we underestimating the leukemogenic risk of hydroxyurea

Hydroxyurea is an established drug that has been used for the treatment of myeloproliferative disorders and some solid tumors for some time. In recent years it has also been found to be effective in the treatment of sickle cell disease. Short term side effects are not serious, and are manageable. The major concern is the potential leukemogenesis with long term use. The risk of leukemogenesis is not defined with its use in benign hematological conditions. We report a case of acute myeloid leukemia with no preceding myelodysplastic syndrome, occurring after 2 years of hydroxyurea therapy in a patient with sickle cell disease

Isoxsuprine in the treatment of sickle cell painful crises: a double-blind comparatives study with narcotic analgesis

Isoxsuprine is a tocolytic agent which improves erythrocyte deformability. It was accidentally found to be effective in the management of sickle cell disease [SCD] painful crises. The experience with the drug in the treatment of sickle cell disease is limited. This double-blind randomized comparative study was undertaken to test its efficacy and safety in the treatment of SCD painful crises. Patients and Forty-three SCD patients [33 males and 10 females] with 44 episodes of pain were included in the study [i.e., one patient was included twice]. Only those with painful crises requiring hospital admission were included. Patients were randomized to receive either isoxsuprine 5-10 mg, or meperidine 50-100 mg intramuscularly, according to body weight, every four hours. A random selection of 23 patients received isoxsuprine, and 21 received meperidine. Pain score, duration of crisis, hospital stay, and side effects were monitored. No significant difference was found in any parameter except for pain score at 30 and 60 minutes. We conclude that isoxsuprine appears to be effective in the treatment of sickle cell painful crises. Confirmation of its efficacy in studies involving a larger number of patients is warranted

Falciparum malaria: an uncommon cause of acute renal failure in Saudi Arabia: a case report and review

A successfully treated case of severe falciparum malaria presenting with acute renal failure [ARF] and jaundice, is reported. Despite the fact that ARF is a common complication of severe falciparum infection, there has been no report of such a complication from Saudi Arabia up to the present time. Discussion on the pathophysiology of renal lesions and treatment is presented. High index of suspicion is required in non endemic areas for malaria, for rapid and correct diagnosis and treatment of a treatable otherwise fatal condition

patterns of oropharyngeal microflora in patients with homozygous sickle cell disease

A controlled prospective study was carried out between 16 July 1991 and 31 January 1992 to evaluate the oropharyngeal flora in homozygous sickle cell disease [SS]. Throat swabs for cultures were taken from 163 asymptomatic SS patients attending sickle cell clinics [90 children and 73 adults]. Throat swabs were also taken from 84 asymptomatic healthy individuals as a control group [44 children and 40 adults]. Group A beta hemolytic Streptococci were isolated from 16.6% of children with SS compared to 4.5% of children without SS [P<0.05]. Staphylococcus aureus was isolated from 16.4% of adults with SS compared to 0% of adults without SS [P<0.0001]. Group G beta hemolytic Streptococci and nongroupable Streptococci were also isolated from 11% and 13.7% of adults with SS compared to 0% and 2.5% of adults without SS respectively. Several other organisms were isolated only from SS patients. We conclude that the oropharyngeal flora of patients with SS is disturbed. This disturbance may contribute to morbidity of these patients

Avascular necrosis of the femoral and humeral heads in the sickle cell disease: Experience at Qatif central hospital, Saudi Arabia

To estimate the frequency of avascular necrosis of femoral heads [ANFH] and humeral heads [ANHH] in sickle cell disease patients. Retrospective study. A secondary care hospital, which is the main referral hospital in Qatif area. Eastern Province of Saudi Arabia. A total of 105 patients, native to the Qatif area with sickle cell disease; 59 patients were males; 46 patients were females; mean age was 22.3 years, [range 5-70 years]. Review of radiographs of femoral and humeral heads. Main outcome measures: Presence of avascular necrosis of femoral and humeral heads. Abnormalities were graded according to Ficat's classification of ANFH. The frequency of ANFH was 41.9%, and that of ANHH was 47.6%. The disease was bilateral in 63.6% and 84% of patients with ANFH and ANHH respectively; 72.7% of patients with ANFH had also ANHH. The frequencies of ANFH and ANHH as reported in this study from Qatif area are much higher than previously reported from the Eastern Province of Saudi Arabia

Ocular manifestations of homozygous sickle cell disease in Qatif, Saudi Arabia: a preliminary report

To determine the frequency of ocular manifestations in Saudi homozygous sickle cell disease patients [SSD].A prospective study.Paediatric and adult sickle cell clinics at Qatif Central Hospital, Eastern Province, Saudi Arabia.The subjects were 51 consecutive patients with confirmed SSD: 31 males and 20 females [M:F ratio 1.6:1]. Mean age 18.6 years [range 5-38 years].Detailed ocular examination during steady state, including indirect ophthalmoscopic retinal examination. Presence of SCO-related ocular manifestations in the conjunctiva, sclera, anterior segment and retina.Ocular abnormalities were found in 13 patients [25.5%]. Conjunctival abnormalities in ten patients [19.6%],and peripheral retinal abnormalities in six patients [11.8%]. No proliferative retinal abnormalities were found.The frequency of ocular manifestations is much lower in our patients compared with SSD patients of African descent