WBC DISORDERS

Deficiency in Sphingomyelinase

• Gaucher's disease

• Taysach's

• Sandhoff's disease

• Niemann-pick

Deficiency in B-glucocerebrosidase

with crumpled tissue paper cytoplasm/ onion-skin like appearance

• Gaucher's disease

• Taysach's

• Sandhoff's disease

• Niemann-pick

Deficiency in hexosaminidase

• Gaucher's disease

• Taysach's

• Sandhoff's disease

• Niemann-pick

fusion of primary and secondary granules

• chediak-higashi

• Jordan's anomaly

• may-hegglin

• pelgeg-huet

mucopolysaccharide 1

• hurler's

• hunter's

• sanfilippo's

• scheie's

w/ presence of giant platelets

• dohle bodies

• pelger-huet

• may-hegglin

• alder-reilly

mutations of genes responsible for NADPH oxidase

• JOB SYNDROME

• CGD

• LAZY LEUKOCYTE SYNDROME

• MPO DEFICIENCY

lab test to identify hairy cell

• trap

• toluidine blue

• lap test

• e-rosette

characterized by defects in respiratory burst oxidase system

• alder-reilly

• Cgd

• job syndrome

• lazy leukocyte SYNDROME

Naegeli monocytic leukemia

• M3

• M4

• M5

• M6