The Roles of Acute and Chronic Marrow Dysfunctions in the Aetiology of Anaemia in Sickle Cell Disease: Pathogenesis and Management Marrow dysfunction in SCD

Abstract

Background: Majority of the literature regarding the pathophysiology of anaemia in sickle cell disease (SCD) is focused on intravascular and extravascular red cell haemolysis with relatively little reference to reduced red cell production due to a myriad of marrow dysfunctions. Objectives: The aim of this overview is thus to present a comprehensive but concise narrative review of the aetiopathogenesis and management of anaemia due to acute and chronic marrow dysfunctions in patients with SCD. Methodology: Online literature search was conducted using search terms relevant to anaemia due to acute or chronic marrow dysfunctions in patients with SCD. Only articles that examined aetiopathogenesis and/or management of marrow dysfunction vis-à-vis anaemia in SCD were selected. Results: Literature search revealed that in addition to ineffective erythropoiesis, SCD patients also suffer from both benign and malignant forms of acute and chronic marrow dysfunctions. Marrow dysfunctions in SCD arise from separate or combined effects of marrow ischemia, deficiencies of micronutrients, renal insufficiency, infection of marrow precursors, haemophagocytic syndrome and myelosuppressive, dysplastic, and oncogenic effects of chemotherapy, gene therapy, and stem cell transplant. Conclusion: Marrow dysfunction is an important cause of anaemia in SCD. While transfusion support provides general short-term management, blood and marrow analyses are often necessary for accurate aetiological diagnosis. The Long-term and definitive management of marrow dysfunctions in SCD is determined by the aetiology, and it ranges from haematinics, antimicrobials, immune modulation, differentiation therapy, chemotherapy, dialysis, and/or erythropoietin supplements.