<prompt> Neutropenia is not a feature ofWiskott aldrich syndromeKostmann syndromeCongenital aspleniaX linked hyper IgM syndrome  <response>: Ans: c Congenital asplenia1 X linked Immmunodeficiency with Hyper IgM syndrome Defective isotype switching leads to increased Ig M and decreased Ig G and A Due to abnormal CD- 40 ligand leading to defective B-T cell interactions: so T cell response is also abnormal pneumocytosis, aspergillosis, CMV infections also Neutropenia is frequent in affected males Harrison 17th edition Defective NFK essential modulator NEMO causes Hyper IgM with ectodermal dysplasia Achranon induced cytidine deaminase AID, URAGIDNA glycosylase UNG. So Hyper IgM may be due to abnormal CD 40 C NEMOAIDUNG.2 Wiskott Aldrich syndrome: Eczema increased IgE; thrombocytopenia small and short lived and infections decreased IgM; T cell deficiency WASP gene mutation IgM is low; IgG and A normal; IgE increased Lymphoreticular malignancy Failure to make Antibody to polysaccharide antigen protein antigen also if advanced Neutropenia; defective chemotaxis of neutrophils also may be seen.3 Kostmann syndrome:Is a hereditary neutropenic syndrome due to mutation in anti-apoptosin gene HAX -1.Other Congenital Neutropenias:Mutation Neutrophil elastase: Severe chronic neutropeniaHereditary cyclic neutropeniaCartilage hair hypoplasia syndromeSchwachmann - Diamond syndromeWHIM syndrome;G-CSF receptor mutation associated with Wiskott Aldrich CD-40 L defect Harrison 17th editionCongenital asplenia: is a cause of NeutrophiliaJobs syndrome:Hyper IgE recurrent infection syndrome eczema, skin abscess, Staphylococcus aureus pneumonia 