chapter 29: nonmalignant leukocyte disorders

a genetic disorder characterized by bilobed dumbell shaped nuclei in neutrophils

none. neutrophils are functional and individuals are healthy

• usually caused by maturation of neutrophils 
• iron deficiency anemia

• gaucher disease
• niemann pick disease

• 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)

yes using enzyme replacement therapy

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

having little acid sphingomyelinase (ASM)

having over 10% of ASM

neimann pick disease

gaucher disease

gaucher disease

gaucher disease

• an autosomal disorder caused by accumulation of protein carbohydrate complexes (granules) in WBC due to malfunction in lysosomes
• toxic granulation

dwafism and gargolism

alder reilly anomaly

• 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

chediak higashi syndrome

characteristics: blood platelet disorder characterized by abnormally large platelets and WBC defects contains dohle leukocyte inclusions

• red purple colored spots on skin
• nose bleeds (epitaxis)
• excessive bleeding from mouth

may hegglin anomaly