November 2025, Volume 3, Issue 11

Author

Deepak Sitaram Laddhad, Vishal Pralhad Gaikwad, Neha Vijay Nidre, Saurabh Valji Bhanushali and Aishwarya Dhruv Laddhad

Abstract

Background: Sickle cell disease is a haemolytic disorder usually diagnosed in paediatric age group. In regions where proper screening lacks it goes undiagnosed in childhood and presents itself in adulthood with multisystem complications, diagnosis and treatment of these patients in adulthood becomes challenging. The incidence of sickle cell disease Indian adults is ~1.1% and prevalence of sickle cell trait is ~5.9% making it very hard to diagnose and needs a vigilant approach. Case Presentation: A 31-yr old female conscious oriented came walking by herself with chief complaints of pain in her b/l upper and lower limbs since 1 week with fatigue and headache since 15 days. No seizure, vomiting or other signs of increased ICP. CT Brain-normal. Lab investigations showed microcytic anaemia, indirect hyperbilirubinemia, positive sickling test and leucocytosis. Haemoglobin electrophoresis confirmed homozygous sickle cell disease. She was treated with hydration, oxygen, analgesic and hydroxyurea. On day 4 she developed acute hypoxemic respiratory failure for which invasive ventilation was needed. 2D Echocardiography revealed moderate pulmonary hypertension with severe tricuspid regurgitation, right atrial and ventricular dysfunction with mild pericardial effusion. Bone marrow aspiration revealed hypercellular marrow with erythroid hyperplasia. Also, on day 7 she developed status epilepticus for which MRI Brain showed acute infarct in rt splenium of corpus callosum in the pericallosal branch of posterior cerebral artery, suggesting embolic stroke also CSF study was normal. With adequate blood transfusions, antibiotic, antiepileptics supportive management patient improved and was discharged in stable condition. Conclusion: This case is an example of considering SCD even in adults with Vaso-occlusive symptoms with unexplained anaemia, as it as a much grave and multisystemic complications where diagnosis and treatment becomes challenging and can lead to life threatening events.