Priapism in sickle-cell disease; incidence, risk factors and complications - an international multicentre study.

Objective: To define the incidence, risk factors and complications of priapism in a large population of patients with sickle-cell anaemia in five centres in the UK and Nigeria, as priapism is common among these patients, but the precise characteristics of the condition in this population are poorly documented.

Patients And Methods: A questionnaire was developed and administered to patients with sickle-cell disease. Questions were designed to define the incidence, nature, precipitants, duration, treatment and complications of priapism.

Ethnicity study and non-selective screening for haemoglobinopathies in the antenatal population of central Manchester.

The objective of this study was to determine the frequency of ethnic groups within the antenatal population in central Manchester and thereby ensure that the haemoglobinopathy service was targeting the correct population and their needs. Ethnic data collection records of 6718 patients were analysed over a 7 month period. Of these 62.

An evaluation of the Bio-Rad Variant Haemoglobin Testing System for the detection of haemoglobinopathies.

The Bio-Rad Variant Haemoglobin Testing System is an automated analyser which uses the principle of cation exchange high performance liquid chromatography. This evaluation was undertaken to examine the effectiveness of the instrument as a screening mechanism to assist in the diagnosis of haemoglobinopathies. The ability to quantify haemoglobins A2 and F and to 'flag' other haemoglobin variants was tested.

Pregnancy after treatment with hydroxyurea in a patient with primary thrombocythaemia and a history of recurrent abortion.

A 28 year old patient with primary thrombocythaemia, who had had two stillbirths in the third trimester, is reported. She was successfully treated with hydroxyurea and delivered a healthy 6lb boy by elective caesarean section. The ease with which this treatment can be given, its high level of tolerance among patients, and its low cost are likely to increase its use as a safe and effective treatment in pregnant patients with primary thrombocythaemia.

Three cases of the myelodysplastic syndrome with pericentric inversion of chromosome 16.

Inversion of chromosome 16, inv(16)(p13q22), is characteristic of acute myeloid leukaemia (AML) with eosinophilia and is rarely found in the myelodysplastic syndrome (MDS). We report three cases of MDS in which inv(16) was observed. They were classified to FAB subtypes RA, RARS and RAEBT; eosinophilia or abnormal eosinophils were not observed.

Bone marrow necrosis complicating chronic myeloid leukaemia.

Two women with chronic myeloid leukaemia in chronic phase were found to have bone marrow necrosis when severe bone pains and falling blood counts prompted a marrow examination to exclude blast transformation. One patient survived for 12 months following the event without transforming. The second patient died soon after and was found to have widespread extramedullary disease.

Failure of mithramycin to control the myeloid blast phase of chronic granulocytic leukemia: a report on nine patients and review of the literature.

After reports of the successful use of mithramycin and hydroxyurea in the myeloid blast phase of chronic granulocytic leukemia, we treated nine patients according to the protocol devised by Koller and Miller (1986). There were no complete responses, but one patient had a partial response with a transient return to the chronic phase. Of the remaining eight patients, two experienced lessening of bone pains, and one a reduction in spleen size, but without hematological improvement.

Fulminant hepatic necrosis caused by adenovirus type 5 following bone marrow transplantation.

A 34-year-old patient was transplanted from an HLA-identical sister for high grade non-Hodgkin's lymphoma in first complete remission. One month post-transplant, he developed hepatitis and haemorrhagic cystitis. He died 2 months post-transplant from fulminant hepatic failure.

A proposal for further standardization of red blood cell glucose 6-phosphate dehydrogenase determinations.

A number of factors were identified which influence the determination of red blood cell glucose 6-phosphate dehydrogenase (G6PD). White blood cells were effectively removed from whole blood by filtration, resulting in a mean reduction of G6PD activity of greater than 1.0 U/g Hb.

Iron deficiency in ethnic minorities: associations with dietary fibre and phytate.

Routine full blood counts and serum ferritin determinations were carried out after admission to hospital in 112 children which included a white caucasian group (n = 65) and two ethnic minority groups of West Indians (n = 24) and Asians (n = 23). In these 3 groups those children between the ages of 1 week and 6 months were found to have similar haemoglobin, mean corpuscular volume, and ferritin levels. In the remaining children (aged from 7 months to 14 years 5 months) serum ferritin levels were lower in the ethnic minority groups than in white caucasians, but the haemoglobin and mean corpuscular volume were not significantly different.

Expression of myeloid differentiation antigens on myeloblasts in bone marrow of patients with myelodysplastic syndromes.

Bone marrow myeloblasts in 15 patients with myelodysplastic syndromes were quantitated with monoclonal antibodies using the immunoalkaline phosphatase technique. Positive blasts were identified in 7 of the 15 cases with at least one of three antibodies reactive with acute myelomonocytic leukaemic cells (PMN-6, PMN-29, AML 2-23) which were non-reactive with normal myeloblasts. In 5 of these cases increased PM-81 positivity was associated with expression of at least one of the other antigens (PM-81 antibody reacts with all types of acute myeloid leukaemic cells and a certain percentage of normal myeloblasts).

Natural history of smouldering leukaemia.

The natural history of 45 cases of smouldering leukaemia has been studied. Males and females were equally represented, with a median age of 60.5.