PDF(Pubmed)
Abstract：
Acquired amegakaryocytic thrombocytopenia (AATP) is a rare disorder in which severely low platelet levels occur due to reduced or complete absence of megakaryocytes in the bone marrow. The pathophysiology of this disease is not fully understood, although anti-thyroid peroxidase antibodies (anti-TPO) binding to cellular-myeloproliferative leukemia (c-mpl) receptors is a proposed mechanism. Currently, no standard published guideline for treatment exists, but immunosuppressive therapies have been used based on the proposed mechanism and associated conditions. We present a case of a 57-year-old male who presented to the hospital with a 3-day history of progressive weakness and dysphagia. He had recently been discharged from an outside health system after evaluation for suspected gastrointestinal bleeding, although esophagogastroduodenoscopy and colonoscopy did not uncover a source of bleeding. Fifteen days later, he was admitted to our hospital for septic shock and acute renal failure with suspected lower gastrointestinal bleeding (melena on presentation). He was found to have a rapidly declining platelet count with a nadir of 0. Due to severe thrombocytopenia, filgrastim was administered. A bone marrow biopsy revealed findings consistent with amegakaryocytosis with otherwise preserved cell lines. Hematologic labs improved with the initiation of appropriate treatment for severe sepsis. After performing an extensive workup, the likely etiology of transient AATP in this case was severe sepsis-induced immune dysregulation and bone marrow suppression.
摘要：
获得性变核细胞性血小板减少症（AATP）是一种罕见的疾病，由于骨髓中巨核细胞的减少或完全不存在，导致血小板水平严重降低。这种疾病的病理生理学尚未完全了解，尽管抗甲状腺过氧化物酶抗体（抗TPO）与细胞骨髓增殖性白血病（c-mpl）受体结合是一种已提出的机制。目前,没有标准的治疗指南，但免疫抑制疗法已根据提出的机制和相关条件使用。我们介绍了一例57岁的男性，他有3天的进行性虚弱和吞咽困难病史。他在评估怀疑有消化道出血后,最近已从外地医疗系统出院,尽管食管胃十二指肠镜检查和结肠镜检查未发现出血来源。15天后,他因感染性休克和急性肾功能衰竭并怀疑下消化道出血(出现黑便)入院。发现他的血小板计数迅速下降，最低点为0。由于严重的血小板减少症,给予非格司汀。骨髓活检显示发现与其他保存的细胞系的阿米迦勒细胞增生症一致。血液学实验室随着严重脓毒症适当治疗的开始而改善。在进行了广泛的检查后，在这种情况下,短暂AATP的可能病因是严重脓毒症诱导的免疫失调和骨髓抑制.
