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. A distinction was made between acute (severe) priapism and the recurrent, 'stuttering' type. RESULTS: The questionnaire was completed by 130 patients (mean age 25 years, sd 11, range 4-66) from the five centres; 102 (78%) were homozygous Hb SS genotype, 19 (15%) were Hb SC genotype and two (1.5%) were Hb Salpha-thalassaemia. Of the patients, 46 (35%) reported a history of priapism, and of these, 33 (72%) had a history of stuttering priapism, while 24 (52%) had had an acute episode of priapism. The mean age of onset of priapism was 15 years, with 75% of patients having the first episode before their 20th birthday. Sexual activity was the most frequent precipitating factor, with fever and/or dehydration being the next most common. Of the 46 patients, 10 (21%) with a history of priapism reported having erectile dysfunction. A similar proportion reported dissatisfaction with sexual intercourse, including a fear of engaging in sexual activity. CONCLUSION: The incidence of priapism among patients with sickle-cell anaemia is high (35%). The implications of priapism for erectile and sexual function are significant and documented in this large series. The treatment of this condition in these patients remains unstandardised. This study highlights the need for an increased awareness of the problems associated with priapism among patients, families and medical professionals.

MRC trial of alpha2b-interferon maintenance therapy in first plateau phase of multiple myeloma. MRC Working Party on Leukaemia in Adults.

Plateau phase has been achieved in 64% of all newly diagnosed patients with multiple myeloma treated with the ABCM (adriamycin, BiCNU, cyclophosphamide and melphalan) regimen in the Medical Research Council (MRC) trials; this stable clinical stage of the disease is associated with no more than minimal symptoms. Several studies have found that alpha-interferon (alpha-IFN) maintenance therapy increases the duration of plateau phase, but it is less clear if this translates into prolonged survival. We report the effect of alpha-IFN on the duration of plateau phase and overall survival in a trial with 284 patients who were randomized to receive alpha2b-IFN (Intron-A) or no maintenance therapy during first plateau phase. The minimum follow-up after randomization was 21 months. There was no significant difference in the overall survival between the two treatment groups (X2=0.32, P=0.57). There was a trend towards longer relapse-free survival in the patients allocated alpha-IFN, but this trend to longer plateau phase was not statistically significant (X2 = 1.62, P = 0.2). Disease progression at relapse on alpha-IFN appears to be more severe with greater elevations from plateau levels of serum paraprotein (P = 0.06) and beta2-microglobulin (P= 0.03) levels. Physicians tended to start chemotherapy sooner after diagnosis of relapse when patients had received alpha-IFN (P = 0.16). Although, in common with most other studies, there is a trend for patients treated with alpha-IFN to have a longer plateau phase, this is counteracted by morbidity attributable to the treatment and a somewhat shortened survival post relapse. Meta-analysis of interferon trials is required to assess whether the minor trend for longer survival in patients maintained on alpha-IFN found in some studies is significant and, if so, the extent of this advantage.