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PLE Pathology > Red Blood Cell Disorders > Flashcards

Red Blood Cell Disorders Flashcards

1
Q

Definition of anemia

A

Decrease in Red Blood Cell Mass

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2
Q

Microcytic, Normocytic, and Macrocytic Anemia MCV cutoff

A

Normo = 80 - 100

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3
Q

Microcytic anemias have increased/decreased Hgb production

A

decreased. - maintains “concentration” of hgb by decreasing cell size

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4
Q

Mechanism of anemia of chronic disease

A

Iron is stored in macrophage, not available for heme synthesis, leading to decreased Hgb and microcytic anemia

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5
Q

Heme is composed of

A

Iron + Protoporphyrrin

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6
Q

Low protoporphyrin leads to which anemia

A

Sideroblastic anemia

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7
Q

4 Microcytic Anemias

A

IDA
Chronic Disease
Sideroblastic anemia
Thalassemia

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8
Q

Most common type of anemia

A

IDA

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9
Q

Iron absorption occurs in which part

A

Duodenum

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10
Q

How is iron absorbed?

A

Iron absorbed
Ferroportin takes iron from enterocyte to blood
Transferrin transports iron and delivers to liver and bone marow
Iron is stored as ferritin bound in macrophage

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11
Q

Measurements of iron status and function

A

Serum iron - iron in blood
TIBC - number of transferrin molecules present in blood
% Sat - number of bound Transferrin
Serum Ferritin

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12
Q

Red cell morphology in early vs late iron deficiency

A

Early - Normocytic

Late - microcytic, hypochromic anemia

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13
Q

3 clinical features of iron deficiency

A

Anemia
Koilonychia
Pica

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14
Q

Iron deficiency anemia associated with esophageal web and atrophic glossitis, and presents with anemia, dysphagia, and beefy-red tongue

A

Plummer-Vinson syndrome

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15
Q

Most common anemia in hospitalized patients

A

Anemia of chronic disease

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16
Q

Acute phase reactant increased in anemia of chronic disease which sequesters iron

A

Hepcidin

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17
Q

Effects of hepcidin

A
  1. Sequesters iron in storage sites which limits iron transfer and limits iron use
  2. Suppresses EPO production
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18
Q

Logic behind hepcidin

A

Iron is used by bacteria - it is a protective mechanism for infection

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19
Q

Protoporphyrin is produced from which part of Krebs Cycle?

A

Succinyl CoA

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20
Q

Iron-laded mitochondria around nucleus of erythroid precursors seen in sideroblastic anemia

A

Ringed sideroblasts

No porphyrin so iron accumulates in mitochondria

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21
Q

Prussian blue stain identifies which substance in the microscope?

A

Iron

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22
Q

Rate limiting step in porphyrin synthesis, which is most common cause of congenital sideroblastic anemia

A

ALAS

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23
Q

Acquired causes of sideroblastic anemia

A

Alcoholism
Lead poisoning
Vitamin B6 deficiency

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24
Q

Decreased synthesis of globin chains of hemoglobin

A

Thalassemia

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25
Q

Carriers of thalassemia are protected against which infection

A

Plasmodium falciparum

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26
Q

3 normal types of HgB

A

HbF (Fetal Hgb)(A2Y2)
HbA (A2B2)
HbA2 (A2d2)

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27
Q

How many alpha alleles (hgb) are present and in what chromosome are they located?

A

4 alleles

Chromosome 16

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28
Q

Number of gene deletions in alpha thalassemia and effect

A

1 gene - asymptomatic
2 genes - mild anemia, slightly increased RBC
(cis deletion - risk of severe thalassemia in offspring)
3 genes - Hemoglobin H disease (beta chain tetramers damage RBC) (severe anemia)
4 genes - Hydrops fetalis (gamma chain tetramers, Hb Barts)

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29
Q

Number of beta genes and in what chromosome

A

2 beta genes

Chromosome 11

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30
Q

Alpha vs Beta thalassemia in terms of genetics

A

Alpha - deletions

Beta - mutations

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31
Q

Red blood cell with a stained area in the center, found in beta thalassemia

A

Target cell

32
Q

What is increased in beta thalassemia minor

A

HbA2

HbA needs beta chain so this is very low/none

33
Q

What is massive erythroid hyperplasia

A

Hematopoeisis occuring in skull and facial bones as a result of increased EPO

34
Q

X ray finding in massive erythroid hyperplasia

A

Crew cut appearance

Chipmunk like face

35
Q

In megaloblastic anemia - why is there less cell divisions

A

Less DNA precursors

36
Q

Function of Vitamin B 12 in DNA precursor synthesis

A

Takes methyl group from tetrahydrofolate so that it can participate in DNA synthesis, and transfers methyl to homocysteine to convert it to methionine

37
Q

Macrocytic anemia causes not caused by B12 and folate

A

Alcoholism
Liver Disease
5-FU use

38
Q

Folate is absorbed in

A

Jejunum

39
Q

Vitamin B 12 is absorbed in

A

Vit B 12 binds to intrinsic factor and absorbed in ileum

40
Q

Autoimmune destruction of parietal cells in stomach, leading to intrinsic factor deficiency

A

Pernicious anemia

41
Q

Parasite associated with vitamin B12 deficiency

A

Diphyllobothrium latum

42
Q

Homocysteine and methylmalonic acid levels in Folate and B12 deficiency

A

homocysteine - both high

methylmalonic acid - normal in folate, high in B12

43
Q

Major causes of normocytic anemia

A

Peripheral destruction

Underproduction

44
Q

Reticulocyte morphology

A

larger cells with bluish cytoplasm - residual RNA

45
Q

How to differentiate normocytic anemia etiology based on retic count

A

> 3% = peripheral destruction (hemolytic anemia)

<3% = marrow problem, underproduction

46
Q

Extravascular hemolysis involves destruction by>

A

Reticuloendothelial system (spleen, macrophages)

47
Q

Unconjugated bilirubin from hemolysis comes from ____ and is transported bound to ____

A

Protoporphyrrin

albumin

48
Q

What molecule binds hemoglobin which escaped from RBC in intravascular hemolysis

A

Haptoglobin

it takes it to spleen

49
Q

Pathophysio of hemosiderinuria

A

Hemoglobin is absorbed in renal tubular cells, then iron accumulates, and the cells are sloughedo off in urine

50
Q

RBC defect where cytoskeleton is not tethered to the cell membrane due to defect in tethering proteins ankyrin, spectrin, and band 3.1

A

Hereditary Spherocytosis

51
Q

Cause of anemia in hereditary spherocytosis

A

Cells more easily consumed by spleen due to shape, more hemolysis

52
Q

CBC parameters increased in hereditary spherocytosis

A

Increased RDW, MCHC

53
Q

Increased risk of aplastic crisis with which infection in erythroblasts

A

Parvovirus B19

54
Q

Diagnostic Test for hereditary spherocytosis

A

Osmotic fragility test - cell is round, so when water comes in, it bursts easity

55
Q

Treatment for Hereditary Spherocytosis

A

Splenectomy

56
Q

Peripheral blood smear feature of Hereditary Spherocytosis post splenectomy

A

Howell-Jolly bodies - DNA fragments unremoved by missing spleen

57
Q

Type of mutation in Sickle Cell Anemia

A

Autosomal Recessive

58
Q

Amino acid change in sickle cell anemia

A

Glutamic acid to Valine

59
Q

Primary hemoglobin present in sickle cell anemia

A

HbS - which aggregate into needle like structure when polymerized

60
Q

Factors which increase/protect against sickling

A

Increase - hypoxemia, Dehydration, Acidosis

Decrease - HbF (why it doesnt present until 6mo), Hydroxyurea

61
Q

Effect of repeated sickling

A

RBC damage - hemolysis, irreversible sickling leading to vasoocclusion

62
Q

Common presenting sign of sickle cell disease in infants

above 6 months

A

Dactylitis

63
Q

Sickle Cell trait patients do not have sickle cells, except in which organ

A

Renal Medulla

64
Q

Laboratory test for Sickle Cell Disease and Trait

A

Metabisulfite screen: any amount of HbS will cause cell to sickle
Hemoglobin Electrophoresis: confirms presence and amount of HbS

65
Q

In Hemoglobin C, what is the protein change in hemoglobin?

A

Glutamic Acid to Lysine (Ly-C-ine)

66
Q

Peripheral blood smear feature of Hemoglobin C

A

Hemoglobin C crystal

67
Q

Acquired defect in myeloid stem cell leading to absent GPI making it susceptible to complement

A

Paroxysmal Nocturnal Hemoglobinuria

68
Q

Why does intravascular hemolysis occur at night in PNH

A

Mild respiratory acidosis from shallow breathing at night activates complement

69
Q

Laboratory test for PNH

A

Screen - sucrose test

Confirmatory - acidified serum test, flow cytometry

70
Q

Main cause of death in PNH

A

Thrombosis from destroyed platelet fragments

71
Q

G6PD deficiency is what type of mutation

A

X linked

72
Q

Pathophysio of G6PD

A

Reduced half life of G6PD. G6PD is enzyme in reaction which produces NADPH, which is required to convert used glutathione back. Low NADPH makes patient susceptible to oxidative stress

73
Q

Examples of oxidative stress in G6PD deficiency

A

Infections, drugs (primaquine, sulfa drugs, dapsone), fava beans

74
Q

Precipitated hemoglobin presents as ____ (morphology), and the cells where this Hgb is removed are called ______

A

Heinz bodies

Bite Cells

75
Q

Antibody mediated IgG or IgM destruction of RBC

A

Immune hemolytic anemia

76
Q

IgG mediated hemolytic anemia leads to (morphology)

A

Spherocytes

PLE Pathology (10 decks)

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