Hematology and Oncology Flashcards
1
Q
List the 3 most common cancers in women
A
- breast
- lung (most deaths)
- colorectal
2
Q
Cancer immunotherapy often includes administration of monoclonal antibodies against a target overexpressed on the cancer cell surface. The Fc portion of the antibody binds ____ cells via CD___ leading to cytotoxicity
A
natural killer
CD16
3
Q
What clotting factors are produced initially by the liver in an inactive form and then activated by vitamin K dependent carboxylatoin
A
II
VII
IX
X
4
Q
PT will be prolonged in cirrhosis primarily due to what deficiency
A
factor VII (shortest half life, produced by the liver, and part of the extrinsic pathway which effects PT)
5
Q
Nitrates are oxidizing agents that are effective in treating ___ poisoning due to their ability to induce methemoglobinemia which contains ___ instead of _____
A
cyanide
ferric (FE3+)
ferrous (Fe4+)
(cyanide binds ferric iron m ore avidly than to mitochondrial cytochrome enzymes, diminshing cyanides toxic effect)
6
Q
Patients with sickle cell disease become functionally asplenic within the first few years of like which dramatically increases the risk for infections with encapsulated organsims, particularly ____
A
streptococcus pneumoniae
also at increased risk for salmonella osteolmyelits
7
Q
What medications are most commonly known to cause pancytopenia
A
antiseizure: carbamazepine, valproic acid
sulfonamides
nifedipine
8
Q
Thrombotic thrombocytopenic purpura is a thrombotic microangiopathy resulting in ____ and ____ which are both findings that are essential for making the diagnosis
A
microangiopathic hemolytic anemia
thrombocytopenia
9
Q
Thrombotic thrombocytopenia purpura is triggered by a severe deficiency in ______
A
ADAMTS13
10
Q
What kind of receptor is HER2
A
tyrosine kinase
11
Q
Tx for HER2 positive breast cancer
A
trastuzumab
12
Q
What is the result of mycoplasma pneumoniae causing cross-reacting IgM antibodies
A
mild, transient hemolytic anemia
13
Q
CD19 is only expressed by what cells
A
B cells
14
Q
Vincristine has what classic toxicity and it stops the cell cycle at what stage
A
neurotoxicity (peripheral neuropathy) M phase (disrupts neuronal microtubules)
15
Q
HbF contains __ globin instead of ___ globin seen in HbA
A
gamma
beta
16
Q
Diagnostic lab values for DIC
A
prolonged PT/PTT
thrombocytopenia (due to consumption of coagulation factors and platelets)
17
Q
Erythropoietin signal transduction is primarily mediated by _____ which promotes erythrocyte precursor survival
A
JAK2/STAT
18
Q
Porphyria cutanea tarda is causes photosensitivity (blistering) and hyperpigmentation and is caused by a deficiency in what enzyme
A
late steps in porphyrin synthesis: uroporphyrinogen decarboxylase
19
Q
acute intermittent porphyria causes abdominal and neurological symptoms and is caused by a deficiency in what enzyme
A
early steps of porphyrin synthesis: porphobilinogen deaminase
20
Q
Parvovirus is what type of virus? (DNA/RNA, positive sense, negative sense, single strand, double strand, enveloped, nonenveloped)
A
nonenveloped sing-stranded DNA virus
21
Q
In Von Willebrand disease, how platelet levels, PT and PTT be affected
A
platelets and PT normal
PTT prolonged due to low levels of factor VIII
22
Q
What are the main side effects of cyclin dependent kinase inhibitors
A
inhibit cellular replication in rapidly dividing cells (bone marrow, neutropenia, leukopenia, anemia)
23
Q
What can cause peripheral neuromuscular excitability (paresthesia, muscle spasms) following a blood transfusion
A
citrate anticoagulants can chelate plasma calcium –> hypocalcemia
24
Q
What is the underlying pathology of primary/hereditary hemochromatosis
A
mutation in HFE protein that causes enterocytes and hepatocytes to detect falsely low iron levels –> increased DMT1 expression on enterocytes to increased iron intestinal absorption and hepatocytes decrease hepcidin synthesis which increases ferroportin expression allowing increased iron secretion into circulation
25
what are the clinical features of primary hemochromatosis
micronodular cirrhosis
diabetes mellitus
skin pigmentation (bronze diabetes)
increased risk for hepatocellular carcinoma, congestive heart failure, and testicular atrophy
26
enoxaparin MOA
low molecular weight heparin
27
What type of leukemia can cause respiratory symptoms such as a child presenting with dyspnea, tachypnea, and inspiratory stridor as well as dysphagia with solid foods
T-cell acute lymphoblastic leukemia (ALL)
| can cause a mediastinal mass
28
What is the MOA of emicizumab treating hemophilia A
mimics activity of factor VII
bispecific monoclonal antibody that binds both factor IXa and factor X bringing them in close proximity to allow for factor X activiation
29
Sickle cell anemia results from a point mutation that substitutes ____ in the place of ____
valine
| glutamic acid
30
Why does unfractionated heparin have equal activity against factor Xa and thrombin while LMWH has greater activity against factor Xa than thrombin
only unfractionated heparin has pentasaccharide chain long enough to bind to both antithrombin and thrombin which is required to inactivate thrombin
both of them can bind anitithrombin, though, which is all that is needed to increase its ability to inactivate factor Xa
31
Symptoms of colicky abdominal pain, constipation, and peripheral neuropathy in the presence of a microcytic anemia with normal iron studies suggests what diagnosis
lead poisoning
32
what does a peripheral smear in lead poisoning show
microcytic anemia that may reveal basophilic stippling
33
What are causes of target cells (RBCs)
reduced cell volume: thalassemia, iron deficiency
| excessive cell membrane: obstructive liver disease, postsplenectomy
34
How does carbon monoxide poisoning affect O2 binding to hemoglobin
heme binds CO stronger than O2 but it also decreases hemoglobin O2 unloading
35
A 50 yr old man with painless mass in his neck that fluctuates in size and has a t(14;18) chromosomal translocation has overexpression of what gene and type of lymphoma is this
```
BCL2 (18)
follicular lymphoma (a non hodgkin lymphoma)
```
36
Apixaban blocks the conversion of ___ to ____
prothrombin
thrombin
(direct factor Xa inhibitor)
37
In erythrocytosis, a normal red blood cell mass indicates what as the cause of polycythemia
plasma volume contraction (relative erythrocytosis)
38
How does multiple myeloma cause bone pain
secretes RANKL and destroys osteoprotegerin --> increased RANK activity --> osteoclast differentiation and inhibition of osteoblasts --> purely radiolucent/osteolytic bone lesions
39
What chemotherapy agent can cause lung toxicity that usually manifests as pulmonary fibrosis
bleomycin
40
what is the MOA of bleomycin
binds to iron and oxygen molecules to create free radicals that cause DNA strand breaks
41
```
What location of enlarged lymph nodes is unlikely to be caused by common childhood infections and is concerning for malignancy:
anterior cervical
posterior cervical
submandibular
submental
supraclavicular
```
supraclavicular
42
How does the BCL2 protein normally inhibit apoptosis
blocking the release of proapoptotic factors (cytochrome C protein) from the mitochondria
43
What lymphoma presents with a painless waxing and waning (fluctuating) lymphadenopathy
```
follicular lymphoma
(most common indolent (painless) non hodgkin lymphoma in adults)
```
44
Vitamin B12 is an essential cofactor in the recycling of ____ to ____ which is required for amino acid, purine, and thymidine synthesis
5-methyl-tetrahydrofolate
tetrahydrofolate
(B12 = colbalamin)
45
____ is a polypeptide synthesized by the _____ that acts as a regulator of intestinal iron absorption and iron release by macrophages
hepcidin
liver
(hypoxia and high erythropoiesis --> low hepcidin --> increased intestinal absorption and stimulate iron release from macrophages)
46
what complication of multiple myeloma can lead to renal failure, heart failure, and neurologic dysfunction
amyloidosis (light chains form insoluble fibrils)
47
Acute promyelocytic leukemia is associated with what chromosomal translocation
t(15;17)
48
Auer rods = ______
acute myelogenous leukemia (includes acute promyelocytic leukemia)
49
```
Tumor lysis syndrome has what effect on the following:
potassium
uric acid
phosphorus
lactate dehydrogenase
```
potassium: increased (--> hypocalcemia)
uric acid: increased
phosphorous: increase
lactate dehydrogenase: increased
50
What causes the delay in warfarin's therapeutic effect
it only blocks the generation of new clotting factors, therapeutic effect is delayed 3-5 days until preexisting clotting factors are consumed
51
What 3 things can cause a left shift in the hemoglobin oxygen dissociation curve
increased pH
decreased 2,3-bisphosphoglycerate (increased in low O2 and decreases O2 affinity allowing release in peripheral tissues)
decreased
temperature (stabilizes bonds)
52
hemolytic anemia, hypercoagulability, and pancytopenia are characteristic of what disease
paroxysmal nocturnal hemoglobinuria
53
Paroxysmal nocturnal hemoglobinuria is caused by an acquired mutation of the ___ gene which is involved in the synthesis of _______
PIGA
| glycosylphosphatidylinositol (GPI) anchor
54
what is the pathogenesis of paroxysmal nocturnal hemoglobinuria
mutation in gene for GPI anchor synthesis --> CD55 (decay-accelerating factor) and CD59 (MAC inhibitory protein) deficiencies --> complement mediated hemolysis (no inhibition of complement on RBC)
55
flow cytometry is the gold standard for diagnosis of paroxysmal nocturnal hemoglobinuria which will show absence of ___ and ____
CD55
| CD59
56
megaloblastic anemia with neurologic dysfunction is due to what deficiency
B12
57
What lymphoma has presence of macrophages and apoptotic bodies in a see of medium-sized lymphocytes
Burkitt
58
Burkitt lymphoma is associated with what chromosome translocation
t(8;14)
| 8: c-Myc
59
Acute intermittent porphyria can cause neurologic symptoms and abdominal pain due to deficiency of what enzyme and can be treated by decreasing activity of what enzyme
```
porphobilinogen deaminase
aminolevulinate synthase (rate limiting enzyme in hepatic pathway of heme)
```
60
Methotrexate is a _____ antagonist which competitively inhibits the enzyme _____ which catalyzes the synthesis of ______.
folic acid
dihydrofolate (DHF) reductase
tetrahydrofolate
61
Lecovorin/folinic acid has what affect on methotrexate vs 5-FU chemotherapeutic effects
methotrexate: inhibited (bc leucovorin bypasses the DHF reductase step that is inhibited by methotrexate)
5-FU: potentiates toxicity (5-FU requires presence of reduced folate inorder to form complexes with thymidylate synthase)
62
list 3 B cell markers
CD19
CD20
CD23
63
A patient with anemia, thrombocytopenia, and leukocytosis with a predominance of CD-20 positive cells has high suspicion of what diagnosis
chronic lymphocytic leukemia (CLL)
64
The restocetin cofactor assay measures platelet agglutination via binding of ____ receptors to ____. It is abnormal in what deficiency?
glycoprotein 1b
| vWF deficiency
65
The primary immune response to EBV is through what cells which appear in the peripheral blood smear as what
CD8 T lymphocytes
| reactive (atypical) CD8 T cells
66
jaundice, dark urine, normocytic anemia, reticulocytosis, and bite cells are indicative of what diagnosis
glucose 6 phosphate dehydrogenase deficiency
67
Rasburicase MOA
recombinant version of urate oxidase
converts uric acid into allantoin that is more soluble
used in tumor lysis syndrome
68
Erythrocytes always convert glucose to pyruvate but have what alternate pathway of doing so that does not generate ATP
bisphosphoglycerate mutase converts 1,3BPG into 2,3BPG
| 2,3BPG is converted to 3 phosphoglycerate by bisphosphoglycerate phosphatase and neither of these steps yield ATP
69
vWF functions as a promotor of platelet adhesion at sites of vascular injury by binding ____ to ____
```
platelet glycoproteins (primarily Gp1b)
subendothelial collagen on injured blood vessel walls
```
70
Hemoglobin C is caused by a ___ mutation that results in a ___ residue being substituted by ____ in the beta globin chain
missense
glutamate
lysine
71
Rank the speed of hemoglobin movement during gel electrophoresis:
hemoglobin A
hemoglobin S
hemoglobin C
hemoglobin A> HbS>HbC
72
Deferoxamine MOA in blood transfusion
chelation therapy
| prevents iron-induced cardiotoxicity and congestive heart failure from developing
73
chromosome translocation t(15;17) causes what leukemia and forms a fusion of what genes
acute promyelocytic leukemia (M3 variant of AML)
15 = PML gene
17 = RARA gene (retinoic acid receptor alpha)
74
____ is a monoclonal antibody directed against the CD20 antigen
rituximab
75
If a patient has elevated WBC with an increase in myelocytes and decreased leukocyte alkaline phosphatase, what is the diagnosis
CML (chronic myelogenous leukemia)
76
Isoniazid inhibits pyridoxine phosphokinase leading to impaired activation of pyridoxine (B6) which is a cofactor for ____. Inhibition of this could cause _____ anemia
delta aminolevulinic acid synthase (catalyzes rate limiting step of heme synthesis)
sideroblastic (anemia caused by impaired heme production)
77
explain how HUS causes thrombocytopenia and hemolytic anemia
shiga toxin causes capillary endothelial damage --> platelet activation --> microthrombi --> erythrocyte damage
78
How does G6PD effect the following: reticulocytes, lactate dehydrogenase, haptoglobin
reticulocytes: increased
lactate dehydrogenase: increased
haptoglobin: decreased (binds free circulating hemoglobin)
(all due to RBC lysis)
79
Multiple myeloma is a malignancy of what cell type
plasma cells
80
What is the diagnosis of a patient presenting with thrombocytopenia, microangiopathic hemolytic anemia, renal insufficiency, neurologic symptoms and fever
TTP (thrombotic thrombocytopenic purpura)
81
Describe the pathogenesis of thrombotic thrombocytopenic purpura (TTP)
formation of autoantibody inhibitors against ADAMTS13 which usually cleaves ultralarge vWF --> aggregation and activation of platelets
82
Which is more suggestive of malignancy: polyclonal proliferation of lymphocytes or monoclonal
monoclonal
83
How does demsmopressin help treat patients with symptomatic vWF disease
transiently increases vWF and factor VIII release from endothelial cells
84
Burkitt lymphoma is associated with what chromosome translocation
t(8;14)
| Myc overexpression which functions as a transcription activator --> cell growth and proliferation
85
What causes a increase in mean corpuscular hemoglobin concentration in spherocytosis
mild dehydration of the red blood cells
86
Precursors to what cells stain positive for Tdt, CD10, CD19
Bcell
87
What is the pathogenesis of disseminated intravascular coagulation (DIC)
widespread formation of microvascular thrombi leads to consumption of platelets, coagulation factors, and fibrinogen. Subsequent activation of anticoagulant proteins leads to low protein C/S. Most patients have bleeding complications and end organ damage to lungs or kidneys
88
How does myoglobin oxygen binding differ from hemoglobin
myoglobin reaches saturation at a much lower partial pressure of oxygen than hemoglobin (1 mm Hg vs 26) and it does not experience heme-heme interactions bc it only has a single heme group so does not have sigmoid shape curve
89
A patient on LMWH that develops thrombocytopenia and deep venous thrombosis 5-10 days after starting LMWH after a right hip arthroplasty is suspicious of what diagnosis
HIT type 2
| heparin induced thrombocytopenia
90
what causes thrombocytopenia in HIT type 2
destruction of antibody coated platelets by splenic macrophages
91
what platelet receptor normally promotes platelet binding binding to fibrinogen
glycoprotein iib/iiia
92
The factor V leiden mutation, causes factor Va resistance to what? which causes atypical venous thrombosis
resistant to inactivation by activated protein C
93
Multiple myeloma is sometimes treated with lenalidomide, what is its MOA
increases the binding affinity of E3 ubiquitin ligase complex to specific transcription factors that are overexpressed in myeloma cells --> destruction by proteasome
94
```
How are the following affected in anemia secondary to menstrual blood loss:
serum ferritin
circulating transferrin
MCV
hypersegmented neutrophils
serum folate
```
```
serum ferritin: low
circulating transferrin: high
MCV: low
hypersegmented neutrophils: none
serum folate: normal
```
95
What type of leukemia is a B cell neoplasm that infiltrates bone marrow causing fibrosis and bone marrow failure and infiltrates the splenic red pulp causing massive splenomegaly
hairy cell leukemia
| lymphocytes have cytoplasmic projections
96
What is the best test to monitor the anticoagulation effect of warfarin? Heparin?
warfarin: PT (or INR)
heparin: PTT
97
In DIC due to gram-negative sepsis, the coagulation cascade is activated by ____ leading to widespread deposition and the consumption of coagulation factors and platelets with eventual bleeding
bacterial endotoxins
98
What is seen on peripheral blood smear of DIC
schistocytes
99
What effect does anemia of chronic disease have on total iron binding capacity
its either normal or low
100
PCR requires what 4 things
primers that are complementary to the regions of DNA flanking the segment of interest
thermostable DNA polymerase
deoxynucleotide triphosphates
source DNA template strand
101
What is the most common cause of cancer death in both women and men
lung cancer
102
How does intravenous glucose help treat acute intermittent porphyria which may present with abdominal pain, peripheral neuropathy, neuropsych symptoms, and reddish brown urine
downregulates ALA synthase activity
103
how does methemoglobinemia affect oxygen binding
methemoglobin is unable to carry oxygen
104
how is PO2 of arterial blood altered in CO poisoning, anemia, and polycythemia
it is unchanged in all of these conditions bc PO2 is a measure of dissolved O2 in blood and does not account for O2 bound to hemogobin
105
A patient with factor VII deficiency likely has normal or abnormal of the following: PT, PTT, bleeding time
PT: prolonged
| PTT and bleeding time: normal
106
What effects does unfractionated heparin have on thrombin time
PTT
activity of factor Xa
thrombin time: prolonged
PTT: prolonged
Activity of factor Xa: decreased
107
What is the genetic cause of beta thalassemia
DNA mutations that effect the transcription, processing, and translation of beta globin mRNA most commonly causing aberrant precursor mRNA splicing or premature chain termination during translation --> beta globin deficiency
108
In a patient with beta thalassemia who has routine blood cell transfusions a liver biopsy shows Kupffer cells containing coarse, yellow-brown cytoplasmic granules which are most likely composed of what
hemosiderin (iron storage complex)
109
MOA hydroxyurea used in sickle cell patients
increases expression of gamma globin chains --> increased circulating fetal hemoglobin
110
What has the classic histopatholgy description of uniform, round, medium sized tumor cells with basophilic cytoplasm and a proliferation fraction of >99%
```
Burkitt lymphoma
(basophilic= lymphocytes)
```
111
What chromosome translocation is seen in CML
t(9;22)
| BCR-ABL --> constitutive tyrosine kinase activity
112
____ converts heme to _____, a pigment that causes the greenish color to develop in bruises several days after injury
heme oxygenase
| biliverdin
113
Patients of advanced age have increased bone marrow ___ and reduced bone marrow ____, leading to impaired bone marrow reserve
fat
| mass
114
What is the role of haptoglobin
binds circulating hemoglobin and reduces renal excretion of free hemoglobin, preventing tubular injury
115
How might cystic fibrosis cause coagulopathy
decreased fat soluble vitamin absorption --> low vit K --> decreased activation of II, VII, IX, X
116
6-mercaptopurine (6-MP) is an immunosuppression drug that is inactivated by what 2 enzymes?
reduced function of either of these enzymes causes increased levels of active metabolites of 6-MP, leading to increased risk for toxicity
xanthine oxidase
| thiopurine methyltransferase
117
Pyruvate kinase deficiency causes splenomegaly how?
failure of glycolysis --> insufficient ATP to maintain erythrocyte structure --> spleen parenchyma removes deformed erythrocytes --> red pulp hyperplasia
118
What anticoagulant prolong both the PTT and PT but have no affect on the thrombin time
direct factor Xa inhibitors
119
what medications prolong the thrombin time
medications that directly or indirectly inhibit thrombin (unfractionated heparin and dabigatran)
120
Desmopressin can alleviate bleeding through vWF release from what cells?
endothelial
121
What inherited bleeding disorder causes prolonged bleeding time and prolonged PTT but does not affect the PT
von Willebrand disease
122
If a 2 wk old baby has 70% Hemoglobin F, 20% hemoglobin A, and 10% hemoglobin S do they have sickle cell or sickle cell trait
sickle cell trait
123
Paroxysmal nocturnal hemoglobinuria can cause what renal pathology
hemosiderosis
| iron deposition in kidney from chronic hemolysis
124
what cause of hemolytic anemia will display an absence of CD55 on RBC
paroxysmal nocturnal hemoglobinuria
125
What causes autosplenectomy in sickle cell patients
repetitive splenic infarctions caused by microvessel occlusion --> small, firm splenic remnant
126
Anemia of chronic disease is caused by elevated inflammatory cytokine levels, mainly _______
hepcidin
127
How does hepcidin cause anemia of chronic disease
increases sequestration of iron within the reticuloendothelial system and reduce circulating iron levels
128
In anemia of chronic disease, how are circulating iron levels and bone marrow iron levels affected
circulating: low
| bone marrow: high
129
What is the pathogenesis of primary myelofibrosis
atypical megakaryocytic hyperplasia which stimulates fibroblast proliferation, resulting in progressive replacement of the marrow space by extensive collagen deposition
130
Apart from chronic myelogenous leukemia, the chronic myeloproliferative disorders frequently harbor a mutation in what
nonreceptor cytoplasmic tyrosine kinase, JAK2
| --> constitutive tyrosine phosphorylation activity --> STAT activation and enters nucleus --> transcription
131
What cause of hepatocellular carcinoma will show intranuclear fragments of foreign DNA within hepatic lesions
```
Hepatitis B
(integrates into host genome and produces oncogenic viral protein: HBx)
```
132
_____ deficiency is associated with coiled hair, perifollicular keratosis, hemorrhage, easy bruising, bleeding gums and loose teeth. The PT, PTT, and platelet count are normal.
vitamin C
133
Primary myelofibrosis causes massive splenomegaly how?
extramedullary hematopoiesis --> expansion of red pulp
134
____ is characterized by aggregates of packed follicles that obscure the normal lymph node architecture
follicular lymphoma
135
90% of patients with follicular lymphoma have the ___ translocation, which causes the overexpression of the ___ protein
t(14;18)
| BCL-2 (antiapoptotic)
136
Primary myelofibrosis is a myeloproliferative disorder associated with the clonal expansion of ____
megakaryoctes
137
Pure red cell aplasia is associated with what 3 pathologies
thymoma
lymphocytic leukemias
parvovirus B19
138
Howell-Jolly bodies on peripheral smear reflect what?
```
splenic dysfunction
(seen in sickle cell)
```
139
Where does uric acid precipitate in the nephron as seen in tumor lysis syndrome
distal tubules and collecting duct
| acidic environments --> precipitate; alkalization of urine prevents crystallization
140
```
Which of the following would decrease warfarin's therapeutic effects:
amiodarone
cimetidine
phenytoin
phenobarbital
metronidazole
rifampin
carbamazepine
```
```
carbamazepine
phenytoin
phenobarbital
rifampin
(the others listed are CYP450 inhibitors which would increase warfarin effects)
```
141
Megaloblastic anemia in a patient who developed a heavy drinking problem in this past year is due to what
```
folate deficiency
(B12 deficiency would take years to deplete)
```
142
What lysosomal storage disorder presents with bone pain, hepatosplenomegaly, and cytopenias and is due to a beta glucocerebrosidase deficiency
Gaucher
143
What causes rouleaux formation and what pathologies is it seen in
elevated levels of circulating proteins which disrupts the repulsive electrostatic charge on the erythrocyte surface and causes stacked aggregation
can been seen in inflammatory conditions that increase acute phase reactants (fibrinogen), it is classicaly linked with multiple myeloma and Waldenstrom macroglobunemia which generate high levels of monoclonal paraprotein (immunoglobulins)
144
How do proteasome inhibitors (bortezomib) treat cancer
they blog the degradation of ubiquitinated proteins by the proteasome, which leads to accumulation of abnormally folded proteins that trigger cellular apoptosis
145
Cancer cells avoid immune recognition by overexpressing _____ which binds its receptor on cytotoxic T cells and inhibits their response (T-cell exhaustion)
programmed death ligand 1 (PD-L1)
146
The most common cause of elevated platelet counts in all age groups is what?
reactive thromobocytosis (high levels of inflammatory cytokines prompt liver to release thrombopoietin. It is seen in chronic infection, rheumatologic disease, and burns)
147
What is seen on blood smear of leukemoid reaction
numerous mature neutrophils which may have reactive morphologic features such as Dohle bodies, toxic granulation, and cytoplasmic vacuoles
148
what lab value can help distinguish leukemoid reaction from CML
leukocyte alkaline phosphatase ( enzyme found in maturing neutrophils)
leukemoid reaction: normal or increased
CML: decreased (bc abnormal maturing neutrophils have decreased levels)
149
95% of patients with polycythemia vera have what gene mutation
V617F mutation involving the JAK2 gene --> hematopoietic cells more sensitive to growth factors
150
what are the clinical manifestations of polycythemia vera
increased RBC and plasma volume and low EPO levels
also can have elevated platelet and/or WBC count
thrombotic events from blood hyperviscocity
peptic ulceration and aquagenic pruritus from histamine release form neutrophils
gouty arthritis from increased cell turnover
151
what is seen on peripheral blood smear of lead poisoning
basophilic stippling
| on a background of hypochromic microcytic anemia
152
EBV infection induces _______ which react to antigens from animal erythrocytes
heterophile antibodies
153
EBV is associated with what malignancies
Burkitt lymphoma
nasopharyngeal carcinoma
(and central nervous system lymphoma in HIV positive patients)
154
Why is nephrotic syndrome a hypercoaguble state
loss of anticoagulant factors, especially antithrombin III
155
LMWH binds _____ and predominately acts on _____
antithrombin III
| factor Xa
156
etoposide MOA
inhibits sealing activity of topoisomerase II
157
what are topoisomerase I inhibitors
irinotecan
| topotecan
158
An anterior mediastinal mass with elevated serum levels of alpha fetoprotein and beta hCG is classic for a ____ tumor
nonseminomatous germ cell tumor
159
After exposure to various anticancer agents, tumor cells can become resistant due to what cell function
human multidrug resistance (MDR1) gene codes for P-glycoprotein, a transmembrane ATP-dependent efflux pump protein that has a broad specificity for hydrophobic compounds that can both reduce influx of drugs into cytosol and increase efflux from the cytosol thereby preventing the action of chemotherapeutic agents
160
what are two characteristic markings of myelodysplastic syndrome
1 or more cytopenias
| dysplasia of erythrocytes and neutrophils
161
What deficiency presents with dysphagia (from esophageal web formation), koilonychia (spoon-shaped nails), and a shiny red tongue
iron deficiency
162
enoxaparin MOA
a LMWH that functions like heparin in that it binds and activated antithrombin III
163
What causes DIC in abruptio placentae (placental bleeding between uterine wall and placenta)
decidual damage and ischemia --> tissue factor release into maternal circulation --> coagulation
164
How can ceftriaxone lead to a hemolytic anemia
penicillins and cephalosporins are common triggers for drug-induced hemolytic anemia because they can bind to the erythrocyte surface and act as haptens for IgG attachement --> clearance by splenic macrophages
165
Auer rods are deformed azurophilic granules found in the cytoplasm of myeloblasts that stain positively for ______
myeloperoxidase
166
Hemophilia is an X linked recessive bleeding disorder due to decreased levels of ____ (hemophilia A) or ____ (hemophilia B)
A: factor VIII
B: factor IX
167
A patient's red blood cells are incubated in a hypotonic solution with glycerol and hemoglobin is released, the control sample does not release hemoglobin. What is the diagnosis
hereditary spherocytosis
168
what test of hemostatic function is generally abnormal in hemophilia
partial thromboplastin time (PTT)
169
What is the purpose of the Kozak consesnus sequence position before and AUG codon
identifies the specific AUG codon that serves as the initiator of translation, mutations affecting the Kozak consensus sequence can significantly impair protein translation
170
with increased bone marrow erythropoiesis, accelerated release of immature RBC into the blood stream cause them to contain bluish cytoplasm due to what
residual ribosomal RNA
171
diminished thymidine synthesis is likely due to wat cause of megaloblastic anemia
vitamin B12 deficiency
172
Parvovirus primarily attacks erythroid progenitor cells due to tropism for an erythrocyte cell surface receptor, infection of the erythroid progenitor cells prevents RBC maturation leading to formation of ______ which can be seen on blood smear
giant pronormoblasts (several times larger than surrounding RBCs with glassy, intranuclear viral inclusion
173
Auer rods = ____
AML
174
How does renal disease cause excessive bleeding
uremic toxins in circulation impair platelet aggregation and adhesion --> qualitative platelet disorder (numbers are normal)
175
30% of patients with SLE have _______ antibodies which can caue paradoxical aPTT prolongation and a false positive RPR/VRDL
antiphospholipid
176
Patients with antiphospholipid antibodies are at risk for ____ and ____
venous and arterial thromboembolism
| unexplained, recurrent pregnancy loss
177
all trans retinoic acid induces remission in about 90% of patients with what cancer
acute promyelocytic leukemia (M3 variant of acute myelogenous leukemia)
