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pathoma > Red Blood disorders > Flashcards

Red Blood disorders Flashcards

1
Q

Anemia

A

Decreased in RBC mass

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2
Q

Anemia

A

Decreased in RBC mass

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3
Q

Anemia in males vs females

A

Males

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4
Q

Microcytic anemia

A

MCV

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5
Q

Heme

A

Iron and protoporphyrin

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6
Q

Hemoglobin made of

A

Hene and globin

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7
Q

Microcytic anemia caused by deficiencies in what 4 things

A

Heme, globin, iron, protoporphyrin (sideroblastic anemia)

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8
Q

What are the 4 microcytic anemias

A

Iron deficiency anemia
Anemia of chronic dz
Sideroblastic anemia
Thalassemia

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9
Q

Anemia in males vs females

A
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10
Q

Microcytic anemia

A

MCV

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11
Q

Heme

A

Iron and protoporphyrin

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12
Q

Hemoglobin made of

A

Hene and globin

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13
Q

Microcytic anemia caused by deficiencies in what 4 things

A

Heme, globin, iron, protoporphyrin (sideroblastic anemia)

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14
Q

What are the 4 microcytic anemias

A

Iron deficiency anemia
Anemia of chronic dz
Sideroblastic anemia
Thalassemia

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15
Q

Iron deficiency anemia

A

Decreased iron = decreased heme = decreased Hb = microcytic anemia

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16
Q

How is iron absorbed

A

Heme/non-heme forms

Duodenum
Enterocytes have DMT1 transporters
(heme form more readily absorbed) and transport iron across cell membrane into blood via ferroportin

Transferrin stores iron in blood & delivers it to liver & bone marrow macrophages for storage

Stored intracellularly bound to ferritin

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17
Q

Lab iron status measurements

A

Serum iron
TIBC
% saturation
Serum ferritin

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18
Q

Iron in body

A

Iron in blood bound to transferrin

Every 3 transferrin - 1 will be carrying iron

In macrophages iron bound to ferritin

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19
Q

Serum iron

A

Iron in blood

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20
Q

Transferrin molecules

A

TIBC - total iron binding capacity

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21
Q

% saturation

A

How many transferring bound to iron

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22
Q

Serum ferritin

A

How much iron present in liver and bone marrow macrophages

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23
Q

Dietary deficiency or blood loss

A

Breast feeding infants
Poor diet in children
Adults: PUD - males, pregnancy/menorrhagia (females)
Elderly - Western world - colon polyps, carcinoma vs developing world - hook worm (necator and ancylostoma)

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24
Q

Other causes of iron deficiency

A

malnutrition
malabsorption (acid aids in iron absorption)
gastrectomy

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25
Q

Fe2+ or Fe3+ absorbed by body?

A

Fe2+ goes into the body and acid keeps it the Fe2+ state.

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26
Q

Stages of iron deficiency

A

Storage iron depleted (ferritin) - TIBC will go up
Serum iron depleted
Normocytic anemia
Microcytic, hypochromic anemia

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27
Q

If ferritin is down - liver and macrophages will pump out more transferring molecules out to find iron

A

Ferritin down

TIBC will go up

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28
Q

What type of anemia is in the very early stage of iron deficiency?

A

Normocytic anemia

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29
Q

Lab findings of microcytic anemia

A

MCV

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30
Q

Tx of microcytic anemia

A

ferrous sulfate

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31
Q

Plummer-Vinson syndrome

A

Iron deficiency anemia with esophageal web/atrophic glossitis

Anemia, dysphagia, beefy-red tongue

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32
Q

Anemia of chronic disease

A

Chronic inflammation/cancer

Most common anemia in hospitalized patients

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33
Q

ACD anemia

A

increase in acute phase reactants - Hepcidin which locks iron in storage sites so it can’t be used

Limits iron transfer from macrophages to erythroid precursors

Hepcidin Suppresses EPO production

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34
Q

Lab findings in ACD

A

Increases ferritin, Decreased TIBC
Decreased serum, decreased % saturation
Increased FEP (not a problem with protoporphyrin)

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35
Q

Tx of ACD

A

Addressing underlying cause

Exogenous epo - especially cancer patients

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36
Q

Sideroblastic anemia due to

A

defective protoporphyrin synthesis - resulting in low heme - low Hb - microcytic anemia

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37
Q

Protoporphyrin synthesized via series of reactions

A

Final reaction attaches protoprphyrin to iron to make heme (7-8 reactions)
(ferrochelotase)

*Occurs in mitochondria

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38
Q

Steps of protoporphyrin production

A 
Succinyl CoA converted to aminolevulinic acid by Aminolevulinic acid synthetase
S CoA (ALAS) ALA (rate limiting step) - VIT B6 is cofactor

ALA to prophobilinogen by Aminolevulinic acid dehydrogenase
ALA (ALAD) Prophobilinogen

Final reaction - protoporphyrin + iron = heme by ferrocheletase in mitochondria

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39
Q

Iron transferred to precursor

A

Iron from bone marrow macrophages to erythoblasts - iron and protoporphyin meet in macrophage to make heme

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40
Q

What happens if protoporphyrin is deficient?

A

Iron gets trapped in mitochondria

Iron laden mitochondria form ring around nucleus of erythroid precursors - cells called sideroblasts

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41
Q

What stain marks iron?

A

prussian blue stain

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42
Q

What enzyme is involved most commonly in congenital sideroblastic anemia?

A

ALAS (rate limiting enzyme)

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43
Q

Common causes of acquired sideroblastic anemia?

A 
Alcoholism (mito poisoning) 
Lead poisoning (LAD/ferrochelatase)
Vit B6 deficiency (for ALAS) - common in isoniazid deficiency
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44
Q

Lab findings in sideroblastic anemia

A

Iron overloaded state
increased ferritin, decreased TIBC
Increased serum iron, increased % saturation

Iron - damages cells/death by free radicals - so iron leaks out and bone marrow eats iron, some can leak into blood

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45
Q

Thalassemia

A

decreased synthesis of globin chains - decreasing Hb

Thalasemmia is decrease in synthesis!

Inherited mutation

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46
Q

What kind of malaria are people with thalassemia protected against?

A

Plasmodium falciparum malaria

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47
Q

What are the three normal types of Hb?

A

HbF (alpha2,gamma2)
HbA (alpha2,beta2)
HbA2 (alpha2,delta2)

alpha common - most important in all globin chains

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48
Q

How many copies of alpha allele?

A

4 on chromosome 16

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49
Q

Alpha thalassemia - is due to

A

Gene deletion

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50
Q

What happens when one alpha is deleted?

A

Asymptomatic

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51
Q

What happens when 2 alpha genes are deleted?

A

Mild anemia with slightly increased RBC count

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52
Q

What are the possibilities for 2 alpha gene deletions

A

Cis - worse than trans - increased risk of severe thalassemia in offspring (seen in Asians)

Trans - one deletion of gene on each chromosome (Africa)

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53
Q

What about when 3 alpha genes are deleted?

A

Severe anemia

Beta chains form tetramers (HbH) that damage RBCs

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54
Q

What is HbH

A

beta chain tetramer

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55
Q

What happens when 4 alpha genes deleted?

A

Lethal in utero - hydrops fetalis
Gamma chains form tetramers (Hb Barts) that damage RBCs

Hb Barts seen on electrophoresis

56
Q

Beta thalassemia due to

A

Gene mutations
2 genes present on chromosome 11

Mutations in absent (Betanull) or diminished (Beta+) production of beta globin chain

57
Q

Mildest form of beta thalassemia

A

B/B+
Beta thalassemia minor

Usually asymptomatic with increased RBC count

Microcytic, hypochromic RBCs and target cells on smear

Increased HbA2

58
Q

Target cell

A

decreased cytoplasm or increased membrane

59
Q

Most severe form of beta thalassemia

A

Beta(null)/Beta(null) - beta thalassemia major

Severe anemia few months after birth
HbF (alpha2gamma2) at birth is temporarily protective

form alpha tetramers that damage RBC - ineffective erythropoiesis/extravascular hemolysis

60
Q

Beta thalassemia - massive erythroid hyperplasia

A

Expansion of hematopoeisis into marrow of skull/facial bones
(EPO released by kidney) - skull - crew cut appearnace on x-ray, facial bones thickened “chipmunk like face”

Extramedullary hematopoiesis with HSM

Risk of aplastic crisis with parvovirus B19

61
Q

Tx for beta thalassemia

A

Chronic transfusion

At risk for secondary hemochromatosis

62
Q

Beta thalassemia blood smear

A

Microcytis, hypochromic target cells, nucleated RBC

63
Q

Beta thalassemia

A

No HbA

Increased HbA2 and HbF

64
Q

Macrocytic anemia

A

MCV>100
larger than normal
d/t most commonly - folate/VitB12 deficiency

one less division than normal (DNA precursor deficiency)

65
Q

THF

A

comes in and quickly gets mehtylated - VIT B12 takes methyl group

VIT B12 passes methyl group to homocysteine and becomes methionine - transfers methyl groups

66
Q

Lack of VIT B12 or folate

A 
Megaloblastic anemia
Hypersegmented neutrophils (greater than 5 lobes)
Megaloblastic change in all rapidly dividing epithelial cells
67
Q

Other causes of macrocytic anemia

A

Alcoholism
Liver dz
Drugs (5-FU)

68
Q

Where is folate absorbed?

A

Jejunum

69
Q

Folate deficiency

A 
poor diet (minimal stores) - alcoholics, elderly
Increased demand (pregnancy, cancer, hemolytic anemia)
Folate antagonists (MTX)
70
Q

Lab findings of macrocytic anemia

A 
Macrocytic RBC and hypersegmented neutrophils
Glossitis
Decreased serum folate
Increased serum homocysteine
Normal methylmalonic acid
71
Q

B12

A

complexed to animal derived proteins

Cleaved then bound to R-binder from salivary gland
Then goes to small bowel - R binder cleaved by pancreatic proteases

VITB12 bound by IF (parietal cells of stomach) and absorbed by ileum

72
Q

Most common cause of B12 deficiency

A

AI destruction of parietal cells leading to intrinsic factor deficiency

73
Q

Parietal cell

A

proton pumps
pink - upon staining
Pernicious anemia

“P”

74
Q

Other causes of VIT B12 deficiency

A 
Pancreatic insufficiency (can't cleave from R-binder) 
Damage to terminal ileum d/t Chron's dz or diphyllobthrium latum 
Dietary deficiency (rare execpt in vegans)
75
Q

Clincal findngs B12 deficiency

A

Macrocytic anemia with hypersegmented neutrophils
Glossitis
Subacute combinded degeneration of spinal cord (from methylmalonic acid building up - can’t be converted to succinyl-CoA - builds up in myelin)

76
Q

Lab findings in B12 deficiency anemia

A

Decreased serum vit B12
Increased serum homocysteine
Increased methylmalonic acid (can’t be converted to succinyl CoA)

77
Q

Normocytic anemia definition/cause

A

RBC are normal size but decreased amount

d/t peripheral destruction or underproduction

78
Q

How do you determine normocytic anemia is peripheral destruction or underproduction

A

Reticulocyte count - young RBC from bone marrow

Bluish cytoplasm from residual RNA

79
Q

Reticulocyte count falsely elevated in

A

macrocytic anemia

Decreased in total RBC falsely elevated % reticulocytes

80
Q

Corrected reticulocyte count

A

Multiply reticulocyte count by Hct/45

> 3% = good marrow response and suggests peripheral destruction

81
Q

Extravascular hemolysis invovles RBC destruction by

A

RE system: macrophages of spleen, liver, lymph nodes - break down Hb

82
Q

Break down of Hb

A

Globin - amino acids
Heme - iron and protoporphyrin
Protoporphyrin - unconjugated bilirubin

83
Q

Clinical findings of extravascular hemolysis

A

Anemia with splenomegaly
Jaundice - unconjugated bilirubin
Increased risk for bilirubin gallstones
Marrow hyperplasia w/ corrected reticulocyte count >3%

84
Q

Intravascular hemolysis and findings

A

Hb binds haptoglobin - (complex) - so free haptoglobin levels decrease

Hemoglobinemia
Hemoglobinuria
Hemosiderinuria
Decreased serum haptoglobin

85
Q

Normocytic anemia with predominant extravascular hemolysis

A

Hereditary spherocytosis
Sickle cell anemia
Hemoglobin C

86
Q

Hereditary spherocytosis

A

Inherited defect in RBC cytoskeleton membrane tethering proteins
*ankryin, spectrin, band 3.1

Membrane blebs formed and lost over time - so cells round

87
Q

Spherocytes less able to maneuver thru

A

splenic sinuses so they’re consumed

88
Q

Clinical and lab findings hereditary spherocytosis

A

Spherocytes w/ loss of central pallor
Increased RDW
Increased MCHC
Splenomegaly, jaudice w/ unconjugated bilirubin - increased risk for bilirubin gallstones
Increased risk for aplastic crisis (parvo B19) - erythroid precursor

89
Q

Dx of hereditary spherocytosis

A

osmotic fragility test: increased spherocyte fragility in hypotonic soln

90
Q

Tx of hereditary spherocytosis

A

Splenectomy - anemia resolves but spherocytes persist

Howell Jolly bodies

91
Q

Sickle Cell anemia

A

AR mutation in Beta chain in hemoglobin

Glutamic acid (hydrophilic) replaced with valine (hydrophobic)

92
Q

What malaria is protected against in SCD?

A

Falciparum malaria

93
Q

Sickle cell dz vs trait

A

SCD:
2 abnormal beta genes present
>90% HbS in RBC

SCT:
one mutated and one normal beta chain

94
Q

HbS polymerizes when

A

deoxygenated

Aggregates into needle like structures resulting in Sickle cells

(hypoxemia, dehydrogration, acidosis)

95
Q

Protective factor against sickling

A

HbF

Tx: hydroxyurea increases levels of HbF

96
Q

Extravascular hemolysis symptoms SCD

A

Anemia

Jaundice with unconjugated hyperbilirubinemia

Increased risk for bilirubin gallstones

97
Q

Intravascular hemolysis with SCD

A

decreased haptoglobin

Target cells on blood smear

98
Q

SCD - massive erythroid hyperplasia

A

Expansion of hematopoiesis into skull/facial bones
Extramedullary hematopoiesis with hepatomegaly
Aplastic anemia crisis (Parvo B19)

99
Q

Dactylitis

A

Common in infants

Swollen hands/feet d/t vao-occlusive infarct of bone (irreversible sickling)

100
Q

Autosplenectomy

A

Irreversible sickling
Increased risk of infection with encapsulated organisms (most common cause of death in children)

Increased risk of salmonella osteomyelitis

Howell Jolly bodies

101
Q

What is the most common cause of death in children w/ SCD?

A

Encapsulated organsims

102
Q

Acute chest syndrome

A

Vaso-occlusive in pulm microcirculation

Chest pain, SOB, lung infiltrates, often precipitated by pneumonia

Most common cause od death in adults (SCD)

103
Q

Most common cause of death in adults w/ SCD?

A

Acute chest syndrome

104
Q

Pain crisis in SCD

A

vaso-occlusion crisis

105
Q

Renal papillary necrosis

A

Gross hematuria and proteinuria

Vaso-occlusive crisis

106
Q

Sickle cell trait

A

One normal beta gene
One mutated beta gene

HbA and HbS
More HbA than HbS
50% S to sickle so these patients asymptomatic

107
Q

Sickle cell trait doesn’t sickle except for…

A

Renal medulla
Extreme hypoxia and hypertonicity cause sickling

Microinfarctions that lead to microscopic hematuria and decreased ability to conc urine

108
Q

Sickle cell trait

A

Metabisulfite screen: causes HbS to sickle

No sickle cell dz or target cells

109
Q

HbC

A

Ar - Beta chain
Glutamic acid replaced by lysine

Less common than SCD

Mild anemia d/t extravascular hemolysis
HbC crystals

110
Q

Normocytic anemia with predominant intravascular hemolysis

A

Paroxysmal nocturnal hemoglobinuria (PNH)

G6PD deficiency

Immune Hemolytic anemia

Microangiopathic hemolytic anemia

Malaria

111
Q

Paroxysmal nocturnal hemoglobinuria (PNH)

A

DAF on surface
MIRL on surface
by GPI anchoring protein in normal cells *acquired defect in myeloid stem cell so GPI not present

In PNH - no GPI so no DAF/MIRL so susceptible to complement (intravascular hemolysis at night)

Retain carbon dioxide causing acidosis activating complement when we sleep
Dark urine in morning

112
Q

Intravascular hemolysis - PNH

A

Hemoglobinermia
Hemoglobinuria
Hemosiderinuria seen days after hemolysis

113
Q

Screen for PNH

A

Sucrose test

Confirmatory test: acidified serum test of flow cytometry to detect lack of CD55 (DAF)

114
Q

Most common cause of death in PNH

A

Thrombosis

Platelet fragments activated coagulation cascade - hepatic, portal, cerebral veins

115
Q

Complications of PHN

A

Iron deficiency Anemia

Myeloid stem cell mutation - can result in AML

116
Q

G6PD deficiency

A

X linked R
Cells susceptible to oxidative stress (glutathione antioxidant neutralizes H2O2 + GSH - GS-SG) - needs to get back to GSH by NADPH which is produced by G6PD

If no G6PH - don’t make reduced glutathione so increased oxidative stress to red blood cells

117
Q

2 major variants G6PD variants

A

African - mildly reduced half like g6pD

Mediterranean variant - markedly reduced half life G6PD

118
Q

G6PD deficiency protective against

A

Falciparum malaria

119
Q

What are the oxidative stress inducers of Heinz bodies

A

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

120
Q

Screen for G6PD deficiency

A

Heinz prep - do this after resolved acute hemolytic episode resolved

121
Q

What mediates extravascular hemolysis

Immune mediated anemia

A

Immune mediated anemia
IgG (extravascular hemolysis - warm) or IgM (intravascular - cold)
Results in spherocytes

122
Q

Immune mediated anemia associated with

A

SLE, CLL,certain drugs

123
Q

TX: immune mediated anemia

A

Cessation of offending drug
IVIG
Steroid
Splenectomy

124
Q

IgM mediated intravascular hemolysis (immune mediated anemia) associated with what to infections

A

Mycoplasma pneumoniae

Infectious mono

125
Q

What test to dx IHA

A

Direct/Indirect coombs test

Direct: do I have RBC already bound by IgG - anti IgG added

Indirect: Does pt have antibodies in the serum?

126
Q

Microangiopathic hemolytic anemia

A

Intravascular hemolysis from vascular pathology

Iron deficiency anemia occurs with chronic hemolysis

Occurs with mircothrombi: TTP-HUS, DIC, HELLP

microthrombi, prosthetic heart valves, aortic stenosis (calcified valves crushes RBC)

127
Q

Malaria causes hemolysis

A

Transmitted by Anopheles mosquito

Infection of RBCs and liver plasmodium

128
Q

RBC ruptures as part of plasmodium life cycle

A

Intravascular hemolysis and cyclical fever

129
Q

Fever cycle P falciuparum

A

daily

130
Q

Fever cycle P vivax and ovale

A

Fever every other day

131
Q

Anemia d/t underproduction

A

Decreased production of RBC by bone marrow

Low corrected RC

Microcytic and marcocytic anemia
Renal failure
Damage to bone marrow precursor cells

132
Q

Parvo virus

A

Temporary halts erythropoiesis
Significant anemia in preexisting marrow stress
Tx; supportive

133
Q

Aplastic anemia

A

Damage to HSC
Pancytopenia w/ low RC

Drugs/chemicals, viral, AI damage

Biopsy reveals empty, fatty marrow

134
Q

TX aplastic anemia

A 
Cessation of causative drugs
Transfusions
Marrow stimulating factors (EPO, GM-CST, G-CSF)
Immunosuppression
BMT (last resport)
135
Q

Myelophthisic process

A

pathologic process replaces bone marrow

Pancytopenia

pathoma (12 decks)

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