Hematologic Flashcards
(110 cards)
1
Q
Most important diagnostic test when evaluating anemia?
A
CBC
2
Q
Life span of erythrocyte (RBC): ___ days
A
100-120 days
3
Q
Primary responsibility of erythrocyte (RBC) is ____? Do mature erythrocytes have a nucleus?
A
oxygenation
no
4
Q
What’s the main type of WBC (60-70%)?
A
Neutrophils
Chief phagocytes in early inflammation;
Bands (immature ones) and Segs
mature ones
5
Q
Do platelets have nucleus or DNA? What’s the life span and what are they removed by? What are they formed from? They are essential for blood ____.
A
NO nucleus or DNA
life span: 10 days, removed by spleen
formed from megakaryocytes
Essential for blood coagulation and control of
bleeding
6
Q
Hematopoiesis occurs in ___ and ___ of fetus; only in ____ after birth
A
liver and spleen of fetus
bone marrow after birth
7
Q
Stimulation of undifferentiated cells –> differentiated cells
Can increase to replenish destroyed cells or
during infection
A
Hematopoiesis
8
Q
9 components of CBC
A
WBC RBC Hgb Hct MCV RDW MCH MCHC Platelets
9
Q
Stimulates hematopoietic stem cells to
differentiate into proerythroblasts
Can also induce cells to pass through
differentiation and maturation more quickly
A
Erythropoietin
10
Q
Retic count marker of ____ function
A
bone marrow
11
Q
What is the WBC in the CBC, and what does it tell us?
A
Measure of # of leukocytes in volume
Tells us: Infection, bone marrow suppression
12
Q
What is the Hgb in the CBC, and what does it tell us?
A
Measure of the component of
RBC that binds O2, g/dL
Tells us: Oxygen carrying capacity
13
Q
What is the Hct in the CBC?
A
Fractional volume of whole blood occupied by RBCs; %
14
Q
What is the MCV in the CBC, and what does it tell us?
A
Indicated the average size of the RBC
Tells us: Classify anemia as microcytic, normocytic or macrocytic
15
Q
What is the RDW in the CBC, and what does it tell us?
A
Measure of variability in RBC size in sample
Tells us: Elevated means greater cell size
variability; increases w/nutritional anemias
16
Q
What is the MCH in the CBC, and what does it tell us?
A
Measures average weight of hemoglobin per RBC
Usually follows the pattern of the
MCV
17
Q
What is the MCHC in the CBC, and what does it tell us?
A
Concentration of hgb in RBC; Calculated value of Hgb/Hct
Classify anemia as hypochromic, normochromic, hyperchromic
18
Q
What are the neutrophils in the Diff and what can it tell us?
A
1st line of defense in infection
Bacterial infection,
neutropenia; bands, stabs
19
Q
What are the Lymphocytes in the Diff and what can it tell us?
A
B and T cells produced in lymphatic system
Acute viral or chronic
bacterial infection
20
Q
What are the Monocytes in the Diff and what can it tell us?
A
2nd line of defense in infection
Viral infection
21
Q
What are the Eosinophils in the Diff and what can it tell us?
A
Commonly produced in
response to allergic infxn
Allergic disorders, parasitic infections
22
Q
What are the Basophils in the Diff and what can it tell us?
A
Responsible for histamine
release
Systemic allergic reaction
23
Q
The reticulocyte count is a measure of the ___ response to anemia
A
bone marrow
24
Q
After newborn period, normal retic count is ___-___%
A
0.5-1.5%
25
Definition of anemia
Hemoglobin and/or
hematocrit at or below
2.5th percentile for
age, race, sex
26
Normal nadir for physiologic anemia from birth-3 months occurs at ____ weeks
6-9
27
From 3-6 months, anemia is likely ____
hemoglobinopathy
28
From 6 months-teens, ___ causes are the more likely cause of anemia
acquired (iron-deficiency anemia)
29
Screen all children at ___ months for IDA
12 months
30
Pathologic anemia during birth-3 month period definition (4 components):
- HgB <13.5 in 1st month
- Hgb <9 otherwise
- signs of hemolysis
- sx of anemia
31
3 main examples of Microcytic anemia
Iron Deficiency Anemia
Thalassemia
Anemia of chronic
disease
32
3 main examples of normal anemia
Sickle cell disease
G6PD
Hemolytic anemia
33
2 main examples of microcytic anemia
Vitamin B12 or
folate deficiency
Congenital aplasia
34
Definition of iron-deficiency anemia
Insufficient iron to maintain normal function
such that iron stores are reduced
Hgb >2 SDs below normal as a consequence
of iron deficiency
35
risk factors for iron-deficiency anemia
- Low SES
- Prematurity, low birth weight
- Lead exposure
- Exclusive BF >4 mo w/o iron supplementation
- Weaning to whole milk early
- Feeding problems
- Poor growth
- Inadequate nutrition
36
tx for iron deficiency anemia
Iron therapy 6 mg/kg/day divided TID
37
Anemia of Chronic Disease often have Hb in range of ___-____
8-12
38
Anemia of Chronic Disease Likely due to:
inflammatory cytokines that inhibit erythryopoeitin
39
3 components of Diagnosis for Anemia of Chronic Disease
low Hb, microcytosis, low retic count
40
Tx for Anemia of Chronic Disease
correct underlying disease
41
* Group of inherited disorders of Hgb synthesis
| * Autosomal recessive
Thalassemias
42
Think of these if microcytic anemia not responsive to
| iron therapy?
Thalassemias
43
Thalassemias not typically symptomatic until __ months of age
6
44
Thalassemias Pathophysiology
– Alpha thalassemia: ___ genes control alpha globin
synthesis
– Beta thalassemia: ___ gene controls beta globin
synthesis
alpha: 4
beta: 1
45
α-Thalassemia predominantly affects what ethnicities?
black, Mediterranean, Middle Eastern,
| Chinese, or SE Asian descent
46
What is the most fatal α-Thalassemia?
α-thalassemia major
47
Which is more common, α-Thalassemia or β-Thalassemia?
β-Thalassemia
48
β-Thalassemia predominantly affects what ethnicities?
black, Mediterranean, Middle Eastern, or Asian
| descent
49
Most severe type of β-Thalassemia? Is it symptomatic at birth?
β-thalassemia major/Cooley’s anemia
NO asymptomatic
at birth
50
α or β trait Thalassemias Clinical Manifestations (2)
mild microcytic anemia,
| asymptomatic
51
Hb H: Thalassemias Clinical Manifestations (4)
mild hemolytic anemia, jaundice, HSM,
| gallstones
52
β-thalassemia intermedia: Clinical Manifestations (2)
moderate hemolytic anemia (Hb >7 g/dL), splenomegaly
53
β-thalassemia major Clinical Manifestations (3)
Massive HSM, growth failure, bony deformities
54
Screening for Thalassemias?
newborn screening
55
α or β trait Thalassemias tx?
none
56
HbH Thalassemia tx?
folic acid supplemenation
57
Beta-thalassemia major tx>
Transfusion therapy (Goal: Hb 9-10 g/dL pre-transfusion)
Iron chelation therapy
58
What is considered the curative tx for Thalassemia?
stem cell transplant
59
Example plan for alpha-thalassemia minor/trait (3 components)
Refer to hematology, monitor, family
| counseling and testing
60
G6PD Pathophysiology: enzyme instability...older red cell instability means less protection against ____
oxidant stress
61
G6PD is __linked recessive. Most common red cell enzyme defect (African, Mediterranean, Asian)
X
62
Clinical Manifestations of G6PD - neonatal? in older children? with oxidant stress?
Neonatal jaundice
* Older children- asymptomatic until oxidant stress
* With oxidant stress- dark urine, Pallor, jaundice, N/V, tachycardia
63
G6PD: Medication Avoidance. Important to avoid oxidative stressors, which includes ____, ____, and ____ (meds/foods)
Note that ___ and ___ can also be oxidative stressors
sulfonamides, antimalarials, fava beans
fever and tachycardia
64
Tx for G6PD
– Avoid triggers
| – No fava beans
65
Diagnosis for G6PD
– CBC, retic count and smear abnormal only with
oxidant stress
– Confirmation: decreased levels of G6PD in erythrocytes
66
• Characterized by Hb S
• Substitution of valine for glutamic acid on surface of hemoglobin S molecule
– Deoxygenation and dehydration --> solidifying and stretching into long, rope-like fiber
– Causes classic crescent shape and decreased RBC
deformability
– Log jam effect
Sickle Cell disease
67
Is sickle cell part of universal newborn screening?
yes
68
Clinical manifestations of sickle cell typically appear by ___ mo. when fetal hemoglobin disappears
6-12
69
2 main clinical manifestations of sickle cell
Vasoocclusion and hemolysis
– Vasoocclusion: recurrent painful episodes + organ
complications
– Hemolysis: chronic anemia + gallstones
70
Common acute complications of sickle cell
```
Vaso-occlusive events
Splenic sequestration
Acute chest syndrome
Stroke
Dactylitis
Aplastic crisis
Priapism
```
71
Prevention of Complications from SCD involves what 4 components?
– Penicillin prophylaxis
– Appropriate, timely immunizations
– Blood transfusions for those at risk of stroke
– Medication therapy: Hydroxyurea
72
Why are SCD patients more at risk for infection? (5)
***functional asplenia, reduced
tissue perfusion, presence of indwelling
catheter, splinting, hypoventilation
73
Infection prevention in SCD patients involves prophylactic ___ <5 y.o. What's the dosage?
penicillin
– Age 3 mo-3 yrs: 125 mg penicillin V PO BID
– Age 3-5: 250 mg penicillin V PO BID
74
Immunizations in Sickle Cell Disease include all routine immunizations PLUS (3)
* Pneumococcus- PCV13 + PPSV23
* Meningococcus
* Influenza
75
– 90% of SCD patients have experienced _____ by age 6
Vasoocclusive Pain
76
Tx for Vasoocclusive Pain? What is it caused by? Is it a diagnosis of exclusion?
```
Treatment: Rapid initiation of analgesics
• Opioids (Morphine or Dilaudid)
• Hydration
• Physical Therapy
• Psychologic
```
Caused by log jam effect
Y Diagnosis of exclusion
77
Dactylitis is a type of ____ pain in SCD patients. Occurs in ___% of patients by 2 y.o. Clinical manifestations of Dactylitis? Tx?
Vasocculsive
40%
– Tender, erythematous,
edematous hands or feet
Tx:– Analgesics
– IVF
78
Splenic Sequestration is most common in SCD patients under age ___. How is diagnosis made? What's the treatment?
2
Diagnosis- enlarged spleen, low H and H, high retic ct
Treatment
• IV fluids
• PRBC transfusion
• Chronic transfusion? Splenectomy?
79
Acute Chest Syndrome incidence is highest in SCD patients ages ____. ___% cause unknown, ___% infection
2-5 y.o.
45% cause unknown, 30%: infection
80
Peak incidence of stroke in SCD patients? Treatment and prevention of stroke in sickle cell patients?
• 10% risk in 1st 20 yrs of life; peak incidence:
4-8 y.o.
• Treatment: RBC transfusion
• Prevention: transcranial doppler 2-16 y.o.
81
30% of boys with SCD will experience ____ by 15 y.o.
priapism
82
Clinical manifestations and tx for priapism?
• Clinical manifestations: prolonged, painful
erection of penis (>4 hours) or stuttering course
(cluster of episodes)
```
Treatment
– Analgesia
– Reduction of engorgement
• Pseudephedrine
• Aspiration and irrigation
```
83
Aplastic Anemia in SCD patients is typically secondary to ____. Mostly what?
secondary to infection
Mostly parvovirus B19
84
Chronic, compensated hemolytic anemia is known as? Usual range __-___ g/dL; many function at 5-6
Chronic anemia
6-9
85
Acute anemia definition? How to manage?
• Definition:
– Hgb 2.0 g/dL below baseline
– Hgb <6.0 g/dL if baseline unknown
* Evaluate CBC and reticulocyte count
* Evaluate for etiology requiring urgent care
86
Megaloblastic Anemias - 2 types? common in children?
* Vitamin B12 deficiency
* Folate deficiency
* Combination of the two
* Very rare in children
87
Phases of hemostasis: primary hemostasis
vasoconstriction --> formation of platelet plug
88
Phases of hemostasis: secondary hemostasis
propagation of clotting process --> termination of clotting
89
Disorders of primary hemostasis (4) - Purpuric Disorders
* ITP
* Congenital thrombocytopenias
* Von Willebrand Disease
* Platelet function abnormalities
90
Disorders of secondary hemostasis (3) - Disorders of Coagulation
* Hemophilia A
* Hemophilia B
* Type 3 VWD
91
```
Purpuric Disorders (Primary Hemostasis) such as
• ITP
• Congenital thrombocytopenias
• Von Willebrand Disease
• Platelet function abnormalities
```
Describe the following....
- Petechiae
- Ecchymoses
- Soft tissue hematoma
- Joint hemorrhages
- Delayed bleeding
- Bleeding from superficial cuts
- Family hx of bleeding
- Sex of patient
- Petechiae: Y
- Ecchymoses: Y -small, many scattered
- Soft tissue hematoma: Rare
- Joint hemorrhages: Not usual
- Delayed bleeding: Rare
- Bleeding from superficial cuts: Common, persistent
- Family hx of bleeding: Rare
- Sex of patient: Mostly female
92
Disorders of Coagulation (Secondary hemostasis) such as
• Hemophilia A
• Hemophilia B
• Type 3 VWD
Describe the following....
- Petechiae
- Ecchymoses
- Soft tissue hematoma
- Joint hemorrhages
- Delayed bleeding
- Bleeding from superficial cuts
- Family hx of bleeding
- Sex of patient
- Petechiae: Not usual
- Ecchymoses: Common - 1 large or more
- Soft tissue hematoma: characteristic
- Joint hemorrhages: characteristic *HALLMARK*
- Delayed bleeding: common
- Bleeding from superficial cuts: uncommon
- Family hx of bleeding: common
- Sex of patient: mostly male
93
Initial screening for disorders of primary or secondary hemostasis: (4)
– CBC + peripheral blood smear
– PT/INR
– aPTT
– ?Fibrinogen activity
94
most common serious congenital
| coagulation deficiencies? are they x-linked recessive?
Hemophilia A and B
yes
95
Pathophysiology of hemophilia
decreased levels of FVIII or FIX leads to dysfunctional clotting
96
___ is hallmark of hemophilia
Hemarthrosis
97
Hemophilia diagnosis
– Prolonged PTT, normal PT, INR and thrombin
– Factor VIII or IX activity level
– Genetic testing
98
Most common bleeding disorder among Caucasians? what's the most common type?
Von Willebrand Disease
type 1:70-80%
99
Clinical manifestations of Von Willebrand Disease
• Clinical Manifestations
– Mucocutaneous bleeding
– Post-surgical/traumatic
bleeding
100
Most common cause of thrombocytopenia
| secondary to increased platelet destruction
Immune Thrombocytopenic Purpura
101
Immune Thrombocytopenic Purpura is typically post___ in children
viral
102
Initial clinical manifestations of Immune Thrombocytopenic Purpura?
Sudden onset of petechiae
+/- purpura post viral illness
– Can progress to major hemorrhage from
mucosal sites
103
ITP: Treatment - observation, first line, second line?
– Observation: skin manifestations only
– First line: IVIG, corticosteroids, or WinRho
– Second line (chronic or refractory ITP): rituximab, immunosuppressive drugs, chemotherapy, thrombopoietin agonists, splenectomy?
104
A 2-day old boy has excessive bleeding after circumcision and labs reveal an aPTT time of 85 seconds and a factor VI activity level of <1%. The mother states that no one in the family has bleeding symptoms. Is this patient at risk for spontaneous bleeding in all joints?
yes
105
Most important proteins in plasma (2)
albumin, fibrinogen
106
If someone is dehydrated, ___ can go up - falsely elevated bc plasma volume is decreased so fraction of RBC seems higher than it is
HCT
107
Most important lab in the CBC when it comes to anemia?
MCHC - classifies as hypochromic, normochromic, hyperchromic
108
If hemoglobin is low, there would be ____ Retic count
elevated
109
If Retic is low and hemoglobin is low, you’re wondering
why isn’t the body compensating by making more blood cells?
110
Diagnostic findings consistent with beta-thalassemia intermedia and major are
hypochromia, microcytosis
