Non-Neoplastic WBC Abnormalities - Usera Flashcards
1
Q
what are the four methods to measure WBCs in the lab?
A
- automated hem. analyzers
- bone marrow aspirate and biopsy
- flow cytometry
- peripheral smear
2
Q
what are the three general factors that affect neutrophil concn in the blood?
A
- bone marrow production and release
- rate of egress to tissue and survival time in the blood
- ratio of marginating to circulating neutrophils in peripheral blood
3
Q
What is the number at which someone has neutrophilia?
A
ANC> 7.0 x 10^9/L
4
Q
T/F: Neutrophilia can be in response to a physiologic, not just pathologic process
A
true
5
Q
How long does it take for marginated neutrophils to return the to circulating pool?
A
20-30 minutes
6
Q
Stress, steroids, epinephrine, and what cytokine can move neutrophils from margination into the ciruclating pool?
A
IL6
7
Q
How long does it take for neutrophils from the marrow storage pool to be released into the blood?
A
4-5 hours; released by IL6
8
Q
How long does it take to increase the marrow mitotic pool?
A
days
9
Q
How does epi increase the neutrophil count?
A
causes them to demarginate
10
Q
What are six major categories of causes of neutrophilia?
A
DIPINN Drugs Inflammation Physiologic Infection Neoplastic Necrotic
11
Q
Vascular collagen and vasculitis are (blank) causes of neutrophilia
A
inlfammatory
12
Q
Bacteria, fungi, parasites, viruses, and spirochetes are (blank) causes of neutrophilia
A
infectiious
13
Q
stress, exercise, and pregnancy are (blank) causes of neutrophilia
A
physiologic
14
Q
Carcinoma, sarcomas, and MPDs are (blank) causes of neutrophilia
A
neoplastic
15
Q
burns, ischemic necrosis, and tissue damage are (blank) causes of neutrophilia
A
necrotic
16
Q
What is the ANC in REACTIVE neutrophilia?
A
<30
17
Q
Reactive neutorphilia shows with a (blank) shift in myeloid maturation to band cells
A
left shift
18
Q
Toxic granulation, Dohle bodies, and vacuolization are morphologic changes of neutrophils seen in (blank)
A
left shifting during reactive neutrophilia
19
Q
T/F: the leukemoid reaction is benign
A
true
20
Q
The ANC in the leukemoid reaction is usually (blank) and has many circulating (blank) leuokcyte precursos
A
> 50 with many immature leukocyte precursors
21
Q
What type of cell is ocassionally present during a leukemoid reaction
A
blast celss
22
Q
What disease do you need to exclude when the leukemoid reaction is on you DDx?
A
CML
23
Q
The (leukemoid/leukoerythroblastic) rxn has the presence of nucleated RBCS
A
LEB
24
Q
the LEB reaction has a left shift in (blank) maturation
A
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: <1.3
27
What is the ANC to define agranulocytosis?
<0.5
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?
```
iCHOAD
intrinsic defects
Cachexia
Hematologic disorders
OVERWHELMING infection
Autoimmune
Drugz
```
32
What are the 4 intrinsic defects that cause neutropenia?
F*CCK!!
1. Fanconi's
2. Kostmann's
3. Cyclic neutropenia
4. 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 hypoplasia
Heterogenous presentation due to csome instability
Presents 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<200/uL
Variable modes of inheritance
Early myeloid precursors in the marrow that do not mature,
ELA2 and HAX1 implicated
```
Kostmann's Syndrome,
39
What is the medical name for Kostmann's Syndrome?
Infantile genetic agranulocytosis
40
```
Name the disease:
Presents in infancy or childhood
Auto. Dom with variable exrpression
21-30 DAY PERIODICITY
ANC
```
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: blue
CGD: 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 test
MPO: 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 syndrome
2. ACTH
3. Acute stress/ Epi
4. Inflammation
5. Prostaglandins
6. 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?
<0.2
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 Disease
Neimann-Pick Disease
Tay-Sachs Disease
Fabry Disease
Wolman’s Disease
Tangier Disease
are 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: >4.0
kids: >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: <2.0
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 hypoplasia
Autosomal: 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 Agammaglobulinema
causes 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 Agammaglobulinema
| presents 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)
