[Post partum acquired hemophilia A with fatal outcome: a case report]. / À propos d'un cas d'hémophilie A acquise du post partum à évolution fatale.

We report the case of a 19-year-old women who developed on post partum day 1 a large hematoma in the vaginal wall. A surgical intervention for evacuating the hematoma was complicated with profuse bleeding of the genital and abdominal cavities. Postoperative coagulation studies revealed a prolonged activated partial thromboplastin time (87/30 s; ratio: 2.9), a decreased factor VIII level at 7% and the presence of an anti-factor VIII inhibitor in a titer of 64 Bethesda Unit (BU). Due to the active bleeding, the patient received Novoseven(®) (recombinant factor VIIa: 2 doses on post partum day 3 and 1 dose on day 6) and Feiba(®) (activated prothrombin complex concentrates: 1 dose on post partum day 8). These few administrated doses were not enough efficacious to achieve a continuous control of the bleeding. Immunosuppressive treatment used prednisone alone for 3 days, then combined to cyclophosphamide. The outcome was marked by the aggravation of the bleeding and an increase in the inhibitor level (132 BU). Finally, the patient died on post partum day 8 following an hemorrhagic shock.

[Extra medullary hematopoiesis associated to congenital dyserythropoietic anemia II in adult]. / Hématopoïèse extramédullaire associée à une dysérythropoïèse congénitale II chez l'adulte.

The congenital dyserythropoietic anemias comprise a group of rare hereditary disorders of erythropoiesis characterized by anemia with ineffective erythropoiesis and morphological abnormalities of erythroblasts in the bone marrow. Congenital dyserythropoietic anemia type II or HEMPAS is the more frequent type. It is rare in adults. Extra medullary hematopoiesis is also a rare entity; it is a physiological response to chronic anemia observed in certain hemopathies like congenital dyserythropoietic anemia type II. We report the observation of a patient for who diagnosis of extra medullary hematopoiesis associated to congenital dyserythropoietic type II was made in adulthood.

[Acquired haemophilia B: a case report and literature review]. / Hémophilie B acquise : à propos d'un cas avec revue de la littérature.

Acquired haemophilia is a rare disease; it occurs most frequently in elderly patients. The majority of cases are due to autoantibodies to factor VIII, which deplete circulating factor VIII or acquired haemophilia A. Only few cases of acquired haemophilia B are reported until today. We report a case of a 7-year-old girl with no past medical history of bleeding disorder and who present an extensive haematoma in the left calf. The diagnosis was established by the demonstration of an isolated prolongation of the activated partial thromboplastin time (APTT) with a reduced factor IX level and evidence of factor IX inhibitor activity to 2 Bethesda Unit (2UB). Diagnosis of acquired haemophilia B confirmed, patient received recombinant factor VIIa and corticosteroid treatment. Bleeding symptoms had completely disappeared and coagulation tests become normal. In conclusion, if bleeding symptoms are associated with unexplained prolongation of APTT, an inhibitor against factor must be searched for not missing an acquired coagulation disease.

Megaloblastic anemia in North Africa.

We prospectively studied 478 patients with megaloblastic anemia living in Tunisia. Overall, 98% of patients had vitamin B12 deficiency. Pernicious anemia accounted for most of these cases, and median age at presentation was 45 years. Megaloblastic anemia occurred in 19 subjects under 15 years of age, and of these, nine had the Immerslund-Graesbeck syndrome.

[Gaucher's disease uncovered late]. / Maladie de gaucher de decouverte tardive: a propos de deux cas.

Gaucher's disease is an uncommon inborn recessive autosomal disease, due to a deficient activity of the lysosomal enzyme beta glucocerebrosidase. This disease is usually diagnosed in the first or second decade of life with the arising of bone pains, splenomegaly and hemorragic manifestations due to thrombocytopenia. When the enlarged spleen is not evident, or after splenectomy, patients may be mis-identified as having Gaucher's disease. We present here two cases of elderly patients aged 70 and 46 years respectively, in whom the disease was a surprising finding of bone marrow examination, during check up for pancytopenia.