Acute chest syndrome and COVID-19 in hydroxyurea naïve sickle cell disease patient in a low resource setting.

Acute chest syndrome (ACS) is a severe complication of sickle cell disease (SCD) and one of the leading causes of mortality in SCD patients. The management of ACS is challenging and requires prompt intervention to halt clinical deterioration. With the outbreak of the Coronavirus Disease 2019 (COVID-19) pandemic, which also primarily results in acute respiratory illness, the clinical picture and treatment outcome in SCD patients with ACS remain unknown. We present a case of a 30-year-old male who came in with features of painful vaso-occlusive episode and haemolysis that later evolved to acute chest syndrome. Chest X-ray showed pneumonic changes and mild bilateral pleural effusion, and nasal Reverse Transcription-Polymerase Chain Reaction (RT-PCR) for COVID-19 test came out positive. He was managed supportively with simple transfusion, antibiotics, dexamethasone and oxygen support with a good clinical outcome. Presenting with non-specific symptoms and similar respiratory symptoms and signs, the clinical picture of COVID-19 can prove difficult to discern from that of ACS due to other causes. This report emphasizes a need for a higher index of suspicion whenever a SCD patient presents with symptoms of acute respiratory distress.

Limited Exchange Transfusion Can Be Very Beneficial in Sickle Cell Anemia with Acute Chest Syndrome: A Case Report from Tanzania.

Acute chest syndrome (ACS) is a life-threatening complication of sickle cell disease (SCD) with blood transfusion an integral part in its management. Red cell exchange (RCE) transfusion is usually regarded as preferable to top-up transfusion, because it reduces the proportion of Hemoglobin (Hb) S while at the same time avoiding circulatory overload. Despite its obvious benefits, RCE is underutilized, particularly in low-resource settings which may be due to scarcity of blood products and of expertise in carrying out exchange transfusion. We report on a young woman with SCD with severe ACS who responded promptly and dramatically to a RCE of only 0.95 L (instead of the recommended 1.4 L) and had in the end an HbS level of 48% (instead of the recommended level below 30%). Limited RCE resulted in significant clinical improvement. We suggest that limited RCE may be of benefit than no RCE in SCD patients with ACS, particularly in settings where RCE is not available.

Audit of clinical-laboratory practices in haematology and blood transfusion at Muhimbili National Hospital in Tanzania.

In Tanzania, there is paucity of data for monitoring laboratory medicine including haematology. This therefore calls for audits of practices in haematology and blood transfusion in order to provide appraise practice and devise strategies that would result in improved quality of health care services. This descriptive cross-sectional study which audited laboratory practice in haematology and blood transfusion at Muhimbili National Hospital (MNH) aimed at assessing the pre-analytical stage of laboratory investigations including laboratory request forms and handling specimen processing in the haematology laboratory and assessing the chain from donor selection, blood component processing to administration of blood during transfusion. A national standard checklist was used to audit the laboratory request forms (LRF), phlebotomists' practices on handling and assessing the from donor selection to administration 6f blood during transfusion. Both interview and observations were used. A total of 195 LRF were audited and 100% of had incomplete information such as patients' identification numbers, time sample ordered, reason for request, summary of clinical assessment and differential diagnoses. The labelling of specimens was poorly done by phlebotomists/clinicians in 82% of the specimens. Also 65% (132/202) of the blood samples delivered in the haematology laboratory did not contain the recommended volume of blood. There was no laboratory request form specific for ordering blood and there were no guidelines for indication of blood transfusion in the wards/ clinics. The blood transfusion laboratory section was not participating in external quality assessment and the hospital transfusion committee was not in operation. It is recommended that a referral hospital like MNH should have a transfusion committee to provide an active forum to facilitate communication between those involved with transfusion, monitor, coordinate and audit blood transfusion practices as per national guidelines.