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Block 7 Week 1 > Non-neoplastic WBC abnormalities-Usera > Flashcards

Non-neoplastic WBC abnormalities-Usera Flashcards

1
Q

What are the four major lab measurements of WBCs?

A

Automated hematology analyzers
Bone marrow aspirate and biopsy
Flow cytometry
Peripheral blood smears

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2
Q

What does automated hematology do?

A

gives cell count

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3
Q

Are aspirate and biopsy the same thing?

A

no biopsy is taken from the core of bone marrow and aspirate is the jelly fluid stuff but you take both and look at both separetly

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4
Q

What does flow cytometry do?

A

helps to identify lineage by looking at cell markers

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5
Q

When do you do peripheral blood smears?

A

ONLY WHEN THERE IS NOT OTHER EXPLAINABLE CAUSE

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6
Q

What are the three factors that affect neutrophil concentration in blood?

A

Bone marrow production and release
Rate of egress to tissue or survival time in blood
Ratio of marginated to circulating neutrophils in peripheral blood (MGP/CGP)

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7
Q

Where do you find neutrophils when you have an infection? What do you call this and why is this significant?

A

line up around the periphery (next to endothelium)-> called margination, When you take needle aspirate you will get low number of neutrophils cuz none of them will be in the middle

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8
Q

Absolute neutrophil count greater than (blank) X 10^9/L in adults is considered neutrophilia

A

7

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9
Q

What is neutrophilia a response to?

A

physiologic or pathologic processes

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10
Q

why can you get immediate neutrophilia and how long does it take?

A

redistribution from marginated to circulating pool-> 20-30 minutes

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11
Q

What are some things that can cause immediate neutrophilia?

A

stress, steroids, epinephrine, IL-6

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12
Q

Why can you get an acute neutrophilia and how long does it take?

A

release from maroow storage pool to blood (IL-6) 4-5 hours

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13
Q

Why can you get a chronic neutrophilia and how long does it take?

A

increase in marrow mitotic poo

days

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14
Q

(blank) will immediately increase your neutrophil count

A

epinephrine

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15
Q

If you have a cell population with more bands than mature neutrophils, what do you call this? WHat does this mean?

A

a left shift

means you have an acute inflammatory response

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16
Q

Explain the matureation of neutrophil

A

Myoblast-> promylocyte-> myelocyte-> metamylocyte-> band-> mature neutrophil

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17
Q

What phase of maturation of a neutrophil is this:

have a giant N to C ratio (i.e the nucleus is huge compared to the cytoplasm) should only be found in bone marrow.

A

Myoblast cells

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18
Q

What phase of maturation of a neutrophil is this:
A cell you with a slightly smaller N but still high N to C ratio than myoblast cells and then there are some granules present.

A

Promyelocyte

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19
Q

What phase of maturation of a neutrophil is this is this:

semicircle nucleus and lots of condensed granules

A

myelocyte

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20
Q

What phase of maturation of a neutrophil is this:

looks like a pacman

A

metamyelocyte

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21
Q

What phase of maturation of a neutrophil is this:

looks like a C shaped nucleus

A

Band cell

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22
Q

What phase of maturation of a neutrophil is this:

multi lobed nucleus

A

neutrophils

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23
Q

Lifespan of neutrophils is only a couple of (blank) so they are just for acute responses

A

hours

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24
Q 
What are these causes of:
Acute inflammation
Acute infection
Tissue necrosis
Drugs, toxins, metabolic
Physiologic
Neoplastic
A

Causes of neutrophilia

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25
Q

What do these cause:

collagen vascular, vaculitis

A

Acute inflammation -> which causes neutrophilia

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26
Q

What do these cause:

Corticosteroids, smoking, growth factors, uremia, ketoacidosis, lithium

A

Neutrophilia

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27
Q

Can carcinomas, sarcomas and MPDs cause neutrophilia?

A

yes!

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28
Q

What physiological things can cause neutrophlia?

A

pregnancy, exercise, stress

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29
Q

What can cause acute infections and thus neutrophilia?

A

bacteria, fungi, parasites, viruses, spirochetes

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30
Q

What are features associated with REACTIVE neutrophilia?

A

Usually <30 x 109/L

Shift to the left in myeloid maturation

Frequently associated with morphologic alterations in neutrophils and precursors
Toxic granulation
Döhle bodies
Vacuolization

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31
Q

Is there a heritable cause for neutrophilia?

A

no

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32
Q

WHo has higher amounts of neutrophils, adults or children?

A

Children

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33
Q

What morphologic alterations in neutrophils and precursors are seen in reactive neutrophilia?

A

toxic granulation, Dohle bodies, vacuolization

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34
Q

What is a leukemoid raction?

A

a reaction that resembles leukemia but isnt

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35
Q

What is this: a benign leukocyte proiferation with WBC usually greater than 50 X 10^9/L with many circulating immature leukocyte precursors.

A

Leukemoid reaction

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36
Q

Since blasts are occasionally present in leukemoid reactions, how can you exclude CML?

A

with cytogenetics and LAP score

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37
Q

What is this:

characterized by presence of nucleated RBC and a shift to the left in granulocyte maturation

A

Leukoerythroblastic reaction

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38
Q

What are leukoerythroblastic reactions aften associated with?

A

myelopthisic processes, severe hemorrhage, hemolytic anemia, or myelodysplastic syndromes

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39
Q

Why will you get a leukemoid reaction?

A

perforated appendicitis (neutrophils), whooping cough (lymphs), cutaneous larva migrans (eos).

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40
Q

Why can you get a leukoerythroblastic reaction?

A

marrow infiltration (fibrosis, mets)

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41
Q

What drug can make it look like a leukemoid or leukoerythroblastic reaction? WHy?

A

neupogen

it is used after bone marrow transplants to increase hemmopoietic activity

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42
Q

What is the definition of neutropenia?

A

less than 2.0 X 10^9 /L in whites and less than 1.3 X 10 ^ 9/L in blacks

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43
Q

What is the definition of agranulocytosis?

A

less than 0.5 X 10 ^9 /L

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44
Q

What are mechanisms for neutropenia?

A

decreased or ineffective marrow production
increased cell loss or tissue egress
pseudoneutropenia (endotoxin)

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45
Q

What are the 6 major causes of neutropenia?

A 
Drugs
Intrinsic Defects
Overwhelming Infection
Hematologic disorders
Autoimmune
Cachexia and Debiiatate States
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46
Q

WHat are the drugs that can cause neutropenia?

A

EtOH, benzene, chloramphenicol, chemotherapy, antibiotics, benzodiazepines
AND
CLOZAPINE!!!!!!!! (Anitpsychotic)

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47
Q

What hematologic disorders can cause neutropenia?

A

megaloblastic anemia
myelodysplasia
marrow failure
hyperslepnism (splenomegaly)

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48
Q

What autoimmune diseases cause neutropenia?

A

Lupus, Rh Arthritis

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49
Q

What intrinisic defects (rare) cause neutropenia?

A

Fanconi’s, Kostmann’s, Cyclic neutropenia, chediak-higashi

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50
Q

What overwhelming infections can cause neutropenia?

A

miliary TB, sepsis, brucellosis

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51
Q

Many conditions causing agranulocytosis affect both (blank) and (blank) lines

A

erythroid and myeloid lines

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52
Q

How do you remember what Brucella does?

A

Besty the Bovine had Brucella in her Bone marrow-> brucella causes bone marrow problems and you get it from cattle

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53
Q

What infections are associated with neutropenia?

A

Viral, Bacterial, Rickettsial, Protozoal

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54
Q

What do these cause:
Influenza, Measles, Chicken pox, Colorado tick fever, Dengue, Infectious mononucleosis, Poliomyelitis, Psittacosis, Sand-fly fever, Smallpox, Rubella, Infectious hepatitis

A

neutropenia via infection

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55
Q

What do these cause:

Typhoid, Bacillary dysentery, Paratyphoid, Brucellosis, Ehrlichiosis

A

Neutropenia via infection

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56
Q

What are these:
Rickettsial pox, Typhus, Rocky Mountain Spotted Fever
What do they cause?

A

Rickettsial infections

infections that cause neutropenia

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57
Q

What are these:
Malaria, Kala-azar, Relapsing fever
What do they cause?

A

protozoal infections

Infections associated with neutropenia

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58
Q

What causes quantitative neutrophil disorders?

A

Myeloid hypoplasia and maturation defects

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59
Q

What are the three major disorders that cause myeloid hypoplasia?

A

Fanconi’s anemia
Kostmann’s syndrome
Cyclic neutropenia

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60
Q

What are the maturation defects that cause quantitative neutrophil disorders?

A

chediak-higashi

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61
Q

What is this:
Panmyeloid hypoplasia
Heterogenous disease caused by chromosomal instability
Presents in childhood with aplastic anemia and congenital physical malformations
Susceptible to hematopoietic and solid organ malignancies

A

Fanconi’s anemia

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62
Q

What is a tell tale sign of fanconi’s anemia?

A

no thumbs, hand deformity

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63
Q 
What is this:
ANC <200/ul
Variable modes of inheritance
Early myeloid precursors in marrow, but do not mature
Ela2 and hax-1 implicated
A

Kostmann’ syndrome

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64
Q

What exactly is Kostmann’s syndrome?

A

an infantile genetic congenital agranulocytosis

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65
Q

What are the 2 genes super implicated in Kostmann’s syndrome?

A

ELA2 and HAX-1

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66
Q 
What is this:
Presents in infancy or childhood
Rare autosomal dominant trait with variable expression 
21-30 day periodicity
ANC
A

Cyclic neutropenia

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67
Q

What is the periodicity of cyclic neutropenia?

A

21-30 days

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68
Q

What gene is associated with cyclic neutropenia?

A

ELA2 gene mutation (neutrophil elastase)

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69
Q

How do you diagnose cyclic neutropenia?

A

routine CBC

then Bone marrow aspirate and biopsy

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70
Q

If you have an infection what will the neutrophil count be like?

A

low because they are making love to the endothelium

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71
Q

What are the most common ways to get congenital neutropenia?

A

Pregnancy induced hypertension (i.e eclampsia)

Babies born to pregnant moms who have overwhelming infections (babies get chorionamniotitis)

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72
Q

What are the five ways to get spurious neutropenia?

A

EDTA-dependent agglutinin
Old specimen
WBC fragility
Paraprotein

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73
Q

How can paraproteins cause spurious neutropenia?

A

a lot of antibodies circulating can effect N counts

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74
Q

What is spurious neutropenia?

A

fake/false neutropenia

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75
Q

What do toxic granullations look like?

A

large, blue-black granules

Primary (azurophilic) granules retain basophilia (easily stain with basic dye), perhaps due to lack of maturation.

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76
Q

What are toxic granulations associated with?

A

Dohle bodies and vacuolization

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77
Q

When do you see toxic granulations?

A

septicemia, sepsis

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78
Q

Toxic granulations found in neutrphils make (blank) activity decreased compared to normal granulocytes

A

peroxidase activity

79
Q

What are Dohle bodies?

A

remnants of free ribosomes and RER

80
Q

When do you see Dohle bodies?

A

in severe bacterial infections, pregnancy, burns, cancer, aplastic anemia, toxic states

81
Q

What do you often see associated with Dohle bodies?

A

occur with toxic granulation and vacuolization

82
Q

What is this:

end stage of phagocytosed material, or fat or other substance

A

Vacuolization

83
Q

What is vacuolization often associated with?

A

Dohle bodies and toxic granules

84
Q

What is vacuolization a predictor for?

A

sepsis

85
Q

What is a shistocyte?

A

a fragmented RBC

86
Q

(blank) is an autosomal dominant disorder characterized by abnormal nuclear shape and chromatin organization in blood granulocytes. Heterozygotes show hypolobulated neutrophil nuclei with coarse chromatin

A

Pelger-Huet anomaly

87
Q

Is pelger huet anomaly detrimental?

A

no, it is clinically asymptomatic with normal neutrophil function

88
Q

Why is it important to know what pelger huet is?

A

so that you dont confuse it with leukemia.

89
Q

What is the definition of hypersegmentation?

A

greater than 5% neutrophils with five lobes or any with six

90
Q

What is hypersegmentation associated with?

A

Megaloblastic anemia (B12 deficiency), chronic infection, myelodysplastic syndromes

91
Q

What is the characteristics of hereditary hypersegmentation?

A

autosomal dominant, rare, not associated with disease

92
Q

Which deficiency will you have neurological symptoms; folate deficiency or B12 deficiency?

A

B12 deficiency

93
Q

What is this:
autosomal recessive trait,
large purplish granules in cytoplasm
granules stain toluidine blue

A

Alder-Reilly anomaly

94
Q

What is Alder-Reilly anomaly associated with?

A

mucopolysaccharidoses (hunters syndrome, hurler’s syndrome)

95
Q

So when you see dark granules what diseases should you be thinking?

A

hunters or hurlers syndrome

96
Q

What is this:

  • autosomal recessive trait
  • Giant gray-green peroxidase positive bodies in cytoplasm of leukocytes and other cells.
  • defects in fusion of cytoplasm membranes, locomotion and chemotaxis
A

Chediak-Higashi

97
Q

What are some notable systems of Chediak-Higashi?

A

abnormal melanosomes (skin hypopigmentation , photophobia, lymphadenopathy, hepatosplenomegaly

98
Q

If you see inclusions in neutrophils what does this narrow your differential to?

A

Chediak higashi or Ehrlichia

99
Q

What is this

partial albinism, photophobia, immunodeficiency, frequent pyogenic infections

A

Chediak-higashi

100
Q 
What is this:
autosomal dominant trait
Large Dohle body-like inclusions in granulocytes
Involves myosin heavy chain 9
inclusions contain RNA
A

May-Hegglin Anomaly

101
Q

What is may-hegglin anomaly associated with?

A

thrombocytopenia, giant oval platelets with few granules

102
Q

What does may hegglin anomaly typically present with?

A

bleeding disorder due to platelet defect

103
Q

If you are looking at a histo slide and you see May Hegglin anomaly, what will you see in the slide?

A

giant platelets and inclusions in granulocytes

104
Q

Does may hegglin anomaly have normal granulocyte function?

A

yes!

105
Q

What is this:

  • defect in respiratory burst oxidase system
  • presents in childhood with recurrent infections with low-grade pathogens
  • formation of granulomas when neutrophils phagocytose, but do not kill organisms
  • defects in membrane-associated cytochrome b (subunits gp91 and p22) and cytosol-associated p47 and p67
  • MESSED UP NADPH OXIDASE SYSTEM
A

Chronic granulomatous disease

106
Q

How is chronic granulomatous disease passed on

A

sex linked and autosomal recessive inheritance patterns

107
Q

Chronic granulomatous disease is caused by defects in membrane associated (blank) and cytosol associated (blank)

A

cytochrome b

p47 and p67

108
Q

If you have a messed up NADPH oxidase system what cant you make?

A

hydrogen peroxide (means you have a defect in respiratory burst)

109
Q

How do you diagnose chronic granulomatous disease?

A

Nitroblue tetrazollum test (NBT)

Normal neutrophils reduce H202 and 02 to a blue product, CGD neutrophils cannot reduce NBT so you dont have blue

110
Q

What is the treatment for CGD?

A

prophylactic antibiotics

111
Q

To diagnose CGD, Incubate with NBT and if your NADPH peroxidase (NADPH) is working then you will have (blank) cells cuz the catalase bugs wll break it down, if you don’t have it it will be (blank) becase the catalase bugs will have nothing to break down.

A

purple

pink

112
Q

What are the characteristics of MPO (myeloperoxidase deficiency)?

A

autosomal recessive inheritance (can be acquired)
absence of MPO in neutrophils and monocytes
Infections not a usual complication

113
Q

How do you diagnose MPO?

A

histochemistry: myeloperoxidase stain
NB: may be recognized on WBC histogram using technicon instruments

114
Q

How do you get acquired myeloperoxidase deficiency (MPO)?

A

myeloid neoplasms, drugs, severe infections, DM, pregnancy

115
Q

WHy dont you have a lot of recurrent infections with myeloperoxidase deficiency (MPO)?

A

because you still got your other lethal oxygen radicals

116
Q

In leukocyte adhesion deficiency type I, what is missing?

A

integrins; CD11a and CD18

117
Q

In leukocyte adhesion type II, what is missing?

A

you have integrin but you dont have your selectins (i.e. nothing to bind to)

118
Q

In leukocyte adhesion type III, what is missing?

A

you dont have activation of your integrins (super rare) so you get margination but no diapedesis

119
Q

What are these symptoms of:
delayed separation of umbilical cord
recurrent pyoderma gangrenosum

A

Leukocyte Adhesion deficiencies

120
Q

What is the summary of LAD?

A

cant fight cells and get things where they need to go

121
Q

If you have a neonate with delayed separation of the umbilical cord, what type of LAD do they have?

A

1

122
Q

What is the definition of eosinophilia?

A

greater than 0.45 X 10^9 /L adults

123
Q

What is eosinophilia associated with?

A

cellular immune response

124
Q

What are the causes of reactive eosinophilia?

A 
parasitic infection
allergic disorders
infections
addisons disease
malignancies
collagen disease
idiopathic
leukemias
GI disorders
125
Q

What infections cause a reactive eosinophilia?

A

leprosy, brucellosis, TB, fungal infections, scarlet fever

126
Q

What interleukins produce IgE?

A

IL-4

127
Q

What are 2 hypereosinophilic syndromes?

A

Eosinophilic leukemia

Loffler’s syndrome

128
Q

What is eosinophilic leukemia?

A

persistent absolute eosinophil count greater than 1.5 X 10^9 /L in adults with tissue infiltration and no apparent cause

129
Q

What is lofflers syndrome?

A

pulmonary infiltrate with eosinophilia syndrome

-tropical eosinophilia

130
Q

Hypereosinophilic syndromes may cause extensive tissue damage, especially (blank) due to release of granule contents.

A

heart

131
Q

Where do you find Charcot-leyden crystals in exudates and what are they?

A

Hypereosinophilic syndromes-> condensation of granule proteins

132
Q

If you see a lot of crystals, what does this tell you and why is it worrisome?

A

a lot of eosinophils were there and it is worrisome because this is very damaging to heart valves.

133
Q

What are the clinical features of hypereosinophilic syndromes?

A 
Fever
Fatigue
Cough
Angioedema
Pruritis
Diarrhea
134
Q

What causes eosinopenia?

A

hypercortilism

135
Q

What do corticosteroids due to eosinophils?

A

it sequesters eosinophils in lymph nodes

136
Q

What can cause hypercortisolism?

A 
Cushing’s syndrome
ACTH
Acute Stress/Epinephrine
Inflammation
Prostaglandins
Bacterial infection
137
Q

What is the definition of basophilia?

A

absolute basophil count greater than 0.2 X 10^9 /L in adults

138
Q

What are causes of basophilia?

A 
Immediate hypersensitivity reactions
Chronic myeloproliferative disorders (PV)
CML
Basophilic Leukemia
Irradiation
139
Q

If you have degranulation of basophils you will be super itchy because of the (blank) degranulation

A

histamine

140
Q

Basophils are sensitive to (blank) so they will often itch after taking a shower

A

temperature

141
Q

What is the definition of monocytosis?

A

absolute monocyte count greater than 0.8 X 10^9 /L in adults

**monocyte count varies with age*

142
Q

Unexplained (blank) is a frequent finging in malignancies

A

monocytosis

143
Q

What is the definition of monocytopenia?

A

Absolute monocyte count ess than 0.2 X 10^9/L in adults

144
Q

What are the causes of monocytosis (6)?

A 
Inflammatory
monocyte disorders
infections
hematologic malignancy
recovery states
post-splenectomy
145
Q

What can cause inflammation that leads to monocytosis?

A

collagen vascular,

UC/Chrons, PAN, TA

146
Q

What are monocyte disorders that cause monocytosis?

A

langerhand cell
histoiocytosis
letterer siwe disease

147
Q

What are infectious disorders that cause monocytosis?

A

TB, SBE, Syphillis, protozoa/ricketssiae

148
Q

What hematologic malignancies cause monocytosis?

A

AML, MDS, MPD, HD, NHL, MM

149
Q

(blank) is a marker of chronic disease.

A

monocytosis

150
Q

What are causes of monocytopenia?

A

stem cell dsorders (aplastic anemia)
Hairy cell leukemia
Glucocorticoid therapy

151
Q

(blank) appear within th emonocyte gate on flow cytometry, which often masks the cytopenia.

A

Hairy cells

152
Q

What are the lipid storage disorders (abnormalities of monocytes)?

A 
Gaucher Disease
Neimann-Pick Disease
Tay-Sachs Disease
Fabry Disease
Wolman's Disease
Tangier Disease
153
Q

What is this:

AR, glucocerebrosidase def.

A

Gaucher Disease

154
Q

What is this:

AR, sphingomyelinase def.

A

Neimann-Pick Disease

155
Q

What is this:

AR, hexamindise A def.

A

Tay-Sachs Disease

156
Q

What is this:

XLR, alpha-galactosidase def.

A

Fabry disease

157
Q

What is this:

AR, lysosomal acid lipase def.

A

Wolman’s Disease

158
Q

What is this:

AR, alpha-lipoprotein def.

A

Tangier Disease

159
Q 
What is this:
Autosomal recessive trait
Glucocerebrosidase deficiency
Macrophages in bone marrow, lymph node, liver, spleen
Serum acid phosphatase increased
Types I, II, and III
A

Gaucher Disease

160
Q

What are the three types of Gauchers disease?

A

Disease manifested in macrophages.
Type 1 non neurological
Type 2 neurologic and fatal
Type 3 neurologic but can live

161
Q

What is lymphocytosis?

A

absolute lymphocyte count greater than 4.0 x 10^9/L in adults and greater 9.0 X 10 ^9/L children

162
Q

Most changes in lymphocyte count are due to changes in numbers of (blank), which normally acounts fo 60-80% of peripheral blood lymphocytes.

A

T lymphocytes

163
Q

What cells are the majority of the circulating cells in the lymph?

A

CD4+ T helper cells

164
Q 
What are these:
Acute infectious lymphocytosis
Persistent polyclonal B cell lymphocytosis
Infectious mononucleosis (EBV)
Bordatella pertussis infection
Cytomegalovirus infection (CMV)
Toxoplasmosis
Retrovirus infection (HTLV-1)
lymphocytic leukemias
A

absolute lymphocytosis with leukocytosis

165
Q

What is this:

  • contagious, mainly occuring in children
  • caused by coxsackie virus A, B6, ehovirus, adenovirus
  • 12-21 day incubation
A

Acute infectious lymphocytosis

166
Q

How long does acute infectious lymphocytosis last?

A

3-5 weeks

167
Q 
What is this:
happens in a lot of female smokers
postsplenectomy
uncommon event
should raise suspicious for a chronic leukemia
diagnosis-> use flow cytometry
A

Persistent polyclonal B cell lymphocytosis

168
Q

What is this:
Self-limited, usually unnoticed (3-5 wk incubation)
Virus gains entry via cd21 (receptor), infects oropharyngeal mucosa and lymphoid tissue
Sore throat, malaise, lymphadenopathy, splenomegaly
WBC 12-25 x 109/L
Atypical lymphocytosis (NOT unique to this disease)
Positive heterophil antibody
INFECTS B CELLS NOT MONOCYTES

A

Infectious mononuleosis (monomer)

169
Q

How does infectious mononucleosis infect B cells?

A

virus gains entry via CD21 (receptor),

170
Q

What kind of antibody will you find in infectious mononuleosis?

A

Positive heterophil antibody

171
Q

What is the definition of lymphocytopenia?

A

absolute lymphocyte count less than 1.0 X 10^9/L in adults and less than 2.0 X 10^9/L in children

172
Q

What are the causes of lymphocytopenia?

A 
Destructive
Debilitative
Infectious
AIDS
Congenital Immunodeficiency
Abnormal Lymph Circ
173
Q

What are some destructive causes of lymphocytopenia?

A

radiation, chemo, steroids

174
Q

What are some debilitative causes of lymphocytopenia?

A

starvation, aplastic anemia, cancer, renal failure CV

175
Q

What are some infectious causes of lymphocytopenia?

A

viral hep, influenza, TB

176
Q

What are the congenital causes for lymphocytopenia?

A

wiskott-aldrich syndrome

177
Q

What abnormalities in lymph circulation can cause lymphocytopenia?

A

Intestinal lypmhangiectasia, thoracic duct drainage/rupture, CHF

178
Q

What is the most severe immunodeficiency?

A

severe combined immunodeficiency syndrome

179
Q

What is severe combined immunodeficiency syndrome?

A

75% of males affected
Both T and B lymphoid systems deficient
recurrent infections, failure to thrive

180
Q

What are the characteristics of the X-linked (Xq13) form of SCIS?

A

absent to severely reduced T cells, thymic hypoplasia

181
Q

What are the characteristics of the autosomal form of SCIS?

A

severe deficiency of T and B cells (ADA deficiency)

182
Q

How do you treat SCIS?

A

bone marrow transplant

183
Q

What is this:
X-linked recessive inheritance (Xp11.3-Xp11.22)
Eczema, thrombocytopenia, and immunodeficiency
Increased risk of secondary neoplasm
Progressive decrease in thymus-dependent immunity
Absent antibodies to blood group antigens
Abnormal antibody production by B cells
No mitogenic response to CD43
May present with abnormal bleeding in neonatal period

A

Wiskott-Aldrich Syndrome

184
Q

In Wiskott-Aldrich syndrome, there is not mitogenic response to (blank)

A

cd43

185
Q

In wiskott-aldrich syndrome, there is abnormal (blanK) production by B cells

A

antibody

186
Q

What can wiskott aldrich present as in the neonatal period?

A

abnormal bleeding

187
Q 
What is this:
absence or hypoplasia of thymus
hypoparathyroidism
congenital heart defects
dysmorphic facies
hypocalcemia
NORMAL B CELL function
A

DiGeorge Syndrome

188
Q

WHat is the pathogenesis of DiGeorge syndrome?

A

del(22)(q11.2)

189
Q

How can you tell someone has DiGeorge syndrome by looking at them?

A

dysmorphic faces

190
Q

What is this:
Frequent respiratory and skin infections
Xq21.3-22
Block in B cell maturation at pre-B cell stage due to failure of variable and constant regions of IgM to connect
Decreased B lymphocytes and absent plasma cells
Marked decrease in serum immunoglobulins

A

X-link agammaglobulinemia

191
Q

How do you treat x-linked agammaglobulinemia?

A

gammaglobulin

192
Q

What is this:
autosomal recessive inheritance
Progressive ataxia, immune dysfunction, increased risk of malignancy
Defects in cell-mediated immunity with thymic hypoplasia or dysplasia

A

Hereditary ataxia-telangiectasia

193
Q

How do you diagnose hereditary ataxia-telengiectasia?

A

increased chromosome breakage, t(14;14)

(this gene is what helps repair DNA :(

Block 7 Week 1 (10 decks)

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