Thrombotic microangiopathy (TMA) in patients with sickle cell disease (SCD) is a rare complication. These patients manifest microangiopathic hemolytic anemia (MAHA) with laboratory evidence of hemolytic anemia, schistocytosis, and thrombocytopenia. This is the first report of the syndrome in a group of these patients. A retrospective chart analysis of 10 consecutively diagnosed patients in SCD crisis who were referred for therapeutic plasma exchange (TPE) after developing MAHA was done. Patients had chest pain, respiratory distress, fever, pulmonary infiltrates, jaundice, and neurological dysfunction with abnormal liver function and coagulation tests. MAHA was diagnosed after a median hospital stay of 5 days. Nine patients recovered completely following TPE with fluid replacement by fresh frozen plasma with or without cryo-poor plasma. Incomplete response to TPE in one case was due to the development of fresh complications. During a median follow-up period of 77 months, there was one recurrent episode and one death in SCD crisis but without evidence of MAHA. TMA is not a very rare complication among Bahraini SCD patients in crisis. Characteristic features of this disorder are acute chest syndrome, organ failure, leuco-erythroblastosis, and a combination of thrombocytopenia, LDH level >1,000 U/l, and schistocytes in blood smears. Management with TPE usually leads to complete recovery with little chance of short-term recurrence. Multiple pathogenetic mechanisms leading to increased von Willebrand factor and its multimers may form the basis of this syndrome.

Reactive proliferation and inappropriate activation of mature histiocytes with haemophagocytosis (HP) may occur in association with a wide variety of infections, neoplasms, collagen vascular diseases, and acquired and inherited immunodeficiency states. The association with infections is particularly important because overwhelming HP can obscure the typical clinical features of the primary disease and negatively affects outcome. A high index of suspicion is required for early recognition of associated HP as the cause of cytopenias. Institution of specific therapy is crucial for survival. This study highlights the wide spectrum of tropical diseases that can have associated reactive HP. Thirty cases with documented prominent HP on bone marrow aspiration smears were reviewed. Twenty-one (69%) of the marrows were from patients who had common tropical infections: malaria, typhoid and visceral leishmaniasis and 11 of 15 patients (73%) who were followed up improved on specific infection-directed and supportive measures. The presence of severe HP in bone marrow smears correlated with marked cytopenias. Recognition of HP in this geographical region should stimulate the search for one of these infections as early institution of specific therapy is crucial for patient survival.

Background and Aims:In sickle cell disease (SCD) patients admitted for intensive care, evaluation of platelet counts in different types of sickle cell complications and its prognostic relevance are not well-studied. Illuminating these aspects were the objectives of this study.Materials and Methods:A chart review of 136 adult SCD patients consecutively admitted to our Intensive Care Unit (ICU) was done. The prognosis on day 1 was assessed by Acute Physiology and Chronic Health Evaluation (APACHE II) and multiple organ dysfunction scores (MODS). Receiver operating characteristic (ROC) curves evaluated the ability of platelet counts, MODS, and APACHE II scores to predict survival.Results:The most common types of crises were severe pain (n = 53), acute chest syndrome (n = 40), and infection (n = 18); 17 patients were nonsurvivors. Platelet counts varied widely (range, 19–838 × 109/L) with thrombocytopenia (n = 30) and thrombocytosis (n = 11). Counts correlated directly with leukocytes and reticulocytes; inversely with lactate dehydrogenase, APACHE, and MODS scores. Areas under ROC curve for platelets, MODS, and APACHE scores to predict survival were 0.73, 0.85, and 0.93, respectively.Conclusions:In severe sickle cell crisis thrombocytopenia is more common than thrombocytosis. In the ICU, day 1 platelet counts correlate inversely with prognostic scores and are significantly reduced in multi-organ failure and nonsurvivors. A platelet count above 175 × 109/L predicts patient survival with high specificity and positive predictive value but lacks sensitivity.

Hydroxyurea (HU) is used as a disease-modifying agent in sickle cell disease (SCD). Its beneficial effects have been ascribed to inhibition of the sickling process through increase of fetal hemoglobin (HbF) levels and influence on multiple factors affecting adhesion of erythrocytes to vascular endothelium. The present study investigates the effect of HU in SCD patients who were grouped on the basis of association with α- and β-thalassemia using routine laboratory methods. A retrospective cross-sectional chart-review was done of 51 adult Bahraini SCD patients attending Salmaniya Medical Complex, Bahrain. Four sub-groups of cases were identified: (i) homozygous sickle cell anemia, 24 cases; (ii) SCD with microcytosis, 16 cases; (iii) sickle α-thalassemia, seven cases; and (iv) sickle β thalassemia, four cases. Documented laboratory and clinical data included hemoglobin level (Hb), hematocrit (Hct), red cell indices, hemoglobin fractions, hospital admissions (frequency), number of inpatient-days, pain episodes (frequency) and red cell transfusion requirement (number of units). Pre- and post-treatment data were compared. Hydroxyurea treatment led to highly significant reduction of HbS % and pain crisis episodes in all patient groups. Other changes such as increases of total hemoglobin, Hct and HbF and reduction of hospital admissions, inpatient days and red cell units transfused also occurred but with less consistent levels of significance within patient sub-groups. Treatment with HU is beneficial for all subgroups of Bahraini SCD patients, without or with α- and β-thalassemia interactions.