The most common inherited from of aplastic anaemia-
Ans. is 'a' i.e., Fanconi's Anemia o Most common inherited congenital anemia is Fanconis anemia.Fanconis anemiao It is the commonest hereditary marrow failure disease constituting two third of the cases,o It is an autosomal recessiveQ constitutional disorder affecting D.N.A. repair.o The Three affected genes are : -FANCAFANCCFANCGThese mutations result in defect in hematopoietic stem cell and the cells are ineffective in D.N.A. repair.FANCONIS ANEMIA|||PancytopeniaCancers whichare likely to developCongenital anomalies associated witho AnemiaInitiallyo Myelodysplastic syndromeo Acute Myelocytic leukemiao Short statureo Microcephaluso Broad nasal boneo Micrognathiao Hypopigmented patcheso Renal malformationo Epicanthal foldo Cafe-u-lait patcheso Hypoplasiao Skeletal defects# Normocytic# Low reticulocyteLater on# Macrocytico Neutropeniao Thrombocytopeniao Bone marrow # Hypocellular# Marrow replaced by fat # Radial ray anomalies (thumb hand and radii)
Fanconi anaemia
Schwachman-Diamond syndrome
Diamond-Blackfan anaemia
Dyskeratosis Congenita
Anemia and RBC Disorders