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巨幼细胞性贫血继发全血细胞减少和 TTP 样表现。

Pancytopenia and TTP-like picture secondary to pernicious anaemia.

机构信息

Hematology/Oncology, Brookdale University Hospital Medical Center, Brooklyn, New York, USA.

Hematology/Oncology, Brookdale University Hospital Medical Center, Brooklyn, New York, USA

出版信息

BMJ Case Rep. 2020 Jul 5;13(7):e235288. doi: 10.1136/bcr-2020-235288.

Abstract

A 21-year-old man presented to the emergency department with generalised weakness, weight loss and decreased appetite for few weeks. He had evidence of severe pancytopenia and haemolysis. His peripheral smear with many schistocytes was suspicious for thrombotic thrombocytopenic purpura (TTP). He was supported with blood transfusions and daily plasmapheresis. His platelet counts worsened despite 4 days of therapy. Bone marrow biopsy was significant for hypercellular bone marrow with megaloblastic changes. Further workup revealed normal ADAMTS13 level, low vitamin B, positive intrinsic factor antibodies and high methylmalonic acid. Diagnosis of pernicious anaemia was established and he was started on daily treatment with intramuscular vitamin B which subsequently improved his symptoms and haematological parameters. This report highlights the importance of checking vitamin B level in patients presenting with pancytopenia and TTP-like picture before making a diagnosis of TTP.

摘要

一位 21 岁男性因全身乏力、体重减轻和食欲减退数周就诊于急诊科。他有严重全血细胞减少和溶血性贫血的证据。他的外周血涂片有许多裂体细胞,提示为血栓性血小板减少性紫癜(TTP)。他接受了输血和每日血浆置换治疗。尽管已经治疗了 4 天,但他的血小板计数仍在恶化。骨髓活检显示骨髓增生明显,伴有巨幼细胞改变。进一步检查发现维生素 B 水平正常,内因子抗体阳性,甲基丙二酸水平升高。诊断为恶性贫血,并开始每日肌内注射维生素 B,随后他的症状和血液学参数均得到改善。本报告强调了在诊断 TTP 之前,应对出现全血细胞减少和 TTP 样表现的患者检查维生素 B 水平的重要性。

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Grave and Pernicious Anemia Presenting as Pancytopenia.以全血细胞减少为表现的严重及恶性贫血
Am J Med. 2020 Jun;133(6):e302-e303. doi: 10.1016/j.amjmed.2019.11.013. Epub 2019 Dec 17.

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