Word association Flashcards
1
Q
c-Kit
A
on stem cell suface to allow proper maturation
2
Q
MPL
A
TPO receptor
3
Q
GPIIb/IIIa autoimmune receptor
A
ITP
4
Q
G-CSF
A
granulocyte
5
Q
M-CSF
A
macrophage
6
Q
mutated neutrophil elastase
A
familial cyclinc neurtopenia
7
Q
<50% fat
A
normal bone biopsy
8
Q
low MCHC
A
hereditary spherocytosis
9
Q
DMT1
A
uptake of iron in gut
10
Q
ferritin
A
storage of iron
11
Q
ferroportin
A
transporter of iron
12
Q
transferrin
A
iron binder in blood
13
Q
hepcidin
A
suppress release of iron
14
Q
angular stomatitis
A
iron deficiency
15
Q
central pallor
A
iron deficiency
16
Q
upregulation of hepcidin
A
anemia of chronic disease
17
Q
basophilic stippling in RBCs
A
lead inhibition of heme synthesis
18
Q
nucleocytoplasmic asynchrony
A
pernicious anemia
19
Q
hemoglobin H
A
alpha thalassemia
20
Q
increase antithrombin 3 activity
A
heparin
21
Q
heparin inhibits
A
thrombin 2, factor 9, 5 and 11
22
Q
EPO and IL3
A
stimulate red cell growth
23
Q
TPO and IL6
A
act on megakaryocyte lineage
24
Q
four inherited aplastic anemias
A
fanconi anemia - DNA repair
Schwachman-Diamond syndrome - SBDS
dyskeratosis congeneta - affect telomerase
congenital amegakaryocytosis thrombocytopenia - mutation in MPL
25
parvovirus B19
can cause aplastic anemia
26
PIG-A
paroxymal nocturnal hemoglobinuria - used for coupling cell surface protein to GP1
27
CD 55 and CD59
lacking in paroxymal nocturnal hemoglobinuria
28
worms wheezes and weird disease
eosinphils - TB, sarcoidosis, addisons and hodgkins
29
- high basophils
CML
30
C5a
chemoattractant for neutrophils
31
integrin and selection
integrin (vessel) and selection (white cell)
32
disorder of selctins or integrins
LAD
33
disorder of poor formation of lysozyme granules
chediak higashi
34
hyperimmunoglobulin E
Job's syndrome - mutation of STAT3 leading to less INF-gamma
35
NBT test
for CGD
36
toxic granules
show infections in neutrophils
37
Dohle bodies
in sepsis, seen in neutrophils, ribosome rich ER
38
Pelger Huet abnormality
bilobed nuetrophils - can lead to myelodysplastic syndrome
39
stress reticulocytosis
after bleed, increase in reticulocytes due to storage in bone marrow
40
elevated MHCH
hereditory spherocytosis
41
microcytic anemia with elevated retic
thalessemia
42
ringed sideroblasts
sideroblastic anemia - problem with heme formation
43
example of microcytic anemia that is hemolytic
thalassemia
44
defective HFE
hemochromatosis
45
blackfan diamond syndrome
inherited pure red cell aphasia
46
folate and iron absorption
both in duodenum and folate in proximal ileum
47
increases in iron absorption
vitamin C, pregnancy, EPO and intake of iron
48
source of B12
animals
49
parietal cell autoantibodies
pernicious anemia
50
elevation of MMA
diagnostic test for B12 deficiency
51
metformin use
can lead to B12 deficiency due to impaired gut absorption
52
hemoglobinuria in hemolytic anemia
only in intravascular anemia
53
haptoglobin
heoglobin scavenging protein
54
march hemoglobinuria
extrisic destruction of RBC to due intense activity
55
mechanical devices can cause
DIC - extrisic hemolytic anemia
56
target cells
phospholipid loading in early liver disease
| also thalassemias
57
acanthocytes
in advanced liver disease due to cholesterol, trouble leaving the spleen leading to advance hemoglytic anemia
58
cold agglutination
IgM
59
warm agglutination
IgG
60
IgM/C3b removal of red cells
in the liver
61
IgG removal of red cells
in the spleen, slower blood flow
62
chromic AIHA is a sign of
underlying illness
63
cold agglutination associated with ....
CLL and lymphoma, viral infections and HIV, EBV
64
penicillin as a hapten
can lead to drug induced hemolytic anemia
65
methyldopa and l dopa
can produce autoantibodies to red cells
66
spektrin
major cytoskeletal protein in RBC
67
spektrin or ankrin defeciency
hereditary spherocytosis
68
defective horizontal interactions of membrane proteins
hereditary elliptocytosis
69
thermal instability of rbc membrane
Hereditary Pyropoikilocytosis
70
Heinz bodies
G6PD - oxidized hemoglobin
71
treatments for IgG hemolysis
targeted at spleen, steriods, IVIG, chemo and spleenectomy
red cell transfusion
72
treatments for IgM hemolysis
plasmapheresis
red cell transfusion
73
spleenomegaly in intravascular or extravascular hemolysis
in extravascular, red cell destruction is mediated by RES system
74
mono is associated with...
Igm cold aggultination
75
increased RBC destruction leads to increase in what bilirubin
indirect
76
bite cells
caused by Heinz bodies in G6PD
77
Tissue factor (TF)
initiates coagulation cascade
78
Gp Ib binds to
vWf
79
GP Ia/IIb binds to
collagen
80
GpIIB/IIIa binds to
fibrinogen
81
activator of protein C and S
thrombomodulin
82
prostacyclin
inhibits platlet activation in quiescent state
83
bleeding from the umbilicus
factor 13 deficiency
84
hemorrhagic disease of newborn
failure of liver to form resulting in vitamin K deficiency
85
factors that thrombin activates
TF:7a pathway and 5, 8 and 13
86
contents of dense granules
ADP, calcium and serotonin
87
Bernard Solier syndrome
deficiency in GPIb - cant bind to vWf
88
ristocetin
aggregation agonist, requires vWf and intact surface membrane
failure implies von willebrand or bernard solliers disease
89
PFA-100
used to test platelet function
90
DDAVP
used for von willebrand treatment, relseases vWf
91
gray platelet
absent alpha granules
92
TAR syndrome
low platelet, absent radii
93
may-hegglin anomaly
giant platelets, thrombocytopenia, dohle bodies
94
pentatd of TTP
fever, thrombocytopenia,
| hemolytic anemia, acute renal failure, and neurologic dysfunction.
95
infection with e coli 157
HUS - hemolytic uremic syndrome
96
ADAMS13
in TTP, leads to production of large multimers
97
antibodies against PF4
heparin induced thrombocytopenia, usually unfractionated heparin
98
paroxymal thrombosis
in heparin induced thrombocytopenia
99
Hb H disease
three deletions of hemoglobin A
100
gene deletion (alpha/beta)
alpha
101
constant spring
non-deletion of alpha gene, premature stop codon
102
point mutations (alpha/beta)
beta
103
Hb E
beta thalassemia
104
Hb barts
gamma tetramers, form in utero in alpha thal
105
deferoxamine
used for treatment of iron overload with chelation
106
Howell Jolly bodies
Sickle cell - DNA left over after leaving bone marrow
107
positive sickle cell test
cell are lyzed and polymerization equals sickle disease
108
4 crises state
vasooclusive crisis
hemolytic crisis
aplastic crisis
sequestration crisis
109
moyamoya
sickle cell - new blood vessels form around clots and are prone to bleeding
110
sickle cell and NO
release of hemoglobin leads to destruction of NO
111
vWf stabilized factor __
8
112
alloimmunization occurs in patients with...
chronic transfusions (sickle cell)
113
most common source of infection in dontions
platelets
114
TRALI
acute lung injury from transfusion
115
TACO
heart overload state from transfusion
116
bridging
while pt on hep,start warfarin, check PT, INR
117
tumor lysis syndrome
ALL
118
20% blasts
acute leukemia
119
myeloperoxidase positive
AML
120
Tdt positive
ALL
121
CD 13, 33, 117
AML
122
T and B cell blasts
ALL
123
cytogenetics of AML
```
t8:21 AML-ETO1 - favorable
inv (16) - favorable
t15:17 - favorable
del(5 or 7)
11q23
inv(3)
```
124
12:21
good prognosis
125
pulmonary toxicity
bleomycin
126
top two NHL
diffuse large B cell and follicular
127
prognosis in NHL
```
A - age
P - performance
L - LDH
E - extranodal
S - stage
```
128
c-myc, bcl1/2, IgG heavy chain
NHL
129
IL3
erythroid progenitor
130
IL6
lymphoid progenitor
131
bone aspiration is good for
morphology, percentage, analyses, flow, cytogenetics
132
bone biopsy is good for
fixitave, cellularyity, arcitechture, antibodies to suface antigens
133
myeloid to erythroid ratio
3:1
134
PMH
mutation in PIG-!, decresed expression of GPI , CD55, CD59, sensitive to pomplement lysis
**thrombosis
135
two causes of aplastic anemia
immune mediated suppression or stem cell damage
136
two treatments for aplastic anemia
1. immunsuppression - ATG, cyclosporin, steriods
| 2. BMT
137
margination
neutrophils stick to vessel wall
138
diapedesis
WBC moves through vessel
139
pelger huet anomaly
WBC variant - bilobed nucleus, begnign hereditary disorder, can lead to myelodysplastic syndrome
140
dohle body
WBC variant - cytoplasmic inclusions, ribosome rich seen in sepsis and G-CSF use
141
toxic granulation
WBC variant - dark blue granules seen in infection or G-CSF use
142
Chediak-Higashi
failure of phagalsome
143
Jobs syndrome
Mutations in STAT3, elevated IgE, defect in chemotaxis*
144
NBT test
negative in CGD
145
orthostatic hypertension
anemia
146
ferroportin
gets iron in from the gut
147
transferrin
iron is bound to it in the blood
148
hepcidin
upregulated when iron is high
149
ferritin
iron stroage`
150
hemosiderin
inron storage - unavilable
151
1 ml of packed RBC contained how much iron
1 mg
152
B12 or folate - neurologic?
B12 - posterior column and peripheral nerves
153
Macrocytic vs megaloblastic
Macrocytic (large) vs megaloblastic (large with nuclear/cytoplasmic dysnchromy)
154
haptoglobin
binds heme
155
intra vs extravascular hemolysis
intra - RBC break and relsese of free heme
extra - RES
dark urine vs enlarged spleen
156
abnormal ostmotic fragility
HS
157
increased MCHC
HS
158
vertical interaction
HS
159
horizontal interactions
ES
160
polychromasia
hemolytic anemia
161
basophilic stippling and spherocytes
hallmark of hemolysis
162
IgG vascular?
extra, removing IgG coated cells
163
IgM vascular?
intra - requires plasmapheresis, steriods and spleenectomy dont work
164
malaria, bartonellosis, babesiosis
infections causing hemolytic anemia
165
hemoglobin A2
Alpha and delta
166
hydrops fetalis
deletion of 4 alpha
167
Hb H disease
deletion of 3 alpha
168
hemoglobin deletion
alpha
169
hemoglobin mutation
beta
170
asian alpha thal
Hb constant spring
171
Hb Barts
gamma tetramers in utero
172
alpha thal diagnosed by
PCR
173
NO-hemoglobin interaction
intravascular hemolysis, NO binds to hemoglobin leading to vasoconstriction
174
deoxygenation leads to
sickling
175
acute dactylitis
sickle cell
176
howell jolly bodies
sickle cell - DNA remnants after nucleus extrusion from marrow
177
4 sickle cell crises
vasooclusive
aplastic - due to parvo B19
hemolytic
sequestration
178
indication for transfusion in sickle cell
ACS and stroke prophylaxis
179
L-glutamine
less oxidant stress, decrease pain crisis in sickle cell
180
TF:FVIIa activates
FX and FIX
181
FXIII
transglutaminse
182
thrombin time
fibrinogen to fibrin clot
183
GPIb receptor deficiency
Bernard Soulier - prolonged bleeding, big platelets
184
GPIIa/IIIb defect
binds to fibinogen - glasmanns thrombasthenia abnormal aggregation
185
vWB type 2B
defect is in the platelet, can use mixing study with patients platelets
186
anti thrombotic factors
tissue factor pathway inhibitor (TFPI) ATII (9,10,11) Protein C and S (5,8)
187
APC
activated protein C resistance - similar to favor V leiden
188
hyperhomocysteinemia
inheritied thrombophilic disorder (MTHFR)
189
estrogen and clotting
acquired cause of thrombophilia
190
APLA
antibodies against GP1 and prothrombin, leads to thrombosis, does not cause bleeding with with PTT long
191
PF4 complex
HIT
192
storage conditions
RBC - 4-6
platelets - RT
FFP and cryo - -65
193
72 hour rule
cross must be sent every 72 hours to check for new antibodies
194
irradiation
for less GVHD, immune compromised
195
filtration
for leukoreduction
196
leukodepletion
CMV safe, reduces risk of allergic reactions
197
presentation of AML/ALL
1. BM failure
2. Leukostatsis
3. Coagulopathy
4. Extra medullary manifesations
5. Metabolic disturbances
198
metabolic disturabance in AML/ALL
1. tumor lysis
2. hypokalemia (AML - kdney)
3. hypoglycemia - artificial
199
auer rods
linear aggregates of primary granules seen in myelobalsts in AML
200
diagnosis with myleoperoxidase
AML
201
diagnosis with Tdt
ALL
202
favorable AML
t15:17
t8:21
inv16
203
unfavorable AML
11q23
| del 5 or 7
204
favorable ALL
t12:21
205
unfavorable ALL
9:22
MLL on 11
4:11
206
meyloid markers
CD 33 CD 117 CD13
207
monocytic subtypes of ALL
skin and gingiva
208
PML-RAR
APL - type of 15:17 AML
209
CHOP
Cyclophosphamide
H - doxorubicin
O - vicristine
P - predisone
210
EBV
associated with burkitts lymphoma and hogskins lymphoma
211
cell markers of HL
NOT CD20, instead CD15 and CD30
212
types of HL
1. nodular sclerosis
2. lymphocyte rich
3. mixed
4. lymphocyte poor
213
ABVD
```
for HL
A - adriamycin (cardio)
B - bleomycin (pulmonary)
V - vinblastine (neurologic)
D - dacarbizine (hematologic)
```
214
sequential involvement of lymph nodes
HL
215
isolated CD5 expression
in CLL, with no other T cell markers
216
immune complications of CLL
hemolytic anemia
RBC aplasia
ITP
neutropenia
217
what factors help with CLL prognosis
CD38
Zap70
FISH
IgVh
218
Staging of CLL
low: lymphocytosis
intermediate: lymph/hepatosplenomegaly
high: anemia/thrombocytopenia
219
smudge cell
CLL
220
CRAB
```
multiple myeloma
C - calcemia
R - Renal dysfunction
A - anemia
B - bone lesions
```
221
environment needed for multiple myeloma
adhesion molecules and IL6
222
major and minor criteria in MM
Major: increased plasma cells, increased M spike
Minor: 10-30% plasma cells, minor M spike, bone lesions, low antibodies
223
progression of MM
MGUS - low spike, low PC
smolering MM - high spike or higher PC
- both above with no CRAB
symptomatic - high spike and high PC and symptoms
224
renal disease in MM
```
cast nephropathy - light chains injure tubules
amyloidosis - light chain in interstitium
deposition disease (MIDD)- proteinuria and renal insufficiency
```
225
two components for scoring in MM
albumin and beta-2 microglobulin
226
treatments in MM
melphalan, steroids, transplant, thalidomide, lenalidomide, proteasome inhibitors
227
Waldenstrom’s Macroglobulinemia
type of lymphobpasmactyic lymphoma (type of NHL) - Igm spike, no bone lesions
228
stacked coin appearance
MM
229
type 2 cyroglobulinemia associated with...
Hep C
230
PMF cell
megakaryocytes drive fibroblast production
231
ringed sideroblasts
myelodysplascit red cells
232
newly acquired puget huer anolmly
myelodysplastic white cells
233
myelodysplastic staging based on what 3 things
1. peripheral blood cytopenias
2. BM morphology
3. BM blasts
234
5q is MDS is special why
marcrocytic anemia, response to lenoliomide (paradox is that t is poor in AML)
235
hallmark of CML
big spleen
