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Hematology > Hematology Week 2: Benign WBC Disorders > Flashcards

Hematology Week 2: Benign WBC Disorders Flashcards

1
Q

The CBC

A
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2
Q

The differential

A
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3
Q

Abnormalities of WBC count

A
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4
Q

Leukocytosis

A

High WBC count

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5
Q

Leukopenia

A

Low WBC count

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6
Q

Leukocytosis Lab reference ranges

A
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7
Q

WBC abnormalities

A

if blasts are increased, consider neoplastic causes

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8
Q

Abnormal neutrophil counts

A
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9
Q

Neutropenia

A

Low neutrophil count

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10
Q

Neutrophilia

A

High neutrophil count

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11
Q

Agranulocytosis

A
  • absence of neutrophils
  • Makes patients highly susceptible to bacterial and fungal infections
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12
Q

Neutrophil Levels in racial and ethnic groups

A
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13
Q

Severe Neutropenia AKA

A

Agranulocytosis

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14
Q

Case 1

A
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15
Q

Case 1 Question

A
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16
Q

Peripheral Blood smear of neutropenia

A
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17
Q

Clinical Presentation of Neutropenia: Symptoms

4 Listed

A
  • Malaise
  • Chills
  • Fever
  • Weakness
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18
Q

Clinical Presentation of Neutropenia: Common Infections

A
  • Infections commonly occur in the oral cavity
  • Aphthous stomatitis
  • less common in skin, vagina, anus, GI tract, lungs and kidneys
  • can be bacterial or fungal (Aspergillus, Candida)
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19
Q

Oral ulcers may be indicative of

A

Severe neutropenia and Agranulocytosis

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20
Q

Common causes of Neutropenia

6 listed

A
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21
Q

Mechanisms of Neutropenia

3 listed

A
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22
Q

Cyclic Neutropenia Overview

A
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23
Q

Cyclic Neutropenia Etiology

A

Autosomal Dominant disorder

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24
Q

Cyclic Neutropenia Clinical Presentation

A

3-6 days of neutropenia occur every 21-30 days in a periodic pattern

Fever

Infection

  • Stomatitis
  • Cellulitis
  • Vaginitis
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25
Q

Work up of Neutropenia

3 main subjects

A
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26
Q

Clinical Diagnosis of Case 1

A
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27
Q
A

Neutrophilia

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28
Q

Question 2

A
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29
Q
A

Toxic granulation

Neutrophilia

cytoplasmic vacuoles

no significant left-shift

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30
Q

The most common WBC abnormality and causes

A

Any stressful event can cause neutrophilia

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31
Q

Work up of Neutrophilia

2 main subjects

A
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32
Q

Toxic Changes of Neutrophils

A
  • Heavier granulation
  • Dohle Body
  • Cytoplasmic vacuoles
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33
Q

Smears

A
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34
Q

Left Shift description

A
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35
Q

Benign causes of increased blasts in the blood

A
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36
Q

Leukemoid reaction

A
  • Left shift of the granulocytes to the blast stage
  • WBC count is very high Usually >50
  • seen with severe bone marrow stress
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37
Q

Leukoerythroblastic reaction

A
  • Left shift of the granulocytes to the Blast Stage
  • Circulating nucleated RBCs are present
  • Seen with severe bone marrow stress
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38
Q

Leukoerythroblastic smear

A
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39
Q

Mechanisms of Neutrophilia

4 listed

A
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40
Q

Clinical Diagnosis of Case #2

A
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41
Q

Lymphopenia

A

Low lymphocyte count

also Lymphocytopenia

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42
Q

Lymphocytosis

A
  • High lymphocyte count
  • can have activated or non-activated appearance
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43
Q

Lymphopenia prevalence

A

Rare

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44
Q

Lymphopenia causes

5 listed

A
  • Drugs (glucocorticoids)
  • Infection (HIV, other viral)
  • Congenital immunodeficiencies
  • Autoimmune Diseases
  • Malnutrition
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45
Q

Lymphocyte Morphology

A
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46
Q

Causes of Non-activated Lymphocytes

5 listed

A
  • Pertussis
  • Smoking
  • Transient stress lymphocytosis
  • Thymoma
  • Polyclonal B Lymphocytosis
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47
Q

Causes of Activated Lymphocytes

5 listed

A
  • Infectious Mononucleosis (Epstein-Barr Virus
  • Cytomegalovirus infection
  • Viral hepatitis
  • Other viral infections
  • Post-vaccination
48
Q

Non activated vs activated lymphocytosis causes

49
Q

Question 3

50
Q

Question 4

51
Q
A

Non-activated lymphocytosis

Cleft is weird

52
Q

Question 5

What is the most common cause of non-activated lymphocytosis in a young child?

A

Pertussis (Whooping Cough)

53
Q

Pertussis Pathogen

A

Gram - coccobacillus Bordetella pertussis

54
Q

Pertussis causes?

A

Laryngotracheobronchitis

55
Q

Pertussis is characterized by?

A

Violent coughing with a loud inspiratory “Whoop”

56
Q

Pertussis Vaccination

A

Vaccination with DPT has greatly reduced the prevalence of whooping cough

57
Q

Pertussis Lymphocytosis Etiology

A

Caused by the inability of lymphocytes to migrate from blood to tissue due to down-regulation of a cell surface adhesion molecule by a toxin produced by B. pertussis

58
Q

Case 4

60
Q

Cause?

A

Activated Lymphocytosis

61
Q

Infectious Mononucleosus Pathogen

A

EBV of B lymphocytes

62
Q

Infectious Mononucleosis Cellular changes

A
  • EBV infection of B lymphocyte Causes
  • The proliferation of Activated cytotoxic/suppressor t cells occurs
  • activated lymphocytes are large reactive with abundant basophilic cytoplasm
  • changes are not unique to IM and may be seen with other viral infections
63
Q

Infectious Mononucleosis Epidemiology

A

most common in adolescence and young adults

64
Q

Infectious Mononucleosis Clinical Presentation

4 listed

A
  • Fever
  • Sore throat
  • Lymphadenopathy
  • Splenomegaly
65
Q

Lab tests for Infectious Mononucleosis

2 listed

A
  • Monospot
  • EBV serologic tests
66
Q

Monospot test Properties

67
Q

EBV Specific Serological Test Properties

68
Q

Moncytosis

A

High monocyte count

69
Q

Monocytopenia

A

Low monocyte count

70
Q

Causes of monocytosis

6 main categories

71
Q

Work up of Monocytosis

72
Q

Eosinophilia

A

high eosinophil count

73
Q

Basophilia

A

High basophilia count

74
Q

Reactive/Benign Basophilia

A

Rare Allergy or hypothyroidsim

75
Q

If a patient has basophilia

A

need to think about malignant diagnoses

76
Q

Case 5

77
Q

Case 5 Diagnosis

78
Q

Causes of Eosinophilia

7 Main Listed

79
Q

Loffler Syndrome

A

basically eosinophilic pneumonia that can cause cardiac damage

80
Q

Loffler Syndrome Clinical Presentation

5 listed

A
  • Fever
  • Malaise
  • Cough
  • Wheezing
  • Urticaria
81
Q

Leukocytosis overview

82
Q

Some examples of disorders of neutrophil functions

3 main mechanisms

83
Q

Humoral Disorders

84
Q

Humoral Disorders result in inadequate generation of?

A

Chemotactic factors or opsonins

  • quantitative immunoglobulins
  • specific complement components
85
Q

Humoral Disorders immunoglobulin deficiency states lead to susceptibility to?

A

Pyogenic infections especially from encapsulated organisms

86
Q

Humoral Disorders abnormality in complement pathway

A

may explain susceptibility to infection of patients with sickle cell anemia

87
Q

Causes Defects of cellular movement

4 listed

A
  • Drugs
  • Chediak-Higashi Syndrome
  • Thermal Injury
  • Neutrophil actin dysfunction
88
Q

Chediak-Higashi Syndrome Etiology

A

Rare Autosomal Recessive disease

LYST gene (CHS1 gene) which is the lysosomal trafficking regulatory protein

89
Q

Chediak-Higashi Syndrome Pathophysiology

A
  • microtubule dysfunction prevents the transport of materials into lysosomes
  • Abnormal granule fusion in many granular cells including skin, hair, adrenal glands and CNS
  • Can cause Oculocutaneous albinism, peripheral neuropathy (nerve), severe pyogenic infections (neutrophil granules)
90
Q

Chediak-Higashi Syndrome

A

usually have neutropenia as well because dysfunctional cells

91
Q

Treatment of Chediak-Higashi Syndrome

A
  • Ascorbic acid
  • G-CSF
  • Prophylactic antibiotics
  • Stem Cell transplant is curative
92
Q

Neutrophils in Chediak-Higashi syndrome

93
Q

Defects in Oxidative Microbicidal Action

4 listed

94
Q

Chronic Granulomatous disease Etiology

A

Defects in phagocyte NADPH Oxidase (phox)

defective respiratory burst in neutrophils

Absence or malfunction of oxidase

usually from defective cytochrome b function (no reactive oxygen radicals, no respiratory burst)

X-linked

95
Q

Chronic Granulomatous disease Neutrophils

A
  • Unable to destroy microves
  • imparied intracellular microbial killing by phagocytes
  • recurrent bacterial/fungal infections with granuloma formation
  • osteomyelitis & abcesses in the first year of life is typical
96
Q

Chronic Granulomatous disease Epidemiology

A

Rare

Male more common (X linked)

Median age of diagnosis is 3 years

97
Q

Chronic Granulomatous Disease Susceptibility to?

A
  • Catalase Positive organisms
  • S aureus, enterobacteriaceae
  • aspergillus
  • organisms resistant to non-oxidative killing
98
Q

Chronic Granulomatous disease Infections common in?

4 listed

A
  • lung
  • skin
  • lymph nodes
  • Liver
  • Granulomas in GI/GU tracts
99
Q

NADPH Oxidase

6 listed

100
Q

Chronic Granulomatous disease Overview

101
Q

Phagosome formation and oxidative killing

102
Q

Chronic Granulomatous disease most common protein mutated

A

gp91 protein but can be any of the 5 proteins

103
Q

Chronic Granulomatous disease Genetics

A

CYBB Gene on the X chromosome

104
Q

Autosomal Recessive Chronic Granulomatous disease

A

milder disease usually p47

105
Q

Treatment of Chronic Granulomatous disease

A
  • prophylactic antibiotics
  • interferon
  • G-CSF
  • Stem Cell transplant is curative
106
Q

CGD AKA

A

Chronic Granulomatous Disease

this is a granuloma

107
Q

NBT AKA

A

Nitroblue Tetrazolium Dye Reduction Test

108
Q

Nitroblue Tetrazolium Dye Reduction Test

A

can detect chronic granulomatous disease CGD

No crystals is consistent with CGD

109
Q

MPO Deficiency epidemiology

A

most common inherited disorder of phagocytes

1/2000 - 1/4000

110
Q

MPO Deficiency Etiology

A

usually autosomal recessive

numerous mutations have been identified

111
Q

MPO Deficiency Clinical Presentation

A

Heterogenous clinical manifestations

112
Q

MPO Deficiency AKA

A

Myeloperoxidase Deficiency

113
Q

MPO Deficiency Overview

114
Q

Acquired MPO Deficiency causes

A
  • Pregnancy
  • Lead Poisoning
  • Severe infection
115
Q

MPO Deficiency is usually clinically?

Hematology (26 decks)

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