Heme Malignancy/ Coag Disorders Flashcards
(152 cards)
1
Q
meyloproliferative disorders include
A
CML
PV
ET
IM
2
Q
ALL effects
A
lymphoid progenitor cells - B, T, NK
3
Q
AML effects
A
myeloid progenitor cells - eosinophil, nuetrophil etc
4
Q
CI to BM exam
A
hemophilia
DIC
severe bleeding disorders
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)
6
Q
cellular component that iniates adaptive immunity
A
luekocytes
7
Q
T/F
WBC are nucelated
A
T
RBC anucelated
8
Q
luekocytosis and penia lab values
A
>11k cytosis
<4k penia
9
Q
4 types of leukemia
A
AML
ALL
CML
CLL
10
Q
Immature cells ate called _____ and when there is >20% = ____ if <20% ________
A
blasts
>20% leukemia
<20% myelodysplastic syndromes
11
Q
Risk factors for developing Myelodysplastic syndrome (MDS
A
>60
expsure to chemicals
chemo/radiation
inherited disorders (fancoci, familial PLT)
12
Q
complciations of Myelodysplastic syndrome (MDS
A
- Complications: IRON OVERLOAD – due to frequent transfusions
- 30% progress to AML
13
Q
how do MDS cases initally present
A
low grade anemia <20% blast in periphery
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
15
Q
etiology of AML
A
Issue w/ myeloid progenitor cell – rapid growth of abnormal myelocytes that acccumulate in BM
16
Q
AML results in:
A
- Anemia (fatigue, weakness, pallor)
- Thrombocytopenia (epistaxis, gingival bleeding, petechiae, purpura)
Neutropenia (persistent/exaggerated (sinus
17
Q
AML has ____% blasts
A
>20% blasts
18
Q
•Leukemia cutis - nodular skin lesions (purplish /grayish) is seen with what dz?
A
AML
19
Q
What would you see in peipheral Smear in pt w/ AML
A
•auer rods in AML cells
20
Q
stages of chemo in AML
A
- Induction chemo
- Re-induction – if relapse
- Consolidation – destroy remaining tumor cells, complete remission is achieved
- 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
21
Q
what is associated with a bad prognosis in AML
A
>60 y/o
elevated LDH
cytogenic abnorm, FLT3 mutation
22
Q
CML is when ____ cells go BAD
A
B cells
23
Q
what disorder is due to abnormality in philedaphia chr
A
CML
24
Q
A
25
CML leads to an increase in proliferation of:
Inc proliferation of granulocytes (neutrophils, basophils, eosinophils)
26
3 phases of CML
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)
27
what dz would we order a FISH looking for BCR-ABL gene rearrangement
CML
28
how would we tx CML with a positive phil chr
negative phil chr?
•Imatinib (Gleevac) – targets phil chr. Does not work with phil chr (-)
- phil chr
* TKI + chemo
* SCT
29
in CML the BCR-ABL1 gene results in:
tyrosine kinase which promotes undiff proliferation
30
in CML how many blasts are in blast vs accelaerated phase
blast 30%
accelerated 15%
31
Si/Sx of chronic phae of CML
anorexia
weight loss
fever
night sweats
fatigue
splenomegaly : LUQ discomfot
NO LYMPHADENOPATHY
32
IN CML, unlike in AML _____ still fucntioning so not as many ____ \_\_\_\_\_
granulocytes
chronic infections.
33
si/sx of accelerated phase in CML
anemia
nuetropenia
priapism
splenomegaly
34
si/sx of blast crisis phase in CML
resembles acute leukemia
rapid progression of BM failure
bone pain
35
most common leukemia in adults
AML
36
most common childhood cancer w/ bimodal distribution
ALL
37
infection is stongly linked to ALL - in adults its _____ and in children its \_\_\_\_\_
HLTV-1
varicella
38
si / sx w/ ALL
* 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)
39
stepwise approach to chemo in ALL
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
40
Lymphoid progenitor cells (B cells and T cells) are altered and reproduce unregulated
ALL
40
Expansion of CD5+ B-cells (immuno-incompetent B-cells) over proliferation of BAD white cells
CLL
41
diagnostic test for CLL
•Flow cytometry - B cells w/ surface antigens (CD19, CD20, CD23, CD5
41
complication of CLL
•Richter's transformation (CLL à ALL) slow disease progression to FAST
42
pt presents w/ generalized, painless lymphadenopathy (HUGE LN’s) – cervical, suprclav, axillary most common
Wells syndrome
and
hemolytic anemia
CLL
43
most common luekemia of US
CLL
44
we should tx CLL w/ chemo in early stages?
no - watch and wait
45
tx of severe CLL
* Chemo
* Immunotherapy
* B-cell signaling inhibitors
* SCT – bad prognosis
* Palliative care
45
genes in CLL that indicate worse prognosis
* 13q (most common) ,
* &/or 17p (worst prognosis) deletion)
46
lab differences in ALL vs CLL
ALL - INC LDH and uric acid
CLL - DEC LDH
47
CLL has a DEC Ig\_\_\_
IgG
48
autoimmune hemolytic anemia is commonly seen with what type of leukemia
CLL
49
Present like CLL but no lymphadenopathy
hair cell - B cells gone array
50
Peripheral smear = Hairy cell present
what type of leukemia
hairy cell
51
hair cell luekemia leads to infiltration of ?
spleen, liver, bone marrow -\> pancytopenia
52
abnormal IgG that leads to -\> Neoplastic proliferation of plasma cells -\> plasmacytoma -\> immune complex deposition in BM
MM
53
bone pain worse w/ ROM is a symptom of
MM
54
MM shoes INC ___ but a LOW ____ level.
protein
albumin
55
In MM the peripheral smear will show
rouleaux formation
56
we would order a serumPEP and urine PEP when checking for
MM
57
This disorder presents with CRAB
define CRAB and name disorder
MM
hypercalcemia
renal insufficency INC Cr
anemia
bone disease (lytic bone lesions)
58
does MM present w/ peripheral nueopathy and palpable LN?
Yes - burning of hands and feet
palp LN rare
59
Tx MM
Supportive care
* Ca ® bisphosphonates
* Renal ® hydration
* Hyperviscosity ® plasmapheresis
* Neuro ® palliative radiation
* Anemia ® transfusion
Chemotherapy
Hematopoietic SCT
60
complications of MM
* Progressive myeloma
* Renal failure
* Sepsis
* Myelodysplasia
61
Birketts is what type of lymphoma
NHL - aggresive, ties to EBV, jaw lympadeopathy in young kids
62
diffuse large be cell is what type of lymphoma
NHL
common in caucasion males
63
what type of lymphoma has protective factors if you exposed to measles, mumps or chicken pox as a child
HL
64
Name 3 classificaitons of NHL
indolent
aggressive
hihgly aggressive
65
dexribe ann-arbor staging for NHL
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
describe annarbor staging for HL
•: 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
wht type of lymphoma is associated with painless lympadeopathy ABOVE diapgragm
HL
68
what dz is assoc w Pel-Ebstein fever (intermittent fever)
HL
69
Dz associated w/ pain at malignant nodal sites w/ alcohol use
HL
70
HL has both classical and nodular presentations: what would we see on peripheral smear of both
classic - reed-sternerg
nodular - popcorn
71
this type of lymphoma
## Footnote
SPREAD ADJACENTLY - start in thorax/mediastinum above the diapgragm and spread adjacently
HL
72
CBC in py w/ HL would show
anemia, leukopenia, neutrophilia, eosinophilia
73
CMP in pt w/ HL would show
•uremia,
hyperca,
hypoglycemia,
hyponatremia,
elevated Alk phos
74
In pts with HL - LDH measures
•marker of bulk/burden of disease
75
Dz associated w/ Painless lymphadenopathy, waxing and waning (Waldeyer ring & MALT)
indolent NHL
76
in aggressive NHL we would see (INC?DEC) LDH and uric acid
INC
77
what dz uses the Cotswold Modification of Ann Arbor Staging – and what does this tell us ??)
HL
how many cycles of chemo ?? (Bulky
78
we would likely see a mediatstinal mass in what dz?
HL
79
In HL what does ESR tell us
•staging \<50
or \<30 w/ B symptoms
80
if a pt presents w/ hemarthrosis what should we automatically be thinking
hemophilia
81
hemophillia A is a deficency of \_\_\_\_\_\_
B \_\_\_\_\_
C\_\_\_\_\_\_
acquired \_\_\_\_
A - F8
B - F9
C-F11
acquired - usually F8 (same as A)
82
hemophillia A and B are _____ recessive
C is ____ recessive and seen in ___ \_\_\_\_
83
which type of hemophilia is likely to be dz as an dult during pregnancy or postpartum
acquired
84
Tx for Hemophilia A
* DDVAP
* Factor VIII products
* Emicizumab – functions like factor 8
* Supportive care
mod-severe bypass DDVAP
85
tx for hemo C
* Factor XI product
* Antifibrinolytic
* (ex. EACA (Epsilon-Aminocaproic Acid or Amicar)
* TXA (Tranexamic Acid))
•
* FFP - if no factor XI
* Supportive care
86
tx for hemo B
* Factor IX products
* Supportive care
87
in hemophilia
mild - \_\_\_% of normal factor activity level
moderate \_\_\_% of normal factor activity level
severe \_\_\_% of normal factor activity level
6-40
1-5
\<1
88
tx for acquired hemophillia
* Emicixumab - functions like F8
* Supportive care
89
•Emicixumab can tx?
Hemo A
Acquired
fucntions like F8
90
Vit K is needed to activate what CF?
II prothrombin
VII
IX
X
synthase of protein C and S
91
liver failure is a mjor cause of
Vit K deficency
92
* Splinter hemorrhages
* Melena
* Hematuria
* ICH
sx of what?
vit K deficency
93
Vit K deficency in infants, how did we overcome?
•VKDB ® ICH &/or death in infants – give infants IM vit K
94
PT/INR
PT
are both elevated/dec in Vit K def
PROLONGED / INC
95
dz that results from an exogenous factor putting tissue factor into overdrive - excess throbin and inc coag cascade
DIC
96
•Purpura fulminans presents w/ what dz?
what should we r/o
DIC
Purpura fulminans – changes rapidly (r/o prtien C deficiency as it is one of the causes)
97
vit K def is caused by
* Liver failure
* Chemo/abx (cephs)
* Warfarin (k antag)
98
D-Dimer and fibronlysis are INC/ DEC in DIC
INC
99
PTT
PT/INR
INC/DEC in DIC
BOTH INC
100
name some causes of DIC
Sepsis - G-
acute promyelocytotic luekemia
vasculitis
OBG complications (pre-eclampsia)
snake bites
101
causes of chronic DIC
solid tumors and aortic anyerusms
102
in DIC factor ___ and ___ will be DEC
V and VIII
103
in DIC blood smear will show
shistocytes
104
tx DIC
* Treat underlying condition
* Hemodynamic support
* FFP/cryoprecipitate
* Transfusion (plt and blood)
•
* +/- heparin
* Protein C conc (purpura fulminans
105
what is relative polycythemia
106
Primary polycythemia vera is due to
secondary:
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
what is #1 CAUSE OF BUDD CHIARI SYND
polycthemia
clot in hepatic vein
108
pt CC is Pruritis following warm bath or shower CC
polychythemia
109
in primary polycythemia EPO is \_\_\_\_
in secondary EPO is \_\_\_\_
primar EOP is dec
secondary EPO is normal or INC
110
tx of polyctthemia in a low-risk pt
* Therapeutic phlebotomy
* Low dose ASA
111
tx polycythemia in high-rsik
* Hydroxyurea
* INF – α
* Ruxolitinib – if hydroxyurea fails
112
prognosis of polycythemia
Bone marrow becomes too fibrotic and moves into “spent phase”
* Myelofibrosis
* Patient will present w/ anemia, thrombocytopenia, leukopenia
112
BM bx is essential when diagnosing primary / secondar PV
primary - shows fibrosis
113
in polycythemia the CBC components will ALL be \_\_\_\_?
INC
114
etiology of thrombocytopenia (4)
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
thrombocytopenia is \<\_\_\_\_\_\_UL
150,000
normal is 150-450
116
HIT is most commonly caused by
UFH - bind to antithrombin III
117
Type II HIT is caused by
autoantibodies against PF4, 5-10 days à leads to clots
118
HIT most commonly leads to what type of clots
•DVT (venous clot more common)
•PE
Cerebral venous sinus thrombosis
* CVA (arterial clots less common)
* MI
* Ischemia
119
Tx HIT
* Stop heparin
* Non-heparin anticoagulant (ex. Anti-Xa inhibitor, direct thrombin inhibitors, DOACs)
•
NO WARFARIN – prothrombotic
120
HIT antibodies Ig\_\_ bind to heparin P4 complexes that lead to activation of PLTs
IgG
121
gold dtsnard dx test for HIT
serotonin assay
122
in HIT PTT ____ and D-dimer and fibrinogen are \_\_\_\_
prolonged
INC
in DIC fibrinogen DEC
123
Autoantibodies against PLT antigens GPIIb/IIIa -\> antibody-platelet complexes destroyed by macrophages in spleen
ITP
124
125
ITP is acute/chronic in children after an infection and is acute/chronic in adults
acute - children
chronic - fertile female adults \>6 mo
126
127
adult with 6mo complaint of heavy menorrhagia and lwo PLTs.
dx
ITP - usually CC
128
caused by ADAMTS13 gene mutation
TTP
129
what plt disorder is a dz of exclusion
ITP
130
whay does ADAMS do norammly in body
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
what dz presents wit classic pentad:
name sx of pentad
TTP
BRAIN FART
* Neuro Sx (Ex. confusion, seizures, HA)
* Fever
* Anemia (ex. hemolytic, shistocytes)
* Renal failure
* Thrombocytopenia
* Splenomegaly
132
Tx of ITP
* Observation (\>30,ooouL no bleeding)
* Corticosteroids
* IVIG
* Platelet transfusion \<10,000uL
* Splenectomy – last line
133
on peripehral smear what is seen in pts w/ TTP
schistocytes
134
is TTP coombs potive or neg
NEG
135
what is the most common inherited coagulopathy 1%
vWD
136
Tx TTP
* Plasmapheresis & plasma exchange – replaces ADAMS
* Glucocorticoids – refractory / relapsing
* Rituximab - refractory / relapsing
* Splenomegaly – rare/ controversial
NO PLT transfusion
137
vWD has 3 subtypes
which is more common and which is more severe
type 1 common
type 3 severe
138
vWD can be inherited or acquired:
inherited through
acquired through:
AD
aortic stenosis
mech heart valve
wilm's tumor
139
TTP has a ____ in haptoblobin and an ____ in LDH
DEc
INC
140
most common finding in vWD
•Mucosal bleeding (ex. epistaxis, gingival bleeding, menorrhagia) – most common finding
141
tx fo vWD
* DDVAP
* vWF os DDVAP doesn’t work
* Factor VIII transfusion (type 3)
* Blood transfusion
* Avoid ASA
142
GOLD standard test for vWD
DEC ristocetin cofactor assay – GOLD standard, tests ability of PLT to bind to ristocetin (dec PLT agglutination)
143
factor ____ is decreased in vWD making it similar to _______ \_\_
F8
hemo A
144
define PT
prothrombin time - time it takes to clot (seconds) using extrsinsic and commpn pathway
one T = exes
145
define PTT
146
name factors in extrinsic
I, II, VII, and X
PT/INR
147
name factors in intrinsic
148
common pathway factors
I, II, V, VIII, X
