[Macrohaematuria post-partum: an unusual case of acquired haemophilia after pregnancy].
BACKGROUND
Acquired hemophilia is a rare disorder due to spontaneous development of antibodies directed against the factor VIII molecule in patients with previously normal levels of factor VIII. In this paper we report an unusual case of acquired hemophilia admitted to our department of nephrology due to persisting gross hematuria.


METHODS
A 38-year-old primigravida woman, previously fit and well, had an uncomplicated pregnancy, labor and a caesarean delivery at 38 weeks of gestation. Two weeks after delivery she developed gross hematuria with anemia. She was therefore admitted to a department of urology where urinary system and hematological investigation did not reveal important anomalies. Due to persisting gross hematuria and suspected kidney disease, the patient was admitted to our department of nephrology where serious anemia and prolongation of aPTT were noted; factor VIII procoagulant activity was low with a Bethesda assay confirming the presence of anti human f VIII antibody. Diagnosis of acquired hemophilia was confirmed and treated with corticosteroid therapy, intravenous immunoglobulins and recombinant factor VIII. After 10 days, gross hematuria stopped with improvement of aPTT and level of factor VIII.


CONCLUSIONS
It is postulated that pregnancy leads to an altered immune status whereby the F VIII from the embryo, through transplacental leakage, may be considered as a foreign antigen by the mother, leading to the formation of antibodies. Bleeding symptoms usually became apparent within 3 months of delivery but could be as late as 12 months post-delivery and sometimes coagulation investigation results are normal at the beginning. It is therefore very important to estimate many times the activated partial thromboplastin time in macrohematuria post-partum. Mortality due to hemorrhage varies between 14 and 22 % in previous series. In the vast majority complete remission is achieved spontaneously within a few months. The diagnosis of acquired hemophilia is of fundamental importance for an adequate treatment preventing serious complications and high mortality.
Divisione di Nefrologia e Dialisi, Ospedale Casa Sollievo della Sofferenza, San Giovanni Rotondo, Italy. mikprenc@katamail.com
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Prencipe
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D'Errico
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Di Giorgio
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Gesuete
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Nobile
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Stallone
D000328:Adult; D001323:Autoantibodies / Q000096:biosynthesis / Q000276:immunology*; D001324:Autoantigens / Q000276:immunology*; D005169:Factor VIII / Q000276:immunology* / Q000627:therapeutic use; D005260:Female; D005326:Fetal Proteins / Q000276:immunology; D006417:Hematuria / Q000209:etiology* / Q000276:immunology; D006467:Hemophilia A / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007519:Isoantigens / Q000276:immunology; D008431:Maternal-Fetal Exchange; D008775:Methylprednisolone / Q000627:therapeutic use; D011247:Pregnancy; D011644:Puerperal Disorders / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology; D011994:Recombinant Proteins / Q000627:therapeutic use
D001323:Autoantibodies; D001324:Autoantigens; D005326:Fetal Proteins; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D007519:Isoantigens; D011994:Recombinant Proteins; D005169:Factor VIII; D008775:Methylprednisolone