Heme/Onc/Lymph U WORLD Questions Flashcards
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
glioblastoma multiforme
most common
pseudopalisading necrosis
astrocyte
given several units of packed red blood cells, develop tingling and hypocalcemia due to ______chelation by ______ that is added to stored blood
calcium
citrate
exposure status determined then followed during study =
exposure status determined after study =
prospective cohort
retrospective cohort
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
malignancy
Gottron Papules
perimysial infiltrates
ovarian
sickle cell anemia is autosomal _______ inheritance and can be confirmed by __________ electrophoresis. the abnormal hemoglobin is designated _____
recessive
hemoglobin
HbS
radiation therapy induces DNA damage through ______ and formation of free radicals
DNA double strand breaks
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
Vitamin K
LIver
VII
Prothrombin time
VII
______blotting is used to ID proteins. ________ blotting is used to ID RNA sequences. ______ blotting IDs specific DNA sequences in an unknown sample
Western
Northern
Southern
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
polycythemia
Growth factors
vera
JAK2/V617F
EPO
Tumor
physiologic
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 _________
hemolytic
spherocytes
spherocytosis
ankyrin, spectrin
spherocytosis
Mean corpuscular hemoglobin concentration MCHC
HIV pol genes are responsible for _______ to antiretroviral therapy. Env gene mutations enable _____ from host neutralizing antibodies
acquired resistance
escape
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
erythroblastosis fetalis (Rh sensitization)
maternal anti RhD IgG
RhD+
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
CCR5
enter
CD4
CD4 and CCR5
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
basophilic
Auer
peroxidase
AML
all NSAID impair platelet aggregation except ____ which is a _____ selective inhibitor and has no side effects of bleeding and GI ulceration
Celecoxib
COX2
mature erythrocytes lose their ability to synthesize heme when they ______ their ________
lose
mitochondria
folate deficiency inhibits the synthesis of ______, which leads to defective ______ synthesis and megaloblastic anemia. THis can be overcome w/ _______ supplementation
deoxythymidine monophosphate (dTMP)
DNA
Thymidine
tRNA that is mischarged with the incorrect amino acid will _________________ amino acid into the polypeptide chain
incorporate the wrong
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 _______
Sialyl Lewis X
PSGL1
E/P selectin
LFA-1
ICAM-1
PECAM-1
chemotherapy induced emesis can be treated w/ _______ and _______ that act as 5HT3 receptor antagonists
Ondansetron
Granisetron
mTOR pathway = binding of growth factor, autophosphorylation of _____ residues, activation of _______, activation of ______, then activation of _____ which translocates to the nucleus
tyrosine
PIP3
AKT
mTOR
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
Multiple myeloma
Ig or Ig fragments
bortezomib
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
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
Meningococcal meningitis that progresses to DIC results in _______ on peripheral blood smear, which are fragmented erythrocytes
schistocytes
_______ can reverse the toxicity of methotrexate in non cancerous cells of the GI mucosa and bone marrow. This drug is also called ________
Leucovorin
Folinic acid
6-MP is degraded by _____, which can be inhibited by ____. Both 6-MP and 6-thioguanine are prodrugs that require activation by _______
XO
allopurinol
HGPRT
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
G6PD deficiency
Heinz bodies
X linked
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 _______
vincristine, vinblastine
M
peripheral neuropathy (stocking and glove)
dysplasia is ____ change in epithelial cells, which CIS is _______, and invasive carcinoma shows
reversible
irreversible
invasion of basement membrane
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 ________
anemia
thrombocytopenia
hematopoetic cell aplasia
aplastic anemia
EPO
+TdT, CD10, CD19 =
+CD2-8, TdT, CD1a =
precursor B-ALL
precursor T-ALL
anemia, megaloblastic anemia, neuro deficits =
can be due to a ___diet
B12 deficient
strict vegan
female, fatigue, hypochromic, microcytic anemia =
bluish color on RBCs after treatment due to =
iron deficient anemia
reticulocytosis (increased ribosomal RNA)
CD8 T cell mediated apoptosis results in rapid cell death due to activation of ______
caspases
SLE can present in women w/ arthralgias and decreased erythrocytes, leukocytes, and platelets. This is called ______
pancytopenia
HIV infection can cause reactivation of latent _____ and result in ________ lymphomas, including ______ lymphoma
EBV
EBV induced
Burkitt
aspirin is an NSAID that ________ inhibits _______ via acetylation
irreversibly
COX 1 and 2
sickle cell can present with jaundice, lower back pain, extremity pain, and anemia. this results in ____ of the spleen due to repeated ________ infarcts
fibrosis and atrophy
splenic
sickle cell patients have increased __________ requirements due to high RBC turnover. THis can result in ________ anemia as well
folic acid
megaloblastic
industrial worker or child with weakness, cramping abdominal pain due to ________
peripheral blood smear will show
physical exam will show
lead poisoning
coarse basophilic stippling
blue lead lines on teeth and gingiva
carbon monoxide poisoning will ______ carboxyhemoglobin, but will have no effect on _______
increase
partial pressure oxygen
methemoglobin
PCR requires knowledge of the nucleotide sequence of the regions ______ the target exon
flanking
three most common cancer incidence in women are _______ respectively
three most common cancer death in women, respectively
Breast, Lung, colon
Lung, breast, colon
kid w/ hemolytic anemia, jaundice, splenomegaly, spherocytes (lack central zone of pallor) =
hereditary spherocytosis
sickle cell anemia experiencing aplastic crisis is due to infection of erythroid progenitor cells with _____, which is a nonenveloped ________ virus
parvovirus B19
ssDNA
t(14;18) is associated w/ ______ overexpression and _____ lymphoma
BCL2
follicular
anti RhD Ig administered usually at _____weeks
28
erythematous itch breast rash w/ skin texture of orange peel =
caused by cancerous cells ________ drainage
peu d orange, inflammatory breast cancer
obstructing lymphatic
HbF is composed of 2 ____ units and 2 ____ units
Adult hemoglobin is composed of 2 ____ and 2 _____
alpha
gamma
alpha
beta
cutaneous lymph from the umbilicus down, including the anus below the dentate line, drain to the ________ lymph nodes
exceptions are (2)
superficial inguinal
glans penis
posterior calf
t(15;17) is associated w/ ____ and is a translocation involving _______ and the PML gene
often presents with fatigue, easy bruising, and _____
APML
RARA
gum bleeding
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
cyanosis
nitrates
unchanged
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 ______
asplenic
Strep pneumo
H influenza
Salmonella
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 =
primary hemochromatosis
HFE protein, decreased hepcidin synthesis and DMT1 overexpression
Iron overload
liver cirrhosis and hepatocellular carcinoma
newborn with intracranial hemorrhage, “natural” newborn period, born on time w/o complications?
diagnosis if Vitamin K prophylaxis had been given ?
Vitamin K deficiency = impaired clotting factor carboxylation
abusive head trauma = subdural hemorrhage and ICB
monoclonal antibodies that block ______ and ______ help prevent T cell inhibition and promote T cell mediated destruction of tumor cells
used in?
PD-1 and CTLA-4
advanced melanoma
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 =
paroxysmal nocturnal hemoglobinuria (complement mediated hemolytic anemai)
PIGA
hemosiderosis due to iron deposition
hepatocellular carcinoma can be caused by Hepatitis B which is _____ virus, or Hepatitis C which is a ______ virus
DNA
RNA
renal cortical cells sense _____ and release ____
hypoxia
EPO
Rituximab is a monoclonal AB directed against the _____ antigen and used in aggressive lymphomas
CD20
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
Fc
Heavy
M
a reduced ability to relase oxygen within the peripheral tissues results in renal hypoxia and compensatory
erythrocytosis
family history of osteosarcomas, breast cancer, brain tumors, adrenal tumors, leukemias =
gene
Li Fraumeni
TP53, AD, TumorSuppressor
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
acute, intermittent porphyria
alcohol, drugs, or calorie restriction
IV heme preparation
Aminolevulinate synthase
port wine colored, PBG, ALA +
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
H+ and CO2
oxygen unloading
spleen acts as a blood filter capable of removig circulating encapsulated pathogens such as (3)
Strep Pneumo
H Influ
N. Meningitidis
rate limiting enzyme in pentose phosphate pathway
deficient =
interconversion of ribose 5 - phosphate and fructose 6 phosphate is mediated by
G6PD
no NADPH = hemolytic anemia
transketolase
baby african american boy with painful swelling in hands and feet =
due to
lab value
dactylitis
Sickle cell
low haptoglobin
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
leukemoid reaction
Dohle
basophilic
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
tumor lysis syndrome
rasburicase or allopurinol
uric acid
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
MDR1
glycoprotein
chemo
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 ____
antithrombin III
DVT
enoxaparin
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 =
DIC (gram neg. rod infection)
TTP- HUS (fever, neuro, acute renal failure, thrombocytopenia, and microangiopathic hemolytic anemia)
vWF disease
high peak gamma globulin region on serum protein electrophoresis w/ M protein =
presents w/
multiple myeloma
anemia, lytic bone lesions, renal insufficiency, hypercalcemia, amyloid deposition
HbS aggregates in a _____ state, low ____, and high ______ state
deoxygenated
pH
2.3 BPG
Mediterranean, mild anemia, microcytic, increased hemoglobin A2, hypochromia, poikilocytes (spherocytes + target cells), asymptomatic =
caused by mutations in
beta thalassemia minor
defective transcription, processing, and translation of betaglobin mRNA
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??
hereditary spherocytosis
splenectomy
pigmented gallstones, aplastic crises from parvovirus B19
CD4 virus
CD21 virus
Erythrocyte P antigen
HIV gp120
EBV gp 350
Parvovirus B19
glutamic acid to valine substition at position 6 in beta globin gene =
missense mutation
sickle cell
GAG to GTG in DNA strand
warfarin inhibits ______ which can lead to skin and fat necrosis seen in the first few days of warfarin therapy
protein C and S
high dose opioid treatments in metastatic cancer commonly require _______ and _____ in order to maintain bowel regularity
adequate fluid intake and daily laxatives
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
CDK4
Rb
new onset neurologic symptoms, hemolytic anemia w/ schistocytes, thrombocytopenia, acute kidney injury =
deficiency of ____ leads to large uncleaved cWF multimers
treat with
thrombotic thrombocytopenic purpura
ADAMTS13
plasma exchange therapy
metalloproteinases are zinc containing enzymes that _____ the _______ of the basement membrane, allowing tumor cell invasion
degrade
ECM
extramedullary hematopoiesis in a child causes a range of skeletal problems such as _______ and is due to ______ such as B thalassemia major
maxillary overgrowth (chipmunk facies)
chronic hemolytic anemia
large, binucleated cells w/ an owls eye appearance are called _____ cells and are diagnostic of _____
Reed Steinberg
Hodgkin lymphoma
excessive bruising since childhood with abnormal platelet aggregometry and a defect in platelet glycoprotein surface receptor =
normally binds =
inheritence
treated w/
Glanzmann thrombasthenia
fibrinogen
AR defect in GP IIB/IIIa
abciximab
isotype switching occurs in the ______ of lymph nodes and requires interaction of the _______ receptor on B cells with the ______ ligand on activated T cells
germinal centers
CD40
CD40
hepcidin is synthesized by ________ and acts as the central regulator of _______ homeostasis
hepatic parenchymal cells
iron
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
mycoplasma pneumoniae
immune response
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?
Plasmodium falciparum malaria
trophozoites
chloroquines: mefloquine for resistant bugs
primaquine due to dormant liver forms
sudden onset abdominal pain, ascites, anemia, reticulocytosis, thrombocytopenia, CD55 and 59, DVT or HVT =
gene =
CD55 and 59 role
paroxysmal nocturnal hemoglobinuria
PIGA
complement inhibitors: disease due to complement activation
eczema, recurrent infections, thrombocytopenia with petechiae, purpura, epistaxis in a child, B and T lymphocyte deficiency =
inheritance =
susceptible to =
treatment is =
Wiskott-Aldrich
X linked
encapsulated organisms
HLA matched bone marrow transplant
mutation at RCC before AUG - methionine start codon leads to a block at
initiation of translation
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
microcytic anemia
alpha
damage
DVT, given a drug that increases PT and aPTT but does not effect thrombin time, what drugs (2)?
Mechanism?
drugs that prolong thrombin time?
apixaban, rivaroxaban
direct factor Xa inhibitors
unfractionated heparin, direct thrombin inhibitors (dabigatran)
______ is the mitochondrial enzyme that activates caspases and indirectly brings about cell death through intrinsic pathway apoptosis
cytochrome C
young african american boy, exertional dyspnea, acute chest syndrome, bone pain =
mutation
sickle cell anemia
point mutation : valine substituted for glutamic acid
chemotherapy, followed by progressive hematuria and suprapubic tenderness due to _______
causative agent is nitrogen mustards such as
metabolized by the kidneys into
prevented by
hemorrhagic cystitis
cyclophosphamide, ifosfamide
acrolein
mesna and sulfhydrl compound
black pigmented stones with low cholesterol content arise from conditions that increase _________ in bile such as _______ in sickle cell, thalassemia, and hereditary spherocytosis
unconjugated bilirubin
chronic hemolysis
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
Tissue Factor
Placenta
the change in color of bruise from dark blue to greenish in color is caused by activity of what enzyme?
heme oxygenased: heme to biliverdin (green)
persistent, fluctuating LAD who may have had an unrelated pharyngitis treated with antibiotics on initial presentation most likely has
translocation
follicular lymphoma
t(14;18)
angiogenesis of a hepatic hemangioma is primarily driven by ______ and ______
VEGF and FGF2
pyruvate kinase deficiency causes ______ anemia, and splenic ______ results from increased work of the splenic parenchyma to remove the deformed RBCs from cirulation
hemolytic
red pulp hyperplasia
night sweats, weight loss, cough, apical pulmonary granulomas =
cells that cause lesions =
surface marker
secondary tuberculosis
macrophages
CD14
fresh frozen plasma rapidly reverses ______ effects whereas vitamin K requires time for clotting factor re synthesis
warfarins
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 ______
HbF
Sickle cell anemia
Gardos
dehydration
HbS
different mRNA sizes, same gene, due to
alternative splicing
recurrent epigastric pain, hepatomegaly, macrocytic anemia, pancreatic calcifications =
anemia is due to
chronic alcoholic pancreatitis
diminished thymidine synthesis: megaloblastosis
carbon tetrachloride poisoning causes fatty change and hepatocyte necorsis via _____ injury
free radical
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
HbC
missense
lysine
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
polycythemia vera
JAK2
individuals who demonstrate increased activity of ______ are more susceptible to developing chemical induced cancers
P450 cytochrome microsomal oxygenase
young female with new onset of thrombocytopenia, ecchymoses, petechiae, mucosal bleeding, and no other obvious causes =
due to
Immune thrombocytopenic purpura
autoimmune destruction of platelets
child with cystic fibrosis, seizure, and confirmed intracranial hemorrhage due to decreased _______ as a result of ________ deficiency, a fat soluble vitamin
coagulation factors affected
gamma carboxylation
Vitamin K
II, VII, IX, X
hypochromic, microcytic anemia is most commonly due to ________, especially in the presence of _______ loss
iron deficiency
occult blood
venous thromboembolism arises due to the ______ triad of __________, _________. and ________
virchow
endothelial injury
venous stasis
hypercoaguable state
cytokeratin is a commonly used immunohistochemical marker of _____ cell carcinomas
epithelial
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 _______
transmembrane
tyrosine kinase
epidermal growth factor receptors
trastuzumab
treatment using a double stranded RNA molecule consisting of 20-30 base pairs =
interferes w/ =
small interfering RNA and microRNA
mRNA translation
tumors harboring activating mutations in _____ are resistant to chemotherapy with _______ such as cetuximab and panitumumab
KRAS
anti EGFR drugs
rivaroxaban, apixaban =
fondaparinux =
argatroban, bivalirudin, and dabigatran =
DIrect Factor Xa inhibitors
INidrect Factor Xa inhibitor
Direct Thrombin inhibitors
many transfusions, frontal bossing, HSM, jaundice, liver biopsy showing kupffer cells containing coarse, yellowish brown cytoplasmic granules composed of_____. This is due to______
hemosiderin
iron overload from transfusion therapy
cachexia and muscle wasting are due to _____ in a paraneoplastic setting
TNF-alpha
prognosis of colorectal adenocarcinoma is directly related to __________, not the ________
stage of invasion
anaplastic grade
fever, pharyngitis, LAD in young adult is suggestive of _________
caused by?
associated with
infectious mononucleosis
EBV
nasopharyngeal carcinoma
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
CP450
warfarin
inhibit warfarin
people with down syndrome are at an increased risk of developing
ALL and AML
if chemotherapy induced emesis is not refractory after -setron use, _______ tend to be effective. The drugs in this category are (2)
neurokinin 1 receptor antagonists
aprepitant, fosaprepitant
recurrent infections, pallor (anemia), and ecchymoses with a peripheral blood smear that shows cells with Auer rods =
translocation
APML M3
t15;17 RARa/PML
aggregates of packed follicles, LAD in the cervical area =
translocation
effect
follicular lymphoma
t14;18
BCL-2
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 ________ ______
3rd and 4th
lymph node paracortex
____ 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 _______
CML
elevated
decreased
myelocytes
philidelphia chromosome, BCR-ABL, t(9;22)
cells that express both CD4 and CD8 are classified as
immature cortical T cells
fibrinolytics such as _____ may cause reperfusion arrhythmias that are benign during arterial reopening.
tPA, reteplase, tenecteplase
erythrocytes use ______ as the major pathway to generate energy as they do not have ______
glycolysis
mitochondria
________ 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
Cushing
oat cell
ACTH
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 ___________
isoniazid
pyridoxine
delta aminolevulinate synthase
sideroblastic anemia
atrophic gastritis can result in _______ and elevated methymalonic acid levels. The _______ count increases dramatically once ______ replacement therapy is initiated, but normalize quickly
vitamin b12 deficiecy due to inadequate intrinsic factor production
reticulocyte
b12 replacement
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)
sulfonamides
antimalarials
antibiotics
neuro deficit and hypersegmented neutrophils =
no neuro deficits but still megaloblastic anemia
B12
folate
desmopressin can alleviate bleeding through _______ release of _______
endothelial
vWF
only ________ heparin is able to bind both antithrombin and thrombin to allow antithrombin to inactivate thrombin.
unfractionated
excessive bleeding + hemarthroses =
inheritence =
phenotypically normal parents, probability of a female sibling with give birth to affected child is
hemophilia A or B
X recessive
1/8
somnolence, lethargy, oliguria, diarrhea that become blood, elevated blood urea nitrogen and creatinine, peripheral blood smear show fragmented erythrocytes =
due to =
HUS
EHEC from undercooked beef
_________ can lead to both dose dependent cytopenia and dose independent aplastic anemia
chlorophenicol
flushing, watery diarrhea, bronchospasm, elevated 5-HIAA =
due to =
carcinoid syndrome
carcinoid metastatic to liver
pancytopenia post infection with no LAD or splenomegaly =
infectious causes
diangosis
aplastic anemia
EBV, parvo B19
hypocellular bone marrow
individual subunits of hemoglobin similar to _______ and shows a ______ shift on dissociation curve
myoglobin
left
Kaposis sarcoma presents with a blue violet or brownish skin plaque in ______ + patients and is due to _________
HIV
HHV8
______________ which is modified to resist activated protein C and results in a hypercoaguable state and predisposes to DVT
Factor V Leiden
hypophosphorylated retinoblastoma protein is most likely to
prevent G1/S cell cycle transition
___________ 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
acute intermittent porphyria
ALA synthase
________ lymphoma is characterized by starry sky microscopic appearance. Translocation of _______ on t____, and functions as a trascription activator
Burkitts
C-myc
8;14
bilobed or double nuclei and inclusion like eosinophilic nucleoli =
diagnostic of
Reed-Sternberg cell
Hodgkin lymphoma
kid with pancytopenia, HSM, distended macrophages with wrinkled tissue paper appearance =
inheritence
deficiency in
Gacher disease
autosomal recessive
B glucocerebrosidases
Beta globin with impaired ionic interaction between beta subunit and 2,3 bisphosphoglycerate is most similar to
HbF
patients with sickle cell trait have relative protection from _______
malaria caused by plasmodium falciparum
carbon monoxide ________ binds to hemoglobin with a much greater affinity than oxygen
competitively
Ig___ cannot cross placenta, while Ig___ can
IgM
IgG
undifferentiated (anaplasti) tumors bear ______ to tissue of origin
no resemblence
abnormal ______ is seen in patients with chronic ____ and ________
bleeding time
kidney disease
uremia
tumor with cystic and solid component in cerebellum in a child is most lilkely
a completely solid tumor is most likely =
pilocytic astrocytoma
medulloblastoma
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 _________
osteoblastic, osteolytic
osteoblastic
osteolytic
osteoblastic
mixed
HER2 is a _______ kinase receptor
tyrosine
sore throat, malaise, LAD, myalgias, splenomegaly in young =
cell that attacks EBV in IM?
IM
CD8 CTL activated
patients with ______ present with lifelong mucosal bleeding, heavy menstruation, normal platelet levels, but prolonged bleeding time due to impaired platelet functioning
von Willebrand disease
von Willebrand factor promotes ________ by binding to and crosslinking __________ with exposed ______. It also functions as a protective carrier protein for ______
platelet adhesion
platelet glycoproteins GpIb
collagen
factor VIII
prostate cancer is usually _______ dependent. In combination with long acting gonadotropin releasing hormone agonsts, ________ is added and acts as a ___________ of testosterone receptors
testosterone dependent
Flutamide
competitive inhibitor
ALL is the most common malignancy of childhood. _____ ALL often presents with a _______ that can cause respiratory symptoms, dysphagia, and SVC syndrome
T cell
mediastinal mass
_________, 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 ______
schistocytes
microangiopathic hemolytic anemia
bloody diarrhea
HUS, TTP
DIC
___________ 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
porphyria cutanea tarda - late step porphyria
uroporphyringoen decarboxylase (UROD)
alchol
_______ prevents chromosomal shortening
telomerase
____________ syndrome is characterized by dysphagia from ________ formation and ________ deficiency anemia. Patients also exhibit koilonychia or spoon shaped nails and a shiny red tongue
Plummer Vinson
esophageal web
iron
prolonged bleeding and hemoarthosis =
desmospressin can be used for treatment of mild hemophilia A by increasing the circulating level of ______
hemophilia
Factor VIII
methotrexate competitively inhibits ______, which causes this intermediate to accumulate intracellularly
dihydrofolate reducatse
dihydrofolate
G6PD presents in a similar way to __________ deficiency
glutathione reductase
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 _____
X linked agammaglobulinemia
T cell function
germinal centers
bruton tyr kinase
__________ is a significant adverse of effect of ganciclovir and is increased with co adminstration of zidovudine or TMP SMX
neutropenia
Auer rods are found in blast cells of ______ but not in _____. More mature cells and fewer blasts are found in ______
AML
ALL
CML
HIV with diffuse medium sized lymphocytes with basophilic cytoplasm and high proliferation index (high Ki67 fraction) =
due to =
translocation
burkitt lymphoma
EBV
t8;14 c-myc
hemophilia due to decreased levels of ______ or _______. The addition of _______ to the blood of a patient with hemophilia results in clotting
factor VIII
Factor IX
thrombin
painless gross hematuria with lack of other symptoms =
usual form?
most likely prognostic factor?
bladder cancer
urothelial (transitional cell) carcinoma
depth of invasion into the muscular layer
adhesion of cells to the ECM involves _______ mediated binding to ________, collagen, and laminin
integrin
fibronectin
drug used for heparin reversal
protamine
constitutive active tyrosine kinase lymphoma =
CML: BCR-ABL
BCRA1 and BCRA1 gene mutations are associated with ___________ and are involved in _______
hereditary breast cancer
DNA repair
most common opportunistic mycosis (fever, dyspnea, malaise while immunosuppressed) is
show ______ on light microscopy
candida albicans
yeasts and pseudohyphae
the _______ is the site of ribsomal subunit maturation and assembly and houses ______
nucleolus
RNA polymerase I
polycythemia vera, essential thrombocytosis, and primary myelofibrosis are the
often have mutations in
chronic myeloproliferative disorders
JAK2
severe fatigue, HSM, early satiety, bone marrow fibrosis =
primary myelofibrosis
AL amyloidosis is associated with
multiple myelomas
DNA synthesis can occur only in the ____ direction
_______ are short stretches of DNA that form the discontinous synthesis of DNA on the lagging strand
5 –> 3
Okazaki fragments
alcoholic with neutrophils that have hypersegmented nuclei due to ______ deficiency
folic acid
iron deficiency anemia is associated with _____ ferritin, ________ total iron binding capacity (transferrin) and _______ ______ RBCs
decreased
increased
microcytic hypochromic
HUS presents with ______ diarrhea, hemolyic anemai with schistocytes, ______, AKI, and elevated ________
bloody
thrombocytopenia
elevated serum indirect bilirubin
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 _______
negative selection
thymic medulla
intravascular hemolytic anemias are characterized by decreased____ and increased _______ and _______
serum haptoglobin
LDH
bilirubin
splenic infarcts in SCD are due to
microvascular occlusion
monoclonal lymphocytic proliferation is strong evidence of
malignancy
methotrexate and 5-FU effectively inhibit
thymidylate formation
heparin induced thrombocytopenia is treat with ______ such as argatroban
direct thrombin inhibitors
the most common causes of thrombocytopenia in hospitalize patients is
heparin
back pain not relieved with rest or NSAIDs =
metastatic cancer
nitrites are oxidizing agents used to treat ____ poisoning by inducing methemoglobinemia during occupational exposure from fumes
cyanide
Squares on a pedigree are
circles are
males
females
enlarging jaw mass from east africa =
Burkitt lymphoma
ristocertin aggregation test will show _______ aggregation of platelets in vW disease
decreased
Irinotecan and topotecan inhibit
etoposide inhibits
topoisomerase I
II
conditions that increase RBC turnover rate such as ____ can cause falsely low HbA1c levels
Beta thalassemia trait
_______ 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+
Hairy cell
cytoplasmic projections
BCL2 normally inhibits
cell death cascade
pure RBC aplaisa is associated with (3)
thymoma, lymphocytic leukemias, and parvo B19 infection
thrombotic occlusion of microvasculature with skin necrosis following initiation of warfarin therapy due to
Protein C deficiency
HbS promotes _____ interaction among Hb moleclues and results in HbS polymerization and erythrocyte sickling
hydrophobic
