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Pregnancy in ET can be complicated by first trimester abortion and/or maternal haemorrhage. Our limited observation suggest a positive impact of low-dose ASA during pregnancy followed by low-molecular-weight heparin postpartum on pregnancy outcome in ET; nevertheless, confirmation by prospective documentation is mandatory.

Acquired haemophilia is a rare, life-threatening, acquired bleeding diathesis. No general consensus exists on the best therapeutic approach. We report on the standardized approach at our institution evaluated in ten patients with acquired haemophilia. Factor VIII inhibitors were found in all patients, activities ranging from 1 to 648 Bethesda units (BU). Eight of the ten patients presented with severe bleeding. Two patients died during the acute phase, one from intracranial bleeding and one due to Mycoplasma pneumonia. One patient with mild bleeding was treated with immunosuppression alone. Two patients with factor VIII inhibitor activities below 5 BU were started on factor VIII concentrate therapy. Therapy was successful in one and was changed to recombinant human activated factor VII infusion (rFVIIa) in the other, owing to insufficient factor VIII recovery. Six patients with factor VIII inhibitor activities above 5 BU were started on activated prothrombin complex concentrate (APCC) therapy. APCC treatment was successful initially in all six patients and was changed to rFVIIa infusion in one for rebleeding. One patient did not receive any specific therapy. Immunosuppression with prednisolone (2 mg kg(-1)) was begun in nine patients and was continued with cyclophosphamide (2 mg kg(-1)) in six. A complete remission of the acquired haemophilia was found in seven of the eight patients surviving the acute phase, one had a partial remission. All patients with acquired haemophilia could be managed effectively following our standardized approach. Routine administration of immunosuppression was associated with high inhibitor elimination rates.

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In 40 patients with essential thrombocythaemia (ET) serum erythropoietin (EPO) and thrombopoietin (TPO) concentrations were determined and compared with the EPO and TPO values of a healthy control group. The mean EPO serum concentration for 24 control patients was 9.4 mU/ml +/- 3.7 (range 2-17.9), for 32 untreated ET patients at diagnosis 6.6 mU/ml +/- 7.6 (range 0.5-44.3) and for 8 ET patients treated with cytoreduction 14.1 mU/ml +/- 8.0 (range 4.5-26.1). Serum EPO levels in untreated ET patients at diagnosis were significantly lower compared with serum EPO levels in healthy control patients (p=0.002). Serum EPO levels in treated ET patients were not different from serum EPO levels in healthy controls (p=0.13) but were significantly higher compared with untreated ET patients (p=0.003). Serum TPO levels were determined in 18 of 40 ET patients, the mean TPO serum concentration was 211 pg/ml +/- 109 (range 62,5-345). The mean TPO serum concentration for 10 untreated ET patients at diagnosis was 162 pg/ml +/- 87 (range 62,5-302) and for 8 ET patients who had received cytoreductive treatment 272 pg/ml +/- 106 (range 96-345), respectively (p=0.04). Both serum TPO levels for treated and untreated ET patients were significantly higher (p<0.001) compared with serum TPO levels for healthy controls. The results of our study suggest a difference in the regulation of serum EPO and TPO in patients with ET. While the mean serum EPO level is decreased in untreated ET patients, the corresponding mean serum TPO level is increased. Treatment with cytoreduction, results in normalisation of the mean serum EPO level, whereas the mean TPO serum level remains elevated.