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HPIM Hema > Ch80: Disorders of Granulocytes and Monocytes > Flashcards

Ch80: Disorders of Granulocytes and Monocytes Flashcards

for 63: Leukocytosis and Leukopenia

1
Q

Major cells comprising the inflammatory and immune responses, including neutrophils, T and B lymphocytes, natural killer (NK) cells, monocytes, eosinophils, and basophils

A

Leukocytes

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2
Q

Normal blood leukocyte counts and their components

A

Normal blood leukocyte ct: 4.3-10.8 x 10^9/L

Neutrophils: 45-74%
Bands: 0-4%
Lymphocytes: 16-45%
Monocytes: 4-10%
Eosinophils: 0-7%
Basophils: 0-2%
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3
Q

Regulates the leukocyte maturation

A
  1. Colony stimulating factors (CSFs)

2. Interleukins (ILs)

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4
Q

Presence of immature cells is termed as?

A

Shift to the left

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5
Q

Stage of neutrophil development where classic lysosomal granules, called primary, or azurophil, granules are produced

A

Promyelocyte

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6
Q

The first recognizable precursor cell in the stage of neutrophil development

A

Myeloblast

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7
Q

Primary granules in promyelocyte are active against which type of pathogens?

A

Gram negative bacteria, fungi, enveloped viruses

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8
Q

Family of cysteine-rich polypeptides with broad antimicrobial activity against bacteria, fungi, and enveloped viruses which are contained on Azurophil granules

A

Defensins

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9
Q

Stages of neutrophil development (increasing maturity)

A
  1. Myeloblast (prominent nucleoli)
  2. Promyelocyte (primary granules appear)
  3. Myelocyte (secondary granules appear)
  4. Metamyelocyte (kidney bean-shaped nucleus)
  5. Band form (condensed, band-shaped nucleus/sausage-shaped nucleus)
  6. Neutrophil (condensed, multilobed nucleus)

Fig 80-2, p. 414

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10
Q

Cell responsible for the synthesis of the specific, or secondary granules that do not function as lysosomes (unlike primary granules), but instead is important in modulating inflammation

A

Myelocyte

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11
Q

Nucleus of neutrophils normally contain up to 4 segments. MORE THAN 5 lobes suggests what?

A
  1. Folate or Vitamin B12 deficiency

2. Congenital neutrophenia syndrome of warts, hypogammaglobulinemia, infections, and myelokathexis (WHIM)

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12
Q

Infrequent dominant benign inherited trait, resulting in neutrophils with distinctive BILOBED NUCLEI

A

Pelger-Huet anomaly

Nucleus has spectacle-like, or “pince-nez” configuration
Fig 80-5, p. 415

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13
Q

Immature or abnormally staining azurophil granules produced in severe acute bacterial infection; prominent neutrophil cytoplasmic granules

A

Toxic granulations

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14
Q

Cytoplasmic inclusions seen during infection and are fragments of ribosome-rich endoplasmic reticulum

A

Dohle bodies

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15
Q

Where is the large reserve of neutrophils located?

A

Bone marrow (90%)

Circulation: 2-3%, others: tissues

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16
Q

Glycoproteins expressed on neutrophils and endothelial cells that cause a low-affinity interaction resulting in “rolling” of the neutrophil along the endothelial surface

A

Selectins

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17
Q

Leukocyte glycoproteins that enables the neutrophils to “stick” to the endothelium

A

Integrins

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18
Q

Process of neutrophil migration or crawling of neutrophils between postcapillary endothelial cells into tissues

A

Diapedesis

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19
Q

Diapedesis involves what type of molecule?

A

platelet/endothelial cell adhesion molecule (PECAM) 1 (CD 31)

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20
Q

Examples of anaphylatoxins

A
  1. C3a

2. C5a

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21
Q

Examples of vasodilators

A
  1. Histamine
  2. Bradykinin
  3. Serotonin
  4. Nitric oxide
  5. Vascular endothelial growth factor (VEGF)
  6. Prostaglandin E and I
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22
Q

Half-life of neutrophil in circulation

A

6-7 hours

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23
Q

Where do senescent neutrophils cleared from circulation by macrophages?

A

Lung

Spleen

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24
Q

Duration at which neutrophils die in the tissues

A

1-4 days

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25
Q

Factors that prolong the lifespan of neutrophils

A

Granulocyte colony-stimulating factor (G-CSF)

IFN-y

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26
Q

In delayed type hypersensitivity, how long does monocyte accumulation occur

A

Within 6-12 hours of initiation of inflammation

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27
Q

Confers the characteristic green color to pus and may participate in turning off the inflammatory process by inactivating chemoattractants and immobilizing phagocytic cells

A

Myeloperoxidase

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28
Q

In the presence of fibrinogen, what induces IL-8 production by neutrophils providing autocrine amplification of inflammation

A

f-met-leu-phe OR Leukotriene B4

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29
Q

Four major groups of chemokines recognized based on cysteine structure near N terminus

A
  1. C (lymphotactin)
  2. CC (MIP-1)
  3. CXC (IL-8)
  4. CXXXC (Fractalkine)
30
Q

What neutrophil count increases the susceptibility to infectious diseases?

A

Below 1000 cells/uL

31
Q

Level of ANC where control of endogenous microbial flora is impaired

A

ANC <500/uL

32
Q

ANC level at which the local inflammatory process is absent

A

ANC <200/uL

33
Q

Causes of neutropenia

A
  1. Depressed production
  2. Increased peripheral destruction
  3. Excessive peripheral pooling
34
Q

Cancer chemotherapy can produce what type of neutrophil disorder?

A

Acute neutropenia

With increased risk of infection vs chronic neutropenia

35
Q

Most common cause of neutropenia

A

Iatrogenic

Due to cytotoxic or immunosuppresive therapies for malignancy or control of autoimmune disorders

Decrease production of rapidly growing progenitor (stem) cells of the marrow

36
Q

Drugs that can cause NEUTROPENIA by inhibiting myeloid precursors

A
  1. Chloramphenicol
  2. Co-trimoxazole
  3. Flucytosine
  4. Vidarabine
  5. Zidovudine
37
Q

Drugs can induce immune-mediated peripheral destruction of neutrophil precursors which can be seen within 7 days of exposure to drug. Most frequent causes are:

A
  1. Sulfa-containing compounds
  2. Penicillins
  3. Cephalosporins
38
Q

Treatment for drug-induced neutropenia

A

Discontinuation of offending drug

Recovery within 5-7 days and complete by 10 days

39
Q

Autoimmune neutropenia caused by circulating antineutrophil antibodies are caused by this condition::

A

Viral infections including HIV

40
Q

Management for autoimmune neutropenia

A
  1. Glucocorticoids
  2. Cyclosporine
  3. Methotrexate
41
Q

Congenital form of neutropenia

A
  1. Kostmann’s syndrome (neutrophil ct <100/uL)
    GENE: antiapoptosis gene HAX-1
  2. Severe chronic neutropenia (300-1500/uL)
    MUTATION: neutrophil elastase (ELANE)
  3. Cyclic hematopoiesis
  4. Shwachman-Diamond syndrome
42
Q

Pancreatic insuffiency due to mutations in the gene Shwachman-Bodian-Diamond syndrome gene

A

Shwachman-Diamond syndrome

43
Q

Syndrome characterized by neutrophil hypersegmentation and bone marrow myeloid arrest due to mutations in the chemokine receptor CXCR4

A

WHIM

Warts, hypogammaglobulinemia, infections, myelokathexis (retention of wbc in marrow)

44
Q

Drug when ingested during pregnancy can cause neutropenia in newborn by either depressed production or peripheral destruction

A

Thiazides

45
Q

Triad of rheumatoid arthritis, splenomegaly, and neutropenia

A

Felty’s syndrome

46
Q

Mechanisms of neutrophilia

A
  1. Increased production
  2. Increased marrow release
  3. Defective margination
47
Q

Most important acute cause of neutrophilia

A

Infection

Increased production + Increased marrow release

48
Q

Term for persistent neutrophilia (>30,000-50,000/uL) seen in leukemia where circulating neutrophils are usually mature and not clonally derived

A

Leukemoid reaction

49
Q

Causes of neutrophilia

A
  1. Infection
  2. Chronic inflammation
  3. Myeloproliferative diseases
  4. Glucocorticoids
  5. Exercise, excitement, stress
  6. Cigarette smoking

Table 80-2, p. 418

50
Q

Types of Leukocyte Adhesion Deficiency (LAD) and their clinical manifestations

A

Type 1 : Delayed separation of umbilical cord, sustained neutrophilia, recurrent infections of skin and mucosa, gingivitis, periodontal disease (MC bacteria: Staph aureus, enteric gram(-) bacteria)

Type 2 : Mental retardation, short stature, Bombay (hh) blood phenotype, recurrent infections, neutrophilia

Type 3: Petechial hemorrhage, recurrent infections

Table 80-4, p. 420

51
Q

Also known as congenital disorder of glycosylation IIc (CDGIIc) due to mutation in a GDP-fucose transporter (SLC35C1)

A

Leukocyte Adhesion Deficiency 2 (LAD 2)

52
Q

Most common neutrophil defect; primary granule defect inherited as autosomal recessive trate

A

Myeloperoxidase deficiency

53
Q

A rare disease with autosomal recessive inheritance due to defects in the lysosomal transport protein LYST (gene CHS1 at lq42)

A

Chediak-Higashi syndrome (CHS)

Genetics: Autosomal Recessive
Clinical: Recurrent pyogenic infections esp with S. aureus
Defect: Reduced chemotaxis and phagolysosome fusion, CHS1 gene
Diagnosis: Giant primary granules in neutrophils (Wright’s stain); genetic detection

54
Q

Group of disorders of granulocyte and monocyte oxidative metabolism and an important model of defective neutrophil oxidative metabolism

A

Chronic granulomatous disease (CGD)

Genetics: X-linked recessive trait (70%)
Clinical: Severe infection of skin, ears, lungs, liver and bone with catalase-positive microorganisms (S. aureus, B. cepacia, Aspergillus)
Defect: No respiratory burst due to lack of one of 5 NADPH oxidase subunits in neutrophils, monocytes, eosinophils
Diagnosis: Dihydrorhodamine (oxidation test) OR nitroblue tetrazolium (dye test); genetic detection

55
Q

T or F: Macrophages are not only phagocytic, but secretory cells that produce cytokines (TNF-a, IL1, IL8, IL12, IL18)

A

True

56
Q

Cytokine that initiates fever in hypothalamus, mobilize leukocytes from bone marrow and activates lymphocytes and neutrophils

A

IL-1

57
Q

Pyrogen important in pathogenesis of gram-negative shock and induces catabolic changes contributing to profound wasting (cachexia) in chronic diseases

A

TNF-alpha

58
Q

Some conditions associated with MONOCYTOSIS

A
  1. Tuberculosis
  2. Brucellosis
  3. Subacute bacterial endocarditis
  4. Rocky Mountain spotted fever
  5. Malaria
  6. Visceral leishmaniasis (kala azar)
59
Q

Mutation in TNF-a receptor characterized by recurrent fever in the absence of infection, due to persistent stimulation of TNF-a receptor

A

TNF-a receptor-associated periodic syndrome (TRAPS)

60
Q

Syndrome of Pyoderma gangrenosum, Acne, and sterile Pyogenic Arthritis caused by mutation in PSTPIP1

A

PAPA syndrome

61
Q

Conditions associated with MONOCYTOPENIA

A
  1. Acute infections
  2. Stress
  3. After treatment with glucocorticoids
  4. Due to myelotoxic drugs

Diseases

  1. Aplastic anemia
  2. Hairy cell leukemia
  3. AML
62
Q

Definition of EOSINOPHILIA

A

Presence of >500 eosinophils per uL of blood common in many settings besides parasite infection

63
Q

Common cause of EOSINOPHILIA

A

Allergic reaction to drugs

Iodides, ASA, sulfas, nitrofurantoin, penicillins, cephalosporins

64
Q

Other causes of eosinophilia

A
  1. Allergies
  2. Collagen vascular diseases (RA, periarteritis nodosa)
  3. Malignancy (Hodgkin’s disease, mycosis fungoides, CML, CA of lung, stomach, pancreas, overy, uterus)
  4. Helminthic infections
65
Q

Dominant eosinophil growth factor inhibited by monoclonal antibody Mepolizumab

A

IL-5

66
Q

Most dramatic hypereosinophilic syndromes

A 
Loeffler's syndrome
Tropical pulmonary eosinophilia
Loeffler's endocarditis
Eosinophilic leukemia
Idiopathic hypereosinophilic syndrome (50,000-100,000/uL)
67
Q

T or F: Effect of eosinophilia on the heart can produce restrictive endomyocardiopathy

A

True

Others: thrombosis, endocardial fibrosis

68
Q

Multisystem disease with prominent cutaneous, hematologic, and visceral manifestations characterized by eosinophilia (>1000/uL) and generalized disabling myalgia without other recognized cause

A

Eosinophilia-myalgia syndrome

Cause: ingestion of contaminants in L-tryptophan-containing products
Treatment: Withdrawal of products and administration of glucocorticoids

69
Q

Causes of EOSINOPENIA

A
  1. Stress (acute bacterial infection)

2. After treatment with glucocorticoids

70
Q

Rare multisystem disease in which the immune and somatic systems are affected, including neutrophils, monocytes, Tcells, Bcells, and osteoclasts

A

Hyperimmunoglobulin E-recurrent infection syndrome (JOB’S SYNDROME)

Clinical: Characteristic facies of broad nose, kyphoscoliosis, eczema; cold abscessess; primary teeth that do not deciduate
Defect: reduced chemotaxis, memory T and B cells, STAT3 mutation, DOCK8 deficiency
Diagnostic: somatic and immune features, serum IgE>2000 IU/ml; genetic testing

71
Q

Management of patients with chronic or cyclic neutrophil counts <500/uL

A

May benefit from

  1. Prophylactic antibiotics (e.g. Co-tri 160/800mg BID, levofloxacin, ciprofloxacin)
  2. G-CSF
72
Q

Management of severe, persistent lymphocyte dysfunction due to cytotoxic chemotherapy

(e.g. mga Chemo patients sa ward)

A
  1. Co-trimoxazole : prevents Pneumocystis jiroveci pneumonia
  2. Avoid heavy exposure to airborne soil, dust, decaying matter (rich in Nocardia and spores of Aspergillus and other fungi)

HPIM Hema (1 decks)

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