Patterns of prescribing hydroxyurea for sickle cell disease patients from a central hospital, Saudi Arabia.

Sickle cell disease (SCD) is a group of inherited disorders of hemoglobin synthesis. It is prevalent in different parts of the world, including the Kingdom of Saudi Arabia. The disease is associated with multiple acute and chronic life-threatening complications. Hydroxyurea (HU) is an effective preventive medication; its use has resulted in decreased morbidity and mortality. However, practice variability, including underutilization of HU, has been reported. No local publication has addressed this issue. The aim of this work is to consider the pattern of HU prescription for SCD patients. This is a retrospective study included patients seen in the outpatient clinics in a central hospital. Cases of medications unavailability or patient refusal to take the drug were not included. A total of 152 patients were included, of them 118 were prescribed HU and 34 were not. In 133 (87.5%) patients, the physician's decision was appropriate. Inappropriate decisions including both under prescription and, to much lesser extent, over utilization had been demonstrated in 19 (12.5%) cases. Impact of raising the healthcare providers' awareness and improving compliance with the updated SCD management recommendations and guidelines deserve further studying. In our local experience, although the majority of HU prescriptions were appropriate, both under prescription and to a lesser extent, overutilization was demonstrated.

A 27-Year-Old Patient Fulfilling the Diagnostic Criteria of Both CMML and JMML.

Chronic myelomonocytic leukaemia (CMML) and juvenile myelomonocytic leukaemia (JMML) are two disease entities that come under the myelodysplastic/myeloproliferative neoplasms category. Each of the two conditions has its own diagnostic criteria. In addition, they have different ages of presentation; while CMML is typically a disease of the elderly, JMML is a disease of young children. Here we are presenting the case of a 27-year-old male patient who, at the time of diagnosis, fulfilled the diagnostic criteria of both diseases. In addition he had radiological changes of type 1 neurofibromatosis. Possible explanations of the patient case have been discussed.

Brain infarction and blasts with bilobed nuclei in a patient with monocytic acute myeloid leukemia mimicking acute promyelocytic leukemia.

We are presenting a case of an adult male patient with monocytic acute myeloid leukemia (AML) who had on presentation brain infarction and bilobed nuclei had been demonstrated in many of the leukemic blasts. There was no laboratory evidence of acute disseminated intravascular coagulopathy, on presentation or later on. Initially the diagnosis of acute promyelocytic leukemia (APL) was considered, so all trans-retinoic acid (ATRA) was added to induction chemo therapy. As the diagnosis of APL was ruled out, based on the flow cytometry, fluorescent in situ hybridization and polymerase chain reaction findings, the ATRA was discontinued and the patient continued on the standard AML chemo therapy induction regimen. Later on chromosomal analysis was also normal. Sever dehydration on presentation, would have contributed to brain infarction. AML particularly monocytic, can mimic APL, especially its microgranular variant. The possible ATRA therapy side effects, can be avoided by early confirmation of the diagnosis.