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Emma Heme/Onc > HypERproliferative Anemias, Thalassemia, Sickle Cell Anemia, Blood Bank/Transfusion Medicine > Flashcards

HypERproliferative Anemias, Thalassemia, Sickle Cell Anemia, Blood Bank/Transfusion Medicine Flashcards

1
Q

hypERproliferative anemias

key features

A

low Hb and HCt
elevated reticulocyte index
inc absolute reticulocyte count (reticulocyte % * total rbc)

anemia w/ appropriate/exaggerated EPO response but unable to fully compensate

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2
Q

2 major conditions leading to hyperproliferative anemias

A
  1. blood loss thru bleeding

2. functional “blood loss” via hemolytic anemia

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3
Q

Free plasma Hb released by intravascular hemolysis is bound by a Hb scavenging protein called ______

A

haptoglobin

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4
Q

haptoglobin levels during intravascular hemolysis

A

decreased

haptoglobin binds free Hb

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5
Q

extravascular hemolysis

A

in spleen or liver

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6
Q

congenital hemolysis causes

membrane disorders

A

spherocytosis

elliptocytosis

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7
Q

congenital hemolysis causes

enzyme disorders

A

G6PD deficiency

pyruvate kinase deficiency

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8
Q

congenital hemolysis causes

Hb disorders

A

Sickle cell anemia

Hb CC EE

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9
Q

acute hemolysis clinical pic

A 
pallor
fatigue
dyspnea
icterus
jaundice
splenomegaly
dark urine
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10
Q

chronic hemolysis clinical pic

A 
pigment gallstones
skin ulcers
aplastic crises (parvovirus, severe anemia, reticulocytopenia)
CHF
folic acid deficiency
inc urine urobilinogen
hemoglobinuria
hemoglo binemia
urine hemosiderin
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11
Q

hereditary spherocytosis

A
  • most comm inherited membrane defect
  • usu AD, some AR
  • defects in spectrum, ankyrin, band 3, pallidin (band 4.2)
  • effects on vertical interactions between membrane skeleton/lipid bilayer
  • dec membrane deformability
  • loss of membrane thru RES
  • biconcave –> SPHERE
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12
Q

hereditary spherocytosis dx

suggestive

A
  • inc reticulocyte count
  • inc MCHC
  • spherocytes on peripheral smear
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13
Q

hereditary spherocytosis dx

diagnostic

A

lower EMA binding (eosin-5-maleimide)

abnml osmotic fragility test

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14
Q

aplastic crises

A

due to a temporary drop in hematocrit and reticulocytopenia with inability of bone marrow production to compensate for spherocyte destruction in the RES

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15
Q

hereditary spherocytosis clinical pic

A

hyper hemolytic crises (inc anemia and jaundice due to infections)

aplastic crises

gallstones

splenomegaly

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16
Q

___ supplementation in hereditary spherocytosis is necessary

A

folic acid

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17
Q

hereditary elliptocytosis

A
  • defective spectrin, protein 4.1, glycophorin C
  • effects HORIZONTAL interactions between cytoskeletal proteins
  • AD
  • mild hemolytic anemia (milder than AS)
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18
Q

Which is characterized by a milder hemolytic anemia?

hereditary elliptocytosis or hereditary spherocytosis

A

hereditary elliptocytosis

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19
Q

G6PD deficiency

A
  • XL, many mutations
  • african variant (A-) is milder and only older rbc are env deficient
  • mediterranean variant: all RBCs deficient
  • comm cause of neonatal jaundice
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20
Q

hexose monophosphate shunt

A

G6P –> 6 phosphogluconate

prod NADPH, reduced glutathione

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21
Q

consequences of impaired pesos monophosphate shunt

A
  • inc intracellular free radicals/peroxides
  • oxidative membrane damage
  • MetHgb
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22
Q

G6PD deficiency

dx

A
  • acute intravascular hemolytic episodes –> hemoglobinemia
  • ghost cells/bite cells
    • Heinz bodies (oxidized Hb aggregates)
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23
Q

Heinz bodies

A

oxidized Hb aggregates

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24
Q

oxidant triggers in G6PD

A 
sulfa drugs
anti-malarial: chloroquine, primaquine
nitrofurantoin
aspirin
misc drugs (pyridium)
mothballs
FAVA beans
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25
Q

common enzyme defect in embden-meyerhof pathway

A

pyruvate kinase

–> RBC hemolysis

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26
Q

pyruvate kinase deficiency

A 
AR
defective ATP production --> cell rigidity
rbc hemolysis
splenomegaly
jaundice

dx: enzyme assay
tx: rbc transfusions/splenectomy

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27
Q

warm AI hemolytic anemia

A 
IgG Abs (comm against Rh antigens) 
rbc agglutination @ warm temps

generally extravascular hemolysis with spleen removing Ab coated (opsonized) rbc

tx: underlying cause, immunosuppression, rbc transfusions if severe anemia, splenectomy

causes:
idiopathic 
malignancy (lymphoma, multiple myeloma)
rheumatologist disease
drugs (methyl-dopa, quinidine, PCN)
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28
Q

cold AI hemolytic anemia

A

IgM Abs –> fixes COMPLEMENT on rbc surface
rbc agglutination in cold

hemolysis=intravascular and extravascular

tx: transfusion dep on sx, avoid cold exposure, warm blood prior to transfusion, steroids/splenectomy NOT effective, plasmapheresis, rituximab (modulate B cell activity), ID and tx underlying cause

causes:
idiopathic
infections (mycoplasma/EBV/HIV)
malignancy (lymphomas or solid tumors)

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29
Q

Ig fixes complement on rbc surface

A

cold AIHA

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30
Q

direct antiglobulin (Coombs) test

A

detects AutoAbs and/or complement on rbc surface

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31
Q

indirect antiglobulin (Coombs) test

A

detects Abs against rbc in serum

exp. alloAbs in hemolytic disease, transfusionrxns, autoAb in severe AIHA

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32
Q

splenectomy is effective in warm or cold AIHA?

A

warm

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33
Q

____ is considered a hapten – a non-pro- tein molecule that does not elicit an antibody response by itself but reacts with an antibody when bound to a carrier protein

A

penicillin

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34
Q

innocent bystander AIHA exp

A

Quinidine

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35
Q

AutoAb

AIHA exp

A

methyldopa, fludarabine

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36
Q

march hemoglobinuria

A

rbc damage via constant pounding

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37
Q

rbc hemolysis infectious causes

A

malaria
martonellosis
babesiosis
clostridium perfrinigens

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38
Q

____ toxin contains phospholipase that directly lyses rbc

A

clostridium

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39
Q

microangioapthic hemolysis

A

rbc membrane damage as rbc traverses abnml surfaces or fibrin network deposited on endothelium

smear: schistocytes

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40
Q

microangioapthic hemolysis causes

A 
hemangioma
artificial valves
DIC
TTP
HUS
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41
Q

paroxysmal nocturnal hemoglobinuria

A

acquired clonal disorder of BM stem cells

mut in PIG-A gene

deficient synthesis of GPI membrane anchor

absence of GPI linked proteins (CD59, CD55 (DAF))
^rbc becomes sensitive to complement lysis

assoc w/ thromboses, aplastic anemia, muscle dystonia (bowel spasms), pulm HTN

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42
Q

Hgb

A

lg protein
2 alpha and two beta–> tetramer
each global chain covalently linked to heme moiety

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43
Q

heme

A

ferrous iron and protoprophyrin

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44
Q

most global synthesis occurs from which gene?

A

alpha 2

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45
Q

alpha globin, which chromosome?

A

16

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46
Q

beta global cluster, which chromosome?

A

11

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47
Q

expression of each globin cluster is regulated by

A

upstream locus control region (LCR)

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48
Q

when do alpha and gamma globin gene expression peak?

A

month 3 gestation

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49
Q

HbF

A

alpha2

gama 2

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50
Q

HbA

A

alpha 2

beta 2

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51
Q

HbA2

A

alpha 2

delta 2

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52
Q

__ globin gene is turned on at birth and persists at low level into adulthood

A

delta

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53
Q

__ globin gene expression is down regulated at birth and Hb F levels decline by _ mo

A

gamma

HbF declines by 6 mo.

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54
Q

Hb fxn

A

sequential binding of single O2 to each of 4 heme groups

  • sigmoid, cooperativity
  • lg O2 release w/ small dec in O2 tension
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55
Q

R shift –> inc O2 unloading

causes

A

low pH tissues
hyperthermia
inc tissue 2,3-BPG
low O2 affinity Hb (sickle Hb)

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56
Q

L shift –> inc O2 loading

A

high pH lungs (CO2 elimination)
high affinity Hb (HbF, Fe3+ Hb)
CO poisoning

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57
Q

hemoglobinopathy

A

alpha/beta globin mut that lead to structural or QUALITATIVE change in Hb

sickle gene, Hb C

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58
Q

thalassemia

A

QUANTITATIVE disorder of globin synthesis

mut in globin chains of Hb –> dec prod of globin chains from affected allele

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59
Q

thalassemia: deficient chain synthesis –> imbalance between alpha and B chains –>

A

precipitation of insoluble homo-tetramers of chain in xs

red cell hemolysis, dec rbc life span, ineffective erythropoises, microcytosis, Fe overload

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60
Q

most comm abnormality of Hb

A

thalassemia

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61
Q

clinical classification of alpha thalassemia

A
  • hydrops fetalis
  • HbH disease
  • alpha thalassemia trait/minor
  • alpha thalassemia minima (silent alpha thalassemia)
62
Q

hydrops fetalis

A

deletion of 4 alpha globin genes, incompatible w/ life and designated (–,—)

63
Q

HbH disease

A

deletion of 3 alpha globin genes (-alpha/–)

can be severe phenotype

64
Q

alpha thalassemia trait/minor

A

deletion of 2 alpha globin genes
mild anemia

cis (aa/–) deletions on same haploid chromosome

trans (alpha-/alpha-) on alternate chromosomes

65
Q

alpha thalassemia minima (silent alpha thalassemia)

A

deletion of 1 alpha globin gene (alpha-/alpha,alpha)

micocytosis but no anemia

66
Q

deletion alpha-thalassemia mutations

A

deletions that remove all or part of the alpha globin gene cluster

deletions of 1 of the 2 alpha genes leave a functional gene on haploid chromosome

67
Q

non-deletional alpha-thalassemia mutations

A

less common than deletion mut in alpha allele

point mutations that inactivate alpha globin

substitutions in alpha 2 globin termination codon (asia)

68
Q

HbH

A

adults
beta tetramers precipitate in rbc, inc affinity
can’t release O2 to tissues

69
Q

Hb Barts

A

gamma tetramers in utero
can’t release O2 to tissues
hydrops fetalis

70
Q

hydrops fetalis

A

abnormal accumulation of fluid in multiple fetal compartments, including around the heart, lungs, abdominal cavity, skin
Hb Bart’s

71
Q

beta thalassemia major

A

Cooley’s anemia

  • very severe
  • transfusion dependency
  • homozygous, complete loss of beta globin gene expression
72
Q

beta thalassemia intermedia

A

moderately severe
not transfusion dependent
mut of both globin alleles but quantity of normal HbA prod is greater than Thal major

73
Q

beta thalassemia minor

A

clinically mild/silent

mut of single beta Thal allele

74
Q

beta thalassemia mutations

A

point mutations that down-reg/abolish either transcription of globin genes/translation of globin mRNA

75
Q

beta thalassemia pathophysio

A

b-globin chain deficiency
–> xs of alpha globin chains –> inc minor Hb species –> alpha tetramers precipitate in rbc precursors, damaging cell membranes –> INEFFECTIVE ERYTHROPOIESIS

peripheral hemolysis –> splenomegaly from work hypertrophy

76
Q

complications of beta-thalassemia major

A

-severe anemia
-chronic hemolysis
-bone deformities (inc BM space) includes frontal bossing/cortical thinning
-hepatosplenomegaly
-infection risk
transfusion Fe overload w end organ damage
-high output HF

77
Q

combined hemoglobinopathy and thalassemia

A

> 50 mut that can result in a variant non-A Hb and thalassemia phenotype

  • Hb Constant Spring
  • delta/beta fusion (lepore hemoglobins)
78
Q

Hb E

A

one of the most common mutations worldwide

HbE=variant Hb, activates cryptic mRNA splice site –> reduced synthesis of beta-E globin chain –> thalassemia phenotype

AE (E trait) –> resistance to plasmodium falciparu

Indian subcontinent, SE China, Thailand, Laos, Cambodia, Malaysia, Sri Lanka

79
Q

Thalassemia dx - CBC

A
  • low Hb and HCt
  • low MCV or microcytosis ABSOLUTE REQ
  • high rbc (>1/3Hb val)
80
Q

absolute requirement of thalassemia dx

A

low MCV or mirocytosis

81
Q

Thalassemia dx - smear

A

small, hypo chromic rbc

polychromasia (inc reticulocytosis), basophilic stippling

variable cell shape (poiklocytosis, rbc fragments, target cells)

82
Q

Fe studies - Thalassemia

A

WNL

83
Q

basophilic stippling

A

hemolytic anemias

rRNA deposits from toxic injury or globin chain deposition

84
Q

Hb electophoresis for dx of thalassemia

A

thalassemia=quantitative disorder so Hb bands migrate at normal rate thru gel

HbH=

beta-thalassemia=HbA2 and HbF inc bc xs

85
Q

Hb electophoresis

alpha-thalassemia

A

HbA only

86
Q

Hb electophoresis

HbH

A

fast migrating band of tetramers of beta (HbH) plus HbA

87
Q

Hb electophoresis

beta-thalassemia

A

HbA2 and HbF inc bc xs alpha chains form tetramers w/ delta and gamma globin chains

88
Q

HPLC for thalassemia dx

A

separates Hb on basis of hydrophobicity

RELIABLY QUANTITATES HbA2 and HbF fractions in beta thalassemia

89
Q

beta thalassemia major tx

A

supportive

  • rbc transfusion from birth
  • folic acid
  • Fe chelation
  • hepatitis vaccination
  • endocrine replacement

disease modifying

  • BM transplant
  • gene therapy
90
Q

sickle cell disease

A

AD co-dominance
-single base pair substitution in beta-globin gene of Hb

(A–>T, glutamic acid –> valine)

91
Q

HbS

A

variant Hb (biochemical change)

92
Q

sickle cell trait

A

most benign

  • HbA protects cells from assuming sickle shape
  • NO anemia, hemolysis, pain crises
  • risk of splenic infarct
  • risk of sudden death w/ extreme dehydration/hyperthermia
  • hyphema
  • isothenuria, hematuria, pyelonephritis (preg)
  • medullary CA of kidney
93
Q

sickle cell trait - primary clinical impact

A

child-bearing

94
Q

sickle cell disease manifests at what age?

A

6mo

95
Q

sickle cell disease modifiers

A

tissue oxygenation status
pH
HbF levels
co-inheritance of other Hb gene abnml

96
Q

heterozygous inheritance of HbS offers some protection against

A

malaria

97
Q

SCD epidemiology

A

African Amer

Hispanic Amer

98
Q

Sickle trait epidemiology

A

8% african-amer

99
Q

sickle cell pathophys

–> RBC hemolysis

A

RBC deoxy –> polymerization of HbS (dec solubility) at low O2 tension –> rbc membrane damage

cellular dehydration –> inc cell [HbS], Ca activated K+ (Gardos) channels –> deoxygenation-induced Na-K pump –> oxidative membrane damage –> K loss –> RBC hemolysis

100
Q

sickle cell pathophys

–> vasoocclusion/pain

A

sickle rbc adhere to vascular endothelium –> activation of coag/inflamm –> inc blood viscosity –> occlusion in microvasculature –> no scavenging by release of free intravascular Hb –> tissue ischemia, PAIN

101
Q

HbS hemolysis, intravascular or extravascular?

A

both intravascular or extravascular

102
Q

rbc survival in sickle disease

A

shortened to 10-16 days

epo can’t fully compensate in severe hemolysis

103
Q

vasocclusive phenomena in sickle cell disease

A

rigid banana shaped cells

104
Q

sickle disease

labs

A 
Hb- low
wbc/platelets-normal or elevated 
MCV:
-normal-low in HbSS
-low in sickle-thalassemia (S-beta thal)

classic markers of (intrinsic) hemolysis

  • elevated reticulocyte count
  • low haptoglobin (binds free Hb)
  • elevated total/indirect bilirubin
  • elevated LDH
105
Q

sickle solubility test

A

deoxygenated HbS is insoluble in presence of concentrated phosphate buffer solution –> forms a turbid suspension

used as screening test (can’t distinguish btwn genotypes)

106
Q

Hb electrophoresis to dx sickle disease

A

on cellulose acetate (alkaline pH), Hb migrates from - to +

fast>slow
HbA>HbF>HbS>HbC

107
Q

citrate agar (acid pH)

A

separates HbS from HbD and HbG, and separates HbC from HbA2 and HbE

108
Q

HPLC

A

sep Hb based on hydrophobicity

allows quantification of HbS

109
Q

sickle cell complications

multi-organ effects

A
  • chronic hemolytic anemia
  • chronic pain syndrome
  • chronic leg ulcers
  • endocrinopathies (growth retardation, hypOgonadism)
  • bone disease (dactylics, AVN, osteomyelitis)
  • ocular (proliferative retinopathy, retinal detachment)
  • sensorineural hearing loss
  • pregnancy: PERILOUS, HIGH MORBIDITY/MORTALITY
  • pulmonary (ACS, pulmonary HTN)
  • cardiac (high output HF)
  • stroke/renal a occlusion
  • renal (proteinuria, sickle nephropathy, renal fail, acute papillary necrosis)
  • infections
  • priapism (sustained penile erection)
  • pigment gall stones
110
Q

acute sickle cell crises

A

acute pain (vasoocclusive) crisis

hemolytic crisis

aplastic crisis (erythroid aplasia, often via parvovirus B19)

sequestration crisis (pooling of erythrocytes in spleen w/ acute splenomegaly/shock-like state)

111
Q

acute dactylics in SCD

A

often first sx of SCD babies

presents 6-18 mo as HbF dec

112
Q

acute chest syndrome

A

most common lung complication in sickle disease

freq cause of death in adults

new pulm infiltrate, pleuritic CP, fever >38.5, dyspnea/tachypnea/wheezing/cough

repeated episodes –> pulm HTN, R HF (cor pulmonale)

unclear etiology, pulm infarct/pulm fat embolism/infectious pneumonia –> 1/2 of cases

113
Q

cause of pulm HTN in sickle cell disease

A

NO scavenging by free Hb chains in serum –> inc vascular tone in pulm bed

114
Q

sickle vasculopathy

A

hemolytic anemia –> free Hb in circulation (potent inhibitor of all NO bioactivity –> EC dysfuction, NO resistance)

ferric heme = toxic to endothelium

inc endothelin-1 prod (vasoconstriction)

115
Q

stroke in SCD

A
  1. ischemic infarcts

2. hemorrhagic stroke (adults over 29, formation of MOYAMOYA vessels in brain

116
Q

intracranial hemorrhage in SCD tx

A

aggressive hydration,RBC transfusion/exchange
Nimodipine
neurosurgery - evacuation; aneurysm repair as needed

117
Q

acute pain crisis tx in SCD

A

narcotics/non-narcotics

fluids

O2 support for hypoxia

low threshold for abx

incentive spirometer

118
Q

acure chest syndrome in SCD

A 
O2 support 
abx
inhaled bronchodilators, incentive spirometer
transfusion
mechanical ventilation
inhaled NO
hydrocyurea
119
Q

hydroxyurea

A

anti-neoplastic

shown to decrease the frequency of vasoocclusive crises and acute chest syndrome, reduce the transfusion requirement, decrease chronic pain, and improve survival

MOA:

  • inc HbF
  • inc Hb
  • dec wbc, platelets
  • attenuates hemolysis
  • improves QOL, survival

AVOID IN PREGNANCY

120
Q

ischemic stroke risk management risk SCD

A

chronic exchange transfusion if velocity >200 m/s

no drug therapy

121
Q

indications for rbc transfusion in SCD

A

acute chest syndrome
stroke prophylaxis

complications: Fe overload

122
Q

L-glutamine

A

SCD management

dec intracellular oxidante stress
dec pain crises

123
Q

after collection of whole blood from donor, _____ is completed, then further separation

A

LEUKODEPLETION

reduced risk of alloimmunization, CMV infection, febrile transfusion rxns

124
Q

blood bank TTD

A 
HIV
Hep B/C
Human T-lymphotrophic Virus (HTLV) types I & II 
Trypanosoma cruzi (Chagas)
West Nile virus
125
Q

shelf life of rbc, platelets

A

rbc=42d
platelets=5d

*req constant agitation

126
Q

most common antigens (ABO)

A

O>A>B>AB

127
Q

ABO Abs

A

naturally occurring

present at birth in absence of transfusion

mix of IgM and IgG against A or B

128
Q

Rh blood group

A

D, C, E, c, e

129
Q

newborn hemolytic disease

A

Rh- (D-) mother is exposed to Rh+ (D+) and development anti-D Abs (IgG) which can cross placenta

hemolysis, anemia, CHF in fetus –> hydrops fetalis and fetal death

prevention: Anti-D Ig (binds any circulating fetal RBCs that are Rh+ to prevent the mother from making Abs to these RBCs)

130
Q

in addition to mom developing antibody to D antigen, also see

A

Rh incompatibility due to E antigen

131
Q

direct Coombs test

A

determines whether there is IgG or IgM coating the patient’s RBCs by adding antihuman Abs to a patient’s RBCs

132
Q

indirect Coombs test

A

starts with the patient’s serum

RBCs with known antigens are added to the patient’s serum

Anti-human Its added to solution

clumping=pt’s serum contains Ig against antigens on donor RBCs

133
Q

indications for transfusion of platelets

A

prophylactically w/ -severe thrombocytopenia (platelet count <10,000)
-bleeding

134
Q

platelet compatibility rules

A

platelets do not need to be transfused according to rules of ABO or Rh compatibility

135
Q

Alloimmunization to ___ antigens can cause refractoriness to platelet transfusions

A

HLA

136
Q

fresh frozen plasma contains

A

coagulation proteins needed for thrombus formation/fibrinolysis

also Abs (need to transfuse according to ABO compatibility)

137
Q

indications for FFP transfusion

A

deficiencies of coagulation proteins w/ unavailable specific concentrates/unknown specific fx deficiency

emergent reversal of warfarin (interferes w/ vitamin K salvage)

138
Q

precipitate left after thawing FFP

A

cryoprecipitate

139
Q

cryoprecipitate components

A

VIII
vW antigen
XIII
fibrinogen

140
Q

primary use of cryoprecipitate in transfusion

A

bleeding pt w/ low fibrinogen

141
Q

2 types of VIII concentrates

A
  1. plasma derived (contains vWF)
  2. recombinant (does NOT contain vWF, no risk of viral transmission)

-used prophylactically in hemophilia A to prevent bleeds

142
Q

prothrombin complex concentrates

A

FEIBA
KCENTRA

use: rapid control of bleeding in patients on vitamin K antagonists or in patients with hemophilia AND those w/ acquired inhibitors to specifc factors

risk of thrombosis

143
Q

FEIBA

A

factor VIII, IX, X, proteins C/s

144
Q

albumin

A

used as volume expander in critically ill patients w/ hypOalbuminemia/capillary leak

cotroversial/expensive

145
Q

IVIG

A

replacement of IgG in immunicomprimised patients

-ITP, Kawasak

146
Q

recombinant factor VIIa

A

used to treat hemophilia pts w/ bleeding who have inhibitors to VIII or IX, or congenital VII deficiency

147
Q

leukodepletion renders blood product “ CMV ____ “

A

leukodepletion renders blood product “ CMV SAFE “

148
Q

“CMV negative” blood

A

only blood donated from a donor known to be CMV negative

149
Q

transfusion-related acute lung injury (TRALI)

A

dyspnea, hypoxia, tachycardia, fever, hypOtension

non-cariogenic pulmonary edema

tx: supportive care

dx of exclusion

150
Q

transfusion-associated circulatory overload (TACO)

A

occurs in pt w/ pre-existing congestive HF

dyspnea, tachycardia, HTN, vol overload

Emma Heme/Onc (16 decks)

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