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11 Cards in this Set

  • Front
  • Back

Chronic Granulomatous Disease

NADPH Oxidase deficiency

Infection by Catalase positive organisms

(Same effects in G6PD as well but with anemia)

Leukocyte Adhesion deficiency

Absence of CD 18 (integrin part)




Omphalitis

Chediak-Higashi syndrome

Degranulation defect - absent NK activity




Albinism

Bruxton X-linked hypogammaglobinemia

CD18- (no circulatin Bcells. Maturation stopped at Pre-B stage)
CD3+




Give antibiotics and gammaglobulins

X-linked Hyper IgM syndrome

Deficiency of CD40L


No class switching. High IgM




Giv antibiotics and gammaglobulins

Selective IgA deficiency

Repeated sino-pulmonary and GI infections


Increased atopy




Can't give gammaglobulins as body not familiar with IgA. Will launch hypersensitivity reaction.

Both pathways
C5, C6, C7, C8, C9

Recurrent meningococcal and gonococcal infections

Deficiency in complement regulatory proteins

C1-INH


Heriditary angioedema

Selective T-cell def:


DiGeorge syndrome

CD19+


CD3- (no Tcells)




Failure of formation of 3rd and 4th pharyngeal pouches, thymic aplasia




Facial abnormalities, hypoparathyroidism, poor Tcell response

MHC class 1 deficiency

Failure of TAP1 to transport peptides to ER




CD8+ def, CD4+ normal - recurrent viral inf

MHC Class II def

CD4+ def. similar to SCID.


No graft vs host disease as MHC II missing.