Hematology Week 2: Benign WBC Disorders Flashcards

(115 cards)

1
Q

The CBC

A
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2
Q

The differential

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3
Q

Abnormalities of WBC count

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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

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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

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13
Q

Severe Neutropenia AKA

A

Agranulocytosis

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14
Q

Case 1

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15
Q

Case 1 Question

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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

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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
Work up of Neutropenia 3 main subjects
26
Clinical Diagnosis of Case 1
27
Neutrophilia
28
Question 2
29
Toxic granulation Neutrophilia cytoplasmic vacuoles no significant left-shift
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The most common WBC abnormality and causes
Any stressful event can cause neutrophilia
31
Work up of Neutrophilia 2 main subjects
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Toxic Changes of Neutrophils
* Heavier granulation * Dohle Body * Cytoplasmic vacuoles
33
Smears
34
Left Shift description
35
Benign causes of increased blasts in the blood
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Leukemoid reaction
* Left shift of the granulocytes to the blast stage * WBC count is very high Usually \>50 * seen with severe bone marrow stress
37
Leukoerythroblastic reaction
* Left shift of the granulocytes to the Blast Stage * Circulating nucleated RBCs are present * Seen with severe bone marrow stress
38
Leukoerythroblastic smear
39
Mechanisms of Neutrophilia 4 listed
40
Clinical Diagnosis of Case #2
41
Lymphopenia
Low lymphocyte count also Lymphocytopenia
42
Lymphocytosis
* High lymphocyte count * can have activated or non-activated appearance
43
Lymphopenia prevalence
Rare
44
Lymphopenia causes 5 listed
* Drugs (glucocorticoids) * Infection (HIV, other viral) * Congenital immunodeficiencies * Autoimmune Diseases * Malnutrition
45
Lymphocyte Morphology
46
Causes of Non-activated Lymphocytes 5 listed
* Pertussis * Smoking * Transient stress lymphocytosis * Thymoma * Polyclonal B Lymphocytosis
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Causes of Activated Lymphocytes 5 listed
* Infectious Mononucleosis (Epstein-Barr Virus * Cytomegalovirus infection * Viral hepatitis * Other viral infections * Post-vaccination
48
Non activated vs activated lymphocytosis causes
49
Question 3
50
Question 4
51
Non-activated lymphocytosis Cleft is weird
52
Question 5 What is the most common cause of non-activated lymphocytosis in a young child?
Pertussis (Whooping Cough)
53
Pertussis Pathogen
Gram - coccobacillus Bordetella pertussis
54
Pertussis causes?
Laryngotracheobronchitis
55
Pertussis is characterized by?
Violent coughing with a loud inspiratory "Whoop"
56
Pertussis Vaccination
Vaccination with DPT has greatly reduced the prevalence of whooping cough
57
Pertussis Lymphocytosis Etiology
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
Case 4
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PBS
60
Cause?
Activated Lymphocytosis
61
Infectious Mononucleosus Pathogen
EBV of B lymphocytes
62
Infectious Mononucleosis Cellular changes
* 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
Infectious Mononucleosis Epidemiology
most common in adolescence and young adults
64
Infectious Mononucleosis Clinical Presentation 4 listed
* Fever * Sore throat * Lymphadenopathy * Splenomegaly
65
Lab tests for Infectious Mononucleosis 2 listed
* Monospot * EBV serologic tests
66
Monospot test Properties
67
EBV Specific Serological Test Properties
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Moncytosis
High monocyte count
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Monocytopenia
Low monocyte count
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Causes of monocytosis 6 main categories
71
Work up of Monocytosis
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Eosinophilia
high eosinophil count
73
Basophilia
High basophilia count
74
Reactive/Benign Basophilia
Rare Allergy or hypothyroidsim
75
If a patient has basophilia
need to think about malignant diagnoses
76
Case 5
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Case 5 Diagnosis
78
Causes of Eosinophilia 7 Main Listed
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Loffler Syndrome
basically eosinophilic pneumonia that can cause cardiac damage
80
Loffler Syndrome Clinical Presentation 5 listed
* Fever * Malaise * Cough * Wheezing * Urticaria
81
Leukocytosis overview
82
Some examples of disorders of neutrophil functions 3 main mechanisms
83
Humoral Disorders
84
Humoral Disorders result in inadequate generation of?
Chemotactic factors or opsonins * quantitative immunoglobulins * specific complement components
85
Humoral Disorders immunoglobulin deficiency states lead to susceptibility to?
Pyogenic infections especially from encapsulated organisms
86
Humoral Disorders abnormality in complement pathway
may explain susceptibility to infection of patients with sickle cell anemia
87
Causes Defects of cellular movement 4 listed
* Drugs * Chediak-Higashi Syndrome * Thermal Injury * Neutrophil actin dysfunction
88
Chediak-Higashi Syndrome Etiology
Rare Autosomal Recessive disease LYST gene (CHS1 gene) which is the lysosomal trafficking regulatory protein
89
Chediak-Higashi Syndrome Pathophysiology
* 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
Chediak-Higashi Syndrome
usually have neutropenia as well because dysfunctional cells
91
Treatment of Chediak-Higashi Syndrome
* Ascorbic acid * G-CSF * Prophylactic antibiotics * Stem Cell transplant is curative
92
Neutrophils in Chediak-Higashi syndrome
93
Defects in Oxidative Microbicidal Action 4 listed
94
Chronic Granulomatous disease Etiology
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
Chronic Granulomatous disease Neutrophils
* 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
Chronic Granulomatous disease Epidemiology
Rare Male more common (X linked) Median age of diagnosis is 3 years
97
Chronic Granulomatous Disease Susceptibility to?
* Catalase Positive organisms * S aureus, enterobacteriaceae * aspergillus * organisms resistant to non-oxidative killing
98
Chronic Granulomatous disease Infections common in? 4 listed
* lung * skin * lymph nodes * Liver * **Granulomas in GI/GU tracts**
99
NADPH Oxidase 6 listed
100
Chronic Granulomatous disease Overview
101
Phagosome formation and oxidative killing
102
Chronic Granulomatous disease most common protein mutated
gp91 protein but can be any of the 5 proteins
103
Chronic Granulomatous disease Genetics
CYBB Gene on the X chromosome
104
Autosomal Recessive Chronic Granulomatous disease
milder disease usually p47
105
Treatment of Chronic Granulomatous disease
* prophylactic antibiotics * interferon * G-CSF * Stem Cell transplant is curative
106
CGD AKA
Chronic Granulomatous Disease this is a granuloma
107
NBT AKA
Nitroblue Tetrazolium Dye Reduction Test
108
Nitroblue Tetrazolium Dye Reduction Test
can detect chronic granulomatous disease CGD No crystals is consistent with CGD
109
MPO Deficiency epidemiology
most common inherited disorder of phagocytes 1/2000 - 1/4000
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MPO Deficiency Etiology
usually autosomal recessive numerous mutations have been identified
111
MPO Deficiency Clinical Presentation
Heterogenous clinical manifestations
112
MPO Deficiency AKA
Myeloperoxidase Deficiency
113
MPO Deficiency Overview
114
Acquired MPO Deficiency causes
* Pregnancy * Lead Poisoning * Severe infection
115
MPO Deficiency is usually clinically?
Silent