Thrombotic microangiopathy in patients with sickle cell disease.

IF 2
Gabriella Biasi Carrasco, Patricia Belintani Blum, Josefina Aparecida Pellegrini Braga
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Abstract

Objective: To describe two cases of patients who had thrombotic microangiopathy (TMA) associated with sickle cell disease (SCD).

Case description: Both patients started with a painful crisis and had acute chest syndrome during hospitalization. They showed significant worsening of hemolytic anemia, with very high levels of lactate dehydrogenase, thrombocytopenia, lowered level of consciousness, organ damage and the presence of schistocytes in peripheral blood. Due to the possibility of TMA, despite the very rare association with SCD, they were treated with fresh frozen plasma replacement and plasmapheresis, with good response.

Comments: TMA is a serious, life-threatening disease, characterized by microangiopathic hemolytic anemia, thrombocytopenia, and organ damage. The association of SCD and TMA is difficult to diagnose, since they can share a similar clinical presentation. Recognizing this association and promptly instituting treatment may impact the survival of these patients.

镰状细胞病患者的血栓性微血管病。
目的:描述两例镰状细胞病伴有血栓性微血管病(TMA)的患者:描述两例与镰状细胞病(SCD)相关的血栓性微血管病(TMA)患者:病例描述:两例患者均以疼痛危象起病,住院期间出现急性胸部综合征。他们的溶血性贫血明显加重,乳酸脱氢酶水平非常高,血小板减少,意识水平降低,器官受损,外周血中出现裂形细胞。尽管与 SCD 的关联非常罕见,但由于 TMA 的可能性,他们接受了新鲜冰冻血浆置换和血浆置换治疗,反应良好:TMA是一种严重的、危及生命的疾病,其特征是微血管病性溶血性贫血、血小板减少和器官损伤。由于 SCD 和 TMA 的临床表现相似,因此很难诊断它们之间的关联。认识到这种关联并及时进行治疗可能会影响这些患者的生存。
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