Granulocyte and Monocyte Disorders

Question 1

PMN development

Answer 1

12 days in marrow
6 hours in blood
1-2 days in tissue

Question 2

Monocyte development

Answer 2

7 days in marrow

4 days intravascular

Question 3

Kostmann syndrome

Answer 3

1˚ neutropenia with marrow hypoplasia
Lack G-CSF receptor or elastase mutation
Chronic infection and infant death common

Question 4

Shwachman-Diamond syndrome

Answer 4

1˚ neutropenia

FAS apoptosis of myeloid precursors

Question 5

Cyclic

Answer 5

1˚ neutropenia that swings every 3 weeks
Defects in elastase or apoptosis of precursors
Hypoplastic bone marrow with myelocyte developmental arrest

Question 6

Secondary causes of neutropenia

Answer 6

Chemotherapy - myelopoiesis suppression
Drug induced - immune response
Virus induced - immune response
B12/folate deficiency - hematopoiesis failure

Question 7

Leukocyte Adhesion Deficiency I

Answer 7

Low adherence/chemotaxis

Chronic abscesses and poor healing of soft tissue

Question 8

Leukocyte Adhesion Deficiency II

Answer 8

Decreased rolling and PMN escape to tissue
Recurrent infection
Also mental and physical disabilities

Question 9

Actin deficiency

Answer 9

Decreased chemotaxis and ingestion

Question 10

Myeloperoxidase deficiency

Answer 10

Risk for fungal infection

Complicated by DM

Question 11

Chediak-Higashi syndrome

Answer 11

Defect in killing and degranulation
Recurrent infection of mucus
Hepatosplenomegaly
Giant granules in leukocytes

Question 12

Chronic Granulomatous Disease

Answer 12

ROS defect - cannot kill catalase positives