NON malignant disorders of leukocytes

Question 1

Lymphocytes disorders

Deficiency of hexosaminidase A

Answer 1

Tay-sachs disease

Question 2

Lymphocytes disorders

Reduced Immunoglobulins production in blood

Answer 2

Bruton Agammaglobulinemia

Question 3

Lymphocytes disorders

Reduced production of Ig due to overactivity of t8 cells

Answer 3

Common variable hypogammaglobulin

Question 4

Lymphocytes disorders

Underdevelopment of thymus

Answer 4

Nezelof’s syndrome

Question 5

Lymphocytes disorders

Deletion of small piece of chromosome 22

Answer 5

Di Geroge’s syndrome

Question 6

Lymphocytes disorders

Loss of Both T and B cells function

Answer 6

Swiss-type agammaglobulinemia

Question 7

Lymphocytes disorders

Failure of T cell response

Only IgA and IgG are present, IgM is absent

Answer 7

Wiscott-aldrich syndrome

Question 8

Lymphocytes disorders

Decreased T cell production
Spider veins

Answer 8

Ataxia Telangiectasia

Question 9

Monocyte macrophage disorder

Deficiency of glucocerebrosidase

Answer 9

Gaucher’s disease

Question 10

Most common lysosomal lipid storage disease

Answer 10

Gaucher’s disease

Question 11

Monocyte macrophage disorder

Deficiency of sphingomyelinase

Answer 11

Niemann-Pick disease

Question 12

Monocyte macrophage disorder

Macrophage with cholesterol overload due to increase in foam cells

Answer 12

Schiller-Christian disease

Question 13

Function disorder

Random movement of phagocytes is NORMAL
Directional motility is IMPAIRED

Answer 13

Job’s syndrome

Question 14

Function disorder

BOTH random and directional motility is impaired

Answer 14

Lazy leukocyte syndrome

Question 15

Function disorder

Intracellular mechanism of granulocyte is defective

Answer 15

Chronic Granulomatous Disease

Question 15

Function disorder

Have half the normal C3 Activity
Homozygous carriers fail to opsonize bacteria

Answer 15

Congenital C3 Deficiency

Question 16

Function disorder

MPO is decreased or absent in PMN and monocytes

Answer 16

Myeloperoxidase deficiency

Question 17

Nucleus disorder

Abnormal DNA synthesis

Answer 17

Hypersegmented neutrophil

Question 18

Nucleus disorder

Decreased segmentation in neutrophil

Pince nez appearance

Defect mutation in Lamin B

Answer 18

Pelger-Huet anomaly

Question 19

Nucleus disorder

Has less dense nuclei with hypo granular cytoplasm

Answer 19

Pseudo Pelger-Huet anomaly

Question 20

Cytoplasm disorder

Accumulation of degraded mucopolysaccharides

Answer 20

Alder-reily anomaly

Question 21

Cytoplasm disorder

Has large peroxidase lysosomes inclusions that are deficient in enzymes for phagocytosis

Answer 21

Chediak-Higashi syndrome

Question 22

Cytoplasm disorder

Dohle bodies, Thrombocytopenia, Giant platelets, and leukopenia

Mutation in MYH9 gene

Answer 22

May-Hegglin anomaly