[Heavy haematuria as the presentation of attenuated haemophilia B (author's transl)]. / A propos d'une hématurie grave révélatrice d'une hémophilie B atténuée.

The authors report a rare case of eosinophilic cystitis presenting with heavy haematuria which led to the discovery of attenuated haemophilia B. Bleeding and clotting times in this patient, with no past history of haemorrhage, were normal. Complete coagulation studies led to the diagnosis in the presence of poor plasma prothrombin consumption and a moderate deficit in clotting factor IX, defining haemophilia B. Despite two partial cystectomies for haemorrhagic lesions of the dome of the bladder (eosinophilic cystitis), carried out with transfusion cover and the administration of P.P.S.B., recurrence of haematuria made it necessary to "rest" the bladder by a cutaneous ureterostomy. Subsequently, the restoration of urinary continuity was possible by uretero-ileo-cystoplasty, without any haemorrhagic complications by virtue of the use of P.P.S.B. The value of the association of epsilon amino-caproic acid with P.P.S.B. is emphasised in the prevention of haemorrhagic complications during surgery in haemokphilia B patients. In attenuated forms of haemophilia B (factor IX level between 5 and 15%) haemorrhages may be only occasional and occur only late in life.

[Comparative study of a factor-VIII inhibitor in a non-hemophylic patient and in a patient with hemophylia A gravis]. / Etude comparative d'un inhibiteur du facteur viii chez un malade non hémophile et chez un malade hémophile A gravis.

In this study, we tried to make a comparison between the findings concerning two human strong anti Factor VIII inhibitors. In one case, the strong inhibitor appeared in an old man without any particular antecedant. In the second case, it occurred in a young hemophiliac A. We found in both cases that the inhibitor was a immunoglobulin G with light chain lambda. Its activity site is on the fragment AB. These anticoagulants have a inhibitory action against only the Factor VIII procoagulant fraction and have exclusively neutralising properties. The inhibitor present in the non hemophiliac patient has particularly great resistance to temperature and pH variations.

[Cerebromeningeal hemorrhages and their sequelae in congenital deficiencies of plasma hemostasis factors. Apropos of a case of Stuart factor deficiency with early cerebromengeal hemorrhage leading to hydrocephalus with considerable psychomotor retardation]. / Les hémorragies cérébro-méningées et leurs séquelles au cours des déficits congénitaux en facteurs plasmatiques de l'hémostase. A propos dun cas de déficit en facteur Stuart avec hémorragie cérébro-méningée précoce conduisant à une hydrocéphalie avec retard psychomoteur considérable

We have taken an interest in cerebro meningeal haemorrhages in congenital plasmatic coagulation Factor deficiency with out obvious traumatic origin. We have observed such accidents in a new born child with congenital Stuart factor deficiency and in four cases of haemophilia A. It seems from our experience and from the literature that such haemorragic accidents are not particularly frequent in congenital coagulation deficiency and contribute only for a few to creation of a psychomotor inadapted population.