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N283B/C Patho > Hematologic > Flashcards

Hematologic Flashcards

1
Q

Most important diagnostic test when evaluating anemia?

A

CBC

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2
Q

Life span of erythrocyte (RBC): ___ days

A

100-120 days

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3
Q

Primary responsibility of erythrocyte (RBC) is ____? Do mature erythrocytes have a nucleus?

A

oxygenation

no

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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

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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

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6
Q

Hematopoiesis occurs in ___ and ___ of fetus; only in ____ after birth

A

liver and spleen of fetus

bone marrow after birth

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7
Q

Stimulation of undifferentiated cells –> differentiated cells

Can increase to replenish destroyed cells or
during infection

A

Hematopoiesis

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8
Q

9 components of CBC

A 
WBC
RBC
Hgb
Hct
MCV
RDW
MCH
MCHC
Platelets
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9
Q

Stimulates hematopoietic stem cells to
differentiate into proerythroblasts

Can also induce cells to pass through
differentiation and maturation more quickly

A

Erythropoietin

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10
Q

Retic count marker of ____ function

A

bone marrow

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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

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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

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13
Q

What is the Hct in the CBC?

A

Fractional volume of whole blood occupied by RBCs; %

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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

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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

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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

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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

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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

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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

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20
Q

What are the Monocytes in the Diff and what can it tell us?

A

2nd line of defense in infection

Viral infection

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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

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22
Q

What are the Basophils in the Diff and what can it tell us?

A

Responsible for histamine
release

Systemic allergic reaction

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23
Q

The reticulocyte count is a measure of the ___ response to anemia

A

bone marrow

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24
Q

After newborn period, normal retic count is ___-___%

A

0.5-1.5%

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25
Q

Definition of anemia

A

Hemoglobin and/or
hematocrit at or below
2.5th percentile for
age, race, sex

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26
Q

Normal nadir for physiologic anemia from birth-3 months occurs at ____ weeks

A

6-9

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27
Q

From 3-6 months, anemia is likely ____

A

hemoglobinopathy

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28
Q

From 6 months-teens, ___ causes are the more likely cause of anemia

A

acquired (iron-deficiency anemia)

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29
Q

Screen all children at ___ months for IDA

A

12 months

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30
Q

Pathologic anemia during birth-3 month period definition (4 components):

A
  • HgB <13.5 in 1st month
  • Hgb <9 otherwise
  • signs of hemolysis
  • sx of anemia
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31
Q

3 main examples of Microcytic anemia

A

Iron Deficiency Anemia

Thalassemia

Anemia of chronic
disease

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32
Q

3 main examples of normal anemia

A

Sickle cell disease

G6PD

Hemolytic anemia

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33
Q

2 main examples of microcytic anemia

A

Vitamin B12 or
folate deficiency

Congenital aplasia

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34
Q

Definition of iron-deficiency anemia

A

Insufficient iron to maintain normal function
such that iron stores are reduced

Hgb >2 SDs below normal as a consequence
of iron deficiency

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35
Q

risk factors for iron-deficiency anemia

A
  • 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
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36
Q

tx for iron deficiency anemia

A

Iron therapy 6 mg/kg/day divided TID

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37
Q

Anemia of Chronic Disease often have Hb in range of ___-____

A

8-12

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38
Q

Anemia of Chronic Disease Likely due to:

A

inflammatory cytokines that inhibit erythryopoeitin

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39
Q

3 components of Diagnosis for Anemia of Chronic Disease

A

low Hb, microcytosis, low retic count

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40
Q

Tx for Anemia of Chronic Disease

A

correct underlying disease

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41
Q
  • Group of inherited disorders of Hgb synthesis

* Autosomal recessive

A

Thalassemias

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42
Q

Think of these if microcytic anemia not responsive to

iron therapy?

A

Thalassemias

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43
Q

Thalassemias not typically symptomatic until __ months of age

A

6

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44
Q

Thalassemias Pathophysiology
– Alpha thalassemia: ___ genes control alpha globin
synthesis
– Beta thalassemia: ___ gene controls beta globin
synthesis

A

alpha: 4
beta: 1

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45
Q

α-Thalassemia predominantly affects what ethnicities?

A

black, Mediterranean, Middle Eastern,

Chinese, or SE Asian descent

46
Q

What is the most fatal α-Thalassemia?

A

α-thalassemia major

47
Q

Which is more common, α-Thalassemia or β-Thalassemia?

A

β-Thalassemia

48
Q

β-Thalassemia predominantly affects what ethnicities?

A

black, Mediterranean, Middle Eastern, or Asian

descent

49
Q

Most severe type of β-Thalassemia? Is it symptomatic at birth?

A

β-thalassemia major/Cooley’s anemia

NO asymptomatic
at birth

50
Q

α or β trait Thalassemias Clinical Manifestations (2)

A

mild microcytic anemia,

asymptomatic

51
Q

Hb H: Thalassemias Clinical Manifestations (4)

A

mild hemolytic anemia, jaundice, HSM,

gallstones

52
Q

β-thalassemia intermedia: Clinical Manifestations (2)

A

moderate hemolytic anemia (Hb >7 g/dL), splenomegaly

53
Q

β-thalassemia major Clinical Manifestations (3)

A

Massive HSM, growth failure, bony deformities

54
Q

Screening for Thalassemias?

A

newborn screening

55
Q

α or β trait Thalassemias tx?

A

none

56
Q

HbH Thalassemia tx?

A

folic acid supplemenation

57
Q

Beta-thalassemia major tx>

A

Transfusion therapy (Goal: Hb 9-10 g/dL pre-transfusion)

Iron chelation therapy

58
Q

What is considered the curative tx for Thalassemia?

A

stem cell transplant

59
Q

Example plan for alpha-thalassemia minor/trait (3 components)

A

Refer to hematology, monitor, family

counseling and testing

60
Q

G6PD Pathophysiology: enzyme instability…older red cell instability means less protection against ____

A

oxidant stress

61
Q

G6PD is __linked recessive. Most common red cell enzyme defect (African, Mediterranean, Asian)

A

X

62
Q

Clinical Manifestations of G6PD - neonatal? in older children? with oxidant stress?

A

Neonatal jaundice

  • Older children- asymptomatic until oxidant stress
  • With oxidant stress- dark urine, Pallor, jaundice, N/V, tachycardia
63
Q

G6PD: Medication Avoidance. Important to avoid oxidative stressors, which includes ____, ____, and ____ (meds/foods)

Note that ___ and ___ can also be oxidative stressors

A

sulfonamides, antimalarials, fava beans

fever and tachycardia

64
Q

Tx for G6PD

A

– Avoid triggers

– No fava beans

65
Q

Diagnosis for G6PD

A

– CBC, retic count and smear abnormal only with
oxidant stress
– Confirmation: decreased levels of G6PD in erythrocytes

66
Q

• 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

A

Sickle Cell disease

67
Q

Is sickle cell part of universal newborn screening?

A

yes

68
Q

Clinical manifestations of sickle cell typically appear by ___ mo. when fetal hemoglobin disappears

A

6-12

69
Q

2 main clinical manifestations of sickle cell

A

Vasoocclusion and hemolysis
– Vasoocclusion: recurrent painful episodes + organ
complications
– Hemolysis: chronic anemia + gallstones

70
Q

Common acute complications of sickle cell

A 
Vaso-occlusive events
Splenic sequestration
Acute chest syndrome
Stroke
Dactylitis
Aplastic crisis
Priapism
71
Q

Prevention of Complications from SCD involves what 4 components?

A

– Penicillin prophylaxis
– Appropriate, timely immunizations
– Blood transfusions for those at risk of stroke
– Medication therapy: Hydroxyurea

72
Q

Why are SCD patients more at risk for infection? (5)

A

***functional asplenia, reduced
tissue perfusion, presence of indwelling
catheter, splinting, hypoventilation

73
Q

Infection prevention in SCD patients involves prophylactic ___ <5 y.o. What’s the dosage?

A

penicillin

– Age 3 mo-3 yrs: 125 mg penicillin V PO BID
– Age 3-5: 250 mg penicillin V PO BID

74
Q

Immunizations in Sickle Cell Disease include all routine immunizations PLUS (3)

A
  • Pneumococcus- PCV13 + PPSV23
  • Meningococcus
  • Influenza
75
Q

– 90% of SCD patients have experienced _____ by age 6

A

Vasoocclusive Pain

76
Q

Tx for Vasoocclusive Pain? What is it caused by? Is it a diagnosis of exclusion?

A 
Treatment: Rapid initiation of analgesics
• Opioids (Morphine or Dilaudid)
• Hydration
• Physical Therapy
• Psychologic

Caused by log jam effect

Y Diagnosis of exclusion

77
Q

Dactylitis is a type of ____ pain in SCD patients. Occurs in ___% of patients by 2 y.o. Clinical manifestations of Dactylitis? Tx?

A

Vasocculsive

40%

– Tender, erythematous,
edematous hands or feet

Tx:– Analgesics
– IVF

78
Q

Splenic Sequestration is most common in SCD patients under age ___. How is diagnosis made? What’s the treatment?

A

2

Diagnosis- enlarged spleen, low H and H, high retic ct

Treatment
• IV fluids
• PRBC transfusion
• Chronic transfusion? Splenectomy?

79
Q

Acute Chest Syndrome incidence is highest in SCD patients ages ____. ___% cause unknown, ___% infection

A

2-5 y.o.

45% cause unknown, 30%: infection

80
Q

Peak incidence of stroke in SCD patients? Treatment and prevention of stroke in sickle cell patients?

A

• 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
Q

30% of boys with SCD will experience ____ by 15 y.o.

A

priapism

82
Q

Clinical manifestations and tx for priapism?

A

• 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
Q

Aplastic Anemia in SCD patients is typically secondary to ____. Mostly what?

A

secondary to infection

Mostly parvovirus B19

84
Q

Chronic, compensated hemolytic anemia is known as? Usual range __-___ g/dL; many function at 5-6

A

Chronic anemia

6-9

85
Q

Acute anemia definition? How to manage?

A

• 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
Q

Megaloblastic Anemias - 2 types? common in children?

A
  • Vitamin B12 deficiency
  • Folate deficiency
  • Combination of the two
  • Very rare in children
87
Q

Phases of hemostasis: primary hemostasis

A

vasoconstriction –> formation of platelet plug

88
Q

Phases of hemostasis: secondary hemostasis

A

propagation of clotting process –> termination of clotting

89
Q

Disorders of primary hemostasis (4) - Purpuric Disorders

A
  • ITP
  • Congenital thrombocytopenias
  • Von Willebrand Disease
  • Platelet function abnormalities
90
Q

Disorders of secondary hemostasis (3) - Disorders of Coagulation

A
  • Hemophilia A
  • Hemophilia B
  • Type 3 VWD
91
Q 
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
A
  • 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
Q

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
A
  • 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
Q

Initial screening for disorders of primary or secondary hemostasis: (4)

A

– CBC + peripheral blood smear
– PT/INR
– aPTT
– ?Fibrinogen activity

94
Q

most common serious congenital

coagulation deficiencies? are they x-linked recessive?

A

Hemophilia A and B

yes

95
Q

Pathophysiology of hemophilia

A

decreased levels of FVIII or FIX leads to dysfunctional clotting

96
Q

___ is hallmark of hemophilia

A

Hemarthrosis

97
Q

Hemophilia diagnosis

A

– Prolonged PTT, normal PT, INR and thrombin
– Factor VIII or IX activity level
– Genetic testing

98
Q

Most common bleeding disorder among Caucasians? what’s the most common type?

A

Von Willebrand Disease

type 1:70-80%

99
Q

Clinical manifestations of Von Willebrand Disease

A

• Clinical Manifestations
– Mucocutaneous bleeding
– Post-surgical/traumatic
bleeding

100
Q

Most common cause of thrombocytopenia

secondary to increased platelet destruction

A

Immune Thrombocytopenic Purpura

101
Q

Immune Thrombocytopenic Purpura is typically post___ in children

A

viral

102
Q

Initial clinical manifestations of Immune Thrombocytopenic Purpura?

A

Sudden onset of petechiae
+/- purpura post viral illness

– Can progress to major hemorrhage from
mucosal sites

103
Q

ITP: Treatment - observation, first line, second line?

A

– Observation: skin manifestations only

– First line: IVIG, corticosteroids, or WinRho

– Second line (chronic or refractory ITP): rituximab, immunosuppressive drugs, chemotherapy, thrombopoietin agonists, splenectomy?

104
Q

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?

A

yes

105
Q

Most important proteins in plasma (2)

A

albumin, fibrinogen

106
Q

If someone is dehydrated, ___ can go up - falsely elevated bc plasma volume is decreased so fraction of RBC seems higher than it is

A

HCT

107
Q

Most important lab in the CBC when it comes to anemia?

A

MCHC - classifies as hypochromic, normochromic, hyperchromic

108
Q

If hemoglobin is low, there would be ____ Retic count

A

elevated

109
Q

If Retic is low and hemoglobin is low, you’re wondering

A

why isn’t the body compensating by making more blood cells?

110
Q

Diagnostic findings consistent with beta-thalassemia intermedia and major are

A

hypochromia, microcytosis

N283B/C Patho (3 decks)

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