Ch. 6 - Non-Neoplastic WBC Abnormalities Flashcards Preview

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Flashcards in Ch. 6 - Non-Neoplastic WBC Abnormalities Deck (155):
1

what are the four methods to measure WBCs in the lab?

1. automated hem. analyzers2. bone marrow aspirate and biopsy3. flow cytometry4. peripheral smear

2

what are the three general factors that affect neutrophil concn in the blood?

1. bone marrow production and release2. rate of egress to tissue and survival time in the blood3. ratio of marginating to circulating neutrophils in peripheral blood

3

What is the number at which someone has neutrophilia?

ANC> 7.0 x 10^9/L

4

T/F: Neutrophilia can be in response to a physiologic, not just pathologic process

true

5

How long does it take for marginated neutrophils to return the to circulating pool?

20-30 minutes

6

Stress, steroids, epinephrine, and what cytokine can move neutrophils from margination into the ciruclating pool?

IL6

7

How long does it take for neutrophils from the marrow storage pool to be released into the blood?

4-5 hours; released by IL6

8

How long does it take to increase the marrow mitotic pool?

days

9

How does epi increase the neutrophil count?

causes them to demarginate

10

What are six major categories of causes of neutrophilia?

DIPINNDrugsInflammationPhysiologicInfectionNeoplasticNecrotic

11

Vascular collagen and vasculitis are (blank) causes of neutrophilia

inlfammatory

12

Bacteria, fungi, parasites, viruses, and spirochetes are (blank) causes of neutrophilia

infectiious

13

stress, exercise, and pregnancy are (blank) causes of neutrophilia

physiologic

14

Carcinoma, sarcomas, and MPDs are (blank) causes of neutrophilia

neoplastic

15

burns, ischemic necrosis, and tissue damage are (blank) causes of neutrophilia

necrotic

16

What is the ANC in REACTIVE neutrophilia?

17

Reactive neutorphilia shows with a (blank) shift in myeloid maturation to band cells

left shift

18

Toxic granulation, Dohle bodies, and vacuolization are morphologic changes of neutrophils seen in (blank)

left shifting during reactive neutrophilia

19

T/F: the leukemoid reaction is benign

true

20

The ANC in the leukemoid reaction is usually (blank) and has many circulating (blank) leuokcyte precursos

>50 with many immature leukocyte precursors

21

What type of cell is ocassionally present during a leukemoid reaction

blast celss

22

What disease do you need to exclude when the leukemoid reaction is on you DDx?

CML

23

The (leukemoid/leukoerythroblastic) rxn has the presence of nucleated RBCS

LEB

24

the LEB reaction has a left shift in (blank) maturation

granulocyte

25

The (leukemoid/LEB) rxn is associated with myelophthisic processes, severe hemorrhage, hemolytic anemia, or MDS

LEB; anemias and hemorrhage make you think of RBCs

26

What are the limits in whites and blacks for neutropenia?

whites:

27

What is the ANC to define agranulocytosis?

28

Pseudoneutropenia is caused by (blank)

endotoxin

29

What drug that is used to treat schizophrenia can cause agranulocytosis?

Clozapine

30

T/F: Cachexia often leads to neutrophilia from the presence of a shock state

phony as a 3 dollar bill. Cachexia = neutropenia

31

What are the six general causes of neutropenia?

iCHOADintrinsic defectsCachexiaHematologic disordersOVERWHELMING infectionAutoimmuneDrugz

32

What are the 4 intrinsic defects that cause neutropenia?

F*CCK!!1. Fanconi's2. Kostmann's3. Cyclic neutropenia4. Chediak-Higashi

33

Megaloblastic anemia, myelodysplasia, marrow failure, hypersplennism are all (blank) disoders that lead to neutropenia

hematologic

34

Chloramphenicol, EtOH, chemo, abx, and clozapine can all cause (neutrophilia/neutropenia)

neutropenia

35

What are the three protozoal infections that commonly cause neutropenia?

Malaria, Kala-azar, Relapsing fever

36

Of the intrinisic defects leading to neutropenia, what is the only one caused by a maturation defect? What is the cause of all of the others?

Chediak-Higashi is caused by a maturation defect. All the others are caused by myeloid hypoplasia

37

Name this disease: Panmeyloid hypoplasiaHeterogenous presentation due to csome instabilityPresents in childhood: aplastic anemia, congenital physical deformities.Susceptible to hematopoetic and solid organ malignancies

Fanconi's Anemia-- you'll see the XRay of the weird joints

38

Name the disease:ANC

Kostmann's Syndrome,

39

What is the medical name for Kostmann's Syndrome?

Infantile genetic agranulocytosis

40

Name the disease:Presents in infancy or childhoodAuto. Dom with variable exrpression21-30 DAY PERIODICITYANC

cyclic neutropenia

41

ELA (1/2) is also known as neutrophil elastase

ELA2

42

What are the two most common congenital neutropenias?

Pregnancy induced hypertension and infection

43

EDTA-dependent agglutinin, old speciments, WBC fragility and paraprotein formation can all lead to (blank) neutropenia

spurious

44

What is this morphology?Azurophilic granules associated with Dohle bodies and vacuolization during septicemia and other toxic states

toxic granulation

45

What makes up a Dohle body?

remnants of free ribosomes and the rER

46

When do you see Dohle bodies?

During severe bacterial infections, pregnancy, burns, and toxic states

47

(Toxic granulation/Dohle bodies/Vacuolizatoin) is a precursor to sepsis

vacuolization

48

T/F: vacuolization may be a false finding in stored blood

true

49

Why do we get vacuolization in neutrophils?

Lots of dead and dying material around them gets phagocytosed, that's why its an early marker of sepsis as it indicates everything is fucking dying

50

Shistocyte RBC morphology indicates the pt could be in what life threatening condition?

DIC

51

If you see a bacteria (such as a diplococci like S. aureus) inside a neutrophil, you are looking at what morphologic state?

abnormality

52

What stages of neutrophil maturation do we see toxic granulation?

Meta, band, and neutrophil stages

53

The Pelger-Huet Anomaly is autosomal (dom/rec)

dominant; has homo and heterozygous forms

54

T/F: Pelger-Huet anomaly indicates a functional defect in the neutrophil

false; clinically asymptomatic

55

A pince-nez nucleus indicates what morphologic state?

Pelger-Huet anomaly

56

The Pelger-Huet anomaly can be confused with a reactive (blank) shift

left

57

What are the general characteristics of a Pelger-Huet anomaly?

Hyposegmented neutrophils, sparse granules, single or pince-nez nucleus

58

>5% of neutrophils with (blank) or more lobes or any single one with (blank) lobes constitutes hypersegmentation

5% with 5, or one with 6

59

What states are associated with hypersegmentation?

Megaloblastic anemia (B12 def), chronic infection, MDS

60

T/F: Hereditary hypersegmentation is associated with refractory megaloblastic anemia

false; associated with no disease!`

61

A loss of vibratory proprioception indicates what vitamin def?

B12

62

The Alder-Reilly anomaly is auto (dom/rec)

recessive

63

Large, purplish Granules that stain METACHROMATICALLY with TOLUIDINE BLUE lets you know you're looking at what anomaly?

Alder-Reilly

64

The anomaly that is associated with Hunter's and Hurler's Syndromes is:

Alder-Reilly"Does he REILLY have Hurlers?!"

65

Mucopolysaccharidoses is a disease state assoicated with what neutrophilic change?

Alder-Reilly anomaly

66

Chediak-Higashi is autosomal (dom/rec)

recessive

67

Name the disease:1. Giant gray-green peroxidase- positive bodies in cytoplasm of leukocytes and other cells.2. Abnormal melanosomes (skin hypopigmentation, photophobia)3. Lymphadenopathy and hepatosplenomegaly

Chediak-Higashi

68

The giant peroxidase bodies in Chediak-Higashi are (blank) bodies

inclusion bodies

69

What is the defect that causes the formation of inclusion bodies in Chediak Higashi?

fusion of cytoplasmic membranes, locomotion, and chemotaxis; therefore hypopigmentation because melanosomes can't move their melanin to the skin surface!

70

T/F: Chediak Highashi is both a morphological and functional defect

true

71

What autosomal dominant trait has large Dohle-body like inclusions in granulocytes that contain RNA?

May-Hegglin Anomaly

72

Which myosin heavy chain is fucked up in May-Hegglin anomaly?

myosin heavy chain 9

73

T/F: the dohle-body like inclusions in May Hegglin anomaly cause a functional defect in GRANULOCYTES

false

74

Describe the platelet morpholgoy in May Hegglin? What effect does this have on their function?

Giant oval platelets, associated with thrombocytopenia and bleeding disorders

75

What disease can't handle catalase organisms because of a defect in the respiratory burst oxidase (NADPH oxidase) system?

CGD

76

What are the two methods of inheritance of CGD?

sex linked and auto recessive inheritance

77

In CGD neutrophils form (blanks) when they phagocytize but do not kill

granulomas

78

Defects in membrane-associated cytochrome b (subunits gp91 and p22), and cytosol-associated p47 and p67 are the molecular causes of (blank)

CGD

79

T/F: defects in ANY portion of the NADPH oxidase system will lead to CGD

true

80

What is the difference between CGD and myeloperoxidase deficiency?

MPO still has NADPH oxidase activity but can't make bleach aka HOCl aka hypochlorite; whereas CGD has NO NAPDH activity!

81

On the NBT (nitrobule tetrazolium test), MPO would be (blue/yellow-pink) while CGD would be (blue/yellow-pink)

MPO: blueCGD: yellow-pink

82

What is the treatment for CGD?

Abx prophylaxis

83

T/F: MPO def can be acquired or genetic

true

84

In what two cells do you get an MPO def?

neutrophils and monocytes

85

T/F: infections are a common complication in both CGD and MPO

false; only in CGD

86

How do you diagnose CGD or MPO?

CGD: NBT testMPO: MPO stain

87

Which LAD deficiency involves Selectins?

LAD II

88

Which LAD deficiency involveds CD11a/CD18

LAD I

89

Which LAD deficiency involves CD11a/CD18 activation?

LADIII

90

Recurrent pyoderma gangrenosum or a failure of umbilical cord separation are disroders associated with which LAD deficiency?

LAD I

91

What is the threshold for eosinophilia?

>0.45

92

Eosinophilia is associted with (cell mediated/humoral) immunity

cell mediated

93

When would you see eosiniphilia in kids?

with allergies or GI food allergies

94

What is the most common cause of eosinophilia? Be specific!

Tissue INVASIVE parasites

95

Addison's Disease often causes (neutrophilia/eosinophilia)

eosinophilia

96

Collage disease often causes (eosinophilia/neutrophilia)

eosinophilia

97

Besides parasites, what is a major general class of causes of eosinophilia?

idiopathic reasons; leukemias and GI disorders

98

T/F: allergies and infections can both cause eosinophilia

true

99

What is the threshold for eosinophilic leukemia?

>1.5 x 10^9/L

100

t/f: eosinophilic leukemia has no known cause

true

101

Describe Loffler's syndrome?

Pulmonary infiltrate with eosinophilia syndrome and tropical eosinophilia

102

What parasite often causes Loffler's?

strongyloids

103

Charcot-Leyden crystals from eosoniophil granule fusion are seen in what disorder?

Loffler's syndrome

104

Charcot-Leyden crystals are particularly dangerous to what organ?

heart; release of granule contents

105

Corticosteroids sequester Eos in the (blank) leading to (blank)

lymph nodes; eosinopenia

106

Sequestration of eos in the lymph nodes can be caused by the release of corticosteroids from what disease processes?

1. Cushing's syndrome2. ACTH3. Acute stress/ Epi4. Inflammation5. Prostaglandins6. Bacterial infection

107

What is the threshold for basophilia?

>0.2 x 10^9/L

108

What cancer often causes basohpilia?

CML

109

Immediate hypersensitivity reactions will cause (baso/eos)ophilia

basophilia

110

T/F: basophilopenia is a life threatening condition

false; clinically insignificant

111

What is the threshold for monocytosis?

>0.8

112

monocyte counts vary with (age/gender)

age

113

T/F: unexplained monocytosis is common in cancer

true

114

What is the threshold for monocytopenia?

115

What body fluid in babies has a high monocyte count?

CSF

116

Monocytes are a marker of (acute/chronic) disease processes

chronic

117

Post-splenectomy pts will often shown elevated counts of what cell type?

monocytes

118

Langerhans Cell histiocytosis, Letterer-Siwe disease are both disorders of what cell type?

monocytes

119

(Monocytosis/penia) is a finding common in hematologic malignancy

monocytosis

120

Aplastic anemia, hairy cell leukemia, and glucocorticoid therapy are all causes of (monocytosis/penia)

monocytopenia

121

Gaucher DiseaseNeimann-Pick DiseaseTay-Sachs DiseaseFabry DiseaseWolman’s DiseaseTangier Diseaseare all (blank) storage disorders of monocytes

lipid

122

(blank) disease ppresents with glucocerebrosidase deficiency in macrophages in the bone marrow, lymph node, liver, and spleen

Gaucher disease

123

In Gaucher disease, serum acid phosphatase is (up/down)

up

124

What is the threshold for lymphocytosis in adults and children?

Adults: >4.0kids: >9.0

125

Most changes in lymphocyte counts are due to (T/B) cells

t cells

126

(T/B) cells account for 60-80% of PERIPHERAL blood lymphocytes

T cells

127

Majority of circulating lymphoid cells are CD(4/8) T cells

CD4

128

Coxsackie A and B6, Echovirus, and Adenovirus are common causes of acute infectious (blank) in children

lymphocytosis

129

Persistent polyclonal B cell lymphocytosis should raise your suspicion of:

chronic leukemia

130

What two risk factors increase your chance of having a persistent polyclonal B cell lymphocytosis?

female smoker and post-splenectomy

131

Infectious mono infects (monocytes/B cells)

B cells

132

T/F: mono is self limiting

true

133

Infectious mono gains entry to the cell via what receptor?

CD21

134

What is the wBC range for someone with infectious mono?

12-25

135

What Ab is positive in people with infectious mono?

Heterophile Ab

136

what is the threshold for lymphocytopenia in adults and kids?

adults:

137

What congenital immunodef can cause lymphocytopenia?

Wiskott-Aldritch

138

T/F: abnormal lymph circulation can lead to lymphocytopenia

true

139

T/F: AIDS or HAART can cause lymphocytopenia

true

140

Most SCID pts are (m/f)

male

141

Describe the difference in T and B cell counts in X linked vs. autosomal forms of SCID?

Xq13: absent to severely reduced T cells; thymic hypoplasiaAutosomal: severe def of both T and B cells (ADA def)

142

What is the Tx for SCID?

bone marrow transplant

143

What is the mode of inheritance of WA syndrome?

X linked

144

What are the three hallmakrs of WA syndrome?

eczema, thrombocytopenia, and immunodef

145

Pts with WA syndrome are at an increased risk of secondary (blank)

neoplasm

146

Pts with WA syndrome have a progressive decrease in (blank)-dependent immunity

thymus

147

T/F: WA syndrome patients have absent Abs to blood groups

true

148

Describe the mitogenic response to CD43 in WA syndrome patients?

ABSENT

149

When would a WA syndrome pt present with abnormal bleeding?

neonatal period

150

(blank) syndrome presents with abscence/hypoplasia of thymus, hypoPTH, congenital heart defects, dysmporphic facies, HYPOCALCEMIA, and is caused by a del22(q11.2)

diGeorge syndrome

151

X-linked Agammaglobulinemacauses a block in the B cell maturation at the (blank) phase due to a failure of variable and constant regions of IgM to connect

pre-B cell stage

152

X-linked Agammaglobulinemapresents with absent (blank) cells

plasma cells and decreased B cells

153

What is the Tx for X-linked Agammaglobulinema

gammaglobulin

154

What disease is auto. rec. and presents with Progressive ataxia, immune dysfunction, increased risk of malignancy, Defect in cell-mediated immunity with thymic hypoplasia or dysplasia?

Hereditary Ataxia-Telangiactasia

155

How do you diagnose hereditary ataxia-telangiactasia?

increased csome breakage at t(14;14)