How I manage priapism due to sickle cell disease.

Priapism due to sickle cell disease is a common but less well characterized complication of the disorder. It represents a "medical emergency" with the key determinant of outcome being the duration of penile ischaemia and time to detumescence of <4 h associated with a successful treatment outcome. Management can be outpatient-based and consists of pre-emptive strategies for early stuttering attacks based on prior health education of the association between the 2 disorders, non pharmacological management, outpatient penile aspiration and irrigation with or without instillation of alpha and beta adrenergic agonists for acute episodes and secondary prophylaxis to prevent the high rates of recurrences. The evidence to recommend medical prophylaxis is sparse but based on a consensus of experts and small phase 2 or III clinical trials. A clearer understanding of the molecular mechanism(s) involving normal and dysregulated erectile physiology, scavenger haemolysis and nitric oxide pathway paves way for the use of phosphodiesterase type 5 inhibitors in medical prophylaxis of stuttering attacks. These agents will need to be studied in multi-centre randomized phase III trials before they become standard of care. A multidisciplinary team approach is required to enhance "sexual wellness" and prevent erectile dysfunction in this sexually vulnerable group.

Efficacy and safety of busulfan-based conditioning regimens for multiple myeloma.

Multiple myeloma is a malignancy of B cells characterized by accumulation of abnormal plasma cells in the bone marrow. In the past 20 years, the use of high-dose therapies and novel agents has resulted in significant and meaningful improvements in survival. Autologous stem cell transplantation (auto-SCT) following a high-dose melphalan-conditioning regimen represents the standard of care for younger patients as well as older patients with a good performance status. A number of strategies have been proposed to improve the outcome of auto-SCTs, including the incorporation of new agents such as thalidomide, lenalidomide, and bortezomib into the induction regimen administered before auto-SCT; the administration of maintenance therapy after auto-SCT; the incorporation of novel agents into chemotherapeutic regimens after transplantation as consolidation therapy; and the use of reduced-intensity allogeneic transplantation after an initial autograft. Although these approaches have demonstrated some success in improving responses after auto-SCT, none of these strategies are curative. An additional strategy to improve outcomes after auto-SCT is to enhance the immediate pretransplant conditioning regimens by either increasing the dose of melphalan or by incorporating novel agents, such as busulfan. This literature review focuses on the efficacy and safety of busulfan-based conditioning regimens for auto-SCT in patients with multiple myeloma.

A prospective diary study of stuttering priapism in adolescents and young men with sickle cell anemia: report of an international randomized control trial--the priapism in sickle cell study.

Priapism is defined as a prolonged, persistent, and purposeless penile erection. It is a common (35%) but frequently understated complication in young men and adults with sickle cell disease. We had previously demonstrated an association between stuttering attacks (<4 hours) and an acute catastrophic event with its consequent problems of erectile dysfunction and impotence. We describe a randomized, placebo-controlled, clinical study looking at medical prophylaxis with 2 oral α-adrenergic agonists, etilefrine and ephedrine, in preventing stuttering attacks of priapism. One hundred thirty-one patients were registered into a 2-phase (observational and intervention phase) study, and 86 patients (66%) completed Phase A diary charts. Forty-six patients (59%) completed a 6-month treatment phase (Phase B), and the remaining patients were lost to follow-up despite persistent efforts to contact them. Various reasons are postulated for the high attrition rates. The drugs were well tolerated, and no serious adverse events were reported. There was no significant difference among the 4 treatment groups in the weekly total number of attacks in Phase B (analysis of covariance P = .99) nor among the average pain score per attack after adjusting for attack rates and pain scores in Phase A (analysis of covariance P = .33). None of the patients who completed the study required penile aspiration at study sites while on medical prophylaxis. Young men with sickle cell disease are not comfortable engaging with health care providers about issues relating to their sexual health. The full impact of an improved awareness campaign and early presentation to hospital merits further standardized study. Priapism still contributes seriously to the comorbidity experienced by this previously inaccessible group of patients and medical prophylaxis with oral α-adrenergic agonists is feasible. Future international collaborative efforts using some of the lessons learnt in this study should be undertaken.

Unusual manifestations of primary cytomegalovirus infection in patients without HIV infection and without organ transplants.

Primary cytomegalovirus (CMV) infection with marked constitutional symptoms is rare in immunocompetent individuals and in those with iatrogenic immunosuppression, except transplant recipients. Four patients admitted to hospital with clinical illnesses associated with primary CMV infection were identified over a 12-month period. Their medical records were reviewed with regard to clinical and laboratory data, and outcome. Primary CMV infection was defined by the concomitant presence of CMV IgM and low avidity CMV IgG antibody. Of two patients with no known underlying illness, one presented with thrombocytopenic purpura and the other with vasculitis. Two patients receiving immunosuppressants for underlying ulcerative colitis presented with CMV-induced pancytopenia and CMV colitis. Atypical lymphocytosis was a feature on blood film examination in three of the four cases. One patient with disseminated CMV infection died of progressive multiorgan failure despite antiviral treatment. CMV disease following primary CMV infection should be considered in otherwise immunocompetent individuals with atypical lymphocytosis on blood film analysis, and particularly in patients on immunosuppressants such as those with ulcerative colitis, since early diagnosis and treatment with antiviral drugs may improve outcome.