxysmal nocturnal hemoglobinuria (PNH)-type blood cells was performed in 32%.

摘要

日本特发性骨髓衰竭综合征国家研究小组使用再生障碍性贫血(AA)/骨髓增生异常综合征(MDS)前瞻性登记系统的登记表,对骨髓衰竭诊断检查进行了一项调查。105例患者中有66例(63%)进行了骨髓活检[原诊断:AA 51例(80%),MDS 12例(32%),无法诊断3例(75%)]。所有病例均进行了骨髓穿刺,36例(34%)至少进行了两次穿刺。骨髓穿刺的一线解剖部位是髂后嵴(62%)。93%的病例进行了细胞遗传学检查。在研究的病例中,AA、意义未明的特发性血细胞减少症(ICUS)和MDS总体中原发诊断与中心复查诊断的一致率为93%。32%的病例进行了流式细胞术分析以检测阵发性夜间血红蛋白尿(PNH)型血细胞。

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