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Hematology CCMLS > Anemias > Flashcards

Anemias Flashcards

1
Q

G6PD deficiency hallmarks

A 
inherited x linked
normochrom. normocyt.
heinz bodies
hemolytic signs
Spherocytes
Bite cells
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2
Q

pyruvate kinase deficiency hallmarks

A 
autosomal recessive
normochrom. normocyt.
burr cells
increased DPG, gallstones (increased bili.)
hemolytic signs
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3
Q

anemic enzyme deficencies testing

A

add glucose to sample
cells increase in hemolysis in 48 hrs.
if not corrected by glucose: pyruvate kinase
if corrected by glucose: G6PD

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4
Q

hereditary spherocytosis anemia hallmarks

A 
autosomal dominant
deficiency of spectrin (unstable mem)
hemolytic signs
normochrom. normocyt
MCHC <36%
 spherocytes,, polychromasia
osmotic fragility test
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5
Q

osmotic fragility test

A

tests RBC resistance to lysis by osmotic stress
depends on cell volume, surface area, membrane
as NaCl decre. cells take in H2O
i.e. spherocytes take in less water than normal cells before they hemolyse

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6
Q

hereditary elliptocytosis anemia hallmarks

A 
autosomal dominant inheritance
ab. mem (spectrin)
elliptocytes >25%
3 variants: common, spherocytic, stomatocytic
hemolysis indicators
mildlly incre retic
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7
Q

indicators of hemolysis

A

increased: retics, polychromasia, bilirubin, urobilinogen, LDH, jaundice
decreased: Hgb, Haptoglobin
hemoglobinemia, hemoglobinuria, hemosiderinuria
RBCs with various shapes
erythroid hyperplasia in BM: decre. M:E ratio
nRBCs
RPI>2.0

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8
Q

spur-cell anemia hallmarks

A 
severe liver disease
increased cholesterol in RBC mem
leptocytes (big thin macrocytes)
normochrom. normocyt.
hemolytic signs
acanthocytes, burr cells, target cells
ab. liver tests
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9
Q

paroxysomal nocturnal hemoglobinuria (PNH)

A

acquired idiopathic
ab. mem.
insidious onset- sensitive to pH when sleep cells lyse
BM hypoplasia
indicators of hemolysis
normochrom. normocyt.
tests: sugar water test, acid serum test, immunophenotyping

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10
Q

types of hemolysis

A

intravascular: due to Ab or compliment, trauma, toxins
see hgburia, hemosiderinuria
extravascular: RBC destruction (liver, spleen, BM)
see increas. bili w/o Hgbemia, hgburia, hemosiderinuria, urobilinogen

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11
Q

non-immune hemolytic anemia extrinsic defects

A

chemicals and drugs
venoms
infectious agents (Babesiosis, Bartonella, Clostridium, Malaria)

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12
Q

Malaria smears

A

thin smears: look @ parasites

thick smears: to ID malarial parasites

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13
Q

Abetalipoproteinemia hallmarks

A

absence of beta lipoprotein (ab. mem,)
normochrom, normocyt.
acanthocytes
maybe decreased Hgb and hemolysis maybe not

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14
Q

non-immune hemolytic anemia: physical injury

A

intravascular hemolysis
fragments, helmet cells
normochrom. normocyt

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15
Q

microangiopathic hemolytic anemia hallmarks

A

non-immune hem. anemia
underlying disorder (lesion,, artificial heart valve, TTP, DIC, burns)
indicators of hemolysis
fragments

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16
Q

HELLP syndrom

A

microangiopathic hemolytic anemia
Hemolysis, Elevated Liver enzymes, Low Platelets
3rd trimester obstetric patients
similar to TTP AND HUS

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17
Q

March Hemoglobinuria

A

microangiopathic hemolytic anemia
Extreme exercise
no frags

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18
Q

HUS

A

Hemolytic uremic syndrome
infec., GI, Shigella (toxin), E. coli 0157:H7,
acute onset: pallor, vomiting, red/black urine, renal failure, anuria
mod-severe anemia
indicators of hemolysis
decreased platelets
normochrom. normocyt
frags, burr, polychromasia
increased BUN, creatinine, casts in urine

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19
Q

immune hemolytic anemia types

A

alloimmune: develop Ab that react against factors foreign to them (transfusion, hemolytic dis. or newborn (Ag from Dad- mom Ab against)
Drug induced

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20
Q

Kleihauer-Betke stain

A

for fetal RBCs
hemolytic disease of the newborn
maternal Ab attaching to fetal RBCs w/ Ag from dad
HgbF resistant to alkaline- stain attacks mom’s cells-Hgb denatured-baby cell bright pink

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21
Q

Warm autoimmune hemolytic anemia

A 
IgG Ab
extravascular hemolysis
idiopathic 
normochrom normocytic
increased retics
spherocytes
associated w/ SLE, CLL, viral illness
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22
Q

cold autoimmune hemolytic anemia

A

IgM Ab: directed against I Ag (on every adult RBC)
extravascular hemolysis or complement lysis
idiopathic
associated w/ mycoplasma, MPD, viral
Raynaud’s phenomenon
Icreased Mcv (polychromatic cells)
corrects with higher temperatures

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23
Q

paroxysmal cold hemoglobinuria (PCH)

A

biphasic IgG Ab
Donath Landsteiner Ab (anti-p specificity)
rxn w/ RBC @ <15C, hemolysis @ body temp
DAT =
associated w/ viral, 3 syphilis, idiopathic
massive hemolysis w/ exposure to cold

24
Q

sickle cell anemia- hemoglobinopathies

A 
black population most common
inherited homozygous
valine sub for glutamic acids @ #6 beta chain 
HgbS
ppt. w/ deoxygenation- not carry O2 well
see @ 6 mon. age and older
25
Q

sickle cell anemia clinical presentation

A 
mod-severe anemia
bone changes
vaso-occlusive crises
organ/tissue infarction
necrosis
autosplenectomy
bact. infec.
thrombosis
26
Q

sickle cell anemia lab hallmarks

A

indicators of hemolysis
normochrom normocyt
target, sickle, nRBCs, basophilic stippling, Howell-Jolly bodies, polychromasia, elliptocytes, frags

27
Q

sickle cell screening test

A

see if Hgb S present
lyse RBCs and deoxygenate to prod. sickle cells
if positive can’t see print through tube

28
Q

Hemoglobin electrophoresis

A

diff. Hgb migrate @ diff. rates
cellulose acetate @ ph 8.4: anode to cathode C/A2, S/D, F, A
(a-accelerates, f-is fast, s-slow, c-crawls)
citrate agar ph 6.2: separates-anode to cathode: F, D/A2/A, origin, S, C

29
Q

sickle cell trait

A

inherited heterozygous
HgbS
usually normal until stress event
normal CBC
electrophoresis: A 50-65%, S 35-45%, F <1%
sickle cell screening tests will be + due to HgbS present

30
Q

Hemoglobin C disease- hemoglobinopathies

A

inherited homozygous
lysine for glutamic acid #6 beta chain
usually asymp./joint pain/spenomegaly
indicators of hemolysis
target cells, Hgb C crystals
Hgb electrophoresis: C >90%, slight increase in F
decreased osmotic fragility (much less fragile due to target cells)

31
Q

hemoglobin C trait

A

inherited heterozygous
asymp.
target
hgb electrophoresis: A 60-70%, C 30-40%

32
Q

Hemoglobin SC disease

A

inherited
one gene S hgb one gene C hgb
similar to SSA, splenomegaly
normochrom normocy
indicators of hemolysis
target, sickle, hgb c crystals, bizarre shaped cells
hgb electrophoresis: 50% S, 50% C slight increase F

33
Q

hemoglobin D disease

A 
inherited
glutamine for glutamic acid @ 121
asymp., Punjab/LA
target cells
hgb electro. Hgb D (citrate agar to separate from S)
34
Q

hemoglobin E disease

A 
inhertied
lysine for glutamic acid @ 26
oriental, 3rd most prevalent, asymp
target cells
hgb electro. increase hgb E
35
Q

unstable hemoglobins- hemoglobinopathies

A 
aa mutations that alter hgb stability
inherited, spontaneous, drug 
extravascular hemolytic anemia
form sufhgb and methgb
indicators of hemolysis
normochrom normocyt
frags (bite cells), Heinz bodies
ab heat instability test
36
Q

beta thalassemia major

A 
both beta genes affected
b*/b*, b+/b*, b+/b+
excess alpha chains- ppt
severe anemia, cardiac failure (die by 20), BM hyperplasia, extramedullary hematopoiesis
decreased hgb
indicators of hemolysis
4+poikilocytosis, nRBCs
microcytic hypochromic
hgb electro: decreased hgb A, N/increased Hgb A2, very increased hgb F
serum Fe and ferritin are N/increased
37
Q

beta thalassemia minor

A

inherited heterozygous
b+/b, b*/b
asymp. unless stressed
lots of RBC morph. for mild anemia- doesn’t match
hgb electro; increased A2 5%, N/increased hgb F
microcyt. hypochrom

38
Q

beta thalassemia hemoglobin S

A 
inhertied heterozygous
b+/s, b*/s
b+/s: few sypmp., like sickle cell trait
b*/s: severe anemia like sickle cell
microcyt hypochrom
poikilocytosis, target
increased retic
hgb electro: hgb S 50-95%, hgb A 0-50%, hgb F 2-30%
39
Q

alpha thalassemia

A

blacks, indians, chinese, SE asian, middle east

decreased synth. alpha chains

40
Q

alpha thalassemia silent carrier

A

3 normal alpha genes
1 ab alpha gene
asypm.
benign

41
Q

alpha thalassemia trait (minor)

A

2 norm genes, 2 ab genes
mild microcyt hypochrom
excess beta chains form tetrads= B4= HgbH (very unstable)= Heinze bodies= hemolysis

42
Q

alpha thalassemia Hemoglobin H disease

A 
1 normal gene, 3 ab gene
microcyt hypoch
increased retic
excess beta from tetrads= Hgb H (high affinity for O2)
heinz bodies
43
Q

alpha thalassemia hydrops fetalis

A 
0 normal genes
excess gamma in fetus
gamma 4= hgb barts (high O2 affinity)
hypoxia- stillborn
hypochromic anemia
increased retic and nRBCs
anisocytosis, poikilocytosis
44
Q

hereditary persistence of fetal hemoglobin

A 
inherited
no prod. of beta/delta chains
gamma chains for all life
asymp
increased hgb due to increased o2 affinity of HgbF
no sig. hemolysis
microcy hypochrom
anisocytosis, poikilocytosis
45
Q

iron deficiency anemia

A 
low on Fe (increased demand, low dietary ingest, malabsor)
hypochrom microcyt
increased RDW
elliptocytes
BM erythrocytic hyperplasia
decreased M:E
46
Q

sideroblastic anemia

A

ab. heme synthesis (autosomal sex linked)
dimorphic population: normal & hypochrom microcyt
pappenheimer bodies, basophilic stippling,
increased RDW
RPI <2
ring sideroblasts
decreased M:E erythrocytic hyperplasia

47
Q

megaloblastic anemia

A

ab. in dna syth.; nucleus mature slower
macrocyt normochrom
oval macrocytes, howell jolly bodies, hypersegmented neut.
decrease wbc, plt, rbc= pancytopenia
BM: asynchony= cyto. matures norm. nucleus not mature as fast

48
Q

non megaloblastic anemia

A

liver disease, alcoholism
macrocy normochro
increased MCV normal MCHC
target, macrcocytes

49
Q

acute blood loss anemia

A

single event (surgery/ amputation)
normocyt normochro
normal MCV, MCHC, RDW

50
Q

Chronic disease anemia

A

hospitalized patients, nursing home
normochrom normocyt
increased Ferritin

51
Q

porphyrias anemia

A 
ab. porphyrin synth
inherited
normochrom normocyt
pink/reddish brown urine
fluoresence UV light
52
Q

chronic renal failure anemia

A

increased toxins, decreased erythropoietin
2* anemia
patients bleed- rbc lyse- decreased prod. of erthyropoietin
normochrom normocyt
burr cells, frags
associated renal failure disease test results

53
Q

endocrine disease anemia

A

hypothyrodism (response to decreased need for O2)
nomochrom normocyt
treat underlying disorder

54
Q

aplastic anemia

A 
inherited, idiopathic, drugs, chemo
pancytopenic
normochrom normocyt
lymph cells dominate
BM hypocellular
55
Q

pure red cell aplasia anemia

A

only involves red blood cells
rare
nomochrom normocyt
M:E increased; decreased erythroid precursor

56
Q

myelophthisic anemia

A

due to marrow replacement by fibrotic cells (myelofibrosis, tumors)
pancytopenia
normochrom normocyt
nRBCs, immature wbc= out of proportion to the degree of anemia
teardrops

Hematology CCMLS (13 decks)

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