Heme and Onc. Flashcards
1
Q
What cell type contains dense granules? What are the two products of dense granules?
A
erythrocytes
ADP and calcium
2
Q
What type of cell contains αgranules? What are the two products of αgranules?
A
erythrocytes
vWF and fibrinogen
3
Q
In what organ is approximately 1/3 of platelet pool stored?
A
spleen
4
Q
What is the vWF receptor?
A
GpIb
5
Q
What is the fibrinogen receptor?
A
GpIIb/GpIIIa
6
Q
What four enzymes are contained by neutrophils?
A
ALP
collagenase
lysozyme
lactoferrin
7
Q
Do macrophages function via MHC II or MHC I?
A
MHC II
8
Q
What type of nucleus do eosinophils possess?
A
bilobed
9
Q
What two enzymes are produced by eosinophils granules?
A
histaminase and arylsulfatase
10
Q
What are the three components of basophil granules?
A
histamine, heparin and leukotrienes
11
Q
What form of leukemia can isolated basophilia be indicative of?
A
CML
12
Q
What granulocyte binds Fc of IgE?
A
mast cell
13
Q
What drug prevents mast cell degranulation?
A
cromolyn sodium
14
Q
What two receptors do dendritic cells express?
A
Fc and MHC II
15
Q
What are dendritic cells in the skin called?
A
Langerhans cells
16
Q
What two CD molecules are expressed by B-lymphocytes?
A
19 and 20
17
Q
What is the CD molecule required for T-cell activation?
A
CD28
18
Q
Multiple myeloma is a cancer of what cell type?
A
plasma cells
19
Q
If a person has type A blood, What antigen do they express on RBCs? What antibody is circulating?
A
A = RBC
Anti-B = circulating
20
Q
If a person has type B blood, What antigen do they express on RBCs? What antibody is circulating?
A
B = RBC
anti-A = circulating
21
Q
What type of immunoglobulin targets RBC antigens?
A
IgM
22
Q
anti-Rh is what type of antibody?
A
IgG
23
Q
If a person has type AB blood, What antigen do they express on RBCs? What antibody is circulating?
A
A and B = RBC
no circulating antibodies
24
Q
What can people with AB blood universally donate?
A
plasma
25
What can people with AB blood universally receive?
blood
26
If a person has type O blood, What antigen do they express on RBCs? What antibody is circulating?
no antigen on RBCs
antigens to A and B in plasma
27
What can people with O blood universally donate?
blood
28
What can people with type O blood universally accept?
plasma
29
What three coagulation factors can thrombin activate?
V and VIII and thirteen
30
What coagulation factors activate factor X?
VIIa and VIIIa
31
Which three coagulation factors does Xa require? What is activated?
IIa and Va and VIIIa
32
What coagulation factor catalyzes the conversion of fibrinogen into fibrin?
IIa
33
Other than fibrinogen, what coagulation factor can thrombin activate?
XIII
34
What is the function of factor XIII?
cross-link fibrin
35
What cofactor requires only calcium?
XIII
36
What coagulation factors require calcium and phospholipid?
II, VII, IX, X
37
What two coagulation factors require thrombin for their activation?
five and eight
38
What is the function of plasmin?
to cleave fibrin
39
What complement can plasmin activate?
C3 to C3a
40
What clotting factor activates kallikrein?
XIIa
41
What two reactions does kallikrein catalyze?
HMWK to bradykinin
plasminogen to plasmin
42
What are the three functions of bradykinin?
increase permeability
pain
vasodilation
43
What three things can convert XII into XIIa?
collagen, basement membrane and activated platelets
44
What is the function of factor nine? What coagulation factors does factor nine need for its activation?
activate ten
seven and eight
45
Hemophilia A is a deficiency in what coagulation factor?
eight
46
Hemophilia B is a deficiency in what coagulation factor?
nine
47
What enzyme does warfarin inhibit?
Vitamin K epoxide reductase
48
What coagulation factor is carried by vWF?
VIII
49
Antithrombin inactivates which coagulation factors? What are the two primary targets of antithrombin?
two, seven, nine, ten, eleven, twelve
thrombin and Xa
50
Factor V Leidin disease produces a mutation in what clotting factor? What can't this factor now be deactivated by?
Va
Activated protein C
51
Activated Protein C (APC) destroys which two coagulation factors?
Va and VIIIa
52
What are the two locations where vWF can be found?
endothelial cells and platelets
53
What is the receptor for vWF?
GpIb
54
What two products do platelets release in order to initiate the coagulation cascade?
ADP and Ca2+
55
What is the fibrinogen receptor?
Gp IIb/IIIa
56
What causes expression of GpIIb/IIIa? What is GpIIb/IIIa a receptor for?
ADP
fibrinogen
57
What thromboxane increases platelet aggregation? Where is this thromboxane released from?
TXA2
platelets
58
Decreasing what molecule does aspirin produce anticoagulation effects?
TXA2
59
What is the MOA of clopidogrel? What is another drug that produces this effect?
clopidogrel inhibits ADP induced expression of GpIIb/IIIa
Ticlopidine
60
What drugs inhibits GpIIb/IIIa directly?
Abciximab
eptifabitide
tirofiban
61
What is the MOA of Ristocetin?
induces vWF to bind GpIb
62
What receptor do clopidogrel and ticlopidine block?
ADP receptor
63
What protein is defective in Glanzmann Thrombasthenia?
GpIIb/IIIa
64
What is deficient in Bernard-Soulier?
GpIb
65
What are the two causes of acanthocytes?
abetalipoproteinemia
liver disease
66
What four conditions can produce basophilic stippling of RBCs?
anemia of chronic disease
alcohol abuse
Lead
Thalassemias
67
What is the acronym of basophilic stipling?
BASically, ACiD Alcohol is LeThal
68
What deficiency can lead to bite cells? What is removed?
G6PDase
oxidized Hb
69
What is the role of ferroportin?
to transfer iron out of a cell
70
What is the function of hepcidin?
to block ferroportin
71
Where are the two locations of ferroportin?
enterocytes of GI tract
macrophages
72
What disease produces a macroovalocyte?
megaloblastic anemia
73
What is a ringed sideroblast?
nucleated RBC with iron in mitochondria
74
Name for diseases where schistocytes are present?
DIC, TTP, HUS and traumatic hemolysis
75
What causes tear drop cells?
bone barrow infiltrate
76
What type of RBC features an increased cell membrane surface area?
target cells
77
What is the acronym do remember the causes of target cells?
HALT
78
What does the H in HALT stand for?
HbC
79
What does the A in HALT stand for?
Asplenia
80
What does the L in HALT stand for?
Liver disease
81
What does the T in HALT stand for?
Thalassemia
82
What type of prosthetic group is oxidized in Heinz bodies?
sulfhydryl
83
What type of stain is used to visualize heinz bodies?
crystal violet
84
What type of thalassemia produces Heinz bodies?
alpha-thalassemia
85
How do Howell-Jolly bodies stain?
basophilic
86
What are Howell-Jolly bodies composed of? What cell type?
nuclear remnants
RBCs
87
In what two circumstances do Howell-Jolly bodies arise?
asplenia or hyposplenia
88
What can cause an iron deficient anemia?
chronic bleeding
89
What is the triad of Plummer-Vinson Syndrome?
1. esophageal webs
2. Iron deficient anemia
3. atrophic glossitis
90
Most microcytic RBCs also feature what?
hypochromia
91
In what population is the cis-deletion prevalent in alpha-Thalassemia?
Asians
92
In what population is the trans-deletion prevalent in alpha-Thalassemia?
Africans
93
What does four allele deletion alpha-thalassemia result in? What chain is present?
Hb barts
gamma chain
94
What does three allele alpha-thalassemia result in? What type of globin composes this?
HbH
β-globin
95
What type of mutation produces alpha-Thalassemia?
deletion
96
What type of population is β-thalassemia most commonly found?
Mediterranean
97
What type of mutation produces β-thalassemia? What two locations do these mutations occur?
point
promoter and splice site
98
What chains is HbH composed of?
four beta chains
99
β-thalassemia minor is characterized by what type of Hb? How much?
HbA2
> 3.5%
100
What happens to the β-chain in β-thalassemia minor?
β-chain is underproduced
101
What happens to the β-chain in β-thalassemia major?
β-chain is absent
102
What form of β-thalassemia produces a crew-cut on X-ray?
β-thalassemia major
103
What happens to bones during β-thalassemia major?
extramedullary hematopoiesis
104
A person with extra-medullary hematopoiesis is at risk of what viral infection?
Parvovirus B19
105
What form of hemoglobin predominates in β-thalassemia major?
HbF
106
What two enzymes are inhibited during lead poisoning?
ferrochelatase
ALA Dehydratase
107
What does the L of LEAD stand for?
Lead Lines on gingivae and metaphyses of long bones
108
What two locations do lead lines appear during lead poisoning?
metaphyses of long bones and gingiva
109
What does the E's of LEAD stand for?
Encephalopathy and erythrocyte stipling
110
What does the A's of LEAD stand for?
abdominal colic and sideroblastic Anemia
111
What does the D of LEAD stand for?
wrist and foot Drops
Dimercaprol and EDTA
112
What drug is used to chelate lead in children?
Succimer
113
What is the most commonly defective enzyme in Sideroblastic Anemia?
ALA Synthase
114
Other than iron, deficiency of what metal can cause sideroblastic anemia?
copper
115
What drug can cause sideroblastic anemia?
Isoniazid
116
What is the treatment for sideroblastic anemia? What enzyme will this restore activity to?
B6
ALA Synthase
117
What are the three antimetabolites that can cause megaloblastic anemia?
methotrexate
trimethoprim
phenytoin
118
What type of GI drug can cause a megaloblastic anemia?
PPIs
119
OTCase deficiency will increase what metabolite?
Orotic acid
120
What is the treatment for orotic aciduria?
uridine monophosphate
121
What is the function of haptoglobin?
to bind free Hb
122
Where is Hb-hemoglobin degraded?
Reticuloendothelial system
123
What are the two main laboratory markers of intravascular hemolytic anemia? What happens do their levels during hemolysis?
decreased haptoglobin
increased LDH
124
What is the breakdown of heme that is found in the urine?
urobilinogen
125
What destroys RBCs in paroxysmal nocturnal hemoglobinuria?
complement
126
Is paroxysmal nocturnal hemoglobinuria considered intravascular or extravascular hemolysis?
intravascular
127
What is the most commonly defective protein during PNH?
GPI anchor
128
What two CD molecules are most often mutated in PNH?
CD55/DAF
CD59
129
What is the function of CD55/DAF?
limit the formation of C3 convertase
130
What are the two functions of CD59?
bind MAC and prevent C9 from binding RBC cell membrane
131
Circulating Hb binds with what molecule? What does this lead to? How can this be treated?
NO
ED, esophageal spasm, abdominal pain
Sildenafil
132
Will unconjugated bilirubin increase during intravascular or extravascular hemolysis?
extravascular
133
What cell type is damaged during microangiopathic hemolytic anemia? What damages the RBCs?
endothelial cells
fibrin mesh
134
HUS is characterized by what triad?
hemolytic anemia
acute kidney failure
thrombocytopenia
135
What three bacteria can cause HUS?
E. coli O157:H7
Camplyobacter
Shigella
136
What toxin is implicated in HUS? What cell surface protein does this toxin bind? What is the net effect?
Shiga
Gb3
endothelial cells to become thrombogenic
137
What metalloprotinease is inactivated during HUS? What is the normal function of this protein?
ADAMTS-13
cleave vWF
138
Most cases of TTP arise due to inactivation of what protein? What is the normal function of this protein?
ADAMS-13
to cleave vWF
139
What is an inherited defect in ADAMTS-13 called?
Upshaw-Shulman Syndrome
140
What does myeloid mean?
granulocyte precursor
141
What are the four major granulocytes?
Neutrophil
Eosinophil
Basophil
Monocyte
142
Aplastic anemia is a destruction of what type of stem cells?
Myeloid
143
What four types of drugs can cause aplastic anemia?
benzene, chloramphenicol, antimetabolites, alkylating agents
144
What four virus can cause aplastic anemia?
B19, EBV, HCV and HIV
145
What type of anemia can cause aplastic anemia? Patients with this type of anemia are predisposed to develop what disease?
Fanconi anemia
AML
146
Idiopathic aplastic anemia may follow what infectious disease?
acute hepatitis
147
What are the three major blood cellular deficiencies of aplastic anemia?
pancytopenia (severe anemia)
thrombocytopenia
leukopenia
148
What are the two treatments for aplastic anemia?
immunosuppressive
G-CSF or GM-CSF
149
What four proteins are defective in hereditary spherocytosis?
ankyrin
spectrin
band 3
band 4.2
150
What is missing in RBCs with hereditary spherocytosis?
central pallor
151
What is the organ manifestation of hereditary spherocytosis?
splenectomy
152
What is used as a screening test in Hereditary Spherocytosis?
eosin-5-maleimide
153
What is the treatment of hereditary spherocytosis?
splenectomy
154
What product is decreased in G6PDase deficiency?
glutathione
155
How will a pyruvate kinase deficiency manifest in a new born?
hemolytic anemia
156
What substitution is present in a patient with HbC? What position?
glutamic acid to lysine in beta-globin
position six
157
What two complement proteins does DAF recognize and sequester?
C4b and C3b
158
What does the direct Coombs test detect?
IgG bound to RBCs
159
What does the indirect Coombs test detect?
circulating anti-RBC IgG
160
Would PNH have a positive Coombs test?
no
161
What is the function of CD59?
prevent formation of MAC
162
What is the treatment of PNH? What is the MOA of this drug?
eculizumab
terminal complement inhibitor
163
What glutamic acid substitution is prevent in HbS?
glutamic acid to valine
164
What two blood diseases will feature a 'crew-cut' Xray?
Thalassemia
HbS
165
Patients with an autosplenectomy are at an increased risk of what type of infection?
encapsulated bacteria
166
What disease can produce splenic sequestration crisis?
sickle cell anemia
167
What is the pneumonic for encapsulated bacteria?
Some Nasty Killers Have Some Capsule Protection
168
Salmonella osteomyelitis almost exclusively effects patients with what type of disease?
sickle cell anemia
169
What is the specific type of kidney damage seen in patients with sickle cell disease?
renal papillary necrosis
170
What is the drug Tx for sickle cell? Why?
Hydroxyurea
breaks down cells susceptible to sickle cell damage
171
What type of Hb does hydroxyurea increase?
HbF
172
Warm agglutinin detects what type of Ab? Against what antigen? Acute or chronic?
IgG
RBCs
chronic
173
Would autoimmune hemolytic anemia produce a positive or negative coombs?
positive
174
Other than hemolytic anemia, warm agglutinin test can detect the presence of what other two diseases?
SLE and CLL
175
Warm agglutinin test can be present if a patient is on what drug?
α-methyldopa
176
Cold agglutinin detects what antibody?
IgM
177
Is warm agglutinin chronic anemia or acute anemia? Cold agglutinin?
warm = chronic
cold = acute
178
What type of leukemia will produce a positive Cold Agglutinin test?
CLL
179
What type of bacterial infection will produce a cold agglutinin test?
Mycoplasma pneumoniae
180
What type of viral infection will produce a cold agglutinin test?
mononucleosis
181
Does serum iron increase or decrease in iron deficient anemia?
decrease
182
Does TIBC increase or decrease in iron deficient anemia?
increase
183
Does ferritin increase or decrease during iron deficient anemia?
decreases
184
Does % transferrin saturation increase or decrease during iron deficiency?
decrease
185
Does serum iron increase or decrease during ACD?
decrease
186
Does TIBC increase or decrease during ACD?
decrease
187
Does ferritin increase or decrease during ACD?
increase
188
Does TIBC decrease or increase during hematochromatosis?
decrease
189
Does pregnancy increase or decrease TIBC?
increase
190
What blood related protein increases during pregnancy and the use of oral contraceptives?
transferrin
191
What do corticosteroids do to neutrophils?
increase their count
192
What do corticosteroids do to eosinophils?
decrease
193
What do corticosteroids do to lymphocytes?
decrease
194
What B-vitamin is required for heme synthesis?
B6
195
What enzyme of heme synthesis is effected during sideroblastic anemia? How is this treated?
ALA synthase
B6
196
What are the two reactants of ALA Synthase?
glycine and succinyl-CoA
197
What are the two enzymes of heme synthesis that lead can interrupt?
ALA dehydratase
ferrochelatase
198
What enzyme is interrupted during Acute Intermittent Porphyria?
Porphobilinogen Deaminase
199
What enyzme of heme synthesis is interrupted during Porphyria Cutanea Tarda?
Uroporphyrinogen Decarboxylase
200
What two metabolites accumulate during lead poisoning?
aminolevulinic acid
Protoporphyrin
201
Does lead poisoning cause microcytic or macrocytic anemia?
microcytic
202
What are the two inhibitors of ALA synthase?
glucose and heme
203
What are the five P's of AIP?
```
painful abdomen
port wine urine
Polyneuropathy
Psychological disturbances
Precipitated by alcohol/drugs/starvation
```
204
What metabolite accumulates during Porphyria Cutanea Tarda?
Uroporphyrinogen
205
What factors are measured by the prothrombin time?
one, two, five, seven and ten
206
What factors are measured by PTT?
all except seven and thirteen
207
What does GpIb bind? Name a disease defective in GpIb?
GpIb binds vWF
Bernard-Soulier disease
208
Immune thrombocytopenia features antibodies against what?
GpIIb/GpIIIa
209
What type of illness may trigger immune thrombocytopenia?
viral
210
What enzyme will be present in the serum during TTP?
LDH
211
vWF carries/protects what factor?
eight
212
What is the treatment of von Willebrand Disease? Why?
DDAVP
desmopressin increases vWF release from endothelial cells
213
What is the mnenomic for remember the causes of DIC?
STOP Making New Thrombi
214
What does the S of STOP Making New Thrombi stand for?
gram-negative sepsis
215
What does the T of STOP Making New Thrombi stand for?
trauma
216
What does the O of STOP Making New Thrombi stand for?
obstetric complications
217
What does the P of STOP Making New Thrombi stand for?
acute Pancreatitis
218
What does the M of STOP Making New Thrombi stand for?
Malignancy
219
What does the N of STOP Making New Thrombi stand for? Why does this happen?
nephrotic syndrome
lose antithrombin in urine
220
What does the 2nd T of STOP Making New Thrombi stand for?
transfusion
221
There is a widespread release of what clotting factor in DIC?
Tissue Factor
222
What two coagulation factors are most consumed in DIC?
five and eight
223
What part of coagulation does antithrombin deficiency effect?
diminishes the increase in PTT following heparin administration
224
What can cause acquired antithrombin deficiency?
nephrotic syndrome
225
Warfarin induced necrosis can be though of as a deficiency in what protein?
Protein C
226
What four clotting factors are contained in cryoprecipitate?
VIII, XIII, vWF and fibronectin
227
Is there an increase in leukocyte ALP durig active infection or CML?
active infection
228
Does Hodgkin lymphoma affect a single node or mutiple nodes?
single
229
Is Hodgkin or non-Hodgkin lymphoma characterized by Reed Sternberg cells?
Hodgkin
230
What type of cell are Reed-Sternberg cells derived from?
B-cells
231
What are Reed-Sternberg cells considered?
crippled germinal centers that have not undergone hypermutation to express their Ab
232
What are the two CD markers of Hodgkin Lymphoma?
CD15 and CD30
233
Which form of Lymphoma has non-contiguous spread?
non-Hodgkin
234
Which form of Lymphoma has a bimodal age distribution?
Hodgkin
235
What age does non-Hodgkin lymphoma usually strike?
20-40
236
What virus is often hypothesized to cause Hodgkin lymphoma?
EBV
237
Which has a better prognosis, Hodgkins Lymphoma with or without lymphocyte rich infiltrate?
rich in lymphocyte = better prognosis
238
Regarding cancer, what oncogenic protein is located on chromosome 8?
c-Myc
239
What translocation is present in Burkitt's Lymphoma?
t(8;14)
240
Regarding cancer, what oncogenic protein is located on chromosome 14?
Ig heavy chain
241
What is the histological key word for Burkitt's Lymphoma?
'Starry Sky'
242
What virus is associated with Burkitt's Lymphoma?
EBV
243
How does Burkitt's arise in African? Sporadic form?
African = Jaw
Sporadic = Abdomen
244
What translocation takes place in diffuse large B-cell lymphoma?
t(14;18)
245
Where is the mantle zone located?
corona of germinal center
246
What translocation is present in a Mantle Cell Lymphoma?
t(11;14)
247
Regarding oncogenesis, what protein is located on chromosome 11?
Cyclin D1
248
What CD molecule is present on a mantle cell lymphoma?
CD5
249
What translocation is present during Follicular Cell lymphoma?
t(14;18)
250
Regarding oncogenesis, what protein is present on chromosome 18?
bcl-2
251
What type of non-Hodgkin lymphoma presents with waxing and waning lymphadenopathy?
follicular lymphoma
252
What is HTLV1 associated with?
IV drug use
253
What three populations are predisposed to HTLV1?
Japan, West Africa, Caribbean
254
What are three common presentations for HTLV1?
cutaneous lesions, bone lesions and hypercalcemia
255
What is mycosis fungoides?
cutaneous T-cell lymphoma
256
What type of T-cell is present in Mycosis Fungoides?
CD4+
257
What are the two locations where Mycosis Fungoides can spread?
lymph nodes and viscera
258
Are mutltiple myeloma monoclonal or polyclonal?
monoclonal
259
What two immunoglobulins are most often produced by Multiple Myeloma? Which more?
IgG and IgA
IgG
260
What type of spike takes place during Multiple Myeloma?
M spike
261
What are Bence Jones proteins composed of? Where are Bence Jones proteins found? What disease do Bence Jones proteins suggest?
Ig light chain
urine
multiple Myeloma
262
Clock face chromatin is indicative of what disease?
Multiple Myeloma
263
Which immunoglobulin is overproduced during Waldenstrom Macroglobulinemia?
IgM
264
How is Waldenstrom Macroglobulinemia differentiated from Multiple Myeloma?
WM does NOT have lytic bone lesions
265
What disease has Rouleaux formation? What are Rouleaux formations?
Multiple Myeloma
stacked RBCs
266
What disease is an asymptomatic precursor to Multiple Myeloma?
Monoclonal Gammopathy of Undetermined Significance
| MGUS
267
What is a Pelger Huet anamoly? Caued by?
bilobed neutrophil
chemotherapy
268
Under what age is ALL most likely to arise?
15 years
269
Where can T-cell ALL create a mass?
thymus/mediastinum
270
Down Syndrome patients are at an increased risk of developing what two types of leukemia?
ALL and AML
271
What is the immunohistochemical marker for pre-T and pre-B cells? Function of this?
TdT+
VDJ rearrangement of TCR
272
What is the immunohistochemical for pre-B cells only?
CD10
273
What translocation has the best prognosis in ALL?
t(12:21)
274
What two immunohistochemical does CLL possess? Is CLL an expansion of B-cells or T-cells?
CD20 and CD5
B-cells
275
Hairy cell leukemia features what type of lymphocytes? Mature or immature?
Mature B-cell
276
What is the average age of onset of AML?
65 years
277
What type of leukemia stains with a TRAP stain?
hairy cell leukemia
278
What leukemia causes marrow fibrosis? What is the stain for this leukemia?
hairy cell
TRAP
279
What two drugs are used to treat Hairy Cell Leukemia? What is the MOA of these drugs?
Cladribine
adenosine analgoue that inhibits adenosine deaminase
280
What type of cells are Auer rods present? What disease features Auer rods?
leukemic blasts
AML
281
Patients with previous exposure to alkylating agents are at an increased risk of developing what type of leukemia?
AML
282
What type of leukemia responds to trans-retinoic acid? Why?
M3 AML
causes blasts to mature
283
DIC is a common presentation of what specific type of leukemia?
M3 AML
284
Which type of AML is most likely to cause DIC? Why?
M3
release of Auer rods due to chemo. agents
285
The Philadelphia Chromosome is possessed in what type of Leukemia?
CML
286
What type of translocation takes place in M3 AML?
t(15:17)
287
What three types of lymphocytes increase in number during CML?
neutrophils, metamyelocytes and basophils
288
What two diseases can CML transform into?
ALL or AML
289
Will there be a high or low level of leukocyte alkaline phosphatase during CML?
low
290
What is the tx of CML?
imatinib
291
Birbeck granules are indicative of what disease?
Langerhans Cell Histiocytosis
292
What precursor are Langerhans cells derived from?
monocytes
293
How does LCH present in child?
lytic bone lesions
294
What disease can be recurrent in LCH?
otitis media
295
S-100 is a marker for what cancer?
melanoma
296
S-100 is an IHC marker for what embryological origin?
mesoderm
297
What two IHC markers are expressed by LCH?
S-100 and CD1a
298
What cell signaling molecule is involved in myeloproliferative disorders?
JAK2
299
What does hematocrit have to be greater than to be considered polycythemia vera?
55%
300
What gene is mutated in polycythemia vera?
JAK2
301
How does polycythemia vera often present? Why?
intense itching after a hot shower
release of histamine
302
What hematological issue can present with gouty arthritis?
polycythemia vera
303
Why can essential thrombocytosis produce bleeding?
platelets sequester too much vWF
304
How do RBCs look during myelofibrosis?
tear drop
305
What drug inhibits heparin?
protamine sulfate
306
What is heparin bound to during HIT? What class of Ab?
PF4
IgG
307
What is the one draw back to LMW heparin?
not easily reversible
308
What two drugs are used in patients with HIT? What is their MOA?
Argatroban and Bivalirudin
inhibit thrombin directly
309
Which anticoagulant can cross the placenta?
warfarin
310
What drug is given to reverse warfarin OD?
vitamin K
311
What are the two direct inhibitors of Factor X?
apixiban and rivaroxaban
312
What are the three thrombolytics? What are their MOAs?
Alteplase, Reteplase and Tenecteplase
conversion of plasminogen into plasmin
313
What two proteins does plasmin cleave?
thrombin and fibrin
314
Name two common ADP receptor inhibitors
clopidogrel ticlopidine
315
Which ADP receptor antagonist can cause neutropenia?
Ticlopidine
316
What are the two PDE3 inhibitors?
Cilostazol and Dipyridamole
317
What antibody is a direct inihibitor of GpIIb/GpIIIa?
abciximab
318
What enzyme is inhibited by methotrexate?
DHFR
319
5-FU covalently complexes what molecule? What enzyme is inhibited?
folic acid
thymidylate synthase
320
What is given during a 5-FU overdose?
uracil
321
What enzyme does cytarabine inhibit?
DNA polymerase
322
What is the most notable side effect of cytarabine?
pancytopenia
323
Azathioprine/6-MP are analogues of what?
purines
324
What enzyme activates 6-MP into azathioprine?
HGPRTase
325
What drug can increase the toxicity of 6-MP and azathioprine?
allopurinol
326
What is the MOA of actinomycin (dactinomycin)?
intercalates DNA
327
What is the MOA of doxorubicin? What does this stop the progression of?
intercalates DNA
DNA topoisomerase
328
What type of toxicity does doxorubicin cause?
cardiac
329
What is the MOA of bleomycin?
free radical generation leadign to dsDNA breaks
330
What is the MOA of cyclophosphamide?
alkylating agent at guanine N-7
331
What is the toxicity of bleomycin?
pulmonary fibrosis
332
p450 is require to activate what type of anti-neoplastics?
Alkylating agents
333
What is the by-product of cyclophosphamide? What does this byproduct cause? What drug can overcome this?
acrolein
hemorrhagic cystitis
mesna
334
What is the main toxicity of cyclophosphamide?
myelosuppression
335
What is the MOA of nitrosureas?
cross-links DNA
336
What type of cancers are nitrosureas used for?
brain cancers
337
What are the main toxicities of nitrosureas?
CNS toxicity
338
What is the MOA of busulfan? What is it used for?
cross-links DNA
ablate bone marrow
339
What is the MOA of vincristine and vinblastine?
bind β-tubulin
340
What is the toxicity of vincristine?
peripheral neuropathy
341
What does vinblastine blast?
bone marrow
342
What is the MOA of paclitaxel?
prevent breakdown of mitotic spindle
343
What are the three main toxicitues of taxols?
myelosuppression
alopecia
hypersensitivity
344
What is the MOA for cisplatin/carboplatin?
cross-link DNA
testicular
345
What are the two toxicities caused by cisplatin?
nephrotoxicity and CN VIII damage
346
What drug is used to treat the nephrotoxicity associated with cisplatin?
amifostine
347
What is the MOA of etoposide/tenoposide?
inhibit topo. II
348
What drug can increase HbF?
sickle cell anemia
349
What is the MOA of irinotecan and topotecan?
inhibition of topo. I
350
What enzyme does hydroxyurea inhibit?
ribonucleotide reductase
351
Which SERM can be a partial agonist in the endometrium?
tamoxifen
352
What drug is used to target HER2+ tumors? What is the main toxicity of this drug?
trastuzumab
heart
353
What is another name for imatinib? What two neoplasias is imatinib used to treat?
Gleevec
Philadelphia chromosome CML and GI stromal
354
Rituximab is used to treat which three conditions?
all Bcell lymphomas
rheumatoid
ITP
355
Vemurafenib is used to treat what disease? Which specific marker?
malignant melanoma
B-raf kinase with V600E mutation
356
Bevacizumab is used to treat which two cancers?
colorectal
renal cell carcinoma
357
What is the toxicity of Methotrexate?
Myelosuppression
358
What four proteins are contained within neutrophils?
ALP, collagense, lactoferrin and lysozyme
359
What oxidative enzyme is contained within neutrophils?
myeloperoxidase
360
What two molecules are expressed by Thelper cells?
CD3 and CD4
361
What two molecules are expressed by cytotoxic Tcells cells?
CD3 and CD8
362
What are the two primary targets of antiheparin?
II and X
363
What molecule accumulates in hereditary sideroblastosis? What organelle?
Iron
mitochondria
364
What prosthetic group of Hb is oxidized in bite cells?
sulfhydryl
365
Where is a common location on the body for pallor to develop during iron deficient anemia?
conjuctiva
366
Lead poisoning inhibits the degradation of what sort of nucleic acid? What does this lead to?
rRNA
basophilic stipling
367
B6 is a cofactor for what enzyme of heme synthesis?
ALA synthase
368
Fanconi anemia features a decrease in what type of repair mechanism?
DNA
369
What are the two findings of G6PD deficiency?
back pain
hemoglobinuria
370
Non-Hodgkin lymphoma may be associated with what virus?
HIV
371
Which form of lymphoma presents with low-grade fever and night sweats?
Hodgkins
372
What three clotting proteins decrease during DIC?
fibrinogen, V and VIII
373
Auer rods contain what enzyme?
peroxidase
374
Does PCV have low or high EPO levels?
low
375
What clotting factor does LMW heparin bind to more?
ten
376
What is the MOA of argatroban and bivalirudin?
inhibit thrombin directly
377
How is warfarin metabolized?
CYP 2C9
378
Which anticoagulant is lipid soluble, warfarin or heparin?
warfarin
379
Which anticoagulant is given orally, warfarin or heparin?
warfarin
380
Which anticoagulant acts rapidly, heparin or warfarin?
warfarin
381
Which anticoagulant has a duration of hours, , warfarin or heparin?
heparin
382
What drug is used to treat an overdose of alteplase/reteplase/tenecteplase?
aminocaproic acid
383
What does 5-fluorouracil complex with? The production of what compound is inhibited? What enzyme is inhibited?
folic acid
dTMP
thymidylate synthase
384
Cytarabine is an analogue of purines or pyrimidines?
pyrimidnes
385
What is the side effect of cytarabine?
pancytopenia
386
What enzyme metabolizes azathioprine?
xanthine oxidase
387
What drug is used for childhood tumors?
dactinomycin
388
Which antineoplastic requires bioactivation by the liver?
cyclophosphamide
389
Drugs that inhibit microtubules prevent what phase of the cell cycle?
M phase
390
What is the most commonly used glucocorticoid for antineoplastic medicine?
Prednisone
391
In what tissue are SERMs an agonist?
bone
392
What two diseases can imatinib be used to treat?
CML and GI stromal tumors
393
What is the cofactor for myeloperoxidase?
heme
394
What is the function of CD40?
co-stimulatory molecule of APCs
395
Where are langerhans cells located?
areas of body in contact with external environment
396
What is the funtion of CD3?
to bind the T-cell recpetor
397
What is the function of Tregs?
help immune system differentiate self from non-self
398
What syndrome can develop if Tregs do not function properly?
IPEX syndrome
399
What txn factor is defective during IPEX syndrome?
FOXP3
400
What two compounds bind to protein C? What binds to activated protein C?
thrombin and thrombomodulin
protein S
401
What clotting factor binds exposed collagen?
vWF
402
What two hematological complications arise from Copper Deficiency?
anemia and neutropenia
403
How would a patient with subacute combined deficiency present with regards to their motor movement?
spastic movements
404
Is Hb released into the serum during extravascular hemolysis?
no
405
What type of infections are patients with a splenectomy predisposed to?
encapsulated organisms
406
What five conditions can produce microangiopathic hemolytic anemia?
```
DIC
TTP
HUS
SLE
malignant hypertension
```
407
What two parasite diseases can cause hemolytic anemia?
malaria and Babesia
408
Do corticosteroids cause neutropenia or neutrophilia?
neutrophilia
impair neutrophil migration out of vasculature
409
Do corticosteroids cause eosinopenia or eosinophilia?
eosinopenia
410
Do corticosteroids cause lymphopenia or lymphophilia?
lymphopenia
411
What drug is used to diagnose vWF Disease?
ristocetin
412
What is the most common cause of inheritable hypercoagulability in humans?
Factor V leiden deficiency
413
Warfarin induced necosis is exacerbated by what disease?
Protein C or S deficiency
414
What neoplasm has a 'fried egg' appearance on histlogy?
Multiple Myeloma
415
Myelodysplastic Syndromes have an increased risk of transforming into what disease?
AML
416
What are histiocytes?
activated macrophages or dendritic cells
417
What is Langerhans Cell Histiocytosis also known as?
Hand-Schuller-Christian Disease
418
What is the triad of LCH?
insipidus/exopthalmos/lytic bone lesions
419
What two types of cells compose histiocytes?
activated dendrites and macrophages
420
What three classes of cells are contained within pancytopenia?
erythroblasts
megakaryoblasts
granulocytes
421
What are the three toxicities of aspirin?
gastric ulceration
tinnitus (CN VIII)
interstitial nephritis
422
What is pulmonary side effect of aspirin overdose?
respiratory alkalosis
423
What is the main side effect of clopidogrel or ticlopidine?
acute coronary syndrome
424
What does increased cAMP in platelets do? What two drugs can cause an increase in cAMP in platelets?
inhibits aggregation
Cilostazol and Dipyridamole
425
What is the only drug that leucovorin can be used in combination with to prevent myelosuppression?
methotrexate
426
What is an overdose of 5-FU treated with?
uridine
427
What enzyme does 6-MP/azathioprine inactivate? What rxn is catalyzed by this rxn?
amidophosphoribosyltransferase
PRPP to PRA
428
What is the toxicity of rituximab?
multifocal leukoencephalopathy
429
Aminocaproic acid is an analogue of what amino acid?
lysine
430
What are the three causes of megaloblastic anemia?
B12, Folate and orotic aciduria
431
What type of drug can cause a B12 deficiency?
PPI
432
A patient with PNH has an increased liklihood of developing what disease?
acute leukemias
433
What three situations precipitate Hb Sickling?
hypoxemia, acidosis, dehydration
434
What are the two inhibitors of ALA Synthase?
glucose and heme
435
What cell would increase in number during Immune Thrombocytopenia in the bone marrow?
Megakaryocytes
436
What receptor is defective in Pelger-Huet Anamoly?
Lamin B receptor
437
What type of cell is present in CLL?
Smudge cell
438
Which anticoagulant is known to cause osteoporosis over long time use?
Heparin
439
What are the four ADP receptor inhibitors?
Clopidogrel, ticlopidine, prasugrel, ticagrelor
440
What drug is used in combination with doxorubicin to prevent cardiomyopathy?
Dexrazoxane
441
What is the antigen of Type A blood?
N-acetylgalactosamine
442
What is the antigen of Type B blood?
Galactose
443
Would an infection increase or decrease ESR?
increase
444
Would an auto-immune disease increase or decrease ESR?
increase
445
Would a neoplasm increase or decrease ESR?
increase
446
Would plycythemia vera increase or decrease ESR?
decrease
447
Would SSA increase or decrease ESR?
decrease
448
Would CHF increase or decrease ESR?
decrease
449
Would pregnancy have an increase or decreased ESR?
increased
450
Which three drugs can cause a non-megaloblastic macrocytic anemia?
hydroxyurea
5FU
zidovudine
451
What is the mode of inheritance for orotic aciduria?
autosomal recessive
452
What is the mode of inheritance for G6PDase Deficiency?
X-linked recessive
453
What is the mode of inheritance of Pyruvate Kinase Deficiency?
autosomal recessive
454
What is the most common cause of death in adults with SSA?
acute chest syndrome
455
What hematological abnormality can produce hemarthroses?
Hemophilia A and B
456
What type of RBC is created during TTP?
Schistocyte
457
Does TTP produce more renal or neurological symptoms?
renal
458
Does TTP produce a fever?
yes
459
What is the mode of inheritance for vWF disease?
autosomdal dominant
460
What two forms of Hodgkin Lymphoma does EBV produce?
mixed-cellularity
lymphocyte depleted
461
What gene is activated during EBV induced Hodgkins Lymphoma?
NF-KB
462
What cell is neoplastic during HTLV1?
CD4
463
What is the most common type of Hodgkins Lymphoma?
nodular sclerosing
464
Which form of myelogenous leukemia can produce splenomegaly?
CML
465
Other than crewcut/spleen/liver, where can extramedullary hematopoiesis take place?
serosal surfaces
466
What cell is over-produced during myelofibrosis? Which two cytokines released drive this process?
Megakaryocytes
PDGF and TGF
467
What non-kidney malignancy can produce an increase in EPO?
hepatocellular carcioma
468
Does warfarin monitor the extrinsic or intrinsic clotting cascade?
extrinsic
469
Which vitamin can potentiate the effects of warfarin?
vitamin E
470
Which endocrine disorder can potentiate the effects of warfarin?
Hyperthyroidism
471
Which fibrinolytic has the longest half-life?
Tenecteplase
472
What three types of tumors is doxorubicin used to treat?
solid tumors
leukemia
lymphomas
473
What two types of cancer is bleomycin used to treat?
Hodgkins
testicular cancer
474
What residue and position do nitrosureas alkylate?
O6 guanine
475
What cancer is busulfan used to treat?
CML
476
What four cancers are cisplatin/carboplatin used to treat?
Testicular
Ovarian
Breast
Lung
477
Which two cancers is Hydroxyurea used to treat?
melanoma and CML
478
In which two locations is tamoxifen an agonist?
bone and endometrium
479
What cancer is the ABVD regimen used for?
Hodgkins Lymphoma
480
What are the drugs of the ABVD regimen?
adriamycin/bleomycin/vinblastine/dacarbazine
481
What two cell lines express MPO?
neutrophils and monocytes
482
What three proteins can imatinib inhibit?
BCR/ABL
PDGF
c-Kit
483
Which leukemia can express CD52?
CLL
484
What drug targets CD52?
alemtuzumab
485
Which two neoplastic agents inhibit G1 and S?
Etoposide
Tenoposide
486
Which class of drugs are used during HIT?
Direct thrombin inhibitor
487
Do factor X Inhibitors require monitoring?
no
488
Are Langerhans cells during LCH mature or immature? What cant they do?
immature
activate Tcells
489
Which bone can be involved during LCH?
mastoid
490
What is seen in the bone marrow during Essential Thrombocytosis?
enlarged megakaryocytes
491
Does AML cause splenomegly?
no
492
Does Hairy Cell Leukemia have splenomegaly? Which pulp?
yes
white
493
What is a Stage One Lymphoma?
single node
494
What is a Stage Two Lymphoma?
two nodes on same side of diaphragm
495
What is a Stage Three Lymphoma?
both sides of diaphragm
496
What is a Stage Four Lymphoma?
invaded non-lymphoid tissue
497
Which two drugs can be used to turn down the immune response during Aplastic Anemia?
cyclosporine
antithymocyte globulin
498
Can be Lymphocytes function as an APC?
yes
499
What are eosinophils highly phagocytic for?
antigen/antibody complexes
500
What does anistocytosis mean?
different sizes
501
What does poikilocytosis mean?
different shapes
