With regards to hereditary spherocytosis, which of the following is false -
Ans, is 'd' i.e., Aplastic Crisis are common o Aplastic crisis are a rare/uncommon complication of Hereditary Spherocytosis typically caused bv virailv induced bone marrow suppression.The most common aetiological agent that induces Aplastic Crisis in patients with Hereditary Spherocytosis is Parvovirus B19o Hereditary spherocytosis usually has autosomal dominant inheritance caused bv mutation in senes for proteins such as.spectrin, ankrin or handj, The genes responsible for HS include akyrin, b spectrin, band-3-protein, a-spectrin, and protein 4.2. In approximately two-thirds to three-quarter of HS patients, inheritance is autosomal dominant. In the remaining patients, inheritance is non-dominant due to autosomal recessive inheritance of a de novo mutation.o In patients with hereditary spherocytosis Red blood cells are destroyed in the spleeny The spleen plays a critical role in the pathobiology of HS, as destruction of spherocytes in the spleen is the primary cause of hemolysis in HS patients.o Aplastic Crisis is an Uncommon ComplicationAplastic crisis following virally induced bone marrow suppression are uncommon, but may result in severe anaemia with serious complications including congestive heart failure or even death.The most common aetiological agent in these cases is parvovirus BI9.Parvovirus selectively infects erythropoietic progenitor cells and inhibits their growth.
Usually has autosomal dominant inheritance
Caused by mutations in genes for proteins such as spectrin, ankrin or band 3
Red blood cells are destroyed in the spleen
Aplastic crises are common
Anemia and RBC Disorders