62 Classification and Clinical Manifestation of Neutrophil Disorders

Question 1

Three childhood syndromes that are associated with neutropenia that are omitted because the neutropenia is part of a more global suppression of hematopoiesis:

Answer 1

Pearson syndrome, Fanconi anemia, and dyskeratosis congenita

Question 2

Mutation found in 70% of cases of the autosomal dominant form of severe congenital neutropenia and in most cases of cyclic neutropenia

Answer 2

Neutrophil elastase 2 gene (ELANE)

Question 3

The autosomal recessive form of severe congenital neutropenia and is caused by mutations in the HAX1 gene

Answer 3

Kostmann syndrome

Question 4

The clinical manifestations of decreased concentrations or abnormal function of neutrophils principally result from

Answer 4

Infection

Question 5

The lower limit of the normal neutrophil count in:

European descent:
African descent:

Answer 5

European descent:1.8 × 109/L
African descent: 1.4 × 109/L

An additional small proportion (~5%) of persons of African descent have neutrophil counts between 1.0 × 109/L and 1.4 × 109/L without evidence of associated abnormalities and this finding also may represent “ethnic neutropenia”.

Question 6

Infections in neutropenic subjects who are not otherwise compromised usually result from ________________ and usually are superficial, involving the skin, oropharynx, bronchi, anal canal, or vagina

Answer 6

Gram-positive cocci

Question 7

TRUE OR FALSE

Pus formation increases in patients with severe neutropenia

Answer 7

FALSE

Pus formation decreases in patients with severe neutropenia

Question 8

Condition associated with apparent normal granulopoiesis in the marrow and is asymptomatic even when the neutropenia has been present for prolonged periods, sometimes in the face of neutrophil counts approaching zero for prolonged periods.

Answer 8

Chronic idiopathic (benign) neutropenia

Question 9

Chronic cyclic neutropenia is characterized by periodic oscillations in the number of neutrophils, with the nadir occurring at approximately_________intervals

Answer 9

3-week intervals

Question 10

An alteration in the neutrophil blood pool where neutropenia occurs because a larger fraction of their blood neutrophils is in the marginal rather than the circulating pool.

The total blood neutrophil pool is normal, and infections do not result from this atypical distribution of neutrophils.

Answer 10

Pseudoneutropenia

Question 11

Sample of drugs that cause neutrophilia

Answer 11

Glucocorticoids
Hematopoietic growth factors
Minocycline
Ethylene glycol intoxication

Question 12

Leukomoid reaction has been designated as neutrophilia exceeding ________neutrophils/L not caused by a myeloproliferative neoplasm

Answer 12

50 × 109 neutrophils/L

Question 13

Hematologic malignancy that mimics leukomoid reaction

Answer 13

Chronic myelogenous leukemias (BCR-ABL at p230 rather than the classical p210)

Chronic neutrophilic leukemia

Question 14

Characteristic of leukomoid reaction

Answer 14

(a) is composed largely of mature neutrophils with a low proportion of bands and myelocytes,
(b) has increased leukocyte alkaline phosphatase reaction in neutrophils,
(c) has increased granulopoiesis with normal maturation and morphology of cells in the marrow,
(d) has normal cytogenetics and genetics of marrow cells
(e) has polyclonal-derived cells in women in whom such studies can be conducted (using the human androgen receptor gene assay)
(f) has cytometric analysis of neutrophils indicating a cluster of differentiation CD13 and CD15 phenotype with absent expression of human leukocyte antigen-D related (HLA-DR) and CD34.

Question 15

TRUE OR FALSE

An elevated neutrophil count is a feature of sickle cell disease and is a negative prognostic variable, increasing the likelihood of vasoocclusive events.

Answer 15

TRUE

An elevated neutrophil count is a feature of sickle cell disease and is a negative prognostic variable, increasing the likelihood of vasoocclusive events.

Question 16

This ratio has been shown to be superior to neutrophil counts as a predictor of bacteremia in patients admitted to emergency departments or intensive care units.

Answer 16

NEUTROPHIL/LYMPHOCYTE COUNT RATIO

Question 17

A widened monocyte distribution width (______units) and an abnormal neutrophil count, either above or below normal, is an important finding to point to sepsis.

Answer 17

> 20.0 units

Question 18

The combined deficit of neutrophils and monocytes characteristic of:

Answer 18

Aplastic anemia, hairy cell leukemia, and cytotoxic therapy

Question 19

Increased concentrations of leukemic neutrophil precursors can produce clinical manifestations of

Answer 19

Microcirculatory leukostasis

Question 20

TRUE OR FALSE

One type of drug-induced neutropenia is distinguished by the rapidity of onset.

Answer 20

TRUE

One type of drug-induced neutropenia is distinguished by the rapidity of onset.

Question 21

TRUE OR FALSE

In patients with ischemic vascular disease, an increased neutrophil count is associated with an increased probability of acute thrombotic episodes and the severity of chronic atherosclerosis.

Answer 21

TRUE

In patients with ischemic vascular disease, an increased neutrophil count is associated with an increased probability of acute thrombotic episodes and the severity of chronic atherosclerosis.

Question 22

Refers to the release histones, DNA, proteases, and antimicrobial molecules that form neutrophil extracellular traps (NETs) as part of their inflammatory response

Answer 22

Netosis