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以“典型血栓性血小板减少性紫癜五联征”临床表现的骨髓增生异常综合征

Myelodysplastic Syndrome Clinically Presenting with the "Classic TTP Pentad".

作者信息

Moscoso Martínez Santiago Fabián, Polanco Jácome Evelyn Carolina, Guevara Elizabeth, Mattoo Vijay

机构信息

Department of Hematology and Oncology, The Brooklyn Hospital Center, 121 Dekalb Ave, New York, NY 11201, USA.

Department of Pathology, Hofstra Northwell Health School of Medicine, 6 Ohio Drive, New Hyde Park, NY 11042, USA.

出版信息

Case Rep Hematol. 2017;2017:4619406. doi: 10.1155/2017/4619406. Epub 2017 Feb 1.

Abstract

The clinical presentation of myelodysplastic syndrome (MDS) is not specific. Many patients can be asymptomatic and can be detected only due to an abnormal complete blood cell count (CBC) on routine exam or for other reasons while others can be symptomatic as a consequence of underlying cytopenias. Thrombotic thrombocytopenic purpura (TTP) usually is suspected under the evidence of microangiopathic hemolytic anemia (MAHA) and thrombocytopenia and because it is a life-threatening condition (medical emergency) immediate initiation of plasmapheresis could be life-saving. The following case illustrates an unusual presentation of MDS in a patient who came in to the emergency room with the classic TTP "pentad" of fever, renal involvement, MAHA, mental status changes, and thrombocytopenia. We will focus our discussion in the clinical presentation of this case.

摘要

骨髓增生异常综合征(MDS)的临床表现并不具有特异性。许多患者可能没有症状,仅在常规检查时因全血细胞计数(CBC)异常或其他原因而被发现,而其他患者可能因潜在的血细胞减少而出现症状。血栓性血小板减少性紫癜(TTP)通常在微血管病性溶血性贫血(MAHA)和血小板减少的证据下被怀疑,并且由于它是一种危及生命的疾病(医疗急症),立即进行血浆置换可能会挽救生命。以下病例说明了一名MDS患者的不寻常表现,该患者因发热、肾脏受累、MAHA、精神状态改变和血小板减少等典型的TTP“五联征”而进入急诊室。我们将重点讨论该病例的临床表现。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2eda/5309395/0773847c6028/CRIHEM2017-4619406.001.jpg

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