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Heme II > Anemias > Flashcards

Anemias Flashcards

1
Q

MVC

A

mean cell volume

80-100

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2
Q

mean cell hemoglobin

A

27-33

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3
Q

mean cell hemoglobin concentration

A

33-37

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4
Q

hemoglobin

A

M- 13.6-17.2

F- 12-15

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5
Q

hematocrit

A

M- 39-49

F- 33-43

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6
Q

red cell count

A

M- 4.3-5.9

F- 3.5-5

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7
Q

reticulocyte count

A

.5-1.5

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8
Q

RDW

A

11.5-14.5

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9
Q

general changes of anemia

A

pale, weak, malasie, easy fatiguability
dyspnea on mild exertion
Hypoxia -> fatty changes of liver, myocardium, and kidney -> severe cardiac failure -> worse hypoxia

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10
Q

acute blood loss

A

low hematocrit b/c diluted w/interstitial fluid
increased erythropoietin -> 5 days for increased retic
leukocytosis due to adrenergic compensatory response
initially normocytic, normochromic -> macrocytic, chromatophilic
early recovery thrombocytosis

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11
Q

hemolytic anemia general characteristics

A

life span <120
elevated erythropoietin and increase in erythropoiesis
accumulation of hemoglobin degredation products

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12
Q

types of hemolytic anemias

A 
hereditary spherocytosis
G6PD deficiency
thalassemias
sickle cell
paroxysmal noctural hemoglobinuria
hemolytic disease of newborn
transfusion reactions
drug induced
autoimmune 
HUS
DIC
TTP
malaria, babeosis
hypersplenism
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13
Q

extravascular hemolysis

A 
anemia
splenomegaly
jaundice (unconjugated) -> gallstones
decreased haptoglobin
often benefit from splenectomy
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14
Q

intravascular hemolysis causes

A 
less common
mechanical injury (prosthetic valves, repetitive physical injury)
C' fixation
intracellular parasites (malaria)
exogenous toxins (clostridial sepsis)
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15
Q

intravascular hemolysis clinical

A 
anemia
hemoglobinemia
hemoglobinuria
hemosideriuria
jaundice (unconjugated) -> gallstones
decreased haptoglobin
increased methemoglobin -> red brown urine
renal hemosiderosis
no splenomegaly
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16
Q

HS

A 
hereditary spherocytosis 
red cells are spheroid
highest in northern Europe
autosomal dominant 75%
25% of compound heterozygosity -> more severe present at birth
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17
Q

HS spectrin

A

normal RBC skeleton made of spectrin
HS caused by frameshift mutations in: ankyrin, spectrin, Band 3, Band 4.2
life span 10-20 days

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18
Q

HS morphology

A 
spherocytosis- small hyperchromic red cells
marrow 
marrow erythroid hyperplasia
hemosiderosis
mild jaundince
cholelithiasis 
moderate splenomegaly
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19
Q

HS clinical

A

abnormally sensitive to osmotic lysis
increased MCHC
anemia, splenomegaly, jaundice
20-30 asymptomatic

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20
Q

HS aplastic crises

A

usually triggered by parvovirus

transfusions may be necessary

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21
Q

HS hemolytic crises

A

infectious mono

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22
Q

HS Tx

A

splenectomy

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23
Q

G6PD deficiency

A 
recessive X-linked (males higher risk)
G6PD- and mediterranean subtypes
protective against malaria
episodic episodes of hemolysis due to oxidant stress (self limited b/c only older cells at risk)
intra and extravascular hemolysis
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24
Q

sources of oxidant stress

A

infectious most common (hep, pneumonia, typhoid)
Drugs (antimalarials, sulfonamides, nitrofurantoins)
fava beans

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25
Q

G6PD morphology

A

heinz bodies - dark inclusions on crystal violet stain

bite cells/spherocytes

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26
Q

sickle cell disease

A

point mutation in beta hemoglobin (glu -> val) -> polymerization of deoxygenated hemoglobin -> sickling -> hemolysis and microvascular obstruction

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27
Q

sickle cell protection against malaria

A

increased extravascular hemolysis -> clears infection

intravascular- due to impaired formation of membrane knobs containing viral PfEMP-1 protein (cerebral malaria)

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28
Q

HbF and sickle cell

A

inhibits polymerization of HbS, therefore infants not symptomatic until 5-6months
people with hereditary persistence of HbF less severe disease

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29
Q

HbSC disease

A

heterozygotes for HbC/HbS
HbC common in Africa
increased sickling

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30
Q

factors that affect sickling

A

MCHC- decreased MCHC less sickling therefore patients w/coexisting thalassemias have less sever disease
intracellular pH- decreased pH worse sickling
transit time- slower more obstruction

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31
Q

microvascular occlusion in sickle cell

A

dependent on red cell membrane damage, not number o sickled cells
also attributed to depleted NO

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32
Q

morphology of sickle cell

A

target cells
howell-jolly bodies
bone marrow hyperplastic -> bone resporption and reformation -> crewcut skull
splenomegaly as child -> autosplenectomy as adult

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33
Q

sickle cell morphology

A

hematocrit 18-30%
reticulocytosis, hyperbilirubinemia, sickled cells
vaso-occlusive/pain crises
acute chest syndrome
priapism
stroke and retinopathy (depletion of NO)
occlusions most common cause of morbidity and mortality

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34
Q

vaso-occlusive/pain crises in sickle cell

A

infection, dehydration, and acidosis can trigger, but most have no predisposing cause
bone, lungs, liver, brain, spleen, penis
bone most common in kids -> hand-foot syndrome/dactylitis

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35
Q

acute chest syndrome

A

particularly dangerous involving the lungs, present w/fever, cough, chest pain, and pulmonary rales
usually when infections slow blood flow

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36
Q

other occlusive effects of sickle cell

A

priapism
stroke and retinopathy (depletion of NO)
occlusions common cause of morbidity and mortality
general chronic tissue hypoxia
generalized impairment of growth and development and organ damage

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37
Q

sequestration crises

A

kids w/intact spleen
rapid splenic enlargement, hypovolemia, and sometimes shock
may be fatal

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38
Q

aplastic crises

A

infection of red cells w/parvovirus B19

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39
Q

sickle cell infections

A

increased risk to capsulated organisms due to decreased splenic fnx, defects in alternative C’
S. pneumoniae, H. influenza, meningitis

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40
Q

Dx of sickle cell

A

culture cells w/metabisulfite which consumes oxygen

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41
Q

Tx of sickle cell

A

hydroxyurea

HSC transplant

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42
Q

alpha chain

A

on chrom 15

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43
Q

beta chain

A

on chrom 11

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44
Q

thalassemias endemic in

A

mediterranean basin, middle east, tropical aftrica, india, and asia

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45
Q

beta mutations

A

B0- absent beta synthesis
B+- reduced beta globin synthesis
100 different mutations, mostly point

46
Q

mechanisms of anemia in beta thalassemia

A

1) deficit in HbA synthesis -> underhemoglobinized, hypochromic, microcytic red cells -> submornal O2 delivery
2) diminished survival of red cells:
- unpaired alpha chains precipitate in red cell precursors -> insoluble inclusions -> membrane damage -> apoptosis -> this happens to 80% of red cell precursors
- red cells which are released still have inclusions and damage and cleared by spleen
- massive erythroid hyperplasia in bone marrow -> impairs bone growth -> crew cut
- extramedullary hematopoiesis creates higher O2 demand for already oxygen starved tissues

47
Q

beta thalassemia major

A 
severe, transfusion depended anemia
have 2 mutated Beta alleles
anemic by 6-9months
hemoglobin levels 3-6
elevated HbF
HbA2 sometimes high
48
Q

beta thalassemia minor

A

only 1 allele

mild asymptomatic microcytic anemia

49
Q

beta thalassemia major morphology

A 
microcytosis and hypochromic red cells
target cells, basophilic stippling, and fragmented cells
inclusions of aggregated alpha chains efficiently removed by spleen so usually NOT seen 
retics elevated
expansion of active marrow
crew cut appearance on xray
splenomegaly
Fe overload
50
Q

beta thalassemia major clinical

A

course is short if untreated
suffer from growth retardation and die at early age
hepatosplenomegaly
crewcut
cardiac disease due to Fe overload common COD
HSC transplant only cure

51
Q

beta thalassemia minor

A

usually asymptomatic
anemia if present mild
hypochromic, microcytic, basophilic stippling, and target cells
mild erythroid hyperplasia in bone marrow
increased HbA2
HbF normal, slightly increased
exclude Fe deficiency anemia

52
Q

alpha thalassemias

A

newborns excess gamma from tetramers -> hemoglobin Barts
older children and adults have HbH
free beta and gamma chains more soluble and from stable tetramers hemolysis and ineffective erythropoiesis less severe then in beta thalassemias
deletion mutations most common cause

53
Q

four types of alpha thalassemia

A

silent carrier disease
alpha-thalassemia trait
hemoglobin H disease
hydrops fetalis

54
Q

silent carrier disease

A

only 1 mutated alpha alelle

completely asymptomatic

55
Q

alpha thalassemia trait

A

2 alleles mutated

asymptomatic

56
Q

hemogloin H disease

A

deletion of 3 genes
HbH tetramers -> extremely high affinity for O2 don’t deliver it
HbH prone to oxidation -> precipitate and form intracellular inclusions -> red cell sequestration by spleen

57
Q

hydrops fetalis

A

most severe form, deletion of all 4 alleles
hemoglobin barts in fetus have very high affinity for O2
fatal in utero w/o transfusions
HSC transplant only cure

58
Q

PNH

A

paroxysmal nocturnal hemoglobinuria
acquired mutations in PIGA, an enzyme essential for synthesis of membrane associated C’ regulatory proteins
rare
only hemolytic anemia caused by acquired genetic defect
x-linked and subject to lyonization (mixed normal and abnormal red cells in female)
correlated w/aplasitc anemia b/c expressed when autoimmune reactions against GPI-linked Ags occurs

59
Q

PNH mutations

A

CD55
CD59- most important
C8 binding protein
all regulate C’

60
Q

lysis in PNH

A

intravascular due to MAC

only nocturnal in 25%, maybe due to decreased pH -> more active C’

61
Q

PNH clinical

A

Fe deficient due to hemolysis
venous thrombosis is leading COD
10% develop AML or MDS

62
Q

PNH Dx

A

flow cytometry to detect cells deficient in CD59 and others

63
Q

Tx PNH

A

eculizumab blocks conversion of C5-C5a

only cure HSC transplant

64
Q

direct coombs test

A

patients red cells mixed w/sera w/Abs

65
Q

indirect coombs test

A

patients serum mixed w/red cells expressing specific Ags

66
Q

immunohemolytic anemias

A

aka autoimmune hemolytic anemias

Dx requires detection of Abs or C’ on red cells via direct coombs test -> agglutination

67
Q

immunohemolytic anemia types

A

warm body Ab type
cold agglutinin type
cold hemolysin type

68
Q

warm body Ab type

A 
most common
50% idiopathic
IgG (can be IgA)
extravascular hemolysis
moserate splenomegaly
many Abs against Rh
69
Q

drug induced warm Ab type

A

antigenic drugs: penicillin and cephalosporins
tolerance breaking drugs: antiHTN alpha-methyldopa
1-2 weeks post exposure to IV drug infusion

70
Q

cold agglutinin type

A

IgM that binds red cells at low temp
sometimes post infection w/M. pneumoniae, EBV, CMV, influenza virus, HIV, or mycoplams
self limited
chronic occurs in certain B-cell neoplasms or as an idiopathic condition

71
Q

cold hemolysin type

A 
IgG bind P blood grp Ags
paroxysmal cold hemoglobinuria
rare, sometimes fatal
seen in kids post viral infections
transient most recover w/in 1 month
72
Q

hemolytic anemia due to trauma

A

cardiac valve prosthetics
microangiopathic hemolytic anemia most often seen w/DIC, but also occurs in TTP, HUS, maligant HTN, SLE, and disseminated CA
Red cell fragments -> schistocytes
burr cells, helmet cells, triangle cells

73
Q

megaloblastic anemia

A

lack central palor and are hyperchromic, but MCHS is not elevated
neutrophils also large then normal w/hypesegmentation of nucleus

74
Q

pernicious anemia

A

autoimmune gastritis that impairs production of IF
more prevalent in scandinavian and other caucasian populations
median age 60, rare <30

75
Q

Vit B deficiency

A

impairs DNA synthesis due to reduced availabiltiy of FH4

76
Q

Abs in pernicious anemia

A

Type I- blocks binding of vit B to IF
type II- prevent binding of IF to IFR
type III- most common, recognize alpha and beta subunits of gastric proton pump

77
Q

other disorders associated w/B12 deficiency

A 
achlorhydria and loss of pesin
gastrectomy
loss of exocrine fnx
Ileal resection or diffuse ileal disease
tapeworms
pregnancy, hyperthyroidism, disseminated CA, chronic infection all increase demand
78
Q

vit B12 deficiency morphology

A

intestinlization of stomach
atrophic glosstitis
CNS changes -> demylinating of dorsal spinal tracts

79
Q

vit B12 deficiency clinical

A

moderate to sever megaloblastic anemia
leujopenia w/hypersegmented granuocytes
low serum B12
elevated serum homocysteine and methmylmalonic acid
rise in hematocrit 5 days post parenteral B12
serum Abs to IF
elevated homocysteine -> cardiac risk factor

80
Q

anemia of folate deficiency

A

megaloblastic appears same as B12

humans entirely dependent on dietary source

81
Q

reactions dependent on folic acid

A

purine synthesis
conversion of homocysteine to methionine
dTMP synthesis (can be inhibited by drugs) required for DNA synthesis

82
Q

risks for folate deficeincy

A 
chronic alcoholics (also trapped in chirrhotic liver)
very old
sprue and diffuse disease of small intestines (lymphoma)
83
Q

drugs associated w/folate deficiency

A

phenytoin

oral contraceptives

84
Q

relative folate defiencies

A

pregnancy
infancy
hyperactive hematopoiesis
disseminated CA

85
Q

folic acid anatgonits

A

methotrexate

86
Q

folic acid clinical

A

serum homocysteine increased
methymalonate concetrations normal
no neuro changes

87
Q

Fe deficiency

A

in US most common in toddlers, adolescent girls, and women of childbearing age

88
Q

normal Fe absorption

A

Ferric(3) -> ferrous (2) via ferrireductase
transported across apical membrane via DMTA
basolateral membrane via ferroportin-> ferrous -> ferric binds tranferrin (usually 2/3 saturated)

89
Q

hepcidin

A

regulates Fe absorption by blocking ferroportin

small circulating peptide from liver

90
Q

Fe absorption inhibited by

A

tannates, carbonate, oxalates, and phosphates

91
Q

morphology of Fe deficeincy

A

microcytic, hypochromic

disappearance of stainable Fe from macros in boen marrow Pencil cells

92
Q

Fe deficiency clinical

A

kolionychia
alopencai
atrophic changes in tounge and gastric mucosa
PICA

93
Q

plummer vinson syndrome

A

esophageal webs
microcytic hypochromic anemia
atrophic glossitis

94
Q

Dx of Fe deficeincy

A 
HCT, Hgb decreased
serum Fe decreased
serum ferritin decreased
TIBC increased
hepcidin decreased
95
Q

anemia of chronic disease

A

due to systemic inflammation

most common anemia in US hospitals

96
Q

categories of anemia of chronic disease

A

chronic microbial infections- osteomyelitis, endocarditis, lung abscesses
chronic immune disorders- RA
neoplasms- carcinomas of lung, breast and HL

97
Q

anemia of chronic disease morphology

A

low serum Fe, reduced TIBC, abundant Fe stored in tissue macros
red cells usually normocytic, normochormic, if progresses can becoome hypochromic and microcytic

98
Q

anemia of chronic disease pathology

A

IL6 -> stimulates increase in hepcidin

low erythropoietin which may be due to hepcidin

99
Q

anemia of chronic disease clinical

A

anemia mild, which major symptoms being of the underlying disease

100
Q

aplastic anemia

A

syndrome of chronic primary hematopoietic failure and pancytopenia
majority of patients autoimmune mechanisms suspected
most cases of known etiology are due to drugs
can also be post viral or due to radiation
65% idiopathic

101
Q

drugs that cause aplastic anemia

A

chemo and organic benzene
chloramphenicol
gold salts

102
Q

inherited causes of aplastic anemia

A

fanconi anemia

telomerase defects

103
Q

fanconi anemia

A

rare autosomal recessive disorder due to defects in multiprotein complex necessary for DNA repair
marrow hypoplasia early in life often w/hypoplasia of kidney, spleen, and bones (thumbs and radii)

104
Q

morphology of aplastic anemia

A

markedly hypercellular bone marrow mostly fat and stroma and scattered lymphocytes
dry tap
granulocytopnia, thrombocytopenia
infections, bleeding

105
Q

aplastic anemia clinical

A 
any age, either sex
insidious
Tx- multiple transfusions, bone marrow transplant
pancytopenia
splenomegaly absent
slight macrocytic and normochromic cells
reticulocytopenia
106
Q

pure red cell anemia

A 
neoplasm (thyoma, large granular lymphocytic leukemia)
drugs
autoimmune disorders
parvovirus
immunosupressive tx usually beneficial
plasmapharesis if autoAbs
107
Q

meylophthisic anemia

A

space-occupying lesions replace normal marrow elements
most common cause is metastatic CA (breast, lung, prostate)
any infiltrative process-> granulomatous disease (TB, sarcoidosis, fungal)
spent phase of MPDs
leukoerythroblastosis and tear drop shaped red cells

108
Q

chronic renal failure

A

almost invarialby associated w/anemia proportional to severity of uremia
diminished synthesis of erythropoietin
extracorpuscular defect that reduces red cell life span and Fe deficiency due to platelet dysfunction and incereased bleeding

109
Q

hepatocellular liver disease

A

toxic, infectious, or cirrhotic
decreased marrow fnx
folate and Fe deficiencies
erythroid progenitors preferentially affected
slightly macrocytic due to lipid abnormalities

110
Q

endocrine anemina

A

hypothyroidism

mild normochromic, normocytic anemia

Heme II (7 decks)

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