Card Set Information

2010-04-12 12:03:13

Heme/Onc Markers
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  1. "Fried egg" cell colonies
    Multiple myeloma = Monoclonal plasma cells
  2. Rouleaux
    Chains of RBC's, seen in multiple myeloma
  3. Inside neutrophilic granules
    • Lysozyme, lactoferrin, myeloperoxidase
    • "LMN"
  4. Inside eosinophilic granules
    • Major basic protein (for killing protozoans & helminths)
    • Histaminase & arylsulfatase (anti-mast cells)
  5. Antidote for tPA
    Aminocaproic acid
  6. Antidote for methotrexate
    Leucovorin, to rescue normal cells (when myelosuppression occurs)
  7. Bruton's agammaglobulinemia
    X-linked (Bruton's boys), dec'd B-cells b/c defective tyrosine kinase (but normal # pro-B cells). Bacterial infections after 6 months.
  8. DiGeorge syndrome
    22q11 deletion --> 3rd & 4th pharyngeal pouches fail to develop --> no parathyroids (hypocalcemia --> tetany) or thymus (T-cell deficiency --> viral & fungal infections), plus malformed great vessels.
  9. SCID
    Presents at 3-6 months. Either both B & T cell development failure (adenosine deaminase deficiency) or just T-cells (IL-2 receptor deficiency). Recurrent infections of all kinds (bact, viral, fungal, protozoal); no allograft rejection.
  10. IL-12 receptor deficiency
    Deficiency of Th1 cells --> disseminated TB.
  11. Hyper-IgM syndrome
    Defect in CD40L on T-helper cells, so no Ab class switching. Severe pyogenic infections, early in life.

    (Both Hyper IgM & Job's = helper T-cells fail you with production of something: either CD40L or IFN-gamma)
  12. Wiskott-Aldrich syndrome
    X-linked defect in IgM response, but elevated IgE & IgA. WIPE: Wiskott-Aldrich, pyogenic Infections, Pupura, & Eczema (high IgE!).
  13. Job's syndrome
    Failed IFN-gamma production by T-helper cells --> failed neutrophil chemotaxis. Job was FATED: coarse Facies, non-inflamm pyogenic Abscesses, retained primary Teeth, high IgE, Derm probs (eczema).
  14. Leukocyte adhesion deficiency, type 1
    Defective LFA-1 integrin (CD18) on phagocytes --> recurrent bacterial infections w/no pus formation, neutrophilia, delayed separation of umbilicus
  15. Chediak-Higashi syndrome
    • Pale (partial albinism), recurrent pyogenic infections, numb (peripheral neuropathy).
    • Auto recessive, defective microtubule formation & lysosomal emptying of phagocytic cells.
  16. Chronic granulomatous disease
    NADPH oxidase deficiency --> defective respiratory burst production --> defective killing. Presents in childhood, or later (milder).
  17. Chronic mucocutaneous candidiasis
    T-cells defective against Candida. Chronic skin & mucous membrane infections.
  18. Ataxia-telangiectasia
    IgA deficiency; defective DNA repair enzymes. Presents ~age 1.
  19. CVID (common variable immunodeficiency)
    Often presents in 20s-30s. Deficient plasma cells (vs. Bruton's = deficient immature B-cells). Inc'd risk of NHL & autoimmune disease. Hepatosplenomegaly.