Division of Hematology and Medical Oncology, Department of Medicine, Icahn School of Medicine at Mount Sinai, New York City, NY, USA.
Department of Medicine, Icahn School of Medicine at Mount Sinai Beth Israel, New York City, NY, USA.
Am J Case Rep. 2023 Apr 14;24:e938854. doi: 10.12659/AJCR.938854.
BACKGROUND Sickle cell disease (SCD) is an autosomal recessive hereditary condition characterized by chronic hemolytic anemia and painful vaso-occlusive episodes. Homozygous sickle cell patients are at increased risk of morbidity and mortality from malaria. Autoimmune hemolytic anemia (AIHA) secondary to, or in the setting of, malarial infection is rare. In our case, the concurrence of Plasmodium falciparum malarial parasitemia and AIHA led to severe hemolytic anemia with an extensive packed red blood cell transfusion requirement. The patient's underlying SCD also contributed to the severity of the anemia and persistence of the malarial infection. CASE REPORT We report the case of a 29-year-old woman in the second trimester of pregnancy, with a history of SCD, who presented with severe anemia beyond her typical baseline in the setting of P. falciparum malaria. Hemolysis markers, including lactate dehydrogenase and bilirubin, were elevated. Direct Coombs testing was positive for IgG and C3 antibodies. Treatment with antimalarial agents and steroids led to clinical improvement and eventual clearance of the parasitemia. CONCLUSIONS Our patient's clinical course was most compatible with P. falciparum malaria-induced AIHA. Although she received a short course of steroids, it was treatment and clearance of the parasitemia that led to resolution of the hemolysis and a return to baseline hemoglobin levels. While the exact mechanism of AIHA in malaria is not well characterized, several unique mechanisms have been proposed and should be considered in cases of P. falciparum malaria manifesting with particularly severe hemolytic anemia.
背景 镰状细胞病(SCD)是一种常染色体隐性遗传疾病,其特征为慢性溶血性贫血和疼痛性血管阻塞性发作。纯合子镰状细胞病患者患疟疾的发病率和死亡率增加。疟疾感染继发或伴发的自身免疫性溶血性贫血(AIHA)较为罕见。在我们的病例中,恶性疟原虫疟原虫寄生虫血症和 AIHA 的同时发生导致严重的溶血性贫血,需要广泛输注红细胞。患者的潜在 SCD 也导致了贫血的严重程度和疟疾感染的持续存在。
病例报告 我们报告了一例 29 岁女性,处于妊娠中期,患有 SCD,在恶性疟原虫疟疾的背景下,出现了超出典型基线的严重贫血。溶血标志物,包括乳酸脱氢酶和胆红素,升高。直接抗球蛋白试验阳性,存在 IgG 和 C3 抗体。抗疟药物和类固醇的治疗导致了临床改善和寄生虫血症的最终清除。
结论 我们患者的临床过程最符合恶性疟原虫疟疾诱导的 AIHA。尽管她接受了短期类固醇治疗,但寄生虫血症的治疗和清除导致溶血的缓解和血红蛋白水平恢复到基线。虽然疟疾中 AIHA 的确切机制尚未得到很好的描述,但已经提出了几种独特的机制,在恶性疟原虫疟疾表现为特别严重的溶血性贫血时应加以考虑。