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Clin Med I Exam 3 Heme/ENT > Heme Malignancy/ Coag Disorders > Flashcards

Heme Malignancy/ Coag Disorders Flashcards

1
Q

meyloproliferative disorders include

A

CML

PV

ET

IM

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2
Q

ALL effects

A

lymphoid progenitor cells - B, T, NK

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3
Q

AML effects

A

myeloid progenitor cells - eosinophil, nuetrophil etc

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4
Q

CI to BM exam

A

hemophilia

DIC

severe bleeding disorders

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5
Q

sites for BM aspiration

A

*posterior superior iliac crest and spine

anterior iliac crest

greatcher troch of femur

vertebral body/ rubs

sternum (usually CI)

tibial (children <12-18 mo)

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6
Q

cellular component that iniates adaptive immunity

A

luekocytes

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7
Q

T/F

WBC are nucelated

A

T

RBC anucelated

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8
Q

luekocytosis and penia lab values

A

>11k cytosis

<4k penia

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9
Q

4 types of leukemia

A

AML

ALL
CML

CLL

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10
Q

Immature cells ate called _____ and when there is >20% = ____ if <20% ________

A

blasts

>20% leukemia

<20% myelodysplastic syndromes

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11
Q

Risk factors for developing Myelodysplastic syndrome (MDS

A

>60

expsure to chemicals

chemo/radiation

inherited disorders (fancoci, familial PLT)

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12
Q

complciations of Myelodysplastic syndrome (MDS

A
  • Complications: IRON OVERLOAD – due to frequent transfusions
  • 30% progress to AML
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13
Q

how do MDS cases initally present

A

low grade anemia <20% blast in periphery

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14
Q

tx of MDS

A

•Supportive care (ex. transfusions, EGF, antibiotics)

Chemo (ex. decitabine, lenalidomide)

  • 5-azacytidine
  • Decitabine – CR 43%
  • Lelinomide – dec transfusions (only pts w/ 5q deletions)
  • Hematopoietic SCT
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15
Q

etiology of AML

A

Issue w/ myeloid progenitor cell – rapid growth of abnormal myelocytes that acccumulate in BM

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16
Q

AML results in:

A
  • Anemia (fatigue, weakness, pallor)
  • Thrombocytopenia (epistaxis, gingival bleeding, petechiae, purpura)

Neutropenia (persistent/exaggerated (sinus

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17
Q

AML has ____% blasts

A

>20% blasts

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18
Q

•Leukemia cutis - nodular skin lesions (purplish /grayish) is seen with what dz?

A

AML

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19
Q

What would you see in peipheral Smear in pt w/ AML

A

•auer rods in AML cells

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20
Q

stages of chemo in AML

A
  1. Induction chemo
  2. Re-induction – if relapse
  3. Consolidation – destroy remaining tumor cells, complete remission is achieved
  4. Maintenance – maintain remission, up to 24 mo (more common in ALL)

SCT (if bad prognosis based on cytogenetics or poor response to re-induction) induction à SCT

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21
Q

what is associated with a bad prognosis in AML

A

>60 y/o

elevated LDH

cytogenic abnorm, FLT3 mutation

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22
Q

CML is when ____ cells go BAD

A

B cells

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23
Q

what disorder is due to abnormality in philedaphia chr

A

CML

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24
Q
A
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25
Q

CML leads to an increase in proliferation of:

A

Inc proliferation of granulocytes (neutrophils, basophils, eosinophils)

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26
Q

3 phases of CML

A

Blast phase (blast crisis)® AML (bad progmosis)

  • Rapid progression of bone marrow failure
  • Bone pain (resembles acute leukemia)

Accelerated phase

  • Worsening anemia
  • Profound neutropenia
  • Extreme pleocytosis

Chronic phase (up to 10 yrs)

  • Insidious onset/fatigue
  • Significant leukocytosis
  • Anorexia/weight loss
  • Fever/night sweats
  • Granulocytes functioning so not many chronic infections. (unlike AML)
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27
Q

what dz would we order a FISH looking for BCR-ABL gene rearrangement

A

CML

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28
Q

how would we tx CML with a positive phil chr

negative phil chr?

A

•Imatinib (Gleevac) – targets phil chr. Does not work with phil chr (-)

  • phil chr
  • TKI + chemo
  • SCT
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29
Q

in CML the BCR-ABL1 gene results in:

A

tyrosine kinase which promotes undiff proliferation

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30
Q

in CML how many blasts are in blast vs accelaerated phase

A

blast 30%

accelerated 15%

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31
Q

Si/Sx of chronic phae of CML

A

anorexia

weight loss

fever

night sweats

fatigue

splenomegaly : LUQ discomfot

NO LYMPHADENOPATHY

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32
Q

IN CML, unlike in AML _____ still fucntioning so not as many ____ _____

A

granulocytes

chronic infections.

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33
Q

si/sx of accelerated phase in CML

A

anemia

nuetropenia

priapism

splenomegaly

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34
Q

si/sx of blast crisis phase in CML

A

resembles acute leukemia

rapid progression of BM failure

bone pain

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35
Q

most common leukemia in adults

A

AML

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36
Q

most common childhood cancer w/ bimodal distribution

A

ALL

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37
Q

infection is stongly linked to ALL - in adults its _____ and in children its _____

A

HLTV-1

varicella

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38
Q

si / sx w/ ALL

A
  • Sudden onset of bone marrow failure ® malaise, fatigue, bleeding, bruising, secondary infections
  • Hepatosplenomegaly
  • B symptoms (fever, weight loss, night sweats)
  • CNS involvement (meningeal spread)
  • Painless lymphadenopathy
  • Testicular enlargement (rare)
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39
Q

stepwise approach to chemo in ALL

A
  1. Induction chemo
  2. Re-induction – if relapse
  3. Consolidation – destroy remaining tumor cells, complete remission is achieved

Maintenance – maintain remission, up to 24 mo (more common in ALL

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40
Q

Lymphoid progenitor cells (B cells and T cells) are altered and reproduce unregulated

A

ALL

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40
Q

Expansion of CD5+ B-cells (immuno-incompetent B-cells) over proliferation of BAD white cells

A

CLL

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41
Q

diagnostic test for CLL

A

•Flow cytometry - B cells w/ surface antigens (CD19, CD20, CD23, CD5

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41
Q

complication of CLL

A

•Richter’s transformation (CLL à ALL) slow disease progression to FAST

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42
Q

pt presents w/ generalized, painless lymphadenopathy (HUGE LN’s) – cervical, suprclav, axillary most common

Wells syndrome

and

hemolytic anemia

A

CLL

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43
Q

most common luekemia of US

A

CLL

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44
Q

we should tx CLL w/ chemo in early stages?

A

no - watch and wait

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45
Q

tx of severe CLL

A
  • Chemo
  • Immunotherapy
  • B-cell signaling inhibitors
  • SCT – bad prognosis
  • Palliative care
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45
Q

genes in CLL that indicate worse prognosis

A
  • 13q (most common) ,
  • &/or 17p (worst prognosis) deletion)
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46
Q

lab differences in ALL vs CLL

A

ALL - INC LDH and uric acid

CLL - DEC LDH

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47
Q

CLL has a DEC Ig___

A

IgG

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48
Q

autoimmune hemolytic anemia is commonly seen with what type of leukemia

A

CLL

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49
Q

Present like CLL but no lymphadenopathy

A

hair cell - B cells gone array

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50
Q

Peripheral smear = Hairy cell present

what type of leukemia

A

hairy cell

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51
Q

hair cell luekemia leads to infiltration of ?

A

spleen, liver, bone marrow -> pancytopenia

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52
Q

abnormal IgG that leads to -> Neoplastic proliferation of plasma cells -> plasmacytoma -> immune complex deposition in BM

A

MM

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53
Q

bone pain worse w/ ROM is a symptom of

A

MM

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54
Q

MM shoes INC ___ but a LOW ____ level.

A

protein

albumin

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55
Q

In MM the peripheral smear will show

A

rouleaux formation

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56
Q

we would order a serumPEP and urine PEP when checking for

A

MM

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57
Q

This disorder presents with CRAB

define CRAB and name disorder

A

MM

hypercalcemia

renal insufficency INC Cr

anemia

bone disease (lytic bone lesions)

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58
Q

does MM present w/ peripheral nueopathy and palpable LN?

A

Yes - burning of hands and feet

palp LN rare

59
Q

Tx MM

A

Supportive care

  • ­ Ca ® bisphosphonates
  • Renal ® ­ hydration
  • Hyperviscosity ® plasmapheresis
  • Neuro ® palliative radiation
  • Anemia ® transfusion

Chemotherapy

Hematopoietic SCT

60
Q

complications of MM

A
  • Progressive myeloma
  • Renal failure
  • Sepsis
  • Myelodysplasia
61
Q

Birketts is what type of lymphoma

A

NHL - aggresive, ties to EBV, jaw lympadeopathy in young kids

62
Q

diffuse large be cell is what type of lymphoma

A

NHL

common in caucasion males

63
Q

what type of lymphoma has protective factors if you exposed to measles, mumps or chicken pox as a child

A

HL

64
Q

Name 3 classificaitons of NHL

A

indolent

aggressive

hihgly aggressive

65
Q

dexribe ann-arbor staging for NHL

A

I: single LN
II: multiple LN on same side of diaphragm

•III: multiple LN on both sides of diaphragm

IV: greater then or one extralymphatic site (BM, lung) with distal node involvement

66
Q

describe annarbor staging for HL

A

•: single LN
II: multiple LN on same side of diaphragm

  • III: multiple LN on both sides of diaphragm
  • IV: multiple extranodoal sites or LN and extranodal disease

X: bulk >10cm (unfavorable)

E: extra nodal extension

A/B: B symptoms weight loss >10%, fever, drenching night sweats

**LOOKING AT BULK

67
Q

wht type of lymphoma is associated with painless lympadeopathy ABOVE diapgragm

A

HL

68
Q

what dz is assoc w Pel-Ebstein fever (intermittent fever)

A

HL

69
Q

Dz associated w/ pain at malignant nodal sites w/ alcohol use

A

HL

70
Q

HL has both classical and nodular presentations: what would we see on peripheral smear of both

A

classic - reed-sternerg

nodular - popcorn

71
Q

this type of lymphoma

SPREAD ADJACENTLY - start in thorax/mediastinum above the diapgragm and spread adjacently

A

HL

72
Q

CBC in py w/ HL would show

A

anemia, leukopenia, neutrophilia, eosinophilia

73
Q

CMP in pt w/ HL would show

A

•uremia,

hyperca,

hypoglycemia,

hyponatremia,

elevated Alk phos

74
Q

In pts with HL - LDH measures

A

•marker of bulk/burden of disease

75
Q

Dz associated w/ Painless lymphadenopathy, waxing and waning (Waldeyer ring & MALT)

A

indolent NHL

76
Q

in aggressive NHL we would see (INC?DEC) LDH and uric acid

A

INC

77
Q

what dz uses the Cotswold Modification of Ann Arbor Staging – and what does this tell us ??)

A

HL

how many cycles of chemo ?? (Bulky

78
Q

we would likely see a mediatstinal mass in what dz?

A

HL

79
Q

In HL what does ESR tell us

A

•staging <50

or <30 w/ B symptoms

80
Q

if a pt presents w/ hemarthrosis what should we automatically be thinking

A

hemophilia

81
Q

hemophillia A is a deficency of ______

B _____

C______

acquired ____

A

A - F8

B - F9

C-F11

acquired - usually F8 (same as A)

82
Q

hemophillia A and B are _____ recessive

C is ____ recessive and seen in ___ ____

A
83
Q

which type of hemophilia is likely to be dz as an dult during pregnancy or postpartum

A

acquired

84
Q

Tx for Hemophilia A

A
  • DDVAP
  • Factor VIII products
  • Emicizumab – functions like factor 8
  • Supportive care

mod-severe bypass DDVAP

85
Q

tx for hemo C

A
  • Factor XI product
  • Antifibrinolytic
  • (ex. EACA (Epsilon-Aminocaproic Acid or Amicar)
  • TXA (Tranexamic Acid))

•

  • FFP - if no factor XI
  • Supportive care
86
Q

tx for hemo B

A
  • Factor IX products
  • Supportive care
87
Q

in hemophilia

mild - ___% of normal factor activity level

moderate ___% of normal factor activity level

severe ___% of normal factor activity level

A

6-40

1-5

<1

88
Q

tx for acquired hemophillia

A
  • Emicixumab - functions like F8
  • Supportive care
89
Q

•Emicixumab can tx?

A

Hemo A

Acquired

fucntions like F8

90
Q

Vit K is needed to activate what CF?

A

II prothrombin

VII

IX

X

synthase of protein C and S

91
Q

liver failure is a mjor cause of

A

Vit K deficency

92
Q
  • Splinter hemorrhages
  • Melena
  • Hematuria
  • ICH

sx of what?

A

vit K deficency

93
Q

Vit K deficency in infants, how did we overcome?

A

•VKDB ® ICH &/or death in infants – give infants IM vit K

94
Q

PT/INR

PT

are both elevated/dec in Vit K def

A

PROLONGED / INC

95
Q

dz that results from an exogenous factor putting tissue factor into overdrive - excess throbin and inc coag cascade

A

DIC

96
Q

•Purpura fulminans presents w/ what dz?

what should we r/o

A

DIC

Purpura fulminans – changes rapidly (r/o prtien C deficiency as it is one of the causes)

97
Q

vit K def is caused by

A
  • Liver failure
  • Chemo/abx (cephs)
  • Warfarin (k antag)
98
Q

D-Dimer and fibronlysis are INC/ DEC in DIC

A

INC

99
Q

PTT

PT/INR

INC/DEC in DIC

A

BOTH INC

100
Q

name some causes of DIC

A

Sepsis - G-

acute promyelocytotic luekemia

vasculitis

OBG complications (pre-eclampsia)

snake bites

101
Q

causes of chronic DIC

A

solid tumors and aortic anyerusms

102
Q

in DIC factor ___ and ___ will be DEC

A

V and VIII

103
Q

in DIC blood smear will show

A

shistocytes

104
Q

tx DIC

A
  • Treat underlying condition
  • Hemodynamic support
  • FFP/cryoprecipitate
  • Transfusion (plt and blood)

•

  • +/- heparin
  • Protein C conc (purpura fulminans
105
Q

what is relative polycythemia

A
106
Q

Primary polycythemia vera is due to

secondary:

A

primary - genetic mutation JAK2

secondary

  1. Appropriate compensatory response to hypoxia leading to an increase in EPO production
  2. Inappropriate due to EPO secreting tumors
107
Q

what is #1 CAUSE OF BUDD CHIARI SYND

A

polycthemia

clot in hepatic vein

108
Q

pt CC is Pruritis following warm bath or shower CC

A

polychythemia

109
Q

in primary polycythemia EPO is ____

in secondary EPO is ____

A

primar EOP is dec

secondary EPO is normal or INC

110
Q

tx of polyctthemia in a low-risk pt

A
  • Therapeutic phlebotomy
  • Low dose ASA
111
Q

tx polycythemia in high-rsik

A
  • Hydroxyurea
  • INF – α
  • Ruxolitinib – if hydroxyurea fails
112
Q

prognosis of polycythemia

A

Bone marrow becomes too fibrotic and moves into “spent phase”

  • Myelofibrosis
  • Patient will present w/ anemia, thrombocytopenia, leukopenia
112
Q

BM bx is essential when diagnosing primary / secondar PV

A

primary - shows fibrosis

113
Q

in polycythemia the CBC components will ALL be ____?

A

INC

114
Q

etiology of thrombocytopenia (4)

A

dec production (vit b12/folate def, liver dz, chemo, aplastic anemia

inc destruction - immune (ITP, HIT) nonimmune (DIC, TTP, HELP syndrom)

inc sequesteration - portal HTN, alc, liver dz, splenalomegaly

dilutional - IV fluids/ blood tranfusions

115
Q

thrombocytopenia is <______UL

A

150,000

normal is 150-450

116
Q

HIT is most commonly caused by

A

UFH - bind to antithrombin III

117
Q

Type II HIT is caused by

A

autoantibodies against PF4, 5-10 days à leads to clots

118
Q

HIT most commonly leads to what type of clots

A

•DVT (venous clot more common)

•PE
Cerebral venous sinus thrombosis

  • CVA (arterial clots less common)
  • MI
  • Ischemia
119
Q

Tx HIT

A
  • Stop heparin
  • Non-heparin anticoagulant (ex. Anti-Xa inhibitor, direct thrombin inhibitors, DOACs)

•
NO WARFARIN – prothrombotic

120
Q

HIT antibodies Ig__ bind to heparin P4 complexes that lead to activation of PLTs

A

IgG

121
Q

gold dtsnard dx test for HIT

A

serotonin assay

122
Q

in HIT PTT ____ and D-dimer and fibrinogen are ____

A

prolonged

INC

in DIC fibrinogen DEC

123
Q

Autoantibodies against PLT antigens GPIIb/IIIa -> antibody-platelet complexes destroyed by macrophages in spleen

A

ITP

124
Q
A
125
Q

ITP is acute/chronic in children after an infection and is acute/chronic in adults

A

acute - children

chronic - fertile female adults >6 mo

126
Q
A
127
Q

adult with 6mo complaint of heavy menorrhagia and lwo PLTs.

dx

A

ITP - usually CC

128
Q

caused by ADAMTS13 gene mutation

A

TTP

129
Q

what plt disorder is a dz of exclusion

A

ITP

130
Q

whay does ADAMS do norammly in body

A

vWF (protein that leads to PLT adhesion) usually is cleaved by ADAMS

in TTP:
•Dec ADAMS -> INC vWF multimers -> PLT adhesion -> PLT microthrombi -> RBC sheared -> shistocytes and DEC RBC.

131
Q

what dz presents wit classic pentad:

name sx of pentad

A

TTP

BRAIN FART

  • Neuro Sx (Ex. confusion, seizures, HA)
  • Fever
  • Anemia (ex. hemolytic, shistocytes)
  • Renal failure
  • Thrombocytopenia
  • Splenomegaly
132
Q

Tx of ITP

A
  • Observation (>30,ooouL no bleeding)
  • Corticosteroids
  • IVIG
  • Platelet transfusion <10,000uL
  • Splenectomy – last line
133
Q

on peripehral smear what is seen in pts w/ TTP

A

schistocytes

134
Q

is TTP coombs potive or neg

A

NEG

135
Q

what is the most common inherited coagulopathy 1%

A

vWD

136
Q

Tx TTP

A
  • Plasmapheresis & plasma exchange – replaces ADAMS
  • Glucocorticoids – refractory / relapsing
  • Rituximab - refractory / relapsing
  • Splenomegaly – rare/ controversial

NO PLT transfusion

137
Q

vWD has 3 subtypes

which is more common and which is more severe

A

type 1 common

type 3 severe

138
Q

vWD can be inherited or acquired:

inherited through

acquired through:

A

AD

aortic stenosis

mech heart valve

wilm’s tumor

139
Q

TTP has a ____ in haptoblobin and an ____ in LDH

A

DEc

INC

140
Q

most common finding in vWD

A

•Mucosal bleeding (ex. epistaxis, gingival bleeding, menorrhagia) – most common finding

141
Q

tx fo vWD

A
  • DDVAP
  • vWF os DDVAP doesn’t work
  • Factor VIII transfusion (type 3)
  • Blood transfusion
  • Avoid ASA
142
Q

GOLD standard test for vWD

A

DEC ristocetin cofactor assay – GOLD standard, tests ability of PLT to bind to ristocetin (dec PLT agglutination)

143
Q

factor ____ is decreased in vWD making it similar to _______ __

A

F8

hemo A

144
Q

define PT

A

prothrombin time - time it takes to clot (seconds) using extrsinsic and commpn pathway

one T = exes

145
Q

define PTT

A
146
Q

name factors in extrinsic

A

I, II, VII, and X

PT/INR

147
Q

name factors in intrinsic

A
148
Q

common pathway factors

A

I, II, V, VIII, X

Clin Med I Exam 3 Heme/ENT (3 decks)

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