RBC/WBC Flashcards

1
Q
A
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2
Q

Where is EPO made?

A

Interstitial Fibroblast Cells of the Kidney near peritubular capillaries

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3
Q

Hypoxia shifts the O2 dissociation curve

A

LEFT

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4
Q

Anemia shifts the O2 dissociation curve

A

LEFT

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5
Q

Polycythemia vera has what effect on EPO production? O2 binding curve?

A

Decreases EPO due to high oxygen environment; Shifts O2 curve Right

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6
Q

Altitude has what effect on EPO production?

A

Increases it

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7
Q

Low O2 conditions will cause a _________ in EPO production and shift the O2 curve ______

A

INCREASE; LEFT

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8
Q

What 2 types of cancer may result in EPO production?

A

Renal Cell Carcinoma and to a lesser extent HCC

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9
Q

Clinical Features of Iron Deficiency Anemia

A

Microcytic Anemia, Koilonychia, Pica

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10
Q

Koilonychia

A

spoon-shaped finger-nails

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11
Q

Polycythemia vera is characterized by

A

bone marrow disease that results in an abnormal increased # of RBCs

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12
Q

Normal Reticulocyte count is

A

1-3%

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13
Q

High Reticulocyte count is

A

~10% “Reticulocytosis”

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14
Q

Koilonychia is pathognomonic of

A

Iron deficient Anemia

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15
Q

4 Types of Microcytic Anemia

A

Iron deficiency, Anemia of Chronic Disease, Sideroblastic, and Thalassemia

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16
Q

Microcytic Anemia is a MCV value of

A
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17
Q

The following are features of iron deficiency anemia: _________ Serum Ferritin _________ TIBC _________ Serum Iron _________ % Saturation _________ FEP

A
  1. Decreased serum ferritin (stored Fe)
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18
Q

Reticulocyte counts should be corrected for

A

ANEMIA

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19
Q

Corrected Reticulocyte Count Equation =

A

HCT/45 X reticulocyte count (45=normal HCT)

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20
Q

1st Sign of increased EPO

A

Reticulocyte response/release from BM

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21
Q

How long before immature reticulocytes become mature?

A

~24hrs

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22
Q

An inadequate reticulocyte response implies the marrow is

A

not working well, dysplastic, or aplastic

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23
Q

The following are features of Anemia of Chronic Disease: _________ Serum Ferritin _________ TIBC _________ Serum Iron _________ % Saturation _________ FEP

A

Increased Serum Ferritin > 30 Decreased TIBC Decreased Serum Iron Decreased % Saturation

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24
Q

The following are features of Sideroblastic Anemia: _________ Serum Ferritin _________ TIBC _________ Serum Iron _________ % Saturation

A

Increased Serum Ferritin Decreased TIBC Increased Serum Iron Increased % Saturation

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25
Q

The following are features of Thalassemia: _________ Serum Ferritin _________ TIBC _________ Serum Iron _________ % Saturation

A

Increased Serum Ferritin Decreased TIBC Increased Serum Iron Increased % Saturation

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26
Q

Prussian Blue Stain is used to stain ______; It is diagnostic for __________ type of Anemia

A

Iron; Sideroblastic Anemia - Ringed Sideroblasts

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27
Q

Causes of Iron Deficient Anemia

A

Malnutrition, Malabsorption (Crohn’s), Blood Loss (PUD, carcinoma, hookworms), Pregnancy, Menorrhagia, Gastrectomy

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28
Q

4 Stages of Iron Deficient Anemia

A
  1. Iron Stores Depleted 2. Serum Iron Depleted 3. Normocytic Anemia 4. Microcytic Anemia
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29
Q

TIBC is a measure of

A

Transferrin in blood

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30
Q

% Saturation is a measure of

A

% of Iron-bound Transferrin molecules in blood

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31
Q

Serum Ferritin is a measure of

A

stored iron w/in bone marrow macrophages and liver

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32
Q

Treatment for Iron Deficient Anemia

A

Ferrous Sulfate, Address any underlying conditions

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33
Q

What is PLUMMER-VINSON SYNDROME?

A

a condition characterized by Iron Deficient Anemia + Esophageal webbing + Atrophic glossitis (anemia, dysphagia, beefy-red tongue)

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34
Q

Describe the mechanism involved in Anemia of Chronic Disease

A

Chronic Disease/Cancer -> chronic inflammation -> acute phase reactants - Hepcidin

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35
Q

What is Hepcidin’s role?

A

sequesters Iron into BM macrophages and histiocytes of liver and prevents its transport out; also suppresses EPO (thought to help eliminate a bacterial infection)

36
Q

Stages of Anemia of Chronic Disease

A

Begins as Normocytic Anemia and progresses to Microcytic Anemia

37
Q

Treatment for Anemia of Chronic Disease

A

Address underlying cause of inflammation; Exogenous EPO for subset of cancer patients

38
Q

Describe the mechanism involved in Sideroblastic Anemia

A

Decreased protoporphyrin synthesis -> reduced heme production -> reduced Hb

39
Q

Describe the rate-limiting step of protoporphyrin synthesis

A

1st step: converts Succinyl-CoA to Aminolevulinic Acid (ALA); catalyzed by ALA synthase + required Cofactor Vitamin B6

40
Q

ALA is converted to ____________ by __________

A

porphobilinogen by ALA Dehydrogenase

41
Q

Final Step in protoporphyrin synthesis

A

Ferrochelatase links protoporphyrin and Iron to form HEME

42
Q

Final Step in protoporphyrin synthesis occurs

A

erythroid precursor MITOCHONDRIA (surrounding nucleus)

43
Q

What else is occurring during protoporphyrin synthesis

A

Iron transport to erythroid precursor MITOCHONDRIA

44
Q

In Sideroblastic anemia where there is decreased protoporphyrin synthesis, what occurs in the erythroid precursor cell?

A

Iron accumulates in the mitochondria creating an iron-laden ring around the nucleus –> eventually the toxic effects of iron cause cell death and Iron leaks into marrow (taken up by macrophages) and blood

45
Q

The Iron-laden ring around the nucleus of erythroid precursors are characteristic of __________ cells

A

Ringed Sideroblasts

46
Q

The most common congenital cause of Sideroblastic anemia is:

A

ALA S enzyme defect

47
Q

Common causes of acquired Sideroblastic anemia are:

A

Alcoholism, Lead Poisoning, Vitamin B6 deficiency

48
Q

How can Alcoholism lead to Sideroblastic anemia?

A

Direct Mitochondrial Poison

49
Q

How can Lead Poisoning lead to Sideroblastic anemia?

A

Denatures the enzymes: ALA D and Ferrochelatase

50
Q

How can Vitamin B6 deficiency lead to Sideroblastic anemia?

A

Necessary Cofactor for ALA S, inhibits the RATE-LIMITING Step of protoporphyrin synthesis

51
Q

What Drug Therapy has been linked to Sideroblastic anemia due to its effect on Vitamin B6 depletion?

A

Isoniazid for TB Tx

52
Q

Describe the mechanism involved in Thalassemia

A

Decreased production of globin chains

53
Q

Anemia of Chronic Disease is common, occurring in ____% of Microcytic anemias

A

30%; usually accompanied by little to no morphological changes on peripheral smear

54
Q

Anemia of Chronic Disease lasts

A

longer than 1-2 months

55
Q

Causes of Anemia of Chronic Disease

A

Infection, Non-infectious inflammation, Malignancy

56
Q

Hepcidin sequesters Iron and ALSO

A

suppresses EPO

57
Q

In 25% of cases, the only chronic diseases present in a patient w/ Anemia of Chronic Disease

A

Diseases such as: congestive heart failure and diabetes mellitus

58
Q

In Anemia of Chronic Disease, anemia is usually

A

mild-moderate w/ HCT > 25; mainly due to EPO repression by Hepcidin

59
Q

CBC includes:

A

Hb, HCT, WBC count, Platelet count, RBC count Calculate: MCV, MCH, RDW (NOT LAP)

60
Q

Hemoglobin F shifts the O2 binding curve

A

LEFT - driving EPO production and Hb synthesis

61
Q

Most common anemia in babies?

A

Iron deficiency since breast milk lacks Iron

62
Q

HbF tetramer is composed of

A

2-alpha and 2-gamma

63
Q

Extramedullary Hematopoiesis occurs where in fetuses and premature infants

A

yolk sac -> liver -> BM

64
Q

What anemic condition results in Extramedullary Hematopoiesis in liver, spleen, face, and skull?

A

Beta-thalassemia Major

65
Q

HbF is replaced by _____ ~ ____%

A

HbA ~97%; HbF may persist at ~1%

66
Q

HbA2 may present at ~____%

A

~3%

67
Q

HbF is broken down in the months following birth by

A

Splenic histiocytes thus “physiologic jaundice”

68
Q

Explain how HbF/Fetal RBC breakdown may result in “physiologic jaundice”

A

RBC/HbF is broken down by splenic histiocytes. Hb is broken into heme (Fe and protoporphyrin) and globin. Protoporphyrin is then broken down into bilirubin, unconjugated bilirubin is fat-soluble (+serum albumin), enters the blood & is delivered to the liver for conjugation. If the concentration of unconjugated bilirubin exceeds the liver’s ability to conjugate –> a build up of unconjugated bilirubin in blood results in “physiologic anemia”

69
Q

Reticulocyte response to anemia would result in a CRC of > ___%

A

> 3%

70
Q

In anemia, a CRC

A

dysplastic or aplastic BM or failure to produce EPO

71
Q

How can alcohol lead to macrocytic anemia?

A

DIRECT toxic effects causing macrocytic anemia

72
Q

Haptoglobins function

A

binds free Hb in blood and returns it to to the liver (saves a small amount of Hb)

73
Q

Hemosiderinuria

A

Hb taken up by renal tubular cells, destroys Hb and binds as Hemosiderin in cell. Sloughing of renal tubular cells results in Hemosiderinuria

74
Q

Hemosiderinuria is a common lab finding in

A

Intravascular Hemolytic Normocytic Anemia - PNH, G6PD def.,

75
Q

HbA tetramer is composed of

A

alpha2, beta2 (97%)

76
Q

HbA2 tetramer is composed of

A

alpha2, delta2 (~3%)

77
Q

Children usually have lower Hb levels because they typically have higher _______ and ______

A

Phosphorus levels and 2,3-BPG

78
Q

2,3-BPG shifts the O2 dissociation curve to the

A

RIGHT

79
Q

Parvovirus B19 infects

A

erythroid precursors

80
Q

Risk of aplastic crisis from a Parvovirus B19 infection occurs in

A

beta-thalassemia Major, Hereditary Spherocytosis, Sickle Cell Anemia

81
Q

Howell-Jolly bodies

A

DNA fragments in RBCs - indicative of splenic dysfunction

82
Q

What condition is likely to have Howell-Jolly bodies appear following Treatment?

A

Hereditary Spherocytosis

83
Q

The mechanism of Hereditary Spherocytosis is

A

defective RBC cytoskeletal proteins resulting in cell shape changes (biconcave to sphere) and membrane blebs that are cleaved by splenic histiocytes

84
Q

Which hormone drives EPO production?

A

Testosterone

85
Q

In what condition would you see a large number of nucleated RBCs?

A

beta-thalassemia Major - due to extra medullary erythropoiesis

86
Q

What inherited genetic mutations may be selected for by their ability to protect against Plasmodium falciparum Malaria ?

A

Sickle Cell Anemia, Thalassemia, G6PD deficiency