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Haem/Onc > Anemia, Congen and Acquired Hemolytic, APSHO > Flashcards

Anemia, Congen and Acquired Hemolytic, APSHO Flashcards

1
Q

2 big categories for hemolytic anemia (and egs)?

A

Intrinsic to RBC (enzymopathies, hemoglobinopathies, membranopathies)
Extrinsic (Abs, toxins, mechanical, microangiopathic)

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2
Q

in addition to intrinsic and extrinsic, other way to differentiate hemolytic anemia?

A

Intravascular and extravascular

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3
Q

where does extravascular HA occur?

A

spleen and/or liver (RES)

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4
Q

what type of antibodies mediate intravascular vs extravascular hemolysis?

A
  • Intravasc: IgM (occ IgG)

- Extra: IgGs that don’t fix complement

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5
Q

Do you expect LDH to be higher in intravascular or extravascular hemolysis? Do you expect haptoglobin to be lower in intravascular or extravascular hemolysis?

A

Intravascular for both

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6
Q

Lab-wise, how can you differentiate intra vs extravascular hemolysis?

A

In intravascular, see hemoglobinEMIA and hemoglobinURIA

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7
Q

4 egs of intravascular hemolysis?

A

Paroxysmal cold hemoglobinuria, cold agglutinin, PNH, valves

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8
Q

3 egs of extravascular hemolysis?

A

warm AIHA, hemolytic disease of the newborn, HS

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9
Q

symptoms/complications of hemolysis?

A
  • Anemia
  • Hydrops fetalis
  • intrauterine growth retardation
  • kernicterus (G6PD = #1 cause)
  • blueberry muffin rash= skin extramedullary hematopoeisis
  • prematurity
  • neonatal hepatic failure
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10
Q

symptoms/complications of older kids/teens?

A

palor, fatigue, scleral icterus, jaundice, pulmonary htn, iron overload in transfused/non-transfused patients, GB stones, splenomegaly, low bone densiety, ednocrinopathies from Fe overload, extramedullary hematopoeisis in liver/spleen/mediastinal area, etc

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11
Q

General management of congen hemolytic anemia?

A
  • Follow growth & dev
  • determine baseline hgb and retics
  • follow for splenomeglay
  • educate fam re: gallstones, parvovirus b19 aplastic crisis
  • monitor for pulmonary htn
  • cholecystectomy if symptomatic gallstones
  • monitor for fe overload and def
  • folate supplementation/bone health
  • RBC transfusion, intermittent vs. chronic..avoidance of over-transfusion in infancy; increased transfusions during preg
  • splenectomy: partial or total, laparoscopic
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12
Q

denatured hgb…required supravital stain…evidence of oxidative damage as in G6PD def…what is this?

A

heinz bodies

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13
Q

nuclear remnants seen on ordinary Wrigh stein..eevidence of spleneoctomy and/or ineffective erythropo…what is this?

A

Howell-Jolly bodies

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14
Q

residual rna on polysomes on wright stain, seen with impaired translationi (thal, lead some enzymopathies)…what is this?

A

basophilic stippling

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15
Q

iron inclusions seen in wright stain, as in sideroblastoic anemia. what is this?

A

pappenheimer bodies

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16
Q

Give 10 causes of congenital HA

A 
HBG-opathies
-thal
-SCD
MEMBRANOPATHIES
-HS
-HE
-HPP
-Hereditary stomatocytosis
-hereditary xerocytosis
-Rh Null
ENZYMOPATHIES
-g6pd def
-PK def
-hexokinase def
-aldolase def
-phophofructose kinase def
-pyrimidine 5' nucelotidase def
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17
Q

10 causes of acquired HA?

A 
IMMUNE
-warm AIHA
-cold agglutinin
-paroxysmal cold hemoglobinuria
-transfusion rxn
NON-IMMUNE
HUS
TTP
DIC
mech/heart valve
Kasabach merritt
paroxysmal nocturnal hemoglobinuria
toxins/meds
thermal burns
wilson disease
infection
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18
Q

HS defects (vertical interactions of membrane)?…spheres don’t deform as well in the spleen… 2

A

ankyrin, spectrin, actin

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19
Q

Hereditary elliptocytosis defects (horizontal interactions)? 2

A

band 3, glycophorins

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20
Q

HS inheritance?

A

AD

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21
Q

what’s the most common congen hemolytic aemia?

A

HS

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22
Q

most common mutation in HS?

A

ankyrin ANK1>band 3 SLC4A1>beta spectrin SPTB

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23
Q

recessie HS muation?

A

alpha spectrin

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24
Q

HS: will MCHC be high or low?

A

high (cellular dehydration)

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25
Q

other than spherocytes see what on HS smear?

A

polychromasia

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26
Q

3 ways to dx HS?

A

EMA bidning, incubated osmotic fragility, molecular dx

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27
Q

what makes HS patient more likely to have gallstones?

A

co-morbid gilbert syndrome

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28
Q

how does osmotic fragility test work?

A

incubate red cells in diff concentrations saline (0-0.9%)…with less saline concentration, cells take on water and lyse…normal cells lyse at 0.5%…HS cells lyse at HIGHER saline concentrations…degree of hemolysis detected by spectrophotometry…sens 80%

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29
Q

is osm frag a good test in neonates?

A

no…fetal cells relatively resistant to this test

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30
Q

EMA binding test=?

A

eosin-5-maleimide binding

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31
Q

explain EMA binding test

A

Eosin-based fluorescent dye binds to RBC membrane protiens, including band 3…see reduction of band 3 fluoresnce in pts with HS

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32
Q

sens and spec of EMA binding test?

A

sense 93-96%, spec 93-99%

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33
Q

reason for false + EMA binding?

A

CDA type II, South Asian Ovalocytosis, hereditary pyropoikilocytosis

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34
Q

benefits of full splenectomy vs. partial splenectomy in HS?

A

full: resolution of anemia, no risk of aplstic crisis/gallstones/hyper-hemolysis
partial: signficant imporvment in anemia, maintian some splenic function

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35
Q

cons of full splenectomy vs. partial in hS?

A

full: infections (encapsulated organissms, babesiosis, malaria); thrombosis (10%, due to decreased filtering capacity)
partial: splenic regrowth in 5-10%, compensated hemolysis so continued risk of aplastic crisis, gallstones, etc

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36
Q

splenectomy recommended for severe HS=?…no splenectomy rec’ed for mild HS=? in between = moderate

A

Severe: Hgb <60-80, requires transfusions…mild: Hgb>110, no sx

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37
Q

Hereditary elliptocytosis: most common mutation?

A

abnormal spectrin heterodimer: SPTA, SPTB

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38
Q

HE inheritance?

A

AD

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39
Q

symptoms in most HE pts?

A

usually asymptomatic

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40
Q

tx in HE?

A

usually no need…splenectomy helps if severe

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41
Q

2 subtypes of HE?

A

southeast asian ovalocytosis, alpha LELY

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42
Q

hereditary pyropoikilocytosis: most common abnormaltiy?

A

spectrin

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43
Q

HPP: inheritance?

A

AR…commonly HE SPTA1 mutation in trans to alpha LELY SPTA1

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44
Q

clinical findings in HPP? 3

A

blood smear: bizarre rbc shapes as in thermal burn
molecular testing of spectrin
MCV 55-74 (L)

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45
Q

HPP: prog?

A

severe hemolysis in early childhood, then HE later in life

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46
Q

2 types of stomatocytosis

A

hydrocytosis= overhydrated= low MCHC, xerocytosis= dehydrated= high MCHC

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47
Q

hydrocytosis: gene?

A

RHAG

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48
Q

xerocytosis in term osm fragility?

A

DECREASED (opp of HS, which is increased)

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49
Q

Mutations in xerocytosis?

A

PIEZO1, KCCN4

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50
Q

xerocytosis inheritance?

A

AD

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51
Q

xerocytosis ddx?

A

acute ehtanol intox, liver disease, rh null disease, tangier disease

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52
Q

complications of xerocytosis?

A

fe overload, marked increased risk of thrombosis of splenectomy!!!!! don’t do it!

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53
Q

hgb o2 dissoc curve: x-axis? y-axis?

A

x= pO2 (mmHg); y= % O2 sat

54
Q

what pushes hgb o2 dissoc curve to left?

A

high pH, low temp, decreased 2,3 DPG

55
Q

what pushes hgb o2 dissoc curve to right?

A

low pH, high temp, increaed 2,3 DPG

56
Q

unstable hgbs inheritnace?

A

AD

57
Q

clinical findings in unstable hgb?

A

blood smear: heinz bodies
hb electrophoreiss
urine: pigmenturia
sx: extravascular hemolysis, ineffective erythropoeisis

58
Q

4 subtypes of unstable hgb?

A

Hb Köhn= most frequent, high O2 affin
Hb Zurich, normal O2 affin, increased affin for CO
Hb Poole: unstable gamma chain variant
Hb Indianapolis: too unstable to find in blood

59
Q

g6pd def: caused by?

A

decreased NADPH; inability ot maintain reduced glutathione

60
Q

g6pd inheritance?

A

x-linked…but girls can still have if lyonization

61
Q

smear findings in G6PD?

A

heinz bodies, blister cells

62
Q

WHY should you wait until crisis over until testing for G6PD def?

A

retics have 5x higher g6pd…wait 1-2 months after

63
Q

classes of g6pd?

A

1-4 (roman numerals)

1: <10% normal, chornic
2: <10% normal, episodic severe, mediterranean
3: 10-60%…G6PD A-= 15% of African Americans
4: >60%…so symptoms

64
Q

give 10 oxidative triggers in G6pd

A 
dapsone
methylene blue
fava beans
henna
rasburicase
napthalene
glyburide
nitrofurantoin
primaquine
TNT=trinitrotoluene
65
Q

PK def: associated with increased or decreased 2,3 DPG?

A

increased

66
Q

PK def–> what within the RBC

A

less ATP–> loss of membrane stability, hemolysis

67
Q

PK def inheritance?

A

AR

68
Q

PK def gene?

A

PKLR

69
Q

dx PK def how?

A

enzyme activity AND sequencing of PKLR

70
Q

in pk def: if undergo splenomegaly, what happens?

A

reticulocytosis!!! retics may increased from 10-15–> 30-70%

71
Q

other than g6pd def and pk def, give two other enzympatothies

A

glucose phophsate isomerase def

pyriidine 5’ nucleotidase def

72
Q

normal heme group is Fe what?

A

2+= ferrous….when gets oxidized–> 3+ = ferric–> more oxygen affinity–> decreased O2 delivery

73
Q

drug that can –> methemoglobinemia?

A

lidocaine

74
Q

AD blue baby with brown blood..dx?

A

Hb M variant; methemoglobinemia

75
Q

what type of methemoglobinemia is AR?

A

NADH MetHb reductase def

76
Q

tx for methemoglobinemia?

A

remove inciting agent
give O2
methylene blue to increased reduction of met hgb..can’t give if g6pd def though

77
Q

at what level of methb does pt become cyanotic?

A

10-15%

78
Q

DAT= direct coombs…how does this work?

A

take pt blood sample; add anti-human IgG= coombs reagent–> agglutinate if there were ALREADY antibodies prsent on the pt’s cells that the coombs reagent is responding to as secondary abs

79
Q

3 subtypes of immune mediated hemolysis?

A
  • autoimmune HA
  • paroxysmal cold hemoglobinuria
  • cold agglutinin disease
80
Q

AIHA: ab type?

A

IgG +/-C3

81
Q

PCH: Ab type?

A

IgG= Donath Landsteiner Ab…DAT often neg becuase sample needs to go to lab warm! and lab has to check for it!

82
Q

Cold agglutinin disease Ab type?

A

IgM= DAT C3+

83
Q

temp for AIHA?

A

37C

84
Q

antigen in AIHA?

A

common or panractive…will have direct adn indirect coombs + because reacts to lots of things

85
Q

AIHA: intra or extravasc?

A

extra

86
Q

causes of AIHA?

A 
idiopathic
rhuem
immunodef
evans
infections
malig
87
Q

tx for AIHA?

A 
transfusions
long course of steorids=1st
ritux=2nd
splenectomy'
immunosupp
88
Q

AIHA prog?

A

often recurs

89
Q

pCH temp?

A

Ab binds at 4C, fixes C3 as warms to 37C

90
Q

antigen in PCH?

A

P

91
Q

PCH: intra or extravasc?

A

intra

92
Q

cause of PCH?

A

viral infection

93
Q

PCH tx? 2

A

Blood/IVF warmer

supportive

94
Q

cold agglutinin disease: temp?

A

4C

95
Q

Antigen in cold agglutinin disease?

A

I/i

96
Q

cold agglutinin diseaes site of clearance?

A

intravasc

97
Q

causes of cold agglutinin disease? (2)

A

mycoplasma, EBV

98
Q

tx of cold agglutinin disease?

A

blood/IVF warmer
supportive
plasmapheresis if severe (because IgM!!)

99
Q

prog in PCH?

A

self-limited

100
Q

prog in cold agglutinin diseaes?

A

self-limited

101
Q

hemolytic diseae of newborn: egs?

A

Rh incompat
ABO incopat
minor incompat

102
Q

tx hemolytic disease of newborn how?

A

exchange transfusion, phototherpya, RBC transfusions

103
Q

Rh hemolytic disease: lab findigns? 3

A

baby’s DAT positive
mom’s indirect coombs will be positive because she’ll have ab to paternal antigen
infant smear: nRBCs, polychromasia

104
Q

ABO incompatib: typically IgM or IgG?

A

IgM, but only IgG can cross placenta

105
Q

Can ABO incompat happen in first preg?

A

yes

106
Q

what is ABO incomp usually not severe?

A

ABO antigens not expressed in early fetal RBCs

107
Q

see what on baby’s smear if ABO incompat?

A

polychromasia
nRBCs
spherocytes

108
Q

MAHA= miroangiopathic HA: see hwat on smear?

A

schistocytes

109
Q

MAHA: 5 egs?

A 
DIC
TTP
HUS
Kasabach-Merrit
burns
BMT (allo>auto)
cyclosproine
tacro
110
Q

marcoangiopathic HA: see what on smear?

A

shcistocytes

111
Q

macroangiopathic hemolysis (larger vessels): causes?

A
  • congen heart disease, esp after surgery wtih rough suture line or reisdual high pressure gradient jet
  • march hemolysis
112
Q

TTP: caused by?

A

def or Abs to ADAMTS13

113
Q

lack of ADAMTS13 –>?

A

ultralarge VWF multimers–> fibrin dep, plt trapping, microangipathiy

114
Q

name of congen ADAMTS13?

A

Upshaw-Schulman syndrome

115
Q

causes of secondary ADAMTS13? 4

A

rheum, preg, infections, idiopathic

116
Q

TTP pentad?

A

MAHA, thrombocytopenia, renal dysfunction, neuro changes, fever

117
Q

how to dx TTP?

A

-intravasc hemolysis, low plts, normal PT/PTT/ibrinogen, schistocytes on smear..low ADAMTS13 activity and positive abs but slow turnaround

118
Q

tx for TTP?

A

urgent plasmapheresis for acuired TTP with steroids and/or other immunosupp (ritux); caplacizumab for adults…dont’ wait for ADAMTS13 results!

119
Q

HUS or TTP more common?

A

HUS

120
Q

HUS traid?

A

MAHA< low plts, renal dysfucntion

121
Q

pathophys of classic HUS?

A

shiga toxin from e coli O157:H7 infection..get bloody diarrhea

122
Q

pathophys of ATYPICAL HUS?

A

sporadic = due to infections…or familial = mutations in complememtn control proteins…may not have bloody diarrhea

123
Q

HUS: ADAMTS13 result?

A

normal

124
Q

classic HUS tx?

A

supportive care

125
Q

atypical HUS tx?

A

supportive care, plasma therpay, eculizumab (blcoks C5–> C5a, C5b), renal or liver transplant

126
Q

Paroxysmal noctrunal hemobloginuria: tx?

A

eculizumab

127
Q

drug induced HA eg?

A

pencillin–> haptenization

128
Q

toxin associated hemolysis eg?

A

clostridium sepsis, brown recluse spider bite, wilson’s disease, burns

129
Q

dx wilsons how?

A

serum copper, ceruloplasmin levels

130
Q

Rh null phenotype hemolysis pathophs?

A

absenet or markeldy low Rh expression (unlike Rh- which refer to D antigen ONLY), due to mutations in RHAG gene

131
Q

findings in Rh null phenotype?

A

mild-mod hemolytic anemia
increased osm frag
elevated HbF
smear: stomatocytes

132
Q

management issue in Rh null phenotype?

A

may form ab to Rh antigens when transfused RBCs

Haem/Onc (30 decks)

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