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(***) (U-World) All Subjects > Heme/Onc/Lymph U WORLD Questions > Flashcards

Heme/Onc/Lymph U WORLD Questions Flashcards

1
Q

new HA, intracranial mass, biopsy, age 60s, with areas of necrosis surrounded by columns of tumor cells, capillaries at the periphery =

this is the _____ primary brain neoplasm in adults

pathology feature

origin cell

A

glioblastoma multiforme

most common

pseudopalisading necrosis

astrocyte

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2
Q

given several units of packed red blood cells, develop tingling and hypocalcemia due to ______chelation by ______ that is added to stored blood

A

calcium

citrate

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3
Q

exposure status determined then followed during study =

exposure status determined after study =

A

prospective cohort

retrospective cohort

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4
Q

dermatomyositis occurs during _____ and is characterized by proximal muscle weakness, heliotrope rash in periorbital and cheek region, and ____, which are erythematous plaques over the joints.

muscle biopsy will show

strong associated with lung, colorectal, and _____ cancers

A

malignancy

Gottron Papules

perimysial infiltrates

ovarian

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5
Q

sickle cell anemia is autosomal _______ inheritance and can be confirmed by __________ electrophoresis. the abnormal hemoglobin is designated _____

A

recessive

hemoglobin

HbS

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6
Q

radiation therapy induces DNA damage through ______ and formation of free radicals

A

DNA double strand breaks

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7
Q

factors II, VII, IX, and X are _______ dependent coagulation factors synthesized in the _______. Factor _____ has the shortest half life. Failure of ________, which assess the extrinsic and common pathway of coagulation, to correct with vitamin K supplementation indicates ________ deficiency

A

Vitamin K

LIver

VII

Prothrombin time

VII

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8
Q

______blotting is used to ID proteins. ________ blotting is used to ID RNA sequences. ______ blotting IDs specific DNA sequences in an unknown sample

A

Western

Northern

Southern

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9
Q

dizziness, HA, pruritus after showering, ruddy complexion, splenomegaly =

increased platelets, RBC mass, and decreased EPO is due to increased bone marrow sensitiity to ____ which results in polycythemia _____, typically with mutation _____

normal platelet count, increased RBC mass, and increased EPO is usually due to an inappropriate _____ producing _____

incrased EPO and RBC mass in the presence of decreased SaO2 is a _______ response

A

polycythemia

Growth factors

vera

JAK2/V617F

EPO

Tumor

physiologic

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10
Q

anemia, reticulocytosis, and increased indirect bilirubin indicates _______anemia. RBCs w/o central pallor are called_____, and can be acquired or hereditary. Hereditary _______ can be due to mutations involving ____, band 3, or ______, while acquired ______ is due to autoimmune disorder. Both of these will show an elevation in _________

A

hemolytic

spherocytes

spherocytosis

ankyrin, spectrin

spherocytosis

Mean corpuscular hemoglobin concentration MCHC

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11
Q

HIV pol genes are responsible for _______ to antiretroviral therapy. Env gene mutations enable _____ from host neutralizing antibodies

A

acquired resistance

escape

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12
Q

newborn w/ jaundice, hepatomegaly, edema, low hemoglobin, + direct Coombs, extramedullary hematopoeisis =

due to _______ antibodies developed during a prior _____ pregnancy which cause severe autoimmune hemolytic anemia and hydrops fetalis

A

erythroblastosis fetalis (Rh sensitization)

maternal anti RhD IgG

RhD+

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13
Q

chemokine receptor ______ acts as a coreceptor that enables the HIV virus to ____ cells. HIV virus uses ___ as a primary receptor, and both _____ and ____ are bound to HIV viral outer envelope protein gp120

A

CCR5

enter

CD4

CD4 and CCR5

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14
Q

fever, bleeding gums, sore throat, several myeloblasts with abundant _______ cytoplasm and a large number of coarse rod shaped intracytoplasmic granules called ____ rods that stain for ________ indicate _______ type M3

A

basophilic

Auer

peroxidase

AML

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15
Q

all NSAID impair platelet aggregation except ____ which is a _____ selective inhibitor and has no side effects of bleeding and GI ulceration

A

Celecoxib

COX2

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16
Q

mature erythrocytes lose their ability to synthesize heme when they ______ their ________

A

lose

mitochondria

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17
Q

folate deficiency inhibits the synthesis of ______, which leads to defective ______ synthesis and megaloblastic anemia. THis can be overcome w/ _______ supplementation

A

deoxythymidine monophosphate (dTMP)

DNA

Thymidine

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18
Q

tRNA that is mischarged with the incorrect amino acid will _________________ amino acid into the polypeptide chain

A

incorporate the wrong

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19
Q

Neutrophils roll via the loose binding of ______ or _______ to L -selectin on neutrophils or ________ on endothelial cells

Neutrophils firmly attach to the endothelium (tight adhesion and crawling) via binding of ________ to _______ on enodthelial cells

Neutrophils migrate out of vasculature (transmigration) via attachment to _______

A

Sialyl Lewis X

PSGL1

E/P selectin

LFA-1

ICAM-1

PECAM-1

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20
Q

chemotherapy induced emesis can be treated w/ _______ and _______ that act as 5HT3 receptor antagonists

A

Ondansetron

Granisetron

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21
Q

mTOR pathway = binding of growth factor, autophosphorylation of _____ residues, activation of _______, activation of ______, then activation of _____ which translocates to the nucleus

A

tyrosine

PIP3

AKT

mTOR

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22
Q

bone pain, fatigue, kidney disease, hypercalcemia, anemia is typical of ________ which is due to neoplastic B lymphocytes that mature into plasma cells and synthesize large amounts of ________. These plasma cells are susceptible to the effects of proteasome inhibitors such as ________, a boronic acid containing dipeptide

A

Multiple myeloma

Ig or Ig fragments

bortezomib

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23
Q

a left shift on O2-Hbg dissociation curve can be caused by _________ (3) and indicates

a right shift can be caused by _____(3) and indicates

A

inc in pH, dec 2,3BPG, dec temp

O2 less available to tissues

dec in pH, inc 2,3BPG, inc temp

O2 more available to tissues

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24
Q

Meningococcal meningitis that progresses to DIC results in _______ on peripheral blood smear, which are fragmented erythrocytes

A

schistocytes

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25
Q

_______ can reverse the toxicity of methotrexate in non cancerous cells of the GI mucosa and bone marrow. This drug is also called ________

A

Leucovorin

Folinic acid

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26
Q

6-MP is degraded by _____, which can be inhibited by ____. Both 6-MP and 6-thioguanine are prodrugs that require activation by _______

A

XO

allopurinol

HGPRT

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27
Q

hemolytic anemia due to medication induced oxidative stress is indicative of _______. Antimalarial drugs usually precede the hemolysis. Peripheral blood smear shows RBCs w/ ______, dark intracellular inclusions that stain w/ supravital stain. This disorder follows ______ recessive inheritance

A

G6PD deficiency

Heinz bodies

X linked

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28
Q

diffuse large B cell lymphoma is the most common type of NHL and is typically treated w/ ________, which act during the ______ phase of the cell cycle and can cause _______

A

vincristine, vinblastine

M

peripheral neuropathy (stocking and glove)

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29
Q

dysplasia is ____ change in epithelial cells, which CIS is _______, and invasive carcinoma shows

A

reversible

irreversible

invasion of basement membrane

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30
Q

fatigue, weak, conjuvtival pallor, decrease hemoglobin =

bruising =

high lipid content of bone marrow =

all of which suggestive of ______, which will present w/ increased production of ________

A

anemia

thrombocytopenia

hematopoetic cell aplasia

aplastic anemia

EPO

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31
Q

+TdT, CD10, CD19 =

+CD2-8, TdT, CD1a =

A

precursor B-ALL

precursor T-ALL

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32
Q

anemia, megaloblastic anemia, neuro deficits =

can be due to a ___diet

A

B12 deficient

strict vegan

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33
Q

female, fatigue, hypochromic, microcytic anemia =

bluish color on RBCs after treatment due to =

A

iron deficient anemia

reticulocytosis (increased ribosomal RNA)

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34
Q

CD8 T cell mediated apoptosis results in rapid cell death due to activation of ______

A

caspases

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35
Q

SLE can present in women w/ arthralgias and decreased erythrocytes, leukocytes, and platelets. This is called ______

A

pancytopenia

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36
Q

HIV infection can cause reactivation of latent _____ and result in ________ lymphomas, including ______ lymphoma

A

EBV

EBV induced

Burkitt

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37
Q

aspirin is an NSAID that ________ inhibits _______ via acetylation

A

irreversibly

COX 1 and 2

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38
Q

sickle cell can present with jaundice, lower back pain, extremity pain, and anemia. this results in ____ of the spleen due to repeated ________ infarcts

A

fibrosis and atrophy

splenic

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39
Q

sickle cell patients have increased __________ requirements due to high RBC turnover. THis can result in ________ anemia as well

A

folic acid

megaloblastic

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40
Q

industrial worker or child with weakness, cramping abdominal pain due to ________

peripheral blood smear will show

physical exam will show

A

lead poisoning

coarse basophilic stippling

blue lead lines on teeth and gingiva

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41
Q

carbon monoxide poisoning will ______ carboxyhemoglobin, but will have no effect on _______

A

increase

partial pressure oxygen

methemoglobin

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42
Q

PCR requires knowledge of the nucleotide sequence of the regions ______ the target exon

A

flanking

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43
Q

three most common cancer incidence in women are _______ respectively

three most common cancer death in women, respectively

A

Breast, Lung, colon

Lung, breast, colon

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44
Q

kid w/ hemolytic anemia, jaundice, splenomegaly, spherocytes (lack central zone of pallor) =

A

hereditary spherocytosis

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45
Q

sickle cell anemia experiencing aplastic crisis is due to infection of erythroid progenitor cells with _____, which is a nonenveloped ________ virus

A

parvovirus B19

ssDNA

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46
Q

t(14;18) is associated w/ ______ overexpression and _____ lymphoma

A

BCL2

follicular

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47
Q

anti RhD Ig administered usually at _____weeks

A

28

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48
Q

erythematous itch breast rash w/ skin texture of orange peel =

caused by cancerous cells ________ drainage

A

peu d orange, inflammatory breast cancer

obstructing lymphatic

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49
Q

HbF is composed of 2 ____ units and 2 ____ units

Adult hemoglobin is composed of 2 ____ and 2 _____

A

alpha

gamma

alpha

beta

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50
Q

cutaneous lymph from the umbilicus down, including the anus below the dentate line, drain to the ________ lymph nodes

exceptions are (2)

A

superficial inguinal

glans penis

posterior calf

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51
Q

t(15;17) is associated w/ ____ and is a translocation involving _______ and the PML gene

often presents with fatigue, easy bruising, and _____

A

APML

RARA

gum bleeding

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52
Q

methemoglobinemia causes a dusky discoloration of the skin similar to _____ when patients are exposed to high levels of _______ in labs. The partial pressure of oxygen in these patients is

A

cyanosis

nitrates

unchanged

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53
Q

sickle cell patients are functionally _______, which increases risk for infection by __________ and ________, which are encapsulated.

The most common cause of osteomyelitis in sickle cell patients is ______

A

asplenic

Strep pneumo

H influenza

Salmonella

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54
Q

weakness, fatigue, loss of expression of gene coding for protein on basolateral surface of hepatocytes and enterocytes that interacts w/ transferrin receptor

Disorder =

protein =

leads to =

inc risk of developing =

A

primary hemochromatosis

HFE protein, decreased hepcidin synthesis and DMT1 overexpression

Iron overload

liver cirrhosis and hepatocellular carcinoma

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55
Q

newborn with intracranial hemorrhage, “natural” newborn period, born on time w/o complications?

diagnosis if Vitamin K prophylaxis had been given ?

A

Vitamin K deficiency = impaired clotting factor carboxylation

abusive head trauma = subdural hemorrhage and ICB

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56
Q

monoclonal antibodies that block ______ and ______ help prevent T cell inhibition and promote T cell mediated destruction of tumor cells

used in?

A

PD-1 and CTLA-4

advanced melanoma

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57
Q

patient presents with abdominal pain, low hemoglobin, platelets, inc bilirubin, inc lactate dehydrogenase, low haptoglobin, mesenteric vein thrombosis, absence of CD55 on RBC =

gene =

renal =

A

paroxysmal nocturnal hemoglobinuria (complement mediated hemolytic anemai)

PIGA

hemosiderosis due to iron deposition

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58
Q

hepatocellular carcinoma can be caused by Hepatitis B which is _____ virus, or Hepatitis C which is a ______ virus

A

DNA

RNA

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59
Q

renal cortical cells sense _____ and release ____

A

hypoxia

EPO

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60
Q

Rituximab is a monoclonal AB directed against the _____ antigen and used in aggressive lymphomas

A

CD20

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61
Q

the C1 complement molecule binds to the ____ region of the ______ Ig chain in the region near the hinge point

Ig___ is a better activator of complement

A

Fc

Heavy

M

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62
Q

a reduced ability to relase oxygen within the peripheral tissues results in renal hypoxia and compensatory

A

erythrocytosis

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63
Q

family history of osteosarcomas, breast cancer, brain tumors, adrenal tumors, leukemias =

gene

A

Li Fraumeni

TP53, AD, TumorSuppressor

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64
Q

young, multiday history of nausea, constipation, severe, poorly localized abdominal pain, anxiety, difficulty concentrating, poor sleep, tingling of limbs, several similar episodes in past =

exacerbated by:

treated w/

due to downregulation of

urine

A

acute, intermittent porphyria

alcohol, drugs, or calorie restriction

IV heme preparation

Aminolevulinate synthase

port wine colored, PBG, ALA +

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65
Q

the binding of oxygen to hemoglobin drives the release of ____ and ___ from hemoglobin, called the Haldane effect. High concentrations of CO2 and H+ in peripheral tissues facilitates ______ from hemoglobin, called the Bohr effect

A

H+ and CO2

oxygen unloading

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66
Q

spleen acts as a blood filter capable of removig circulating encapsulated pathogens such as (3)

A

Strep Pneumo

H Influ

N. Meningitidis

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67
Q

rate limiting enzyme in pentose phosphate pathway

deficient =

interconversion of ribose 5 - phosphate and fructose 6 phosphate is mediated by

A

G6PD

no NADPH = hemolytic anemia

transketolase

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68
Q

baby african american boy with painful swelling in hands and feet =

due to

lab value

A

dactylitis

Sickle cell

low haptoglobin

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69
Q

pneumonia + sepsis can result in ______ which is benign leukocytosis (>50,000). Peripheral blood smear will show _____ bodies, which are light blue (______) peripheral granules in neutrophils

A

leukemoid reaction

Dohle

basophilic

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70
Q

chemotherapy that leads to rapid lysis of neoplastic cells can result in =

patients can be given ______ which protects normal organs by converting ______ into more soluble metabolites

A

tumor lysis syndrome

rasburicase or allopurinol

uric acid

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71
Q

the human mutli drug resistant gene ______ encodes for a _______ that is a transmembrane ATP dependent efflux pump and is responsible for preventing the action of various _________ agents

A

MDR1

glycoprotein

chemo

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72
Q

heparin increases the effect of the naturally occurring anticoagulant ______ by binding it and is used for prophylaxis against _____ in nonambulatory post operative patients. This drug, in LMW form is called ____

A

antithrombin III

DVT

enoxaparin

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73
Q

patients bleed, coagulation cascade active, PT and PTT prolonged, low fibrinogen and increased FDP =

not bleeding, only platelets activated, normal PT and PTT, normal fibrinogen

increased partial thromboplastin time, everything else normal =

A

DIC (gram neg. rod infection)

TTP- HUS (fever, neuro, acute renal failure, thrombocytopenia, and microangiopathic hemolytic anemia)

vWF disease

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74
Q

high peak gamma globulin region on serum protein electrophoresis w/ M protein =

presents w/

A

multiple myeloma

anemia, lytic bone lesions, renal insufficiency, hypercalcemia, amyloid deposition

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75
Q

HbS aggregates in a _____ state, low ____, and high ______ state

A

deoxygenated

pH

2.3 BPG

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76
Q

Mediterranean, mild anemia, microcytic, increased hemoglobin A2, hypochromia, poikilocytes (spherocytes + target cells), asymptomatic =

caused by mutations in

A

beta thalassemia minor

defective transcription, processing, and translation of betaglobin mRNA

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77
Q

young man, recurrent jaundice, pallor, icterus, mild splenomegaly no hepatomegaly or LAD, inc lactate dehydrogenase, low Hg, RBCs release hemoglobin in hypotonic solution w/ glycerol (osmotic fragility) =

treatment?

complications??

A

hereditary spherocytosis

splenectomy

pigmented gallstones, aplastic crises from parvovirus B19

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78
Q

CD4 virus

CD21 virus

Erythrocyte P antigen

A

HIV gp120

EBV gp 350

Parvovirus B19

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79
Q

glutamic acid to valine substition at position 6 in beta globin gene =

missense mutation

A

sickle cell

GAG to GTG in DNA strand

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80
Q

warfarin inhibits ______ which can lead to skin and fat necrosis seen in the first few days of warfarin therapy

A

protein C and S

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81
Q

high dose opioid treatments in metastatic cancer commonly require _______ and _____ in order to maintain bowel regularity

A

adequate fluid intake and daily laxatives

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82
Q

proliferation signals activate _____ which results in hyperphosphorylation of the _____ protein, inactivating it. When this protein is inactive, cells are allowed to transition from G1 to S, thus increasing activity of dihydrofolate reducatse and DNA polymerase

A

CDK4

Rb

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83
Q

new onset neurologic symptoms, hemolytic anemia w/ schistocytes, thrombocytopenia, acute kidney injury =

deficiency of ____ leads to large uncleaved cWF multimers

treat with

A

thrombotic thrombocytopenic purpura

ADAMTS13

plasma exchange therapy

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84
Q

metalloproteinases are zinc containing enzymes that _____ the _______ of the basement membrane, allowing tumor cell invasion

A

degrade

ECM

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85
Q

extramedullary hematopoiesis in a child causes a range of skeletal problems such as _______ and is due to ______ such as B thalassemia major

A

maxillary overgrowth (chipmunk facies)

chronic hemolytic anemia

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86
Q

large, binucleated cells w/ an owls eye appearance are called _____ cells and are diagnostic of _____

A

Reed Steinberg

Hodgkin lymphoma

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87
Q

excessive bruising since childhood with abnormal platelet aggregometry and a defect in platelet glycoprotein surface receptor =

normally binds =

inheritence

treated w/

A

Glanzmann thrombasthenia

fibrinogen

AR defect in GP IIB/IIIa

abciximab

88
Q

isotype switching occurs in the ______ of lymph nodes and requires interaction of the _______ receptor on B cells with the ______ ligand on activated T cells

A

germinal centers

CD40

CD40

89
Q

hepcidin is synthesized by ________ and acts as the central regulator of _______ homeostasis

A

hepatic parenchymal cells

iron

90
Q

when the body mounts a response against _________, a bug that shares antigens with human erythrocytes, it also lyses RBCs, leading to anemia. These antibodies are called cold agglutinins. Once the _______ has faded, the anemia resolves

A

mycoplasma pneumoniae

immune response

91
Q

fatigue, fever, return from central Africa, multiple small rings in RBCs on Giemsa staining =

multiple small rings called

treatment of choice

required for P. vivax and P ovale?

A

Plasmodium falciparum malaria

trophozoites

chloroquines: mefloquine for resistant bugs

primaquine due to dormant liver forms

92
Q

sudden onset abdominal pain, ascites, anemia, reticulocytosis, thrombocytopenia, CD55 and 59, DVT or HVT =

gene =

CD55 and 59 role

A

paroxysmal nocturnal hemoglobinuria

PIGA

complement inhibitors: disease due to complement activation

93
Q

eczema, recurrent infections, thrombocytopenia with petechiae, purpura, epistaxis in a child, B and T lymphocyte deficiency =

inheritance =

susceptible to =

treatment is =

A

Wiskott-Aldrich

X linked

encapsulated organisms

HLA matched bone marrow transplant

94
Q

mutation at RCC before AUG - methionine start codon leads to a block at

A

initiation of translation

95
Q

Beta thalassemia results in ________ _________ anemia due to decrease beta globin chain synthesis. unpaired _____ chain precipitate within red cells and cause membrane ______, leading to extravascular hemolysis and ineffective erythropoiesis

A

microcytic anemia

alpha

damage

96
Q

DVT, given a drug that increases PT and aPTT but does not effect thrombin time, what drugs (2)?

Mechanism?

drugs that prolong thrombin time?

A

apixaban, rivaroxaban

direct factor Xa inhibitors

unfractionated heparin, direct thrombin inhibitors (dabigatran)

97
Q

______ is the mitochondrial enzyme that activates caspases and indirectly brings about cell death through intrinsic pathway apoptosis

A

cytochrome C

98
Q

young african american boy, exertional dyspnea, acute chest syndrome, bone pain =

mutation

A

sickle cell anemia

point mutation : valine substituted for glutamic acid

99
Q

chemotherapy, followed by progressive hematuria and suprapubic tenderness due to _______

causative agent is nitrogen mustards such as

metabolized by the kidneys into

prevented by

A

hemorrhagic cystitis

cyclophosphamide, ifosfamide

acrolein

mesna and sulfhydrl compound

100
Q

black pigmented stones with low cholesterol content arise from conditions that increase _________ in bile such as _______ in sickle cell, thalassemia, and hereditary spherocytosis

A

unconjugated bilirubin

chronic hemolysis

101
Q

the most common trigger for DIC in pregnancy is the release of _______ from an injured ________ into the maternal circulation. Presents with bleeding from incision sites, IV sites, and mucosal surfaces due to rapid consumption of clotting factors and platelets

A

Tissue Factor

Placenta

102
Q

the change in color of bruise from dark blue to greenish in color is caused by activity of what enzyme?

A

heme oxygenased: heme to biliverdin (green)

103
Q

persistent, fluctuating LAD who may have had an unrelated pharyngitis treated with antibiotics on initial presentation most likely has

translocation

A

follicular lymphoma

t(14;18)

104
Q

angiogenesis of a hepatic hemangioma is primarily driven by ______ and ______

A

VEGF and FGF2

105
Q

pyruvate kinase deficiency causes ______ anemia, and splenic ______ results from increased work of the splenic parenchyma to remove the deformed RBCs from cirulation

A

hemolytic

red pulp hyperplasia

106
Q

night sweats, weight loss, cough, apical pulmonary granulomas =

cells that cause lesions =

surface marker

A

secondary tuberculosis

macrophages

CD14

107
Q

fresh frozen plasma rapidly reverses ______ effects whereas vitamin K requires time for clotting factor re synthesis

A

warfarins

108
Q

hydroxyurea increase _____ synthesis by an unknown mechanism and is used to treat _______ patients with frequent pain crises

_______ channel blockers hinder the efflux of K and H2O from the cell, preventing ________ of RBCs and reducing polymerization of ______

A

HbF

Sickle cell anemia

Gardos

dehydration

HbS

109
Q

different mRNA sizes, same gene, due to

A

alternative splicing

110
Q

recurrent epigastric pain, hepatomegaly, macrocytic anemia, pancreatic calcifications =

anemia is due to

A

chronic alcoholic pancreatitis

diminished thymidine synthesis: megaloblastosis

111
Q

carbon tetrachloride poisoning causes fatty change and hepatocyte necorsis via _____ injury

A

free radical

112
Q

electrophoresis shows a single band that migrates less than HbA and HbS band in an African American boy with easy fatiguability: indicative of _____ that is caused by a ________ mutation that results in a glutamate residue being substituted by ______ in the beta globin chain

A

HbC

missense

lysine

113
Q

HA, weakness, aquagenic pruritus, facial plethora, and splenomegaly, along with PUD and gouty arthritis =

due to a mutation in ______, which is a cytoplasmic tyrosine kinase

A

polycythemia vera

JAK2

114
Q

individuals who demonstrate increased activity of ______ are more susceptible to developing chemical induced cancers

A

P450 cytochrome microsomal oxygenase

115
Q

young female with new onset of thrombocytopenia, ecchymoses, petechiae, mucosal bleeding, and no other obvious causes =

due to

A

Immune thrombocytopenic purpura

autoimmune destruction of platelets

116
Q

child with cystic fibrosis, seizure, and confirmed intracranial hemorrhage due to decreased _______ as a result of ________ deficiency, a fat soluble vitamin

coagulation factors affected

A

gamma carboxylation

Vitamin K

II, VII, IX, X

117
Q

hypochromic, microcytic anemia is most commonly due to ________, especially in the presence of _______ loss

A

iron deficiency

occult blood

118
Q

venous thromboembolism arises due to the ______ triad of __________, _________. and ________

A

virchow

endothelial injury

venous stasis

hypercoaguable state

119
Q

cytokeratin is a commonly used immunohistochemical marker of _____ cell carcinomas

A

epithelial

120
Q

HER2 oncogene encodes for a _______ glycoprotein with intrinsic ________ activity and is a family of ________. Overexpression is associated witha worse prognosis and an increased risk of disease recurrence. Treatment is a monoclonal antibody against HER2 called _______

A

transmembrane

tyrosine kinase

epidermal growth factor receptors

trastuzumab

121
Q

treatment using a double stranded RNA molecule consisting of 20-30 base pairs =

interferes w/ =

A

small interfering RNA and microRNA

mRNA translation

122
Q

tumors harboring activating mutations in _____ are resistant to chemotherapy with _______ such as cetuximab and panitumumab

A

KRAS

anti EGFR drugs

123
Q

rivaroxaban, apixaban =

fondaparinux =

argatroban, bivalirudin, and dabigatran =

A

DIrect Factor Xa inhibitors

INidrect Factor Xa inhibitor

Direct Thrombin inhibitors

124
Q

many transfusions, frontal bossing, HSM, jaundice, liver biopsy showing kupffer cells containing coarse, yellowish brown cytoplasmic granules composed of_____. This is due to______

A

hemosiderin

iron overload from transfusion therapy

125
Q

cachexia and muscle wasting are due to _____ in a paraneoplastic setting

A

TNF-alpha

126
Q

prognosis of colorectal adenocarcinoma is directly related to __________, not the ________

A

stage of invasion

anaplastic grade

127
Q

fever, pharyngitis, LAD in young adult is suggestive of _________

caused by?

associated with

A

infectious mononucleosis

EBV

nasopharyngeal carcinoma

128
Q

rifampin, phenobarbital, and phenytoin are universal enhancers of ________ and decrease the effect of _____

in contrast, cimetidine, amiodarone, and TMP-SMX ______ _______ metabolism, increasing risk of bleeding

A

CP450

warfarin

inhibit warfarin

129
Q

people with down syndrome are at an increased risk of developing

A

ALL and AML

130
Q

if chemotherapy induced emesis is not refractory after -setron use, _______ tend to be effective. The drugs in this category are (2)

A

neurokinin 1 receptor antagonists

aprepitant, fosaprepitant

131
Q

recurrent infections, pallor (anemia), and ecchymoses with a peripheral blood smear that shows cells with Auer rods =

translocation

A

APML M3

t15;17 RARa/PML

132
Q

aggregates of packed follicles, LAD in the cervical area =

translocation

effect

A

follicular lymphoma

t14;18

BCL-2

133
Q

DiGeorge syndrome is caused by maldevelopment of the ____ and ___ pharyngeal pouch derivatives. This causes a lack of development of the thymus, an extreme deficiency of mature T lymphocytes, leading to poor development of ________ ______

A

3rd and 4th

lymph node paracortex

134
Q

____ and leukemoid reaction can have presentations similar to leukocytosis. Leukemoid reaction has an ________ alkaline phosphatase level, while CML has a ______ level and an increase in precursor _______. Definitive diagnosis of CML is depending on _______

A

CML

elevated

decreased

myelocytes

philidelphia chromosome, BCR-ABL, t(9;22)

135
Q

cells that express both CD4 and CD8 are classified as

A

immature cortical T cells

136
Q

fibrinolytics such as _____ may cause reperfusion arrhythmias that are benign during arterial reopening.

A

tPA, reteplase, tenecteplase

137
Q

erythrocytes use ______ as the major pathway to generate energy as they do not have ______

A

glycolysis

mitochondria

138
Q

________ syndrome in a patient with small cell or _____ lung cancer, and is a result of a paraneoplastic syndrome involving ectopic production of _____ by tumor cells

A

Cushing

oat cell

ACTH

139
Q

TB treatment, ________ inhibits pyridoxine phosphokinase, leading to _________ (b6) deficiency. Pyridoxines active form is the cofactor for ________, which catalyzes the rate limiting step of heme synthesis. Inhibition of this step can result in ___________

A

isoniazid

pyridoxine

delta aminolevulinate synthase

sideroblastic anemia

140
Q

atrophic gastritis can result in _______ and elevated methymalonic acid levels. The _______ count increases dramatically once ______ replacement therapy is initiated, but normalize quickly

A

vitamin b12 deficiecy due to inadequate intrinsic factor production

reticulocyte

b12 replacement

141
Q

G6PD is the rate limiting step of glucose 6 phosphate to 6 phosphogluconate. Deficiency results in bite cells and weakness, malaise, fatigue, and anemia after oxidative stress, such as treatment with (3)

A

sulfonamides

antimalarials

antibiotics

142
Q

neuro deficit and hypersegmented neutrophils =

no neuro deficits but still megaloblastic anemia

A

B12

folate

143
Q

desmopressin can alleviate bleeding through _______ release of _______

A

endothelial

vWF

144
Q

only ________ heparin is able to bind both antithrombin and thrombin to allow antithrombin to inactivate thrombin.

A

unfractionated

145
Q

excessive bleeding + hemarthroses =

inheritence =

phenotypically normal parents, probability of a female sibling with give birth to affected child is

A

hemophilia A or B

X recessive

1/8

146
Q

somnolence, lethargy, oliguria, diarrhea that become blood, elevated blood urea nitrogen and creatinine, peripheral blood smear show fragmented erythrocytes =

due to =

A

HUS

EHEC from undercooked beef

147
Q

_________ can lead to both dose dependent cytopenia and dose independent aplastic anemia

A

chlorophenicol

148
Q

flushing, watery diarrhea, bronchospasm, elevated 5-HIAA =

due to =

A

carcinoid syndrome

carcinoid metastatic to liver

149
Q

pancytopenia post infection with no LAD or splenomegaly =

infectious causes

diangosis

A

aplastic anemia

EBV, parvo B19

hypocellular bone marrow

150
Q

individual subunits of hemoglobin similar to _______ and shows a ______ shift on dissociation curve

A

myoglobin

left

151
Q

Kaposis sarcoma presents with a blue violet or brownish skin plaque in ______ + patients and is due to _________

A

HIV

HHV8

152
Q

______________ which is modified to resist activated protein C and results in a hypercoaguable state and predisposes to DVT

A

Factor V Leiden

153
Q

hypophosphorylated retinoblastoma protein is most likely to

A

prevent G1/S cell cycle transition

154
Q

___________ attacks are characterized by abdominal pain and vomiting, reddish brown urine, and neuropsych symptoms. Treatment consists of IV glucose/dextrose or heme preparations which downregulate _______ activity

A

acute intermittent porphyria

ALA synthase

155
Q

________ lymphoma is characterized by starry sky microscopic appearance. Translocation of _______ on t____, and functions as a trascription activator

A

Burkitts

C-myc

8;14

156
Q

bilobed or double nuclei and inclusion like eosinophilic nucleoli =

diagnostic of

A

Reed-Sternberg cell

Hodgkin lymphoma

157
Q

kid with pancytopenia, HSM, distended macrophages with wrinkled tissue paper appearance =

inheritence

deficiency in

A

Gacher disease

autosomal recessive

B glucocerebrosidases

158
Q

Beta globin with impaired ionic interaction between beta subunit and 2,3 bisphosphoglycerate is most similar to

A

HbF

159
Q

patients with sickle cell trait have relative protection from _______

A

malaria caused by plasmodium falciparum

160
Q

carbon monoxide ________ binds to hemoglobin with a much greater affinity than oxygen

A

competitively

161
Q

Ig___ cannot cross placenta, while Ig___ can

A

IgM

IgG

162
Q

undifferentiated (anaplasti) tumors bear ______ to tissue of origin

A

no resemblence

163
Q

abnormal ______ is seen in patients with chronic ____ and ________

A

bleeding time

kidney disease

uremia

164
Q

tumor with cystic and solid component in cerebellum in a child is most lilkely

a completely solid tumor is most likely =

A

pilocytic astrocytoma

medulloblastoma

165
Q

bone metastases are either ______, _______, or mixed. Increased uptake on radionuclide bone imaging is indicative of _________.

multiple myeloma, non small cell lung cancer, NHL, RCC, and melanoma tend to be ________

Prostate cancer, small cell lung cancer, HL tend to be ________

GI and breast cancer tend to be _________

A

osteoblastic, osteolytic

osteoblastic

osteolytic

osteoblastic

mixed

166
Q

HER2 is a _______ kinase receptor

A

tyrosine

167
Q

sore throat, malaise, LAD, myalgias, splenomegaly in young =

cell that attacks EBV in IM?

A

IM

CD8 CTL activated

168
Q

patients with ______ present with lifelong mucosal bleeding, heavy menstruation, normal platelet levels, but prolonged bleeding time due to impaired platelet functioning

A

von Willebrand disease

169
Q

von Willebrand factor promotes ________ by binding to and crosslinking __________ with exposed ______. It also functions as a protective carrier protein for ______

A

platelet adhesion

platelet glycoproteins GpIb

collagen

factor VIII

170
Q

prostate cancer is usually _______ dependent. In combination with long acting gonadotropin releasing hormone agonsts, ________ is added and acts as a ___________ of testosterone receptors

A

testosterone dependent

Flutamide

competitive inhibitor

171
Q

ALL is the most common malignancy of childhood. _____ ALL often presents with a _______ that can cause respiratory symptoms, dysphagia, and SVC syndrome

A

T cell

mediastinal mass

172
Q

_________, or fragmented RBCs suggest ____________ such as HUS, TTP, DIC, or mechanical damage (prosthetic valve). In childhood, HUS is often preceded by ______. Coaguation studies (Prothrombin time and partial thromboplastin time) are normal in _______ and _______ but abnormal in ______

A

schistocytes

microangiopathic hemolytic anemia

bloody diarrhea

HUS, TTP

DIC

173
Q

___________ is the most common disorder of porphyrin (heme) synthesis and is caused by ________ defciency, and manifests as a vesicle and blister on sun exposed areas along with edema, pruritus, pain, and erythema.

exacerbated by ____, smoking, halogenated hydrocarbons, hep C, HIV

A

porphyria cutanea tarda - late step porphyria

uroporphyringoen decarboxylase (UROD)

alchol

174
Q

_______ prevents chromosomal shortening

A

telomerase

175
Q

____________ syndrome is characterized by dysphagia from ________ formation and ________ deficiency anemia. Patients also exhibit koilonychia or spoon shaped nails and a shiny red tongue

A

Plummer Vinson

esophageal web

iron

176
Q

prolonged bleeding and hemoarthosis =

desmospressin can be used for treatment of mild hemophilia A by increasing the circulating level of ______

A

hemophilia

Factor VIII

177
Q

methotrexate competitively inhibits ______, which causes this intermediate to accumulate intracellularly

A

dihydrofolate reducatse

dihydrofolate

178
Q

G6PD presents in a similar way to __________ deficiency

A

glutathione reductase

179
Q

low immunoglobulins and recurrent infections is indicative of ________. A positive response to candida antigens indicates that ________ is intact. In this disorder, the ______ and primary lymphoid follicles doe not form due to an absence of B cells. This condition results from a mutation in _____

A

X linked agammaglobulinemia

T cell function

germinal centers

bruton tyr kinase

180
Q

__________ is a significant adverse of effect of ganciclovir and is increased with co adminstration of zidovudine or TMP SMX

A

neutropenia

181
Q

Auer rods are found in blast cells of ______ but not in _____. More mature cells and fewer blasts are found in ______

A

AML

ALL

CML

182
Q

HIV with diffuse medium sized lymphocytes with basophilic cytoplasm and high proliferation index (high Ki67 fraction) =

due to =

translocation

A

burkitt lymphoma

EBV

t8;14 c-myc

183
Q

hemophilia due to decreased levels of ______ or _______. The addition of _______ to the blood of a patient with hemophilia results in clotting

A

factor VIII

Factor IX

thrombin

184
Q

painless gross hematuria with lack of other symptoms =

usual form?

most likely prognostic factor?

A

bladder cancer

urothelial (transitional cell) carcinoma

depth of invasion into the muscular layer

185
Q

adhesion of cells to the ECM involves _______ mediated binding to ________, collagen, and laminin

A

integrin

fibronectin

186
Q

drug used for heparin reversal

A

protamine

187
Q

constitutive active tyrosine kinase lymphoma =

A

CML: BCR-ABL

188
Q

BCRA1 and BCRA1 gene mutations are associated with ___________ and are involved in _______

A

hereditary breast cancer

DNA repair

189
Q

most common opportunistic mycosis (fever, dyspnea, malaise while immunosuppressed) is

show ______ on light microscopy

A

candida albicans

yeasts and pseudohyphae

190
Q

the _______ is the site of ribsomal subunit maturation and assembly and houses ______

A

nucleolus

RNA polymerase I

191
Q

polycythemia vera, essential thrombocytosis, and primary myelofibrosis are the

often have mutations in

A

chronic myeloproliferative disorders

JAK2

192
Q

severe fatigue, HSM, early satiety, bone marrow fibrosis =

A

primary myelofibrosis

193
Q

AL amyloidosis is associated with

A

multiple myelomas

194
Q

DNA synthesis can occur only in the ____ direction

_______ are short stretches of DNA that form the discontinous synthesis of DNA on the lagging strand

A

5 –> 3

Okazaki fragments

195
Q

alcoholic with neutrophils that have hypersegmented nuclei due to ______ deficiency

A

folic acid

196
Q

iron deficiency anemia is associated with _____ ferritin, ________ total iron binding capacity (transferrin) and _______ ______ RBCs

A

decreased

increased

microcytic hypochromic

197
Q

HUS presents with ______ diarrhea, hemolyic anemai with schistocytes, ______, AKI, and elevated ________

A

bloody

thrombocytopenia

elevated serum indirect bilirubin

198
Q

the process of ______ in T cell maturation is essential for eliminating T cells that bind to self MHC or self antigens with high affinity. THis occurs in the _______

A

negative selection

thymic medulla

199
Q

intravascular hemolytic anemias are characterized by decreased____ and increased _______ and _______

A

serum haptoglobin

LDH

bilirubin

200
Q

splenic infarcts in SCD are due to

A

microvascular occlusion

201
Q

monoclonal lymphocytic proliferation is strong evidence of

A

malignancy

202
Q

methotrexate and 5-FU effectively inhibit

A

thymidylate formation

203
Q

heparin induced thrombocytopenia is treat with ______ such as argatroban

A

direct thrombin inhibitors

204
Q

the most common causes of thrombocytopenia in hospitalize patients is

A

heparin

205
Q

back pain not relieved with rest or NSAIDs =

A

metastatic cancer

206
Q

nitrites are oxidizing agents used to treat ____ poisoning by inducing methemoglobinemia during occupational exposure from fumes

A

cyanide

207
Q

Squares on a pedigree are

circles are

A

males

females

208
Q

enlarging jaw mass from east africa =

A

Burkitt lymphoma

209
Q

ristocertin aggregation test will show _______ aggregation of platelets in vW disease

A

decreased

210
Q

Irinotecan and topotecan inhibit

etoposide inhibits

A

topoisomerase I

II

211
Q

conditions that increase RBC turnover rate such as ____ can cause falsely low HbA1c levels

A

Beta thalassemia trait

212
Q

_______ leukemia is a indolent B cell neoplasm found in middle aged men and characterized by bone marrow failure, pancytopenia, and massive splenomegaly. Presents with dry tap, lymphocytes with ____ and TRAP+

A

Hairy cell

cytoplasmic projections

213
Q

BCL2 normally inhibits

A

cell death cascade

214
Q

pure RBC aplaisa is associated with (3)

A

thymoma, lymphocytic leukemias, and parvo B19 infection

215
Q

thrombotic occlusion of microvasculature with skin necrosis following initiation of warfarin therapy due to

A

Protein C deficiency

216
Q

HbS promotes _____ interaction among Hb moleclues and results in HbS polymerization and erythrocyte sickling

A

hydrophobic

(***) (U-World) All Subjects (20 decks)

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