Monocytes and Myeloid Cells

Question 1

What disease is characterized by severe neutropenia lasting 5-7 days and recurring every 15-25 days caused by apoptosis of marrow precursors and is either auto recessive or dominant?

Answer 1

cyclic neutropenia

Question 2

What is neutropenia?

Answer 2

decrease in absolute neutrophil count; usually <1500/microL but varies

Question 2

What disease is characterized by the production of Abs that cross react with neutrophils; it presents before 14 mos old and lasts for 20 mos?

Answer 2

chronic benign neutropenia of childhood

Question 2

What causes increased neutrophil production?

Answer 2

infection, inflammation, drugs, tumors, stress, myeloproliferative disorders

Question 3

Name 5 causes of neutropenia due to increased turnover of mature cells.

Answer 3

1) chronic benign neutropenia of childhood 2) autoimmune neutropenia 3) alloimmune neutropenia 4) drugs 5) splenomegaly and hypersplenism

Question 4

What is chronic benign neutropenia of childhood?

Answer 4

production of Abs that cross react with neutrophils; presents before 14 mos old and lasts for 20 mos

Question 4

What causes decreased egress of neutrophils from circulation?

Answer 4

steroids, splenectomy, leukocyte adhesion deficiency

Question 6

Name 4 major causes of inherited decreased neutrophil production.

Answer 6

1) Kostmann Syndrome 2) Shwachman-Diamond Syndrome 3) cyclic neutropenia 4) Chediak-Higashi syndrome

Question 6

What is leukocytosis and what can it mean?

Answer 6

increased total WBCs; infection, inflammation, stress, leukemia

Question 8

Name 3 major causes of acquired decreased neutrophil production.

Answer 8

1) drugs, esp chemo 2) viral infections 3) nutritional deficienciies

Question 9

What is alloimmune neutropenia?

Answer 9

transplacental passage of fetal cells to the mother causes her production of IgG Abs that then attack baby’s neutrophils; lasts weeks to a few months

Question 10

What disease is characterized by the transplacental passage of fetal cells to the mother, causing her production of IgG Abs that then attack baby’s neutrophils; lasts weeks to a few months?

Answer 10

alloimmune neutropenia

Question 11

What is monocytosis and what can it mean?

Answer 11

increased monocyte counts; hematologic malignancies, collagen vascular diseases, granulomatous diseases, infections, carcinoma

Question 12

What is autoimmune neutropenia?

Answer 12

Abs to neutrophils; usu. assoc. with SLE, Evan’s or Felty’s Syndrome

Question 14

What disease is characterized by defects in myeloid precursors causing neutropenia, pancreatic insufficiency, fat malabsorption, bony abnormalities, growth delay; may develop aplastic anemia, leukemia, or MDS; is auto recessive?

Answer 14

Shwachman-Diamond Syndrome

Question 15

What disease is characterized by decreased myeloid stem cell production; very severe neutropenia resulting in death if not treated; may develop leukemia or MDS; and can be either auto recessive or dominant?

Answer 15

Kostmann Syndrome

Question 17

What is cyclic neutropenia?

Answer 17

severe neutropenia lasting 5-7 days and recurring every 15-25 days caused by apoptosis of marrow precursors; auto recessive or dominant

Question 17

What disease is characterized by Abs to neutrophils and is usu. assoc. with SLE, Evan’s or Felty’s Syndrome?

Answer 17

autoimmune neutropenia

Question 18

What is basophilia and what can it mean?

Answer 18

increased basophil #s; food or drug hypersensitivity, urticaria, infection, inflammation, myeloproliferative diseases

Question 19

What is neutrophilia?

Answer 19

increased neutrophil counts; >7500 cells

Question 20

What are the 3 main causes of neutrophilia?

Answer 20

1) increased production 2) increased release from storage pools 3) decreased egress from circulation

Question 21

What is eosinophilia and what can it mean?

Answer 21

>350/microL; allergies or allergic disorders (asthma), parasites, allergic drug reactions

Question 23

What is Kostmann Syndrome?

Answer 23

decreased myeloid stem cell production; very severe neutropenia resulting in death if not treated; may develop leukemia or MDS; auto recessive or dominant

Question 24

What is Shwachman-Diamond Syndrome?

Answer 24

defect in myeloid precursors causes neutropenia, pancreatic insufficiency, fat malabsorption, bony abnormalities, growth delay; may develop aplastic anemia, leukemia, or MDS; auto recessive

Question 25

What causes increased release of neutrophils from storage pools?

Answer 25

steroids, acute infection, stress, endotoxin