MCQ Hematology Answer 36

The correct Answer is B

The neurological complications result from one point mutation that causes vasculopathy of both large and small vessels. Anemia and the resultant cerebral hyperemia produce conditions of hemodynamic insufficiency. Sickled cells adhere to the endothelium, contributing to a cascade of activated inflammatory cells and clotting factors, which result in a nidus for thrombus formation. Because the cerebrovascular reserve becomes exhausted, the capacity for compensatory cerebral mechanisms is severely limited. There is evidence of small-vessel sludging, and a relative deficiency of nitric oxide in these vessels further reduces compensatory vasodilatation. Both clinical strokes and silent infarcts occur, affecting motor and cognitive function.


Reference:
Sickle cell disease: the neurological complications. Ann Neurol. 2003 Jan;53(1):143; author reply 143-4. PMID: 12112099 [PubMed - indexed for MEDLINE]

Category: Hematology MCQs