RESUMO
Neonatal alloimmune thrombocytopenia is caused by maternal antibodies against paternally-inherited alloantigens present on foetal platelets and is a significant cause of morbidity and mortality in neonates. While generally thought of as a human platelet antibody mediated phenomenon, cases of HLA mediated NAIT have been reported. We document the investigation of a patient with two pregnancies affected by HLA-B56 mediated NAIT and a proposal for management of future pregnancies.
Assuntos
Antígenos HLA-B/química , Trombocitopenia Neonatal Aloimune/sangue , Adulto , Antígenos de Plaquetas Humanas/imunologia , Plaquetas/imunologia , Evolução Fatal , Feminino , Morte Fetal , Heterozigoto , Humanos , Recém-Nascido , Hemorragias Intracranianas , Isoanticorpos/imunologia , Masculino , Linhagem , Recidiva , Trombocitopenia Neonatal Aloimune/imunologiaRESUMO
This case report describes a patient with an idiopathic acquired Factor VIII inhibitor and severe bleeding. She was treated with rituximab after failing first-line treatment with steroids and cyclophosphamide. Two months following rituximab treatment, our patient developed a succession of severe opportunistic infections requiring intensive care unit admission. Over a period of 12 weeks she required treatment for Pseudomonas aeruginosa septicaemia, herpes simplex gingivostomatitis and pharyngotonsillitis, clostridium difficile-related diarrhoea, systemic cytomegalovirus infection, pneumocystis jiroveci, and invasive pulmonary aspergillosis lung infections. After significant rehabilitation, the patient was finally discharged following a 5-month admission. This case highlights the complexity of balancing a life-threatening condition with the side effects of treatment. It also raises the issue of routine prophylaxis for immunosuppression in nonmalignant conditions, which will become a common dilemma with the expanding indications for rituximab use.