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首页> 外文期刊>Experimental Hematology Oncology >Bone marrow transplant-associated thrombotic microangiopathy without peripheral blood schistocytes: a case report and review of the literature
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Bone marrow transplant-associated thrombotic microangiopathy without peripheral blood schistocytes: a case report and review of the literature

机译：骨髓移植相关血栓性微肺病理没有外周血血小霉素：文献报告和审查

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Bone marrow transplant-associated thrombotic microangiopathy (TA-TMA) is a relatively frequent but under-recognized and under-treated hematopoietic stem cell transplant (HSCT) complication that leads to significant post-transplant morbidity and mortality. Classic TMA-defining laboratory abnormalities appear at different times in the course of TA-TMA development, with schistocytes often appearing later in the disease course. In some severe TMA cases, schistocytes may be absent due to increased endothelial permeability. Unfortunately, many clinicians continue to perceive the presence of schistocytes as an absolute requirement for TA-TMA diagnosis, which leads to delayed recognition and treatment of this potentially fatal transplant complication. Patient chart review and PubMed literature search using the term, "transplant-associated thrombotic microangiopathy". A 54-year-old male IgG kappa multiple myeloma underwent a reduced intensity allogeneic HSCT from a 9/10 HLA-matched unrelated donor after conditioning with fludarabine and melphalan. On day +?27, the patient developed acute kidney injury followed by repeated episodes of diarrhea and gastrointestinal bleeding attributed to graft versus host disease (GVHD) and cytomegalovirus (CMV) colitis. Repeated colonic biopsies suggested CMV infection and GVHD. Despite appropriate treatment with antiviral therapy and immunosuppressants, the patient's condition continued to deteriorate. He experienced concomitant anemia and thrombocytopenia as well as elevated lactate dehydrogenase and low haptoglobin levels, but a TA-TMA diagnosis was not made due to an absence of schistocytes on peripheral smear. The patient expired secondary to uncontrolled gastrointestinal bleeding. A post-mortem analysis of the resection specimen revealed extensive TMA involving numerous arteries and arterioles in the ileal and colonic submucosa as well as in the muscularis propria and deep lamina propria of the mucosa. TA-TMA can occur in the absence of peripheral blood schistocytes. Our experience underscores the importance of considering the diagnosis of intestinal TA-TMA in patients with refractory post-transplant diarrhea and GI bleeding, even if all classic features are not present.
机译：骨髓移植相关的血栓性微血管病变（TA-TMA）是相对频繁但公认的造血干细胞移植（HSCT）并发症，其导致显着的移植后发病率和死亡率。经典TMA定义实验室异常在TA-TMA发育过程中出现在不同的时期，血清细胞经常出现在疾病课程后面。在一些严重的TMA案例中，由于内皮渗透性增加，血清细胞可能不存在。不幸的是，许多临床医生继续认为血清细胞的存在是对TA-TMA诊断的绝对要求，这导致延迟识别和治疗这种可能致命的移植并发症。患者图表审查和PubMed文献搜索使用术语“移植相关血栓性微疗病”。 54岁的男性IgG kappa多骨瘤在用Fludarabine和Melphalan调节后，从9/10 HLA匹配的无关供体中进行了降低的强度同种异体HSCT。在第+ + 27时，患者发育急性肾损伤，然后归因于移植物与宿主疾病（GVHD）和细胞病毒（CMV）结肠炎的反复发作。反复结肠活组织检查表明CMV感染和GVHD。尽管用抗病毒治疗和免疫抑制剂进行了适当的治疗，但患者的病症继续恶化。他经历了伴随的贫血和血小板减少症以及乳酸脱氢酶和低哈达氟胺水平的升高，但由于外周涂片的不存在而不是由于血清缺血而产生的TA-TMA诊断。患者已过期为不受控制的胃肠道出血。切除试样的后验尸分析显示出含有髂骨和结肠粘膜炎患者的大量动脉和动脉瘤以及粘膜腹膜和粘膜的深层胶版中的广泛TMA。在没有外周血血小细胞的情况下，可以发生TA-TMA。我们的体验强调了考虑难治性后移植后腹泻和GI出血患者肠道TA-TMA诊断的重要性，即使所有经典功能都没有。

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