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Objective: To date, the treatment of priapism in sickle-cell patients has relied on measures aimed at lowering blood viscosity and acidosis and reducing the level of circulating hemoglobin S (hyperhydration, alkalinization, or exsanguinotransfusion...) Surgical cavernous-venous shunt may be proposed if conservative treatment fails. We examined the efficacy of intracavernous etilefrin injections., Patients and Methods: From January 1996 through October 1997 (20 months) we performed 35 consecutive intracavernous injections of an alphastimulant, etilefrin in 7 sickle-cell patients (6 SS, 1 AS) who had experienced one or several episodes of low-flow priapism lasting 2 to 28 hours., Results: Involution of the tumefaction was rapidly achieved in all cases. Tolerance was satisfactory, although some post-injection undesirable effects were reported by certain patients: moderate transient pain (2-5 min) in the retrosternal area, or intense pain in the penis (more intense than the priapism) which lasted 10 to 30 minutes. This work confirmed the earlier reported efficacy of intracavernous injections of etilefrin and suggests that the autonomous nervous system plays an important role in the genesis of this condition in sickle-cell patients. Patients should be informed about the observed undesirable effects which have not been reported previously in the literature., Conclusion: Etilefrin can be proposed as first line treatment for priapism in sickle-cell patients (at least in cases lasting less than 24 h). The pathogenic mechanism could involve neuromuscular dysfunction.

The gene frequency of the most important hemoglobin (Hb) abnormalities is reported in a population of 171 Togolese newborns. Hb phenotypes, hematological parameters, and the more frequently described alpha-gene deletions were analyzed. Structural abnormalities of beta-globin were observed in 35.7% of the children with a gene frequency of 0.105 for beta(S) and 0.091 for beta(C). The frequency of the different alpha-globin genotypes was alpha alpha/ = 0.71, -alpha/ = 0.28, and alpha alpha alpha/ = 0.01. All of the individuals homozygous for the -alpha genotypes, and most of the heterozygous individuals, carried Hb Bart's. Within the alpha alpha/alpha alpha and the -alpha/alpha alpha groups, several individuals with or without Hb Bart's were found; they did not differ from the others by their red blood cell (RBC) parameters but by their levels of fetal hemoglobin (Hb F). The African alpha2 polymorphism marker, characterized by the replacement of G by TCGGCCC at position 7238 (EMBL HSHBA4, 1993) and of T 7174 by G, was found in 21 newborns. The mean value of Hb F was calculated for each genotype, the mean (G)gamma percentage was 69.4 +/- 4.0%, and the gene frequency of the AgammaT marker was 0.10; this marker was linked to the normal beta-globin cluster.

Unlabelled: Idiopathic thrombocytopenic purpurea (ITP) is an autoimmune disease that occurs frequently in Europe and the US, but has rarely been described in Africa. Case report. An 8-year-old girl was admitted for cutaneous and mucosal bleeding. She had a low platelet count (11 x 10(9)/l). ITP was suspected and the diagnosis was confirmed by bone marrow examination. Corticosteroid treatment was effective., Discussion: This is the second case reported in Togo since 1982. The diagnosis of the disease is straightforward, so the lack of cases reported in central Africa suggests that the disease is rare in this region. The rarity of the disease may be due to genetic or environmental factors, or it may simply be that physicians overlook this disease when making their diagnosis. Corticosteroids are now the preferred treatment for ITP because of the risk of transmitting Creutzfeldt-Jacob's disease by intravenous administration of immunoglobulin. Splenectomy is the ultimate treatment for chronic forms., Conclusion: Unlike other diseases, the diagnosis and treatment of which require methods unavailable in parts of Africa, ITP treatment, as currently practiced in countries of the northern hemisphere, is within the reach of most African countries. Further studies are required to determine the true frequency of the disease in central Africa.