chapter 29: nonmalignant leukocyte disorders

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  1. what is pelger huet anomaly
    a genetic disorder characterized by bilobed dumbell shaped nuclei in neutrophils
  2. what are the typical signs and symptoms of individuals with pelger huet anomaly
    none. neutrophils are functional and individuals are healthy
  3. what causes hypersegmentation and what condition is it often seen in?
    • usually caused by maturation of neutrophils 
    • iron deficiency anemia
  4. name the two types of lipid storage disease
    • gaucher disease
    • niemann pick disease
  5. describe gaucher disease and signs and symptoms
    • inability to breakdown lipids due to deficiency in beta glucocerebrosidase causing accumulation of lipids in brain, spleen, liver, lungs, bone marrow, and kidneys
    • occurs in stages from type 1 to type 3 (nnon neuropathic to chronic neuropathic)
  6. is guacher disease treatable?
    yes using enzyme replacement therapy
  7. describe the characteristics of niemann pick disease and signs and symptoms and the types
    caused by dysfunctional metabolism of sphingomlipids due to malfunctional amounts of acid sphingomyelinase (ASM) enzyme which is found in the lysosomes of cells. Having a malfunction in ASM storage may cause cell death

    there are three types NPA, NPB, and NPC

    • lipid accumulation in the brain, spleen, liver
    • loss of muscle tone 
    • brain degeneration
    • ataxia (lack of muscle control in voluntary movement)
    • enlargment of spleen and liver due to overworking
  8. neimann pick A
    having little acid sphingomyelinase (ASM)
  9. neimann pick B
    having over 10% of ASM
  10. what disease is associated with this cell

    Image Upload
    neimann pick disease
  11. what disease is associated with this cellImage Upload
    gaucher disease
  12. what is the associated disease
    Image Upload
    gaucher disease
  13. what is the associated diseaseImage Upload
    gaucher disease
  14. What is alder reilly anomaly
    • an autosomal disorder caused by accumulation of protein carbohydrate complexes (granules) in WBC due to malfunction in lysosomes
    • toxic granulation
  15. what are the symptoms of alder reilly anomaly
    dwafism and gargolism
  16. what is the associated disease
    Image Upload
    alder reilly anomaly
  17. What is chediak higashi syndrome
    • a recessive immunodeficiency disorde caused by malfunction in vesicle transport characterized by large lysosomic inclusions ion the cell and albinism
    • caused by LYST gene malfunction
  18. What is the associated disease
    Image Upload
    chediak higashi syndrome
  19. what is may hegglin anomaly
    characteristics: blood platelet disorder characterized by abnormally large platelets and WBC defects contains dohle leukocyte inclusions
  20. what are the signs and symptoms of may hegglin anomaly
    • red purple colored spots on skin
    • nose bleeds (epitaxis)
    • excessive bleeding from mouth
  21. what is the associated diseaseImage Upload
    may hegglin anomaly
  22. Image Upload

Card Set Information

Author:
tanyalequang
ID:
326521
Filename:
chapter 29: nonmalignant leukocyte disorders
Updated:
2016-12-06 08:13:07
Tags:
pictures disorders leukocyte
Folders:
hematology
Description:
includes pictures and description of disorders
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