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A 27-year-old woman with a history of aplastic anaemia complained of poor control of her right arm and hand, unsteady gait, and headache that increased while in a recumbent position. She was diagnosed with cerebral sinus thrombosis. Additional investigation revealed paroxysmal nocturnal haemoglobinuria (PNH). Treatment with heparin was initiated but stopped after the patient developed a brain haemorrhage. The patient recovered with no signs of residual symptoms and began taking oral anticoagulants as maintenance therapy. PNH is a rare acquired clonal disorder due to a defective expression of the glycosylphosphatidyl-inositol anchor membrane protein. It is characterised by haemolytic anaemia, diminished haematopoiesis, increased susceptibility for infections and a hypercoagulable state. Patients with aplastic anaemia have an increased risk of developing PNH. In patients with cerebral sinus thrombosis PNH should be considered as a possible underlying disorder. These patients should be questioned for possible clinical symptoms of PNH, such as acute abdominal pain or dark urine in the morning. For patients with these symptoms and in those with a history of aplastic anaemia or recurrent thrombosis, additional testing for PNH should be conducted.

To evaluate the results of 30 years of allogeneic HLA-identical bone marrow transplantation (BMT) as the treatment for children with acquired severe aplastic anaemia.Retrospective, descriptive.Of all patients who underwent an HLA-identical sibling-donor BMT for severe aplastic anaemia at the Department of Paediatrics, Leiden University Medical Center, in the period 1971-2000, and had a follow-up period of at least 1 year, the medical data were reviewed. The patients were split into 2 groups: patients transplanted before 1989 (n = 24), and patients who had their BMT from 1989 onwards (n = 20). This was due to a change in the treatment policy, namely a reduction in the period between diagnosis and BMT, resulting in fewer blood transfusions as well as changes in the prophylaxis against graft-versus-host disease (GvHD) from 1989 onwards (combination therapy using methotrexate and cyclosporin).There was an increase in the 1-year actuarial survival rate from 67% in the period before 1989 to 90% thereafter. The incidence of GvHD has significantly decreased since the introduction, in 1989, of the combination therapy using methotrexate and cyclosporin, with only 1/20 patients suffering from acute GvHD versus 13/24 prior to 1989 (p = 0.002). No patients acquired chronic GvHD after 1989, whereas before 1989, 10 patients had acquired this (p = 0.001).The prognosis of allogeneic HLA-identical sibling transplantation for paediatric patients with severe aplastic anaemia has considerably improved over the last 30 years due to improved supportive care, a significant decrease in GvHD and a shorter period between diagnosis and BMT, with the result that less blood transfusions have been required and less sensitisation has occurred. The long-term survival chance has increased to 90%.

A case is reported of a 7 years old boy with Fanconi's Anemia (FA). The aplastic anemia has been treated with androgens. Six years after the confirmation of the diagnosis FA a (pre-)leukemia occurred. Because of the known complications of cytostatics in FA and the bad prognosis, the leukemia has not been treated. The boy died 4 months later. An overview of the literature shows 31 patients with acute non-lymphocytic leukemia in FA, of which the clinical course, chromosomal investigations and therapy are discussed.

The cases of three dogs are reported, in which administration of oestradiol resulted in bone marrow hypoplasia. These case reports gave rise to a discussion leading to the advice to seek other methods of treatment.

In the period 1975-1981 seventeen patients aged from 4 to 30 months were seen with transient normochromic, normocytic anemia and reticulocytopenia caused by erythroblastopenia. The majority of the cases (12/17) were seen in autumn and winter. In 16 of the patients bone marrow aspirates were obtained; they showed erythroblastopenia. In 7 cases we observed young lymphoïd cells, which suggested the diagnosis of leukemia. Distinguishing features of congenital hypoplastic anemia and transient erythroblastopenia of childhood are compared. Except for blood transfusion, therapy e.g. corticosteroïds is not necessary in transient erythroblastopenia of childhood. Spontaneous recovery is a diagnostic feature, contrasting with congenital hypoplastic anemia.

Multiple liver tumors were observed in 2 women, 14 and 30 years of age, who had taken anabolic steroids over a period of years. In one case peliosis hepatis was found. 2 patients, 24 and 34 years of age, who had used oral contraceptives (o.c.s) for 9-10 years, were also examined for liver tumors. In one patient an increased uptake of the left hepatic lobe was observed, which corrected itself after discontinuation of o.c. use. Focal nodular hyperplasia was found in the other patient. 3 types of liver tumors are distinguished: focal nodular hyperplasia, hepaticellular carcinoma, and peliosis hepatis. They can be associated with long-term o.c. use, usually in younger women. Pain and swelling are the first symptoms of such tumors, and tests show slightly elevated liver enzyme values. A diagnosis can be reached using scintigram, angiogram, or angiography. Surgery to remove the tumor is indicated in the event of bleeding.