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Flashcards in RBC Pathology 1 Deck (127):
1

In RBC development, the ______ is the first source of red cell elements. What week of fetus development?

Yolk sac. 3rd week

2

At what month does the liver become the chief site of red cell formation?

3rd month

3

What does it mean when the baby has a hematopoetic liver at birth? Is this pathologic?

Baby had anemia in utero; Yes it is pathologic

4

Sole source of all blood cells and lymphocyte precursors at birth

Bone marrow

5

In utero, when does hematopoesis switch to the bone marrow?

4th month

6

Which bones are the most hematopoetically active in an 18 year old?

Central bones (vertebrae, ribs, sternum, skull, pelvis)

7

In adults, _______ marrow can be reactivated to red marrow with increased demand for blood cells

Yellow / Fat

8

Fetal hemoglobin diminishes after ______ of age

5-6 months

9

Beta Hemoglobin occurs only after _______ postnatally

6 months

10

Hemoglobin with 4 beta chains

Hgb H (nonfunctional)

11

Hgb with 4 gamma chains

Hemoglobin barts (nonfunctional)

12

Fetal hemoglobin has 2 ____ chains and 2 _____ chians

alpha; gamma

13

Hemoglobin ___ has 2 alpha and 2 beta chains and in normal adults constitutes ___% of Hgb

A; 95%

14

Hemoglobin A2 has 2 ____ chains and 2 ____ chains

alpha; delta

15

_____ is the youngest and largest of all cell types with prominent nucleoli

Blast

16

Progression of red blood cell maturation

Myeloid stem cell --> Pronormoblast --> Basophilic Normoblast --> Polychromatic Normoblast --> Orthochromatic Normoblast --> Polychromatic erythrocyte (reticulocyte) --> Eryrthrocyte

17

In RBC maturation, what is the first cell apparent in the PBS?

reticulocyte (polychromatic erythrocyte)

18

General S/S of anemia

weakness, malaise, easy fatigability, dyspnea on mild exertion

19

PE findings in anemia

Pallor, dizziness & headache, angina, anemic murmur (hemic murmur), koilonychias & brittle nails, oliguria, anuria

20

Normal Hgb levels

12-14 g/dl (F); 13-16 g/dL (M)

21

Normal Hct levels

33-42% (F); 39-48% (M)

22

Normal reticulocyte count

0.5 - 1.5%

23

What the the reticulocyte count measure?

Bone marrow activity

24

Normally, reticulocyte count is (elevated / depressed) in anemia

Elevated

25

Normal MCV; formula for MCV; measure of?

80-94 fl; Hct/RBC count x 10; average volume of RBCs

26

Normal MCHC; formula for MCHC; measure of?

32-36 gm/dL; Hb/Hct x 100; Average concentration of Hgb in a given volume of packed RBCs

27

Normal size of central pallor of RBC

1/3 of the diameter

28

Central pallor >1/3 of the diameter

hypochromic

29

Central pallor <1/3 of the diameter

hyperchromic

30

Change in size of RBC

Anisocytosis

31

Change in shape of RBC

Poikilocytosis

32

Normal MCH; formula of MCH; Measure of?

25-34 pg; Hb/RBC count x 10; average content of Hb per RBC

33

Anemias that are hypochromic, microcytic

IDA, Thalassemia, Sideroblastic

34

Macrocytic anemias

Vitamin B12 and Folic Acid deficiency anemias

35

What is the first response to acute blood loss?

leukocytosis and thrombocytosis

36

Type of anemia in acute blood loss

normocytic, normchromic

37

Type of anemia in chronic blood loss; what does it manifest as?

hypochromic, microcytic; IDA

38

Intrinsic hemolytic anemias

G6PD deficiency, sickle cell, pyruvate kinase deficiency, spherocytosis, thalassemia

39

Extrinsic hemolytic anemias

Malaria, Ab-mediated, lead poisoning, SLE

40

Give the 3 characteristics of hemolytic anemia

1) Accumulation of Hb catabolism
2) Increased erythropoesis
3) Premature destruction of RBCs

41

Anemias of defective DNA synthesis

B12 and Folic acid deficiency

42

Anemias of defective Hb synthesis

IDA, thalassemia

43

Anemias d/t disturbance of proliferation of stem cells

Aplastic anemia, Anemia of renal failure

44

Normal Erythroid:Myeloid ratio in BM

1:4

45

In erythroid hyperplasia, myeloid cells are replaced by?

red cell progenitors

46

3 changes in intravascular hemolytic anemia not found in extravascular hemolytic anemia

Hemoglobinuria, Hemoglobinemia, Decrease in hepatoglobin

47

Why is there a decrease in hepatoglobin in intravascular hemolytic anemia?

Free Hb in blood combine with hepatoglobin, get transported to the RES system in the spleen

48

Causes on intravascular hemolytic anemia

Mechanical injury, infection (malaria), complement fixation, exogenous toxic factors

49

Splenomegaly is not seen in intravascular hemolysis (T / F)

True

50

Type of bilirubin in ALL types of hemolytic anemia

Unconjugated

51

Main characteristics of Extravascular hemolytic anemia

Anemia, Jaundice, Splenomegaly

52

Cause of splenomegaly in extravascular hemolytic anemia

splenic trapping of RBCs, usually caused by alterations that render the red cell less deformable
**Splenomegaly does not occur in all cases**

53

Defective membrane proteins in spherocytosis:

Spectrin
Actin
Ankyrin

54

PBS of spherocytosis

Small, dark-staining spheroidal RBCs with loss of central pallor

55

Main sign in spherocytosis

Splenomegaly and Jaundice

56

How is spherocytosis diagnosed?

Family history, hematologic findings, laboratory evidence

57

The course of spherocytosis is stable unless the following happens

1) Aplastic crisis
2) Hemolytic crisis

58

The ONLY red cell disorder that is HYPERCHROMIC and MICROCYTIC

Spherocytosis

59

In spherocytosis, chronic hemolytic anemia happens when...

the bone marrow cannot compensate

60

What can be done in chronic hemolytic anemia d/t spherocytosis?

exchange transfusion

61

Pathogenesis of G6PD deficiency

Abnormalities in HMP shunt or glutathione metabolism --> reduced ability of cells to protect against oxidative injuries --> hemolysis

62

In G6PD deficiency, increasing amounts of Hb+3 causes production of?

Heinz bodies (denatured globin chains)

63

How are bite cells produced?

Macrophages plucking out the Heinz bodies

64

PBS: normocytic, normochromic, with increased reticulocytes, Heinz bodies and bite cells

G6PD deficiency anemia

65

Precipiating factors for G6PD deficiency anemia

Infections (most common), Oxidant drugs (anitmalarials, sulfonamides, nitrofurantoins), Flava beans, Stress

66

G6PD deficiency variant wherein only mature RBC are lacking the enzyme

G6PD -

67

G6PD deficiency variant wherein all RBCs are lacking the enzyme

G6PD Mediterranean

68

During the recovery phase of G6PD deficiency there is...

Reticulocytosis

69

S/S of G6PD deficiency

1) Primarily intravascular hemolysis 2-3 days after exposure to oxidative stress
2) Self-limited
3) Increased reticulocyte count during recovery
4) Mild extravascular hemolysis
5) NO signs of chronic hemolysis

70

HbS formation is from the substitution of _______ for valine at the ____ position of the beta chain

glutamine; 6th

71

Pathogenesis of sickle cell anemia

HbS aggregation in presence of deoxygenation --> distortion --> sickling of RBC (Holly leaf appearance) --> (1) Chronic hemolysis in the spleen (2) Small vessel occlusion (3) Tissue damage

72

Presence of ____ and ____ in liquid chromatography is indicative of sickle cell trait

HbA and HbS

73

Presence of HbA and HbS in liquid chromatography is indicative of sickle cell Anemia (T/F)

False (only HbS)

74

The PBS in sickle cell anemia shows

1) normocytic, normochromic RBCs
2) (+) sickle cells
3) (+) normoblasts

75

In infants with sickle cell anemia, the spleen is _______

Enlarged

76

In adults with sickle cell anemia, the spleen is _______ and this is d/t _________

Small; Autosplenectomy

77

Lab abnormalities in sickle cell anemia

Decreased Osmotic fragility and ESR

78

Lab abnormalities in sickle cell trait

None

79

Pain and infarction in different organs cause by plugging up of capillaries is seen in sickle cell (Anemia / Trait)

Anemia

80

4 Factors that affect sickling

1) Amount of interaction of HbS
2) Increase in MCHC & intracellular dehydration
3) Decrease in pH
4) Length of exposure to decreased O2

81

Sickle cell anemia cannot be seen until the infant is at least _______ of age d/t the increased presence of ______

6 months; HbF

82

Evidence of chronic hemolysis in sickle cell anemia

1) Increased RBC destruction
2) Increased erythropoesis (extramedullary hematopoesis, hyperplastic bone marrow, increased retic and normoblasts)

83

In sickle cell anemia, the skull x-ray show...

"Crew haircut" appearance of the skull

84

Critical episodes that can complicate sickle cell anemia

1) Vaso-occlusive crisis
2) Aplastic crisis
3) Sequestration crisis

85

Sickle cell anemia: Sequestration crisis pathogenesis

Massive RBC destruction --> sudden splenomegaly --> hypovolemia --> shock

86

In children, vaso-occlusive crisis of sickle cell anemia typically affects:

Bone

87

Triggers of vaso-occlusive crisis

Infection, dehydration, acidosis

88

Most common sites of vaso-occlusive crisis

bone, lungs, liver, brain, spleen, penis

89

What is the pathology of Thalassemias?

Defective globin synthesis

90

Type of thalssemia with defective beta globin chain

Beta thalassemia

91

Beta thalassemia minor is also known as

Cooley's trait

92

Critical episodes that can complicate sickle cell anemia

1) Vaso-occlusive crisis
2) Aplastic crisis
3) Sequestration crisis

93

3 types of beta thalassemia

minor, intermedia, major

94

Sickle cell anemia: Sequestration crisis pathogenesis

Massive RBC destruction --> sudden splenomegaly --> hypovolemia --> shock

95

Beta thalassemia major is also known as

Cooley's anemia

96

In sickle cell anemia, what is a vaso-occlusive crisis?

Multiple simultaneous painful thrombosis in different organs, most commonly in bone (in children)

97

Necessary for survival in beta thalassemia major:

Blood transfusion

98

Triggers of vaso-occlusive crisis

Infection, dehydration, acidosis

99

Type of thalassemia with defective alpha chain

alpha thalassemia

100

Most common sites of vaso-occlusive crisis

bone, lungs, liver, brain, spleen, penis

101

Type of Hb found in hydrops fetalis

Hb Barts (4 gamma)

102

Most common cause of mutation in beta thalassemia

Splicing mutations

103

4 classifications of alpha thalassemia and their cause:

1) Silent carrier state (single gene deletion)
2) Alpha thalassemia trait (deletion of 2 genes from a single chromosome)
3) Hemoglobin H disease (Deletion of 3 genes)
4) Hydrops fetalis (deletion of all 4 alpha globin genes)

104

S/Sx of Thalassemia:

Massive erythroid hyperplasia
Skull X-ray "crewcut" appearance
Facial alteration
Hemosiderosis
Massive hepatosplenomegaly
Jaundice

105

PBS of Thalassemia

Hypochromic, microcytic anemia
Marked anisocytosis
Target cells
Basophilic stippling

106

Laboratory diagnosis of thalassemia

1) Hb electrophoesis
2) PBS
3) Evidences of BM hyperplasia (inc retic)
4) Iron overload (increased siderocytes, sideroblasts)

107

Iron administration is therapeutic in ___ but fatal in ___:

IDA; Thalassemia

108

______ provides a sensitive means for detecting red cells that are deficient in GPI-linked proteins

flow cytometry

109

What is the only hemolytic anemia that is caused by an ACQUIRED gene defect?

Paroxysmal nocturnal hemoglobinuria

110

In PNH, why is there a tendency for RBCs to lyse at night?

d/t decreased pH durin sleep = increased complement activity

111

Other S/S of thalassemia (aside from facial and skull)

Expansion of red marrow, hemosiderosis / hemochromatosis, Massive splenomegaly, Jaundice

112

How is PNH diagnosed? What is the only cure?

Flow cytometry; BM transplant

113

Benefit and drawback of C5a monoclonal Ab infusion in PNH patients

Reduces hemolysis BUT increased risk for fatal meningococcal infections

114

3 classifications of Immuno-hemolytic anemias

1) Warm Ab IHA
2) Cold agglutin IHA
3) Cold hemolysis IHA

115

Warm Ab IHA is Ig_ that is active at __ C and __ fix complement:

IgG; 37 degrees; does not fix complement

116

Secondary causes of warm Ab IHA

drugs, lymphomas & leukemias, autoimmune disorders

117

Cold agglutin IHA is Ig_ active from ___ C and ___ fix complement:

IgM; 0-30 degrees; does fix complement

118

Most common causes of cold agglutin IHA

Mycoplasma pneumonia
Infectious mononucleosis

119

Cold hemolysin IHA is Ig_ active at ___ C and ___ fix complement:

IgG; low temperature; does fix complement

120

Common causes of cold hemolysin IHA

mycoplasma pneumonia, measles, mumps, other viral flu-like syndromes

121

Many ___ cells are present in microangiopathic hemolytic anemia with vessel narrowing due to ___:

Schistocytes; fibrin deposition

122

Common pathogenic feature in mircoangiopathic hemolytic anemia

microvascular lesion

123

Common pathogenic feature in mircoangiopathic hemolytic anemia

microvascular lesion

124

Major diagnostic criterion for immuno-hemolytic anemias:

Coomb's antiglobin test

125

In PNH, abnormal __ gene leads to defects in these proteins __:

PIGA; CD 59, CD 55, C8 protein

126

S/Sx of PNH:

Intravascular hemolysis
Episodic venous thrombosis
Acute myeloid leukemia

127

A hemolytic anemia resulting from trauma to RBC d/t narrowing or ostruction in microvaculature

mircoangiopathic hemolytic anemia