Module 6: Non-malignant leukocyte disorders

Question 1

CGD of children

Answer 1

Neutrophils can’t kill catalase positive organisms (eg. Staph, Enterobacteriaceae, Pseudomonas, Candida, Brucella)

Question 2

Myeloperoxidase deficiency

Answer 2

Failure to kill bacteria

Question 3

Pelger-Huet anomaly

Answer 3

Hypo-segmentation of neutrophils (most nuclei are bands or have only 2 lobes)

Question 4

May-Hegglin anomaly

Answer 4

Abnormal bleeding

Question 5

Gaucher’s disease

Answer 5

Enzyme deficiency results in accumulation of glycolipid in macrophages

Question 6

Niemann-Pick disease

Answer 6

Enzyme deficiency results in accumulation of sphingomyelin and cholesterol in macrophages

Question 7

Chediak-Higashi syndrome

Answer 7

Defective lysosome membrane fusion protein results in grossly abnormal granulation in many cells - victims suffer from anemia, abnormal bleeds, endocrine problems, abnormal growth and recurrent infections, often die in their preteens

Question 8

Alder-Reilly anomaly

Answer 8

Enzyme deficiency leads to accumulation of polysaccharides - victims suffer from mental deficiency and physical deformations

Question 9

Infectious mononucleosis

Answer 9

A fairly common acute viral disease with fever, swollen lymph nodes, an absolute reactive lymphocytosis and a positive slide test (increased titre of EBV virus antibody)

Question 10

Etiologic agent of infectious mononucleosis

Answer 10

Infection with Epstein Barr virus