Time to think beyond sickle cell screening and haemoglobin electrophoresis: a case report and review of literature of sickle cell D-Punjab falsely labelled as sickle cell SS from central India

Abstract

Sickle cell haemoglobin D disease (D-Punjab) is a rare variant of sickle cell disease (SCD) reported from central India. Exact incidence of pulmonary thrombosis in patients with HbSD is unknown. Pulmonary thrombosis is known complication of SCD but rare in case of HbSD pattern. We reported a case of 34-year-old male patient with HbSD (D-Punjab) with acute chest syndrome (ACS). CT pulmonary angiogram revealed near complete thrombosis of right middle lobe segmental and subsegmental branches with pulmonary infarct. Our patient responded to anticoagulant therapy. This case report is a reminder that HPLC should be done in all patients with sickle cell disease along with solubility test and hemoglobin electrophoresis to detect exact incidence of hemoglobin D disease in central India.