69 Classification and Clinical Manifestations of Disorders of Monocytes and Macrophages

Question 1

TRUE OR FALSE

The terms histiocyte and macrophage are synonymous.

Answer 1

TRUE

The terms histiocyte and macrophage are synonymous.

Question 2

Subsets of cells in the blood monocyte pool

Answer 2

Classic monocytes (85%)
Intermediate monocytes and patrolling monocytes (15%)

Question 3

Notable examples of disorders accompanied by severe monocytopenia

Answer 3

Aplastic anemia and Hairy Cell Leukemia

Question 4

Severity of bacteremia, organ dysfunction, and mortality has been associated with monocyte counts below ____________

Answer 4

Below 0.25 × 10 9 /L

Question 5

A syndrome of profound monocytopenia, often amonocytosis, associated with susceptibility to mycobacterial avian complex, fungal, and disseminated papilloma virus infections, and a heightened risk of subsequent development of myelodysplasia or acute myelogenous leukemia

Answer 5

MonoMAC syndrome

Question 6

Mutation associated with MonoMAC syndrome

Answer 6

Autosomal recessive or acquired mutations of GATA2

Question 7

Hematopoietic tumors with predominance of monocytic cells in marrow and blood

Answer 7

Acute monocytic leukemia
Chronic myelomonocytic leukemia
Dendritic cell variants of acute myelogenous leukemia

Question 8

Infectious hemophagocytic histiocytosis caused by what virus

Answer 8

Epstein-Barr virus

Question 9

Disease with defective macrophages result from an abnormality in their cell and granule membranes

Answer 9

Chédiak-Higashi disease

Question 10

A disorder that results from an inherited deficiency of the enzyme glucocerebrosidase

Answer 10

Gaucher disease

Question 11

Cytokine that impair monocyte functions

Answer 11

IL10

Question 12

TRUE OR FALSE

Isolated monocytopenia in the absence of any other blood cell deficiency or immune deficiency has not been reported.

Answer 12

TRUE

Isolated monocytopenia in the absence of any other blood cell deficiency or immune deficiency has not been reported.

Question 13

Macrophages can serve as a reservoir for HIV and are the principal locus for the virus in the ___________

Answer 13

Brain and in neural tissue

Question 14

Deficiency in a specific subset of macrophages, the osteoclasts, results in

Answer 14

Osteopetrosis

Question 15

Inhibit osteoclast action by interfering with its function of bone resorption and by inhibiting the mevalonate pathway to geranylgeranyl diphosphate

Answer 15

Bisphosphonates

Question 16

Primary chemical inducer of granuloma formation.

Answer 16

Monocyte-derived TNF

Question 17

TRUE OR FALSE

Benign monocytosis is associated with specific clinical manifestations.

Answer 17

FALSE

Benign monocytosis is not associated with specific clinical manifestations.

Question 18

TRUE OR FALSE

All forms of myelogenous leukemia with a predominance of monocytes are associated with a predisposition to troublesome tissue infiltrates, especially in the skin, gingiva, lymph nodes, meninges, and anal canal.

Answer 18

TRUE

All forms of myelogenous leukemia with a predominance of monocytes are associated with a predisposition to troublesome tissue infiltrates, especially in the skin, gingiva, lymph nodes, meninges, and anal canal.

Question 19

Refers to the accumulation of activated macrophages (histiocytes) in tissue sites

Answer 19

Hemophagocytic lymphohistiocytosis

Question 20

Cell that belong to a family of antigen-presenting cells that express high levels of human leukocyte antigen-D related (HLA-DR)

Answer 20

Dendritic cells

Question 21

HIV-1 usess the ______receptors as an entry pathway for infection of monocytes/macrophages

Answer 21

CD4