RBC Pathology 1 Flashcards
1
Q
In RBC development, the ______ is the first source of red cell elements. What week of fetus development?
A
Yolk sac. 3rd week
2
Q
At what month does the liver become the chief site of red cell formation?
A
3rd month
3
Q
What does it mean when the baby has a hematopoetic liver at birth? Is this pathologic?
A
Baby had anemia in utero; Yes it is pathologic
4
Q
Sole source of all blood cells and lymphocyte precursors at birth
A
Bone marrow
5
Q
In utero, when does hematopoesis switch to the bone marrow?
A
4th month
6
Q
Which bones are the most hematopoetically active in an 18 year old?
A
Central bones (vertebrae, ribs, sternum, skull, pelvis)
7
Q
In adults, _______ marrow can be reactivated to red marrow with increased demand for blood cells
A
Yellow / Fat
8
Q
Fetal hemoglobin diminishes after ______ of age
A
5-6 months
9
Q
Beta Hemoglobin occurs only after _______ postnatally
A
6 months
10
Q
Hemoglobin with 4 beta chains
A
Hgb H (nonfunctional)
11
Q
Hgb with 4 gamma chains
A
Hemoglobin barts (nonfunctional)
12
Q
Fetal hemoglobin has 2 ____ chains and 2 _____ chians
A
alpha; gamma
13
Q
Hemoglobin ___ has 2 alpha and 2 beta chains and in normal adults constitutes ___% of Hgb
A
A; 95%
14
Q
Hemoglobin A2 has 2 ____ chains and 2 ____ chains
A
alpha; delta
15
Q
_____ is the youngest and largest of all cell types with prominent nucleoli
A
Blast
16
Q
Progression of red blood cell maturation
A
Myeloid stem cell –> Pronormoblast –> Basophilic Normoblast –> Polychromatic Normoblast –> Orthochromatic Normoblast –> Polychromatic erythrocyte (reticulocyte) –> Eryrthrocyte
17
Q
In RBC maturation, what is the first cell apparent in the PBS?
A
reticulocyte (polychromatic erythrocyte)
18
Q
General S/S of anemia
A
weakness, malaise, easy fatigability, dyspnea on mild exertion
19
Q
PE findings in anemia
A
Pallor, dizziness & headache, angina, anemic murmur (hemic murmur), koilonychias & brittle nails, oliguria, anuria
20
Q
Normal Hgb levels
A
12-14 g/dl (F); 13-16 g/dL (M)
21
Q
Normal Hct levels
A
33-42% (F); 39-48% (M)
22
Q
Normal reticulocyte count
A
0.5 - 1.5%
23
Q
What the the reticulocyte count measure?
A
Bone marrow activity
24
Q
Normally, reticulocyte count is (elevated / depressed) in anemia
A
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
