Heme Flashcards
(152 cards)
1
Q
intrinsic pathway factors
A
8, 9. 11, 12
aPTT
2
Q
extrinsic pathway factors
A
7
PT
3
Q
hemophilia A factor
A
8
4
Q
hemophilia B factor
A
9
5
Q
hemophilia A sx
A
hemarthrosis
easy bruising
6
Q
hemophilia A labs
A
normal plt
normal PT
high PTT
7
Q
tx hemophilia A
A
factor 8
DDAVP
8
Q
christmas disease
A
hemophilia B
9
Q
hemophilia B sx
A
easy bruising
hemarthrosis
spontaneous bleeding episodes
10
Q
hemophilia B labs
A
plt normal
high PTT
normal PT
11
Q
what to avoid in hemophilia
A
ASA
12
Q
hemophilia B tx
A
factor 9
13
Q
von willebrand factor
A
factor 8
14
Q
von willebrand disease sx
A
mucocutaneous bleeding, gingival bleeding
menorrhagia
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
16
Q
von willebrand dz tx
A
DDAVP
avoid ASA
17
Q
vitamin K deficiency labs
A
prolonged PT
normal PTT
normal bleeding time
normal plt
18
Q
MCC DIC in pregnancy
A
placental abruption: tissue factor released into maternal circulation
19
Q
DIC labs
A
low plt, low fibrinogen
prolonged PT, PTT, and BT
high D dimer
schistocytes
20
Q
immune mediated thrombocytopenia
A
heparin induced thrombocytopenia (HIT)
Immune thrombocytopenia purpura (ITP)
21
Q
non-immune mediated thrombocytopenia
A
thrombotic thrombocytopenic purpura (TTP)
hemolyticouremic syndrome (HUS)
22
Q
thrombocytopenia labs
A
low plt
prolonged BT
PT, PTT normal
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)
24
Q
gold standard HIT
A
serotonin release assay
25
tx HIT
d/c heparin
start anticoagulation w/ argatroban* or non-heparin anticoagulant
26
immune (idiopathic) thrombocytopenic purpura (ITP) sx
children 2-6yo, female following acute viral infection
mucocutaneous bleeding, epistaxis, spontaneous bleeding
no hepatosplenomegaly
27
ITP labs
low plt (isolated thrombocytopenia)
normal coags
NO SCHISTOCYTES
28
tx ITP
observation if asx, mild, or child
CS (first) or IVIG if adult
29
thrombotic thrombocytopenic purpura (TTP) etiology
ADAMTS13 activity decreased --> decreased von willebrand factor
30
TTP pentad
thrombocytopenia
microangiopathic hemolytic anemia
renal dysfunction (hematuria, low urine output)
neurological impairment
fever
31
TTP sx
classic pentad
bleeding
splenomegaly
32
TTP labs
high schistocytes
normal PT/PTT
Neg D dimer
Dec ADAMTS13
33
TTP tx
plasmaphoresis mainstay
IV CS
hematology referral
34
hemolytic uremic syndrome caused by
shiga toxin producing E. coli
35
HUS sx
child after episode of gastroenteritis caused by E. coli and S. dysenteriae
triad: microangiopathic hemolytic anemia, thrombocytopenia, acute kidney injury
MC kid <5yo
36
HUS triad
thrombocytopenia
renal insufficiency
microangiopathic hemolytic anemia
NO FEVER
NO NEURO SX
37
HUS labs
PT/PTT normal
d-dimer negative
schistocytes
elevated serum Creatine
38
tx HUS
supportive
avoid abx and anti motility agents
39
anemia of chronic dz labs
inc ferritin
low transferrin
high serum Fe
low TIBC
40
anemia of chronic dz tx
transfusions
IV EPO
tx underlying
41
iron deficiency labs
low hgb
RDW high
low retic
low serum iron, low ferritin
high TIBC
42
lead poisoning sx
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
43
lead poisoning labs
basophilic stippling
lead line on xray
44
lead poisoning tx
chelation therapy: dimercaprol and EDTA for adults, succimer for children
45
sideroblastic anemia labs
high serum iron, high ferritin
transferrin and TIBC normal
Prussian blue stain: ringed sideroblast appearance
RDW high
46
sideroblastic anemia tx
remove toxins
pyridoxine (B6)
chelation therapy
47
aplastic anemia causes
parvovirus B19
EBV
hepatitis
carbamazepine, methimazole, PTU, NSAIDS
48
asplastic anemia sx
pallor, purpura, petechiae
no splenomegaly
high risk of bleed and infection
49
aplastic anemia labs
bone marrow bx: dry tap
dx of exclusion
pancytopenia, iron studies normal
50
asplastic anemia tx
bone marrow transplant
transfusion
51
hereditary spherocytosis sx
anemia, jaundice, splenomegaly
pigmented black gallstones (calcium bilirubinate)
52
hereditary spherocytosis labs
hyper chromic spherocytes (round RBC lacking central pallor)
inc MCHC
53
hereditary spherocytosis test
osmotic fragility test
Coombs negative
54
hereditary spherocytosis tx
folic acid
splenectomy TOC in severe
55
hemolytic anemia labs
inc LDH, inc direct bili, inc retic, low H&H
direct Coombs test +
spherocytes
56
tx warm hemolytic anemia
steroids
splenectomy if refractory
immunosuppressive meds
57
tx cold hemolytic anemia
avoid cold
plasmapheresis if severe
58
hemolytic dz of newborn caused by
Rh incompatibility
59
hemolytic dz of newborn sx
jaundice
kernicterus hydrops fetalis
60
hemolytic dz of newborn dx
doppler US, Coombs test, blood typing
61
hemolytic dz of newborn tx
eopetin alfa/ darbepoetin
iron
transfusion, IVIG
62
G6PD sx
abd pain, dark (tea colored) urine, jaundice
splenomegaly, back pain
pigmented gallstones
63
G6PD lab
Heinz bodies, bite cells, hemighost
definitive: enzyme assay
64
G6PD tx
iron/folic acid supplement
avoid triggers
65
meds that can trigger G6PD
chloroquine
nitrofurantoin
bactrim
ASA, ibuprofen
66
paroxysmal nocturnal hemoglobinuria sx
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
paroxysmal nocturnal hemoglobinuria dx
flow cytometry: hemosiderinuria
68
tx paroxysmal nocturnal hemoglobinuria
eculizumab: anti-compliment CD5 ab
prednisone: dec hemolysis
marrow transplant
69
Folate alternative name
B9
70
MCC B12 deficiency
defective absorption: gastric bypass, terminal ilium, atrophy
71
B12 deficiency sx
vegan
paresthesia, glossitis, ataxia
neuro sx: memory loss, gait abnormality
72
B12 deficiency dx
PBS: hyperhsegmented neutrophils
+ schillings test: gold
high homocysteine
high methylmalonic acid
73
folate deficiency sx
alcoholics or use of phenytoin/MTX
no neuro sx
74
folic acid deficiency labs
PBS: hyperhsegmented neutrophils
high homocysteine levels
normal methylmalonic acid
75
polycythemia MCC
JAK2 mutation
76
polycythemia sx
red and pain in palms/soles
bruising, bleeding
aquatic pruritus, face blush
budd chiari: abd pain, ascites, hepatomegaly
77
polycythemia labs
high RBC/HH, WBC, plt
dec EPO
78
tx polycythemia
phlebotomy
ruxolitinib: JAK2 inhibitor
79
what is secondary erytheocytosis
inc RBC due to another process
80
secondary erythrocytosis labs
inc RBC/hct w/ normal WBC and plt
normal EPO
81
hemochromatosis triad
cirrhosis (abd, hepatomegaly, jaundice)
DM
skin bronzing
82
hemochromatosis lab
inc iron, ferritin, and transferrin
definitive: liver bx (inc iron deposits/ hemosiderin)
83
hemochromatosis tx
phlebotomy
iron chelation: deferoxamine
84
sickle cell dz etiology
beta globin gene (HbS) mutation
autosomal recessive
glutamic acid --> valine
85
sickle cell sx
dactylics @ 6-9mo
joint pain, jaundice, hepatosplenomegaly
priapism, H shaped vertebrae, splenic sequestration
painful occlusive crisis
86
MCC osteomyelitis in children w/ sickle cell
salmonella
87
assoc w/ aplastic crisis in sickle cell dz
parvo B19
88
sickle cell labs
Howell jolly bodies
low HH, low RBC, reticulocytosis
hemoglobin electrophoresis definitive
hair on end appearance on xray
89
tx sickle cell
fluids, pain control, folic acid, iron
PCNV for infx
hydroxyurea to reduce frequency of crisis
90
sickle cell immunizations
S: S. pneumo
Hi: Hib
N: N. meningococcus
91
thalassemia A labs
teardrop cells
hemoglobin electrophoresis
basophilic stippling
92
thalassemia A tx
folic acid
blood transfusion, splenectomy
bone marrow transplant definitive
93
thalassemia B major sx
sx at 6mo. hepatomegaly, frontal bossing, severe hemolytic anemia (pallor, jaundice), osteopenia,
94
thalassemia B labs
target cells, variation of size and shape of RBC
xray: hair on end
hemoglobin electrophoresis definitive
95
thalassemia B tx
iron transfusion, splenectomy if severe
96
hemolytic transfusion reaction sx
due to ABO incompatibility
immediate F, chill, dark urine, hypoTN
+ Coombs test
97
hemolytic transfusion reaction tx
stop transfusion
crystalloid infusion
diuretic therapy
98
febrile transfusion reaction
fever and chills w/in 6hrs
MC transfusion rxn
stop transfusion and supportive (acetaminophen)
99
transfusion related acute lung injury
SOB w/in 30m
resolves spontaneously
100
allergic reaction to transfusion
urticaria/ pruritus likely d/t donors plasma
symptomatic care- antihistamines
101
delayed transfusion reaction
3-4wks after transfusion
fall in hemoglobin, rise in bilirubin
supportive care
102
graft vs host dz
immunocompromised pts
rash, elevated LFT, pancytopenia
103
leukemia associated w/ downs syndrome
acute leukemias
104
mc pediatric leukemia
ALL
105
ALL sx
fever, joint pain, bleeding, lymphadenopathy
hepatosplenomegaly
CNS sx
106
ALL labs
inc WBC blasts
+TdT
+cALLa
107
ALL tx
chemo
stem cell transplant
CNS prophylaxis: intrathecal MTX
good prognosis
108
AML can be caused by
radiation exposure
109
AML labs
inc WBC myeloblasts
auer rods
110
AML tx
chemo
stem cell transplant
111
MC leukemia in adults
CLL
112
CLL labs
inc smudge cells
113
CLL tx
watch and wait
chemoimmunotherapy: fludarabine
stem cell curative
114
CML etiology
unregulated stimulation of tyrosine kinase system
115
CML sx
fatigue, fever, night sweats
hepatosplenomegaly
116
CML labs
philadelphia chromosome t(9;22)
low LAP score
117
CML tx
tyrosine kinase inhibitor: imatinib
bone marrow transplant curative
118
Hodgkins lymphoma sx
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
Hodgkins lymphoma tests
lymph node bx, PET scan, bone marrow bx
reed Sternberg cells (owl eyes) on CBC
high LDH, mediastinal mass on CXR
120
Hodgkins lymphoma tx
chemo
121
non Hodgkins lymphoma sx
can spread extra nodal, highly malignant
fever, night sweats, weight loss, painless lymphadenopathy
122
burkitt lymphoma
type of non-hodgkins, hx EBV,
enlarging mass over jaw (Africa), abd if outside of Africa
starry sky
123
multiple myeloma sx
AA male, elderly
BREAK (bone lesions, recurrent infx, elevated calcium, anemia, kidney failure)
124
multiple myeloma tests
XR: punched out bone lesions
bence jones protein
rouleaux formation
M spike
125
multiple myeloma tx
chemo, bisphosphonates
abx (infx), GCS (hypercalcemia)
stem cell transplant definitive
126
myelodysplastic syndrome
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
prescribing heparin monitor
monitor PTT
128
enoxaparin
LMWH
no need to monitor PTT
129
fractionated LMWH inhibits
X
130
unfractionated heparin inhibits
X and thrombin
131
heparin antidote
protamine sulfate
132
prescribe warfarin monitor
PT-INR
133
warfarin MOA
decrease synthesis of vitamin K clotting factors (2, 7, 9, 10), protein C and S
134
warfarin antidote
vitamin k
fresh frozen plasma
135
direct factor Xa inhibitors
apixaban
rivaroxaban
no need monitor labs
136
direct thrombin inhibitors
dabigatran
used w/ anti platelet agents (ASA, clopidogrel)
137
direct thrombin inhibitors antidote
idarucizumab
138
coagulopathy of advanced liver disease labs and tx
inc PT
low albumin
advanced dz: high PT and PTT with no response to vitamin K
tx w/ FFP
139
factor v leiden sx
hx of miscarriage and DVT at young age
140
factor v leiden dx and tx
genetic testing (FVL mutation) gold
anticoagulants
141
antiphospholipid syndrome MOA
HLA-DR7 mutation
142
antiphospholipid syndrome sx
recurrent thrombotic events, rapid organ failure
renal failure video reticularis (mottled purple discoloration)
recurrent miscarriage
143
antiphospholipid syndrome tests
positive anticardiolipin (false positive in syphilis)
anti-beta 2 glycoprotein abs
144
antiphospholipid syndrome tx
ASA
warfarin
Pregnant: LMWH
145
protein C and S deficiency difference
protein C: unregulated activation of coagulation cascade
protein S: enhanced activity of coagulation cascade
146
protein C and S sx
hyper coagulable state, recurrent DVT/PE
stroke
neonatal purpura fulminans
skin necrosis
147
protein C and S deficiency tx
heparin for life
may develop warfarin induced skin necrosis
148
antithrombin 3 deficiency
venous thrombosis
recurrent DVT/PE
asx need anticoagulation only before surgery
high dose IV heparin then oral anticoagulation indefinitely
149
PT is which factors
1, 2, 5, 10 (common coag pathways)
7 (extrinsic)
150
HIT presentation
someone who got platelets and then after 1 wk, plt drop to 50%
151
auer rods + myeloperoxidase positive subtype of AML
Acute promyelocytic leukemia
152
burkitt lymphoma presentation
Africa, pain and swelling jaw
stars in a dark sky
assoc w/ epstein barr
