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Hema IM > Hema > Flashcards

Hema Flashcards

1
Q

If HSC damaged, and no longer function, a person would only survive?

A

2-4 weeks

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2
Q

Yolk sac produce?

A

Red blood cells, macrophages

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3
Q

Placenta produce?

A

Stem cells, rbcs, platelets

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4
Q

Thymus produce?

A

Cells of adaptive immunity

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5
Q

Blood cell production before birth?

A

Yolk sac, spleen, liver

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6
Q

Blood cells produced after birth?

A

Bone marrow

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7
Q

Two cardinal functions of HSCs?

A
  1. Self renewal
  2. Differntiation
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8
Q

As the stem cells mature there is loss of?

A

Plasticity and proliferative capacity

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9
Q

how many days does it take for HSC to become mature?

A

10-14 days

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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

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11
Q

Three potential dates of HSC self renewal?

A

Apoptosis
Self renewal
Differentiation

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12
Q

Key limiting parameter for both autologous and allogeneic stem cell transplantation?

A

Stem cell number

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13
Q

This has rich source of stem cells but has inadequate number of stem cells?

A

Umbilical cord

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14
Q

Umbilical cord cells have two advantages over stem cell sources. What are they?

A
  1. Lower incidence of graft versus host disease
  2. Cord blood banks have representation of populations underrepresented in adult donor registries.
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15
Q

Which anemia is associated with normocytic, normochromic red cells and an inappropriately low reticulocyte response ?

A

Hypoproliferative anemias

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16
Q

This category includes early iron deficiency anemias?

A

Hypoproliferative anemias

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17
Q

Reticulocyte index<2-2.5?

A

Hypoproliferative anemias

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18
Q

Which is the critical element in the function of all cells?

A

Iron

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19
Q

Which iron is highly toxic?

A

Free iron

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20
Q

This carries O2 as part of hemoglobin?

A

Iron

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21
Q

This is a critical element in iron containing enzymes, including the cytochrome system in mitochondria?

A

Iron metabolism

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22
Q

Absence of this causes cells to lose their capacity for electron transport and energy metabolism, hemoglobin synthesis is impaired?

A

Iron

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23
Q

Iron transport protein?

A

Transferrin

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24
Q

Almost all iron transported by transferrin is delivered to_______?

A

Erythroid marrow

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25
Q

Excess iron binds to a storage protein named?

A

Apoferritin

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26
Q

Stored iron is called?

A

Ferritin

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27
Q

Average red cell life span?

A

120 days

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28
Q

How many percent of red cells are replaces every day?

A

0.8- 1%

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29
Q

Each millilitre of Red cells contain______ mg of elemental iron?

A

1mg

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30
Q

Male and female how much elemental iron is required per day?

A

1mg and 1.4 mg

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31
Q

Routes of iron loss are?

A

Blood loss
Loss of epithelial cells from skin
Gut
Gi tract

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32
Q

True or false?
Heme iron( red meat) is the most readily absorbed?

A

True

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33
Q

True or false?
Vegetarians are at disadvantage as certain food reduce iron absorbtion to as high as 50% ( phyates and phosphates)?

A

True

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34
Q

When ionisable iron salts are given together with the food the amount of iron absorbed is reduced? True or False?

A

True

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35
Q

Iron in vegetables is lower in compared to non vegetarian? True or false?

A

True

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36
Q

Iron absorbtion occurs in?

A

Duodenum and proximal small Intestine

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37
Q

Iron is Transported across the membrane by?

A

DMT-1

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38
Q

Iron is stored as ferritin and exposed to the surface by?

A

Ferroportin

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39
Q

Ferroportin is negatively regulates by?

A

Hepacidin

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40
Q

Principal regulatory hormone of iron?

A

Hepacidin

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41
Q

Iron interacts with________ which oxidizes iron to ferric form for______ binding?

A

Hephaestin, transferrin binding

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42
Q

One of the most prevalent forms of malnutrition?

A

Iron deficiency anemia

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43
Q

Examples of negative iron balance?

A

Blood loss, pregnancy, growth spurts, inadequate dietary intake

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44
Q

As long as iron stores are available, serum iron , TIBC and red cell protoporphyrin level remains within normal limits? True/ False?

A

True

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45
Q

Marrow iron stores are absent when the serum ferritin level is?

A

<15

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46
Q

Cardinal rule: appearance of iron deficiency in an adult male or post menopausal female means gastrointestinal blood loss until proven otherwise? True/ False?

A

True

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47
Q

Usual signs of anemia?

A

Fatigue, pallor, reduced excerise capacity

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48
Q

Signs For advanced tissue iron deficiency ?

A

Cheilosis( fissure at the corner of the mouth) and Koilonychia( spooning of the fingernails)

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49
Q

Normal serum iron levels?

A

50-150

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50
Q

TIBC normal levels?

A

300-360

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51
Q

That normal level?

A

25-50%

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52
Q

What’s the formula for Tsat?

A

(Serum iron*100)/TIBC

53
Q

Level of Tsat in iron deficiency?

A

Tsat of<20%

54
Q

A TSAT of >50% indicates what?

A

Disproportionate amount of iron is getting delivered to non erythroid tissues. If this persists for extended time, tissue iron overload may occur.

55
Q

Which protein is readily available for iron for extraction?

A

Apoferritin

56
Q

Which protein is less readily available for extraction?

A

Hemosiderin

57
Q

Which is the most convenient test to estimate iron stores?

A

Serum Ferritin level

58
Q

Normal serum ferritin level in male?

A

100

59
Q

Normal serum ferritin level in female?

A

30

60
Q

Serum ferritin level in iron deficiency?

A

<15

61
Q

_________ is an intermediate in the heme synthesis pathway?

A

Protoporphyrin

62
Q

This accumulates within the red cell when heme synthesis is impaired?

A

Protoporphyrin

63
Q

Most common cause of increase in protoporphyrin is?

A

Iron deficiency and lead poisoning

64
Q

This reflects an inadequate iron supply to erythroid precursors?

A

Protoporphyrin

65
Q

These receptors are highest in the erythroid cells?

A

Transferrin receptors

66
Q

This reflects total erythroid marrow mass?

A

Transferrin receptors

67
Q

Normal values of transferrin receptors ?

A

4-9

68
Q

Testosterone and anabolic steroids _________ erythropoiesis?

A

Increase

69
Q

Hypothyroidism and pituitary hormone deficiency may develop______ anemia?

A

Mild

70
Q

Decreased dietary protein intake may cause _______ anemia

A

Mild to moderate hypoproliferative anemia

71
Q

Release of EPO is impaired in patients with________?

A

Marasmus

72
Q

Two major forms of treatment of Hypoproliferative anemia?

A

Transfusion and EPO

73
Q

In whom reversels are not possible in hypoproliferative anemia?

A

End stage kidney disease, cancer, chronic inflammatory states.

74
Q

Patient without serious underlying cardiovascular disease or pulmonary disease,Intervention is needed when hemoglobin level fall down what level?

A

7-8g/dl

75
Q

One unit of packed RBCs increase hemoglobin by?

A

1g/dl

76
Q

For CKD, usual dose of EPO is _______ 3 times a week intravenously?

A

50-150U/kg

77
Q

Disorder of reduced globin biosynthesis?

A

Thalassemia

78
Q

Disorder that affects the amino acid sequence of globin?

A

Hemoglobinopathies

79
Q

Most common Mendelian genetic disease?

A

Thalassemia & hemoglobinopathies

80
Q

Globin is encoded in two nonalielic clusters?

A

Beta- globulin gene cluster & alpha- globulin gene cluster

81
Q

Short arm of chromosome 11?

A

Beta- globulin gene cluster

82
Q

Alpha - globulin gene cluster in which chromosome?

A

Chromosome 16

83
Q

Hemoglobin composition of normal adults?

A

> 95%Hba, ~1%HBF, and 2-3% HbA2 (A2g2).

84
Q

Nitric oxide promotes?

A

Vasodilation

85
Q

_________ is defined by sigmoidal shape of the hemoglobin O2 dissociation curve .

A

Oxygen binding

86
Q

Point that indicates the partial pressure of O2 where hemoglobin is half saturated?

A

P50

87
Q

Hemoglobin has high affinity, less O2 delivered to tissues?

A

Low P50

88
Q

Hemoglobin has low affinity, O2 is released more to tissues?

A

High P50

89
Q

Factors that affect affinity of hemoglobin?

A

Cadet
Co3, acidosis, 2,3-DPG, Exercise, Temperature

90
Q

This gene mutation are expressed in embryo and fetus and persist throughout life?

A

A- Globin gene mutations

91
Q

HbF mutations are expressed in?

A

Fetus and in first month’s of life, vanishing from notice afterwards

92
Q

These mutations are innocuous and usually not detected?

A

Delta globin gene mutations

93
Q

This gene mutation becomes clinically apparent after the synthesis of HbF dwindles to adult levels?

A

B- globulin gene mutation

94
Q

All disorders of hemoglobin are autosomal dominant or co- dominant disorders True or false.

A

False,
It’s autosomal recessive or co- dominant

95
Q

Initial laboratory tests for hemoglobin disorders?

A

CBC with reticulocyte count( increase in reticulocyte count)

96
Q

Confirmatory lab tests for hemoglobin disorder diagnosis?

A

Hemoglobin fraction by high performance liquid chromatography (HPLC) or capillary electrophoresis

97
Q

Mutations in globin gene causes?

A

Sickle cell disease beta Thalassemia

98
Q

This is caused by assortment of genotypes?

A

Sickle cell disease

99
Q

HbS is similar to HbS except?

A

HbS polymerizes when it is deoxygenated

100
Q

Complications of sickle cell disease?

A

Acute painful episodes
Acute chest syndrome
Osteonecrosis
Leg ulcers

101
Q

Most common acute events in sickle cell disease?

A

Acute painful episodes

102
Q

Where does unprovoked severe pain occur in acute painful episodes in sickle cell disease?

A

Extremities & torso

103
Q

Acute painful episodes are often symmetrical &_________?

A

Stereotypical

104
Q

Ape require strong opioids for treatment? True or false?

A

True

105
Q

Some patients die shortly after an acute episodes due to?

A

Arrythmias / pulmonary embolism

106
Q

Second most frequent acute sickle cell related event. Pneumonia like illness?

A

Acute chest syndrome

107
Q

In acute chest syndrome for management, we give supplemental O2 for destruction<95 %? True or false?

A

True

108
Q

Osteonecrosis affects _______ bilaterally?

A

Hips

109
Q

Unna boots in?

A

Leg ulcers

110
Q

Encompasses most of the lower leg in sickle cell disease?

A

Leg ulcer

111
Q

Doing what before and during exercise can prevent most episodes of heat related illness?

A

Optimal hydration

112
Q

Unbalanced globin synthesis is a hallmark of?

A

Thalassemia

113
Q

This improves effective erythropoiesis?

A

Luspatercept

114
Q

Finding an elevated level of HbA2 and HbF by HPLC is sufficient to establish this diagnosis?

A

B thalassemia

115
Q

Mild or no anemia but microcytic/ hypochromic erythrocytes with minimal or no increase in reticulocyte count?

A

B thalassemia

116
Q

Most common B thalassemia mutation?

A

Single nucleotide changes

117
Q

Here B globin chain synthesis is deficient allows a globin chain to accumulate in excess?

A

B thalassemia

118
Q

Mediterranean anemia?

A

B thalassemia

119
Q

One or two A globin genes are missing?

A

A thalassemia

120
Q

Deletion/ malfunction of 3 a globin genes?

A

HBH disease

121
Q

No normally functioning a globin gene?

A

Hb Bart’s hydrops fetalis

122
Q

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?

A

Fetus

123
Q

When Y globin synthesis is mostly silenced, unpaired B globin chains lacking a suitable partner to form HbA , tetramerize as ______& ______ in adults?

A

B4 and HbH

124
Q

Hb Bart’s and HBH have very_______ affinity and do not unload _____ to tissues

A

High O2 affinity, O2

125
Q

Hb Bart’s hydrops fetalis result in severe ________

A

Anemia

126
Q

HbH disease leads to __________ with __________

A

Oxidative membrane damage with extravascular hemolysis

127
Q

Transfusions not needed in permanent basis?

A

a Thalassemia

128
Q

Prevention is best approach in ?

A

A thalassemia

129
Q

This is caused by deficiency in cobalamin( vit B12) or folate?

A

Megaloblastic anemias

Hema IM (1 decks)

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