Niemann pick disease lipid storage disorderlipids

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Niemann Pick Disease (lipid storage disorder)(lipids come from NS) Hurler Syndrome (mucopolysaccharide (sugar) storage) Hunter Syndrome (also mucopolysaccharide storage) Tay – Sachs Disease (lipid storage – most common) All of these have a different enzyme deficiency Morphologic Alterations of Lymphocytes i.e. Reactive Lymphocytes -cells larger than normal lymphocyte a nuclear abnormality – Butt cell – nucleus clefts, sometimes seen in malignancies like lymphomas Cytoplasmic abnormalities -see vacuoles – i.e. Tay Sachs, Gaucher, Niemann Pick
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11/2 WBC Disorders Cont.’d Non-Morphologic Alterations of Lymphocytes Lacking some of the lymphocytes T Helper T Cytotoxic T Suppressor Null Cells What if you can’t make precursors for them? SCIDS Severe Combined Immunodeficiency Disease (no immune system except for phagocytosis – no T cell response – can’t make antibodies – i.e. Boy in the Bubble – used to die by age 2; a cluster disease – many different ways to inherit it; sex linked SCIDS; pleuripotent stem cell can’t mature to lymphoid cell line; David – Boy in the Bubble – germ free environment, thought bone marrow would cure it (to repopulate the normal lymphocyte population) – closest match his sister – had decision to make @ 12 yrs – chance of rejection – died – but he attributed to bone marrow transplantation and the rejection phenomenon T cell Abnormalities Called thymic aplasia w/o tissue = aplasia; so w/o thymus also called DiGeorge Syndrome no T cells ---fungal infections, TB (diseases that are more T cell dependent) B cell Abnormalities B cells make antibodies = agammaglobinemia Also called Brutons Disease Have repeated infections all the time Trx w/ bone marrow transplant if a perfect match; *give them gammaglobulin – amnt of it give based on body wt only lasts to max of 2 mo; may cost $6000 SCIDS = most common
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  • Fall '12
  • professoridon'tknow
  • white blood cell, WBC, Leukemias, WBC Disorders Cont.

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