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Heme Flashcards

1
Q

intrinsic pathway factors

A

8, 9. 11, 12
aPTT

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2
Q

extrinsic pathway factors

A

7
PT

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3
Q

hemophilia A factor

A

8

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4
Q

hemophilia B factor

A

9

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5
Q

hemophilia A sx

A

hemarthrosis
easy bruising

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6
Q

hemophilia A labs

A

normal plt
normal PT
high PTT

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7
Q

tx hemophilia A

A

factor 8
DDAVP

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8
Q

christmas disease

A

hemophilia B

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9
Q

hemophilia B sx

A

easy bruising
hemarthrosis
spontaneous bleeding episodes

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10
Q

hemophilia B labs

A

plt normal
high PTT
normal PT

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11
Q

what to avoid in hemophilia

A

ASA

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12
Q

hemophilia B tx

A

factor 9

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13
Q

von willebrand factor

A

factor 8

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14
Q

von willebrand disease sx

A

mucocutaneous bleeding, gingival bleeding
menorrhagia

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15
Q

von willebrand dz labs

A

low von willebrand factor
increased bleeding time
plt normal
PT/INR normal, PTT normal
ristocetin cofactor assay decreased= gold

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16
Q

von willebrand dz tx

A

DDAVP
avoid ASA

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17
Q

vitamin K deficiency labs

A

prolonged PT
normal PTT
normal bleeding time
normal plt

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18
Q

MCC DIC in pregnancy

A

placental abruption: tissue factor released into maternal circulation

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19
Q

DIC labs

A

low plt, low fibrinogen
prolonged PT, PTT, and BT
high D dimer
schistocytes

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20
Q

immune mediated thrombocytopenia

A

heparin induced thrombocytopenia (HIT)
Immune thrombocytopenia purpura (ITP)

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21
Q

non-immune mediated thrombocytopenia

A

thrombotic thrombocytopenic purpura (TTP)
hemolyticouremic syndrome (HUS)

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22
Q

thrombocytopenia labs

A

low plt
prolonged BT
PT, PTT normal

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23
Q

heparin induced thrombocytopenia (HIT) sx

A

5-10d after starting unfractionated or LMW heparin
paradoxical thrombotic events (DVT, stroke, MI, PE, limb gangrene)

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24
Q

gold standard HIT

A

serotonin release assay

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25
Q

tx HIT

A

d/c heparin
start anticoagulation w/ argatroban* or non-heparin anticoagulant

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26
Q

immune (idiopathic) thrombocytopenic purpura (ITP) sx

A

children 2-6yo, female following acute viral infection
mucocutaneous bleeding, epistaxis, spontaneous bleeding
no hepatosplenomegaly

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27
Q

ITP labs

A

low plt (isolated thrombocytopenia)
normal coags
NO SCHISTOCYTES

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28
Q

tx ITP

A

observation if asx, mild, or child
CS (first) or IVIG if adult

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29
Q

thrombotic thrombocytopenic purpura (TTP) etiology

A

ADAMTS13 activity decreased –> decreased von willebrand factor

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30
Q

TTP pentad

A

thrombocytopenia
microangiopathic hemolytic anemia
renal dysfunction (hematuria, low urine output)
neurological impairment
fever

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31
Q

TTP sx

A

classic pentad
bleeding
splenomegaly

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32
Q

TTP labs

A

high schistocytes
normal PT/PTT
Neg D dimer
Dec ADAMTS13

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33
Q

TTP tx

A

plasmaphoresis mainstay
IV CS
hematology referral

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34
Q

hemolytic uremic syndrome caused by

A

shiga toxin producing E. coli

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35
Q

HUS sx

A

child after episode of gastroenteritis caused by E. coli and S. dysenteriae
triad: microangiopathic hemolytic anemia, thrombocytopenia, acute kidney injury
MC kid <5yo

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36
Q

HUS triad

A

thrombocytopenia
renal insufficiency
microangiopathic hemolytic anemia
NO FEVER
NO NEURO SX

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37
Q

HUS labs

A

PT/PTT normal
d-dimer negative
schistocytes
elevated serum Creatine

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38
Q

tx HUS

A

supportive
avoid abx and anti motility agents

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39
Q

anemia of chronic dz labs

A

inc ferritin
low transferrin
high serum Fe
low TIBC

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40
Q

anemia of chronic dz tx

A

transfusions
IV EPO
tx underlying

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41
Q

iron deficiency labs

A

low hgb
RDW high
low retic
low serum iron, low ferritin
high TIBC

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42
Q

lead poisoning sx

A

abd pain, constipation
Burton’s line (lines in gingiva)
blue lines and long bones of metaphysics (chronic exposure)
neuro sx (encephalopathy: HA/N/V/memory loss)
drop foot, drop wrist
metabolic acidosis

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43
Q

lead poisoning labs

A

basophilic stippling
lead line on xray

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44
Q

lead poisoning tx

A

chelation therapy: dimercaprol and EDTA for adults, succimer for children

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45
Q

sideroblastic anemia labs

A

high serum iron, high ferritin
transferrin and TIBC normal
Prussian blue stain: ringed sideroblast appearance
RDW high

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46
Q

sideroblastic anemia tx

A

remove toxins
pyridoxine (B6)
chelation therapy

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47
Q

aplastic anemia causes

A

parvovirus B19
EBV
hepatitis
carbamazepine, methimazole, PTU, NSAIDS

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48
Q

asplastic anemia sx

A

pallor, purpura, petechiae
no splenomegaly
high risk of bleed and infection

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49
Q

aplastic anemia labs

A

bone marrow bx: dry tap
dx of exclusion
pancytopenia, iron studies normal

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50
Q

asplastic anemia tx

A

bone marrow transplant
transfusion

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51
Q

hereditary spherocytosis sx

A

anemia, jaundice, splenomegaly
pigmented black gallstones (calcium bilirubinate)

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52
Q

hereditary spherocytosis labs

A

hyper chromic spherocytes (round RBC lacking central pallor)
inc MCHC

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53
Q

hereditary spherocytosis test

A

osmotic fragility test
Coombs negative

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54
Q

hereditary spherocytosis tx

A

folic acid
splenectomy TOC in severe

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55
Q

hemolytic anemia labs

A

inc LDH, inc direct bili, inc retic, low H&H
direct Coombs test +
spherocytes

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56
Q

tx warm hemolytic anemia

A

steroids
splenectomy if refractory
immunosuppressive meds

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57
Q

tx cold hemolytic anemia

A

avoid cold
plasmapheresis if severe

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58
Q

hemolytic dz of newborn caused by

A

Rh incompatibility

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59
Q

hemolytic dz of newborn sx

A

jaundice
kernicterus hydrops fetalis

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60
Q

hemolytic dz of newborn dx

A

doppler US, Coombs test, blood typing

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61
Q

hemolytic dz of newborn tx

A

eopetin alfa/ darbepoetin
iron
transfusion, IVIG

62
Q

G6PD sx

A

abd pain, dark (tea colored) urine, jaundice
splenomegaly, back pain
pigmented gallstones

63
Q

G6PD lab

A

Heinz bodies, bite cells, hemighost
definitive: enzyme assay

64
Q

G6PD tx

A

iron/folic acid supplement
avoid triggers

65
Q

meds that can trigger G6PD

A

chloroquine
nitrofurantoin
bactrim
ASA, ibuprofen

66
Q

paroxysmal nocturnal hemoglobinuria sx

A

hemolytic anemiaL dark cola colored urine during night or early AM w/ partial clearing during the day
venous thrombosis of large vessels: hepatic, mesenteric, cerebral, subnormal veins (hyper coagulable state)
pancytopenia: bone marrow failure, often after bone marrow injury

67
Q

paroxysmal nocturnal hemoglobinuria dx

A

flow cytometry: hemosiderinuria

68
Q

tx paroxysmal nocturnal hemoglobinuria

A

eculizumab: anti-compliment CD5 ab
prednisone: dec hemolysis
marrow transplant

69
Q

Folate alternative name

70
Q

MCC B12 deficiency

A

defective absorption: gastric bypass, terminal ilium, atrophy

71
Q

B12 deficiency sx

A

vegan
paresthesia, glossitis, ataxia
neuro sx: memory loss, gait abnormality

72
Q

B12 deficiency dx

A

PBS: hyperhsegmented neutrophils
+ schillings test: gold
high homocysteine
high methylmalonic acid

73
Q

folate deficiency sx

A

alcoholics or use of phenytoin/MTX
no neuro sx

74
Q

folic acid deficiency labs

A

PBS: hyperhsegmented neutrophils
high homocysteine levels
normal methylmalonic acid

75
Q

polycythemia MCC

A

JAK2 mutation

76
Q

polycythemia sx

A

red and pain in palms/soles
bruising, bleeding
aquatic pruritus, face blush
budd chiari: abd pain, ascites, hepatomegaly

77
Q

polycythemia labs

A

high RBC/HH, WBC, plt
dec EPO

78
Q

tx polycythemia

A

phlebotomy
ruxolitinib: JAK2 inhibitor

79
Q

what is secondary erytheocytosis

A

inc RBC due to another process

80
Q

secondary erythrocytosis labs

A

inc RBC/hct w/ normal WBC and plt
normal EPO

81
Q

hemochromatosis triad

A

cirrhosis (abd, hepatomegaly, jaundice)
DM
skin bronzing

82
Q

hemochromatosis lab

A

inc iron, ferritin, and transferrin
definitive: liver bx (inc iron deposits/ hemosiderin)

83
Q

hemochromatosis tx

A

phlebotomy
iron chelation: deferoxamine

84
Q

sickle cell dz etiology

A

beta globin gene (HbS) mutation
autosomal recessive
glutamic acid –> valine

85
Q

sickle cell sx

A

dactylics @ 6-9mo
joint pain, jaundice, hepatosplenomegaly
priapism, H shaped vertebrae, splenic sequestration
painful occlusive crisis

86
Q

MCC osteomyelitis in children w/ sickle cell

A

salmonella

87
Q

assoc w/ aplastic crisis in sickle cell dz

88
Q

sickle cell labs

A

Howell jolly bodies
low HH, low RBC, reticulocytosis
hemoglobin electrophoresis definitive
hair on end appearance on xray

89
Q

tx sickle cell

A

fluids, pain control, folic acid, iron
PCNV for infx
hydroxyurea to reduce frequency of crisis

90
Q

sickle cell immunizations

A

S: S. pneumo
Hi: Hib
N: N. meningococcus

91
Q

thalassemia A labs

A

teardrop cells
hemoglobin electrophoresis
basophilic stippling

92
Q

thalassemia A tx

A

folic acid
blood transfusion, splenectomy
bone marrow transplant definitive

93
Q

thalassemia B major sx

A

sx at 6mo. hepatomegaly, frontal bossing, severe hemolytic anemia (pallor, jaundice), osteopenia,

94
Q

thalassemia B labs

A

target cells, variation of size and shape of RBC
xray: hair on end
hemoglobin electrophoresis definitive

95
Q

thalassemia B tx

A

iron transfusion, splenectomy if severe

96
Q

hemolytic transfusion reaction sx

A

due to ABO incompatibility
immediate F, chill, dark urine, hypoTN
+ Coombs test

97
Q

hemolytic transfusion reaction tx

A

stop transfusion
crystalloid infusion
diuretic therapy

98
Q

febrile transfusion reaction

A

fever and chills w/in 6hrs
MC transfusion rxn
stop transfusion and supportive (acetaminophen)

99
Q

transfusion related acute lung injury

A

SOB w/in 30m
resolves spontaneously

100
Q

allergic reaction to transfusion

A

urticaria/ pruritus likely d/t donors plasma
symptomatic care- antihistamines

101
Q

delayed transfusion reaction

A

3-4wks after transfusion
fall in hemoglobin, rise in bilirubin
supportive care

102
Q

graft vs host dz

A

immunocompromised pts
rash, elevated LFT, pancytopenia

103
Q

leukemia associated w/ downs syndrome

A

acute leukemias

104
Q

mc pediatric leukemia

105
Q

ALL sx

A

fever, joint pain, bleeding, lymphadenopathy
hepatosplenomegaly
CNS sx

106
Q

ALL labs

A

inc WBC blasts
+TdT
+cALLa

107
Q

ALL tx

A

chemo
stem cell transplant
CNS prophylaxis: intrathecal MTX
good prognosis

108
Q

AML can be caused by

A

radiation exposure

109
Q

AML labs

A

inc WBC myeloblasts
auer rods

110
Q

AML tx

A

chemo
stem cell transplant

111
Q

MC leukemia in adults

112
Q

CLL labs

A

inc smudge cells

113
Q

CLL tx

A

watch and wait
chemoimmunotherapy: fludarabine
stem cell curative

114
Q

CML etiology

A

unregulated stimulation of tyrosine kinase system

115
Q

CML sx

A

fatigue, fever, night sweats
hepatosplenomegaly

116
Q

CML labs

A

philadelphia chromosome t(9;22)
low LAP score

117
Q

CML tx

A

tyrosine kinase inhibitor: imatinib
bone marrow transplant curative

118
Q

Hodgkins lymphoma sx

A

potential EBV, bimodal age
painless firm mass or lymphadenopathy w/ nodular sclerosis
extra nodal spread not common
hepatosplenomegaly
fever, night sweats, weight loss
generalized pruritus

119
Q

Hodgkins lymphoma tests

A

lymph node bx, PET scan, bone marrow bx
reed Sternberg cells (owl eyes) on CBC
high LDH, mediastinal mass on CXR

120
Q

Hodgkins lymphoma tx

121
Q

non Hodgkins lymphoma sx

A

can spread extra nodal, highly malignant
fever, night sweats, weight loss, painless lymphadenopathy

122
Q

burkitt lymphoma

A

type of non-hodgkins, hx EBV,
enlarging mass over jaw (Africa), abd if outside of Africa
starry sky

123
Q

multiple myeloma sx

A

AA male, elderly
BREAK (bone lesions, recurrent infx, elevated calcium, anemia, kidney failure)

124
Q

multiple myeloma tests

A

XR: punched out bone lesions
bence jones protein
rouleaux formation
M spike

125
Q

multiple myeloma tx

A

chemo, bisphosphonates
abx (infx), GCS (hypercalcemia)
stem cell transplant definitive

126
Q

myelodysplastic syndrome

A

group of hematological cancers MC elderly
asx, anemia, bleeding, inc risk of infx
hepatosplenomegaly
mild: watch and wait
severe: supportive transfusions
stem cell transplant curative

127
Q

prescribing heparin monitor

A

monitor PTT

128
Q

enoxaparin

A

LMWH
no need to monitor PTT

129
Q

fractionated LMWH inhibits

130
Q

unfractionated heparin inhibits

A

X and thrombin

131
Q

heparin antidote

A

protamine sulfate

132
Q

prescribe warfarin monitor

133
Q

warfarin MOA

A

decrease synthesis of vitamin K clotting factors (2, 7, 9, 10), protein C and S

134
Q

warfarin antidote

A

vitamin k
fresh frozen plasma

135
Q

direct factor Xa inhibitors

A

apixaban
rivaroxaban
no need monitor labs

136
Q

direct thrombin inhibitors

A

dabigatran
used w/ anti platelet agents (ASA, clopidogrel)

137
Q

direct thrombin inhibitors antidote

A

idarucizumab

138
Q

coagulopathy of advanced liver disease labs and tx

A

inc PT
low albumin
advanced dz: high PT and PTT with no response to vitamin K
tx w/ FFP

139
Q

factor v leiden sx

A

hx of miscarriage and DVT at young age

140
Q

factor v leiden dx and tx

A

genetic testing (FVL mutation) gold
anticoagulants

141
Q

antiphospholipid syndrome MOA

A

HLA-DR7 mutation

142
Q

antiphospholipid syndrome sx

A

recurrent thrombotic events, rapid organ failure
renal failure video reticularis (mottled purple discoloration)
recurrent miscarriage

143
Q

antiphospholipid syndrome tests

A

positive anticardiolipin (false positive in syphilis)
anti-beta 2 glycoprotein abs

144
Q

antiphospholipid syndrome tx

A

ASA
warfarin
Pregnant: LMWH

145
Q

protein C and S deficiency difference

A

protein C: unregulated activation of coagulation cascade
protein S: enhanced activity of coagulation cascade

146
Q

protein C and S sx

A

hyper coagulable state, recurrent DVT/PE
stroke
neonatal purpura fulminans
skin necrosis

147
Q

protein C and S deficiency tx

A

heparin for life
may develop warfarin induced skin necrosis

148
Q

antithrombin 3 deficiency

A

venous thrombosis
recurrent DVT/PE
asx need anticoagulation only before surgery
high dose IV heparin then oral anticoagulation indefinitely

149
Q

PT is which factors

A

1, 2, 5, 10 (common coag pathways)
7 (extrinsic)

150
Q

HIT presentation

A

someone who got platelets and then after 1 wk, plt drop to 50%

151
Q

auer rods + myeloperoxidase positive subtype of AML

A

Acute promyelocytic leukemia

152
Q

burkitt lymphoma presentation

A

Africa, pain and swelling jaw
stars in a dark sky
assoc w/ epstein barr

PNCE (33 decks)

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