Hema Flashcards
1
Q
If HSC damaged, and no longer function, a person would only survive?
A
2-4 weeks
2
Q
Yolk sac produce?
A
Red blood cells, macrophages
3
Q
Placenta produce?
A
Stem cells, rbcs, platelets
4
Q
Thymus produce?
A
Cells of adaptive immunity
5
Q
Blood cell production before birth?
A
Yolk sac, spleen, liver
6
Q
Blood cells produced after birth?
A
Bone marrow
7
Q
Two cardinal functions of HSCs?
A
- Self renewal
- Differntiation
8
Q
As the stem cells mature there is loss of?
A
Plasticity and proliferative capacity
9
Q
how many days does it take for HSC to become mature?
A
10-14 days
10
Q
Proliferation of cells is generally not associated with the ability to undergo a self renewing division except?
A
Memory T and B cells
11
Q
Three potential dates of HSC self renewal?
A
Apoptosis
Self renewal
Differentiation
12
Q
Key limiting parameter for both autologous and allogeneic stem cell transplantation?
A
Stem cell number
13
Q
This has rich source of stem cells but has inadequate number of stem cells?
A
Umbilical cord
14
Q
Umbilical cord cells have two advantages over stem cell sources. What are they?
A
- Lower incidence of graft versus host disease
- Cord blood banks have representation of populations underrepresented in adult donor registries.
15
Q
Which anemia is associated with normocytic, normochromic red cells and an inappropriately low reticulocyte response ?
A
Hypoproliferative anemias
16
Q
This category includes early iron deficiency anemias?
A
Hypoproliferative anemias
17
Q
Reticulocyte index<2-2.5?
A
Hypoproliferative anemias
18
Q
Which is the critical element in the function of all cells?
A
Iron
19
Q
Which iron is highly toxic?
A
Free iron
20
Q
This carries O2 as part of hemoglobin?
A
Iron
21
Q
This is a critical element in iron containing enzymes, including the cytochrome system in mitochondria?
A
Iron metabolism
22
Q
Absence of this causes cells to lose their capacity for electron transport and energy metabolism, hemoglobin synthesis is impaired?
A
Iron
23
Q
Iron transport protein?
A
Transferrin
24
Q
Almost all iron transported by transferrin is delivered to_______?
A
Erythroid marrow
25
Excess iron binds to a storage protein named?
Apoferritin
26
Stored iron is called?
Ferritin
27
Average red cell life span?
120 days
28
How many percent of red cells are replaces every day?
0.8- 1%
29
Each millilitre of Red cells contain______ mg of elemental iron?
1mg
30
Male and female how much elemental iron is required per day?
1mg and 1.4 mg
31
Routes of iron loss are?
Blood loss
Loss of epithelial cells from skin
Gut
Gi tract
32
True or false?
Heme iron( red meat) is the most readily absorbed?
True
33
True or false?
Vegetarians are at disadvantage as certain food reduce iron absorbtion to as high as 50% ( phyates and phosphates)?
True
34
When ionisable iron salts are given together with the food the amount of iron absorbed is reduced? True or False?
True
35
Iron in vegetables is lower in compared to non vegetarian? True or false?
True
36
Iron absorbtion occurs in?
Duodenum and proximal small Intestine
37
Iron is Transported across the membrane by?
DMT-1
38
Iron is stored as ferritin and exposed to the surface by?
Ferroportin
39
Ferroportin is negatively regulates by?
Hepacidin
40
Principal regulatory hormone of iron?
Hepacidin
41
Iron interacts with________ which oxidizes iron to ferric form for______ binding?
Hephaestin, transferrin binding
42
One of the most prevalent forms of malnutrition?
Iron deficiency anemia
43
Examples of negative iron balance?
Blood loss, pregnancy, growth spurts, inadequate dietary intake
44
As long as iron stores are available, serum iron , TIBC and red cell protoporphyrin level remains within normal limits? True/ False?
True
45
Marrow iron stores are absent when the serum ferritin level is?
<15
46
Cardinal rule: appearance of iron deficiency in an adult male or post menopausal female means gastrointestinal blood loss until proven otherwise? True/ False?
True
47
Usual signs of anemia?
Fatigue, pallor, reduced excerise capacity
48
Signs For advanced tissue iron deficiency ?
Cheilosis( fissure at the corner of the mouth) and Koilonychia( spooning of the fingernails)
49
Normal serum iron levels?
50-150
50
TIBC normal levels?
300-360
51
That normal level?
25-50%
52
What's the formula for Tsat?
(Serum iron*100)/TIBC
53
Level of Tsat in iron deficiency?
Tsat of<20%
54
A TSAT of >50% indicates what?
Disproportionate amount of iron is getting delivered to non erythroid tissues. If this persists for extended time, tissue iron overload may occur.
55
Which protein is readily available for iron for extraction?
Apoferritin
56
Which protein is less readily available for extraction?
Hemosiderin
57
Which is the most convenient test to estimate iron stores?
Serum Ferritin level
58
Normal serum ferritin level in male?
100
59
Normal serum ferritin level in female?
30
60
Serum ferritin level in iron deficiency?
<15
61
_________ is an intermediate in the heme synthesis pathway?
Protoporphyrin
62
This accumulates within the red cell when heme synthesis is impaired?
Protoporphyrin
63
Most common cause of increase in protoporphyrin is?
Iron deficiency and lead poisoning
64
This reflects an inadequate iron supply to erythroid precursors?
Protoporphyrin
65
These receptors are highest in the erythroid cells?
Transferrin receptors
66
This reflects total erythroid marrow mass?
Transferrin receptors
67
Normal values of transferrin receptors ?
4-9
68
Testosterone and anabolic steroids _________ erythropoiesis?
Increase
69
Hypothyroidism and pituitary hormone deficiency may develop______ anemia?
Mild
70
Decreased dietary protein intake may cause _______ anemia
Mild to moderate hypoproliferative anemia
71
Release of EPO is impaired in patients with________?
Marasmus
72
Two major forms of treatment of Hypoproliferative anemia?
Transfusion and EPO
73
In whom reversels are not possible in hypoproliferative anemia?
End stage kidney disease, cancer, chronic inflammatory states.
74
Patient without serious underlying cardiovascular disease or pulmonary disease,Intervention is needed when hemoglobin level fall down what level?
7-8g/dl
75
One unit of packed RBCs increase hemoglobin by?
1g/dl
76
For CKD, usual dose of EPO is _______ 3 times a week intravenously?
50-150U/kg
77
Disorder of reduced globin biosynthesis?
Thalassemia
78
Disorder that affects the amino acid sequence of globin?
Hemoglobinopathies
79
Most common Mendelian genetic disease?
Thalassemia & hemoglobinopathies
80
Globin is encoded in two nonalielic clusters?
Beta- globulin gene cluster & alpha- globulin gene cluster
81
Short arm of chromosome 11?
Beta- globulin gene cluster
82
Alpha - globulin gene cluster in which chromosome?
Chromosome 16
83
Hemoglobin composition of normal adults?
>95%Hba, ~1%HBF, and 2-3% HbA2 (A2g2).
84
Nitric oxide promotes?
Vasodilation
85
_________ is defined by sigmoidal shape of the hemoglobin O2 dissociation curve .
Oxygen binding
86
Point that indicates the partial pressure of O2 where hemoglobin is half saturated?
P50
87
Hemoglobin has high affinity, less O2 delivered to tissues?
Low P50
88
Hemoglobin has low affinity, O2 is released more to tissues?
High P50
89
Factors that affect affinity of hemoglobin?
Cadet
Co3, acidosis, 2,3-DPG, Exercise, Temperature
90
This gene mutation are expressed in embryo and fetus and persist throughout life?
A- Globin gene mutations
91
HbF mutations are expressed in?
Fetus and in first month's of life, vanishing from notice afterwards
92
These mutations are innocuous and usually not detected?
Delta globin gene mutations
93
This gene mutation becomes clinically apparent after the synthesis of HbF dwindles to adult levels?
B- globulin gene mutation
94
All disorders of hemoglobin are autosomal dominant or co- dominant disorders True or false.
False,
It's autosomal recessive or co- dominant
95
Initial laboratory tests for hemoglobin disorders?
CBC with reticulocyte count( increase in reticulocyte count)
96
Confirmatory lab tests for hemoglobin disorder diagnosis?
Hemoglobin fraction by high performance liquid chromatography (HPLC) or capillary electrophoresis
97
Mutations in globin gene causes?
Sickle cell disease beta Thalassemia
98
This is caused by assortment of genotypes?
Sickle cell disease
99
HbS is similar to HbS except?
HbS polymerizes when it is deoxygenated
100
Complications of sickle cell disease?
Acute painful episodes
Acute chest syndrome
Osteonecrosis
Leg ulcers
101
Most common acute events in sickle cell disease?
Acute painful episodes
102
Where does unprovoked severe pain occur in acute painful episodes in sickle cell disease?
Extremities & torso
103
Acute painful episodes are often symmetrical &_________?
Stereotypical
104
Ape require strong opioids for treatment? True or false?
True
105
Some patients die shortly after an acute episodes due to?
Arrythmias / pulmonary embolism
106
Second most frequent acute sickle cell related event. Pneumonia like illness?
Acute chest syndrome
107
In acute chest syndrome for management, we give supplemental O2 for destruction<95 %? True or false?
True
108
Osteonecrosis affects _______ bilaterally?
Hips
109
Unna boots in?
Leg ulcers
110
Encompasses most of the lower leg in sickle cell disease?
Leg ulcer
111
Doing what before and during exercise can prevent most episodes of heat related illness?
Optimal hydration
112
Unbalanced globin synthesis is a hallmark of?
Thalassemia
113
This improves effective erythropoiesis?
Luspatercept
114
Finding an elevated level of HbA2 and HbF by HPLC is sufficient to establish this diagnosis?
B thalassemia
115
Mild or no anemia but microcytic/ hypochromic erythrocytes with minimal or no increase in reticulocyte count?
B thalassemia
116
Most common B thalassemia mutation?
Single nucleotide changes
117
Here B globin chain synthesis is deficient allows a globin chain to accumulate in excess?
B thalassemia
118
Mediterranean anemia?
B thalassemia
119
One or two A globin genes are missing?
A thalassemia
120
Deletion/ malfunction of 3 a globin genes?
HBH disease
121
No normally functioning a globin gene?
Hb Bart's hydrops fetalis
122
In B thalassemia, absence of or reduced synthesis of a globin allows unpaired Y globin chains to form Y4 or Hb Bart's in whom?
Fetus
123
When Y globin synthesis is mostly silenced, unpaired B globin chains lacking a suitable partner to form HbA , tetramerize as ______& ______ in adults?
B4 and HbH
124
Hb Bart's and HBH have very_______ affinity and do not unload _____ to tissues
High O2 affinity, O2
125
Hb Bart's hydrops fetalis result in severe ________
Anemia
126
HbH disease leads to __________ with __________
Oxidative membrane damage with extravascular hemolysis
127
Transfusions not needed in permanent basis?
a Thalassemia
128
Prevention is best approach in ?
A thalassemia
129
This is caused by deficiency in cobalamin( vit B12) or folate?
Megaloblastic anemias
