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Heme/onc > Word association > Flashcards

Word association Flashcards

1
Q

c-Kit

A

on stem cell suface to allow proper maturation

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2
Q

MPL

A

TPO receptor

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3
Q

GPIIb/IIIa autoimmune receptor

A

ITP

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4
Q

G-CSF

A

granulocyte

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5
Q

M-CSF

A

macrophage

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6
Q

mutated neutrophil elastase

A

familial cyclinc neurtopenia

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7
Q

<50% fat

A

normal bone biopsy

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8
Q

low MCHC

A

hereditary spherocytosis

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9
Q

DMT1

A

uptake of iron in gut

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10
Q

ferritin

A

storage of iron

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11
Q

ferroportin

A

transporter of iron

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12
Q

transferrin

A

iron binder in blood

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13
Q

hepcidin

A

suppress release of iron

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14
Q

angular stomatitis

A

iron deficiency

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15
Q

central pallor

A

iron deficiency

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16
Q

upregulation of hepcidin

A

anemia of chronic disease

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17
Q

basophilic stippling in RBCs

A

lead inhibition of heme synthesis

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18
Q

nucleocytoplasmic asynchrony

A

pernicious anemia

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19
Q

hemoglobin H

A

alpha thalassemia

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20
Q

increase antithrombin 3 activity

A

heparin

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21
Q

heparin inhibits

A

thrombin 2, factor 9, 5 and 11

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22
Q

EPO and IL3

A

stimulate red cell growth

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23
Q

TPO and IL6

A

act on megakaryocyte lineage

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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

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25
Q

parvovirus B19

A

can cause aplastic anemia

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26
Q

PIG-A

A

paroxymal nocturnal hemoglobinuria - used for coupling cell surface protein to GP1

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27
Q

CD 55 and CD59

A

lacking in paroxymal nocturnal hemoglobinuria

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28
Q

worms wheezes and weird disease

A

eosinphils - TB, sarcoidosis, addisons and hodgkins

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29
Q
  • high basophils
A

CML

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30
Q

C5a

A

chemoattractant for neutrophils

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31
Q

integrin and selection

A

integrin (vessel) and selection (white cell)

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32
Q

disorder of selctins or integrins

A

LAD

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33
Q

disorder of poor formation of lysozyme granules

A

chediak higashi

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34
Q

hyperimmunoglobulin E

A

Job’s syndrome - mutation of STAT3 leading to less INF-gamma

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35
Q

NBT test

A

for CGD

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36
Q

toxic granules

A

show infections in neutrophils

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37
Q

Dohle bodies

A

in sepsis, seen in neutrophils, ribosome rich ER

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38
Q

Pelger Huet abnormality

A

bilobed nuetrophils - can lead to myelodysplastic syndrome

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39
Q

stress reticulocytosis

A

after bleed, increase in reticulocytes due to storage in bone marrow

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40
Q

elevated MHCH

A

hereditory spherocytosis

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41
Q

microcytic anemia with elevated retic

A

thalessemia

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42
Q

ringed sideroblasts

A

sideroblastic anemia - problem with heme formation

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43
Q

example of microcytic anemia that is hemolytic

A

thalassemia

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44
Q

defective HFE

A

hemochromatosis

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45
Q

blackfan diamond syndrome

A

inherited pure red cell aphasia

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46
Q

folate and iron absorption

A

both in duodenum and folate in proximal ileum

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47
Q

increases in iron absorption

A

vitamin C, pregnancy, EPO and intake of iron

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48
Q

source of B12

A

animals

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49
Q

parietal cell autoantibodies

A

pernicious anemia

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50
Q

elevation of MMA

A

diagnostic test for B12 deficiency

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51
Q

metformin use

A

can lead to B12 deficiency due to impaired gut absorption

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52
Q

hemoglobinuria in hemolytic anemia

A

only in intravascular anemia

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53
Q

haptoglobin

A

heoglobin scavenging protein

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54
Q

march hemoglobinuria

A

extrisic destruction of RBC to due intense activity

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55
Q

mechanical devices can cause

A

DIC - extrisic hemolytic anemia

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56
Q

target cells

A

phospholipid loading in early liver disease

also thalassemias

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57
Q

acanthocytes

A

in advanced liver disease due to cholesterol, trouble leaving the spleen leading to advance hemoglytic anemia

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58
Q

cold agglutination

A

IgM

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59
Q

warm agglutination

A

IgG

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60
Q

IgM/C3b removal of red cells

A

in the liver

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61
Q

IgG removal of red cells

A

in the spleen, slower blood flow

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62
Q

chromic AIHA is a sign of

A

underlying illness

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63
Q

cold agglutination associated with ….

A

CLL and lymphoma, viral infections and HIV, EBV

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64
Q

penicillin as a hapten

A

can lead to drug induced hemolytic anemia

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65
Q

methyldopa and l dopa

A

can produce autoantibodies to red cells

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66
Q

spektrin

A

major cytoskeletal protein in RBC

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67
Q

spektrin or ankrin defeciency

A

hereditary spherocytosis

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68
Q

defective horizontal interactions of membrane proteins

A

hereditary elliptocytosis

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69
Q

thermal instability of rbc membrane

A

Hereditary Pyropoikilocytosis

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70
Q

Heinz bodies

A

G6PD - oxidized hemoglobin

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71
Q

treatments for IgG hemolysis

A

targeted at spleen, steriods, IVIG, chemo and spleenectomy

red cell transfusion

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72
Q

treatments for IgM hemolysis

A

plasmapheresis

red cell transfusion

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73
Q

spleenomegaly in intravascular or extravascular hemolysis

A

in extravascular, red cell destruction is mediated by RES system

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74
Q

mono is associated with…

A

Igm cold aggultination

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75
Q

increased RBC destruction leads to increase in what bilirubin

A

indirect

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76
Q

bite cells

A

caused by Heinz bodies in G6PD

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77
Q

Tissue factor (TF)

A

initiates coagulation cascade

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78
Q

Gp Ib binds to

A

vWf

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79
Q

GP Ia/IIb binds to

A

collagen

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80
Q

GpIIB/IIIa binds to

A

fibrinogen

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81
Q

activator of protein C and S

A

thrombomodulin

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82
Q

prostacyclin

A

inhibits platlet activation in quiescent state

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83
Q

bleeding from the umbilicus

A

factor 13 deficiency

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84
Q

hemorrhagic disease of newborn

A

failure of liver to form resulting in vitamin K deficiency

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85
Q

factors that thrombin activates

A

TF:7a pathway and 5, 8 and 13

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86
Q

contents of dense granules

A

ADP, calcium and serotonin

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87
Q

Bernard Solier syndrome

A

deficiency in GPIb - cant bind to vWf

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88
Q

ristocetin

A

aggregation agonist, requires vWf and intact surface membrane

failure implies von willebrand or bernard solliers disease

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89
Q

PFA-100

A

used to test platelet function

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90
Q

DDAVP

A

used for von willebrand treatment, relseases vWf

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91
Q

gray platelet

A

absent alpha granules

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92
Q

TAR syndrome

A

low platelet, absent radii

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93
Q

may-hegglin anomaly

A

giant platelets, thrombocytopenia, dohle bodies

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94
Q

pentatd of TTP

A

fever, thrombocytopenia,

hemolytic anemia, acute renal failure, and neurologic dysfunction.

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95
Q

infection with e coli 157

A

HUS - hemolytic uremic syndrome

96
Q

ADAMS13

A

in TTP, leads to production of large multimers

97
Q

antibodies against PF4

A

heparin induced thrombocytopenia, usually unfractionated heparin

98
Q

paroxymal thrombosis

A

in heparin induced thrombocytopenia

99
Q

Hb H disease

A

three deletions of hemoglobin A

100
Q

gene deletion (alpha/beta)

A

alpha

101
Q

constant spring

A

non-deletion of alpha gene, premature stop codon

102
Q

point mutations (alpha/beta)

A

beta

103
Q

Hb E

A

beta thalassemia

104
Q

Hb barts

A

gamma tetramers, form in utero in alpha thal

105
Q

deferoxamine

A

used for treatment of iron overload with chelation

106
Q

Howell Jolly bodies

A

Sickle cell - DNA left over after leaving bone marrow

107
Q

positive sickle cell test

A

cell are lyzed and polymerization equals sickle disease

108
Q

4 crises state

A

vasooclusive crisis
hemolytic crisis
aplastic crisis
sequestration crisis

109
Q

moyamoya

A

sickle cell - new blood vessels form around clots and are prone to bleeding

110
Q

sickle cell and NO

A

release of hemoglobin leads to destruction of NO

111
Q

vWf stabilized factor __

A

8

112
Q

alloimmunization occurs in patients with…

A

chronic transfusions (sickle cell)

113
Q

most common source of infection in dontions

A

platelets

114
Q

TRALI

A

acute lung injury from transfusion

115
Q

TACO

A

heart overload state from transfusion

116
Q

bridging

A

while pt on hep,start warfarin, check PT, INR

117
Q

tumor lysis syndrome

A

ALL

118
Q

20% blasts

A

acute leukemia

119
Q

myeloperoxidase positive

A

AML

120
Q

Tdt positive

A

ALL

121
Q

CD 13, 33, 117

A

AML

122
Q

T and B cell blasts

A

ALL

123
Q

cytogenetics of AML

A 
t8:21 AML-ETO1 - favorable
inv (16) - favorable
t15:17 - favorable
del(5 or 7)
11q23
inv(3)
124
Q

12:21

A

good prognosis

125
Q

pulmonary toxicity

A

bleomycin

126
Q

top two NHL

A

diffuse large B cell and follicular

127
Q

prognosis in NHL

A 
A - age
P - performance
L - LDH
E - extranodal
S - stage
128
Q

c-myc, bcl1/2, IgG heavy chain

A

NHL

129
Q

IL3

A

erythroid progenitor

130
Q

IL6

A

lymphoid progenitor

131
Q

bone aspiration is good for

A

morphology, percentage, analyses, flow, cytogenetics

132
Q

bone biopsy is good for

A

fixitave, cellularyity, arcitechture, antibodies to suface antigens

133
Q

myeloid to erythroid ratio

A

3:1

134
Q

PMH

A

mutation in PIG-!, decresed expression of GPI , CD55, CD59, sensitive to pomplement lysis

**thrombosis

135
Q

two causes of aplastic anemia

A

immune mediated suppression or stem cell damage

136
Q

two treatments for aplastic anemia

A
  1. immunsuppression - ATG, cyclosporin, steriods

2. BMT

137
Q

margination

A

neutrophils stick to vessel wall

138
Q

diapedesis

A

WBC moves through vessel

139
Q

pelger huet anomaly

A

WBC variant - bilobed nucleus, begnign hereditary disorder, can lead to myelodysplastic syndrome

140
Q

dohle body

A

WBC variant - cytoplasmic inclusions, ribosome rich seen in sepsis and G-CSF use

141
Q

toxic granulation

A

WBC variant - dark blue granules seen in infection or G-CSF use

142
Q

Chediak-Higashi

A

failure of phagalsome

143
Q

Jobs syndrome

A

Mutations in STAT3, elevated IgE, defect in chemotaxis*

144
Q

NBT test

A

negative in CGD

145
Q

orthostatic hypertension

A

anemia

146
Q

ferroportin

A

gets iron in from the gut

147
Q

transferrin

A

iron is bound to it in the blood

148
Q

hepcidin

A

upregulated when iron is high

149
Q

ferritin

A

iron stroage`

150
Q

hemosiderin

A

inron storage - unavilable

151
Q

1 ml of packed RBC contained how much iron

A

1 mg

152
Q

B12 or folate - neurologic?

A

B12 - posterior column and peripheral nerves

153
Q

Macrocytic vs megaloblastic

A

Macrocytic (large) vs megaloblastic (large with nuclear/cytoplasmic dysnchromy)

154
Q

haptoglobin

A

binds heme

155
Q

intra vs extravascular hemolysis

A

intra - RBC break and relsese of free heme
extra - RES

dark urine vs enlarged spleen

156
Q

abnormal ostmotic fragility

A

HS

157
Q

increased MCHC

A

HS

158
Q

vertical interaction

A

HS

159
Q

horizontal interactions

A

ES

160
Q

polychromasia

A

hemolytic anemia

161
Q

basophilic stippling and spherocytes

A

hallmark of hemolysis

162
Q

IgG vascular?

A

extra, removing IgG coated cells

163
Q

IgM vascular?

A

intra - requires plasmapheresis, steriods and spleenectomy dont work

164
Q

malaria, bartonellosis, babesiosis

A

infections causing hemolytic anemia

165
Q

hemoglobin A2

A

Alpha and delta

166
Q

hydrops fetalis

A

deletion of 4 alpha

167
Q

Hb H disease

A

deletion of 3 alpha

168
Q

hemoglobin deletion

A

alpha

169
Q

hemoglobin mutation

A

beta

170
Q

asian alpha thal

A

Hb constant spring

171
Q

Hb Barts

A

gamma tetramers in utero

172
Q

alpha thal diagnosed by

A

PCR

173
Q

NO-hemoglobin interaction

A

intravascular hemolysis, NO binds to hemoglobin leading to vasoconstriction

174
Q

deoxygenation leads to

A

sickling

175
Q

acute dactylitis

A

sickle cell

176
Q

howell jolly bodies

A

sickle cell - DNA remnants after nucleus extrusion from marrow

177
Q

4 sickle cell crises

A

vasooclusive
aplastic - due to parvo B19
hemolytic
sequestration

178
Q

indication for transfusion in sickle cell

A

ACS and stroke prophylaxis

179
Q

L-glutamine

A

less oxidant stress, decrease pain crisis in sickle cell

180
Q

TF:FVIIa activates

A

FX and FIX

181
Q

FXIII

A

transglutaminse

182
Q

thrombin time

A

fibrinogen to fibrin clot

183
Q

GPIb receptor deficiency

A

Bernard Soulier - prolonged bleeding, big platelets

184
Q

GPIIa/IIIb defect

A

binds to fibinogen - glasmanns thrombasthenia abnormal aggregation

185
Q

vWB type 2B

A

defect is in the platelet, can use mixing study with patients platelets

186
Q

anti thrombotic factors

A

tissue factor pathway inhibitor (TFPI) ATII (9,10,11) Protein C and S (5,8)

187
Q

APC

A

activated protein C resistance - similar to favor V leiden

188
Q

hyperhomocysteinemia

A

inheritied thrombophilic disorder (MTHFR)

189
Q

estrogen and clotting

A

acquired cause of thrombophilia

190
Q

APLA

A

antibodies against GP1 and prothrombin, leads to thrombosis, does not cause bleeding with with PTT long

191
Q

PF4 complex

A

HIT

192
Q

storage conditions

A

RBC - 4-6
platelets - RT
FFP and cryo - -65

193
Q

72 hour rule

A

cross must be sent every 72 hours to check for new antibodies

194
Q

irradiation

A

for less GVHD, immune compromised

195
Q

filtration

A

for leukoreduction

196
Q

leukodepletion

A

CMV safe, reduces risk of allergic reactions

197
Q

presentation of AML/ALL

A
  1. BM failure
  2. Leukostatsis
  3. Coagulopathy
  4. Extra medullary manifesations
  5. Metabolic disturbances
198
Q

metabolic disturabance in AML/ALL

A
  1. tumor lysis
  2. hypokalemia (AML - kdney)
  3. hypoglycemia - artificial
199
Q

auer rods

A

linear aggregates of primary granules seen in myelobalsts in AML

200
Q

diagnosis with myleoperoxidase

A

AML

201
Q

diagnosis with Tdt

A

ALL

202
Q

favorable AML

A

t15:17
t8:21
inv16

203
Q

unfavorable AML

A

11q23

del 5 or 7

204
Q

favorable ALL

A

t12:21

205
Q

unfavorable ALL

A

9:22
MLL on 11
4:11

206
Q

meyloid markers

A

CD 33 CD 117 CD13

207
Q

monocytic subtypes of ALL

A

skin and gingiva

208
Q

PML-RAR

A

APL - type of 15:17 AML

209
Q

CHOP

A

Cyclophosphamide
H - doxorubicin
O - vicristine
P - predisone

210
Q

EBV

A

associated with burkitts lymphoma and hogskins lymphoma

211
Q

cell markers of HL

A

NOT CD20, instead CD15 and CD30

212
Q

types of HL

A
  1. nodular sclerosis
  2. lymphocyte rich
  3. mixed
  4. lymphocyte poor
213
Q

ABVD

A 
for HL
A - adriamycin (cardio)
B - bleomycin (pulmonary)
V - vinblastine (neurologic)
D - dacarbizine (hematologic)
214
Q

sequential involvement of lymph nodes

A

HL

215
Q

isolated CD5 expression

A

in CLL, with no other T cell markers

216
Q

immune complications of CLL

A

hemolytic anemia
RBC aplasia
ITP
neutropenia

217
Q

what factors help with CLL prognosis

A

CD38
Zap70
FISH
IgVh

218
Q

Staging of CLL

A

low: lymphocytosis
intermediate: lymph/hepatosplenomegaly
high: anemia/thrombocytopenia

219
Q

smudge cell

A

CLL

220
Q

CRAB

A 
multiple myeloma
C - calcemia 
R - Renal dysfunction
A - anemia
B - bone lesions
221
Q

environment needed for multiple myeloma

A

adhesion molecules and IL6

222
Q

major and minor criteria in MM

A

Major: increased plasma cells, increased M spike

Minor: 10-30% plasma cells, minor M spike, bone lesions, low antibodies

223
Q

progression of MM

A

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
Q

renal disease in MM

A 
cast nephropathy - light chains injure tubules
amyloidosis - light chain in interstitium
deposition disease (MIDD)- proteinuria and renal insufficiency
225
Q

two components for scoring in MM

A

albumin and beta-2 microglobulin

226
Q

treatments in MM

A

melphalan, steroids, transplant, thalidomide, lenalidomide, proteasome inhibitors

227
Q

Waldenstrom’s Macroglobulinemia

A

type of lymphobpasmactyic lymphoma (type of NHL) - Igm spike, no bone lesions

228
Q

stacked coin appearance

A

MM

229
Q

type 2 cyroglobulinemia associated with…

A

Hep C

230
Q

PMF cell

A

megakaryocytes drive fibroblast production

231
Q

ringed sideroblasts

A

myelodysplascit red cells

232
Q

newly acquired puget huer anolmly

A

myelodysplastic white cells

233
Q

myelodysplastic staging based on what 3 things

A
  1. peripheral blood cytopenias
  2. BM morphology
  3. BM blasts
234
Q

5q is MDS is special why

A

marcrocytic anemia, response to lenoliomide (paradox is that t is poor in AML)

235
Q

hallmark of CML

A

big spleen

Heme/onc (5 decks)

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