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儿科获得性血友病 A 的诊断和治疗挑战:2 例报告。

Diagnostic and Treatment Challenges for Acquired Hemophilia A in Pediatrics: Report of 2 Cases.

机构信息

Department of Pediatrics, University of California-San Diego School of Medicine, La Jolla.

Division of Pediatric Hematology/Oncology, Rady Children's Hospital San Diego, San Diego, CA.

出版信息

J Pediatr Hematol Oncol. 2021 Nov 1;43(8):e1111-e1114. doi: 10.1097/MPH.0000000000002007.

Abstract

Acquired hemophilia A (AHA) occurs rarely in children. We report 2 cases of adolescent females with AHA. The first case underwent bone marrow aspiration/biopsy during workup, which was complicated by bleeding. Bleeding resolved after initiation of therapy with cyclophosphamide and glucocorticoid, but despite the addition of rituximab, she did not achieve complete remission until treatment with intravenous immunoglobulin. In the second case, we observed that a mixing study without incubation will not detect an acquired factor VIII inhibitor, but further workup based on suspicion for AHA led to the correct diagnosis. Both had significant medication toxicity which required treatment modification.

摘要

获得性血友病 A(AHA)在儿童中很少见。我们报告了 2 例青少年女性 AHA 病例。第 1 例在检查过程中进行了骨髓抽吸/活检,随后出现了出血并发症。经环磷酰胺和糖皮质激素治疗后出血得到缓解,但尽管加用了利妥昔单抗,她仍未完全缓解,直到接受静脉注射免疫球蛋白治疗。第 2 例中,我们观察到不孵育的混合研究将无法检测到获得性因子 VIII 抑制剂,但基于对 AHA 的怀疑进一步检查导致了正确的诊断。这两例患者均有严重的药物毒性,需要进行治疗调整。

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