All of the following are true about sickle cell disease, except: (PGI May 2009)
|A||Mutation in alpha chain|
|B||Symptoms ameliorated by HbF|
|C||Veno-occlusive cries is cause of morbidity|
|D||Bone pain is presenting feature|
Mutation in alpha chain (In SSA, mutation occurs in Beta chain)
1. Sickle cell anemia is associated with granulocytosis (Leukocytosis) Both total and segmental leucocyte number increase during vasocclusive crisis and infections.
2. Chronic acute or subacute pulmonary crisis (Pulmonary vasocclusive disease) lead to pulmonary hypertension and cor pulmonale.
3. The heart is enlarged with a hyperdynamic precordium and systolic murmurs.
4. Reactive hyperplasia of bone marrow with associated widening of medullary cavities produces a characteristic biconcave vertebra i.e. fish mouth vertebra.
5. Most common cause of death: Sepsis & acute chest syndrome