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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 (72 cards)

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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
Factors that prolong the lifespan of neutrophils
Granulocyte colony-stimulating factor (G-CSF) | IFN-y
26
In delayed type hypersensitivity, how long does monocyte accumulation occur
Within 6-12 hours of initiation of inflammation
27
Confers the characteristic green color to pus and may participate in turning off the inflammatory process by inactivating chemoattractants and immobilizing phagocytic cells
Myeloperoxidase
28
In the presence of fibrinogen, what induces IL-8 production by neutrophils providing autocrine amplification of inflammation
f-met-leu-phe OR Leukotriene B4
29
Four major groups of chemokines recognized based on cysteine structure near N terminus
1. C (lymphotactin) 2. CC (MIP-1) 3. CXC (IL-8) 4. CXXXC (Fractalkine)
30
What neutrophil count increases the susceptibility to infectious diseases?
Below 1000 cells/uL
31
Level of ANC where control of endogenous microbial flora is impaired
ANC <500/uL
32
ANC level at which the local inflammatory process is absent
ANC <200/uL
33
Causes of neutropenia
1. Depressed production 2. Increased peripheral destruction 3. Excessive peripheral pooling
34
Cancer chemotherapy can produce what type of neutrophil disorder?
Acute neutropenia With increased risk of infection vs chronic neutropenia
35
Most common cause of neutropenia
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
Drugs that can cause NEUTROPENIA by inhibiting myeloid precursors
1. Chloramphenicol 2. Co-trimoxazole 3. Flucytosine 4. Vidarabine 5. Zidovudine
37
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:
1. Sulfa-containing compounds 2. Penicillins 3. Cephalosporins
38
Treatment for drug-induced neutropenia
Discontinuation of offending drug Recovery within 5-7 days and complete by 10 days
39
Autoimmune neutropenia caused by circulating antineutrophil antibodies are caused by this condition::
Viral infections including HIV
40
Management for autoimmune neutropenia
1. Glucocorticoids 2. Cyclosporine 3. Methotrexate
41
Congenital form of neutropenia
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
Pancreatic insuffiency due to mutations in the gene Shwachman-Bodian-Diamond syndrome gene
Shwachman-Diamond syndrome
43
Syndrome characterized by neutrophil hypersegmentation and bone marrow myeloid arrest due to mutations in the chemokine receptor CXCR4
WHIM Warts, hypogammaglobulinemia, infections, myelokathexis (retention of wbc in marrow)
44
Drug when ingested during pregnancy can cause neutropenia in newborn by either depressed production or peripheral destruction
Thiazides
45
Triad of rheumatoid arthritis, splenomegaly, and neutropenia
Felty's syndrome
46
Mechanisms of neutrophilia
1. Increased production 2. Increased marrow release 3. Defective margination
47
Most important acute cause of neutrophilia
Infection Increased production + Increased marrow release
48
Term for persistent neutrophilia (>30,000-50,000/uL) seen in leukemia where circulating neutrophils are usually mature and not clonally derived
Leukemoid reaction
49
Causes of neutrophilia
1. Infection 2. Chronic inflammation 3. Myeloproliferative diseases 4. Glucocorticoids 5. Exercise, excitement, stress 6. Cigarette smoking Table 80-2, p. 418
50
Types of Leukocyte Adhesion Deficiency (LAD) and their clinical manifestations
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
Also known as congenital disorder of glycosylation IIc (CDGIIc) due to mutation in a GDP-fucose transporter (SLC35C1)
Leukocyte Adhesion Deficiency 2 (LAD 2)
52
Most common neutrophil defect; primary granule defect inherited as autosomal recessive trate
Myeloperoxidase deficiency
53
A rare disease with autosomal recessive inheritance due to defects in the lysosomal transport protein LYST (gene CHS1 at lq42)
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
Group of disorders of granulocyte and monocyte oxidative metabolism and an important model of defective neutrophil oxidative metabolism
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
T or F: Macrophages are not only phagocytic, but secretory cells that produce cytokines (TNF-a, IL1, IL8, IL12, IL18)
True
56
Cytokine that initiates fever in hypothalamus, mobilize leukocytes from bone marrow and activates lymphocytes and neutrophils
IL-1
57
Pyrogen important in pathogenesis of gram-negative shock and induces catabolic changes contributing to profound wasting (cachexia) in chronic diseases
TNF-alpha
58
Some conditions associated with MONOCYTOSIS
1. Tuberculosis 2. Brucellosis 3. Subacute bacterial endocarditis 4. Rocky Mountain spotted fever 5. Malaria 6. Visceral leishmaniasis (kala azar)
59
Mutation in TNF-a receptor characterized by recurrent fever in the absence of infection, due to persistent stimulation of TNF-a receptor
TNF-a receptor-associated periodic syndrome (TRAPS)
60
Syndrome of Pyoderma gangrenosum, Acne, and sterile Pyogenic Arthritis caused by mutation in PSTPIP1
PAPA syndrome
61
Conditions associated with MONOCYTOPENIA
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
Definition of EOSINOPHILIA
Presence of >500 eosinophils per uL of blood common in many settings besides parasite infection
63
Common cause of EOSINOPHILIA
Allergic reaction to drugs Iodides, ASA, sulfas, nitrofurantoin, penicillins, cephalosporins
64
Other causes of eosinophilia
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
Dominant eosinophil growth factor inhibited by monoclonal antibody Mepolizumab
IL-5
66
Most dramatic hypereosinophilic syndromes
``` Loeffler's syndrome Tropical pulmonary eosinophilia Loeffler's endocarditis Eosinophilic leukemia Idiopathic hypereosinophilic syndrome (50,000-100,000/uL) ```
67
T or F: Effect of eosinophilia on the heart can produce restrictive endomyocardiopathy
True Others: thrombosis, endocardial fibrosis
68
Multisystem disease with prominent cutaneous, hematologic, and visceral manifestations characterized by eosinophilia (>1000/uL) and generalized disabling myalgia without other recognized cause
Eosinophilia-myalgia syndrome Cause: ingestion of contaminants in L-tryptophan-containing products Treatment: Withdrawal of products and administration of glucocorticoids
69
Causes of EOSINOPENIA
1. Stress (acute bacterial infection) | 2. After treatment with glucocorticoids
70
Rare multisystem disease in which the immune and somatic systems are affected, including neutrophils, monocytes, Tcells, Bcells, and osteoclasts
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
Management of patients with chronic or cyclic neutrophil counts <500/uL
May benefit from 1. Prophylactic antibiotics (e.g. Co-tri 160/800mg BID, levofloxacin, ciprofloxacin) 2. G-CSF
72
Management of severe, persistent lymphocyte dysfunction due to cytotoxic chemotherapy (e.g. mga Chemo patients sa ward)
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)

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