RESUMO
Pregnancy is a potential trigger of acute thrombotic thrombocytopenic purpura (TTP). The management of pregnancy-associated immune-mediated TTP (iTTP) can be challenging, especially when it is refractory to standard treatment. Caplacizumab, a nanobody to von Willebrand factor (VWF) blocking its A1 domain, is a valuable new therapeutic option. Its use is, however, not approved during pregnancy and breastfeeding. We describe the successful off-label administration of caplacizumab during pregnancy and delivery in a patient with refractory iTTP. The favourable outcome without significant thrombotic or haemorrhagic complications indicates that caplacizumab may be an effective and safe treatment option in refractory iTTP during pregnancy.
Assuntos
Complicações Hematológicas na Gravidez , Púrpura Trombocitopênica Trombótica , Anticorpos de Domínio Único , Humanos , Gravidez , Feminino , Púrpura Trombocitopênica Trombótica/tratamento farmacológico , Púrpura Trombocitopênica Trombótica/imunologia , Anticorpos de Domínio Único/uso terapêutico , Adulto , Complicações Hematológicas na Gravidez/tratamento farmacológico , Fator de von Willebrand/antagonistas & inibidoresAssuntos
Anticorpos Biespecíficos , Antígeno de Maturação de Linfócitos B , Linfoma Plasmablástico , Humanos , Anticorpos Biespecíficos/uso terapêutico , Linfoma Plasmablástico/tratamento farmacológico , Linfoma Plasmablástico/patologia , Antígeno de Maturação de Linfócitos B/imunologia , Masculino , Feminino , Idoso , Pessoa de Meia-Idade , Antineoplásicos Imunológicos/uso terapêuticoRESUMO
Herein we describe a case of febrile non-hemolytic reaction (FNHTR) in a 64-year-old male 20 min after the transfusion of one red blood cell unit. 20 days prior the patient had undergone an allogeneic hematopoietic stem cell transplantation (HCT) from an unrelated donor with minor ABO disparity. The patient had been treated for plasma cell myeloma with multiple transfusions in the past, but no transfusion reactions or alloimmunization had been reported.