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176 Cards in this Set

  • Front
  • Back
which pathway does heparin affect?
intrinsic pathway
which pathway does warfarin affect?
defect in hemophilia A
decreased factor VIII
defect in hemophilia B
decreased factor IX
coag parameter prolonged in hemophilia?
what can be given in mild hemophilia A before minor surgical procedures?
DDAVP, which can increase vWF which increases factor 8
what is the most common hereditary bleeding disorder?
von willebrand's disease
how is von willebrand's inherited?
autosomal dominant
what is the role of vWF
stabilizes factor 8 and enhances platelet aggregation/attachment to injured vascular endothelium
how is bleeding time affected in hemophilia?
it is normal ecause no platelet function abnormality exists
what coagulation parameters are increased in von willebrand's?
PTT and bleeding time
how does heparin work?
catalyzes activation of antithrombin III
how does warfarin work?
interferes with sythesis of vitamin K dependent cofactors
what lab values are decreased in TTP?
platelets and RBCs
test that measures the ability of vWF to agglutinate platelets in the presence of ristocetin
ristocetin cofactor assay
treatment for major bleeding in pt with von willebrands
FFP or cryoprecipitate
what drug can be effective in mild von willebrand's
what is Evan's syndrome?
ITP with autoimmune hemolytic anemia
peak incidence of Evan's syndrome?
20-50 years
pathophys in ITP
IgG autoantibody binds platelets, which causes lysis by splenic macrophages
drug associated with ITP in adults?
test for ITP?
platelet-associated IgG test
what does blood smear show in ITP
megathrombocytes without schistocytes
what should you consider before giving prednisone to a patient with ITP
bone marrow aspiration because it can mask a leukemia
most common inherited cause of hypercoagulability
factor V leiden
presence of blast cells
acute leukemia
most common leukemia in pts <15
most common leukemias in pts 15-59
most common leukemias in pts >60
ALL and philadelphia chromosome is associated with what type of prognosis
philadelphia chromosome
what do blasts stain for in ALL
what disorder is ALL associated with?
down syndrome
which type of AML is associated with DIC?
which type of AML is associated with gingival hyperplasia?
how does AML typically present?
fatigue, hemorrhage, bruising
patients with high WBC and AML may present with what?
leukostasis, which arises from sludging of circulating leukemic blasts in tissue microvasculature
what is pathognomonic for AML
auer rods
eosinophilic needle-like cytoplasmic inclusions
auer rods, found in blast cells
positive stains in AML
sudan black and myeloperoxidase (10%)
AML M3 - translocation involving retinoic receptor alpha and PML genes
chemo for AML
cytarabine plus daunorubicin
what in addition to chemo is M3 type of AML treated with?
all-trans-retinoic acid
CLL is almost always a clonal malignancy of what type of cells?
B cells
median age of onset of CLL?
smudge cells
coexpression of CD19 and CD5 on flow
hypogammaglobulinemia is found in 50% of cases of what leukemia?
indications for chemo in CLL?
progressive fatigue, symptomatic lymphadenopathy, or anemia/thrombocytopenia
first-line therapy for CLL?
fludarabine - avoid in hemolytic anemia
what is Richter's syndrome?
isolated LN transformation into aggressive large cell lymphoma (seen in CLL)
approximately 90% of patients with CML have what?
phases of CML
chronic, accelerated, blast
lab abnormalities in tumor lysis syndrome
increased K, increased phos, increased uric acid, decreased Ca2+
back pain, anemia, renal insufficency
multiple myeloma
what types of infections do pts with multiple myeloma tend to get?
encapsulated organisms (strep pneumo, h flu)
age distribution of Hodgkins
bimodal; 20-30, then >50
most common form of Hodgkin's
nodular sclerosis - primarily affects young females
free kappa and lambda chains on SPEP/UPEP
Bence Jones proteinuria (multiple myeloma)
xray findings in multiple myeloma
lytic lesions, esp in axial skeleton, osteoporosis and fractures
treatment for multiple myeloma
few symptoms: thalidomide; chemo for pts who are refractory to thalidomide or who are acutely symptomatic
M protein without evidence of systemic disease
IgM spike, hyperviscosity, cold agglutinins
waldenstroms macroglobulinemia
G6PD deficiency, membranopathy
blister cell
G6PD deficiency
burr cell
ARF, uremia
heinz body
thalassemia, hemoglobinopathies, enzymopathies
artificial heart valves and microangiopathic hemolytic anemias
target cell
most common metabolic disorder of RBCs
G6PD deficiency
drugs that can precipitate hemolysis in pts with G6PD deficiency
antimalarials, quinolones, sulfa drugs, NSAIDs, nitrofurantoin, fava beans, vitamin C
decreased iron, increased TIBC, decreased ferritin
iron deficiency anemia
decreased iron, decreased TIBC, normal/increased ferritin
anemia of chronic disease
increased iron, normal to increased TIBC, increased ferritin in pt with microcytic anemia
sideroblastic anemia
treatment for sideroblastic anemia
pyridoxine (vit B6)
levels to check in pts with suspected thalassemia
HbA2, HbF
how does G6PD present
jaundice, dark urine, ATN 1-3 days after exposure
pentad of TTP
renal dysfunction
neurologic abnormalities
acute chest syndrome
chest pain, pulmonary infiltrate, increased WBC, hypoxia (indistinguishable from PNA)
what is aplastic crisis often due to in sickle cell?
parvovirus B19
what is hemolytic crisis in sickle cell associated with?
G6PD deficiency
anemia, thrombocytopenia following mild viral illness or gastroenteritis with E. coli O157:H7
LDH level in HUS
causes of TTP
HIV, pregnancy, OCP use
normal, but bleeding time is increased
treatment for TTP
large volume plasmapheresis, corticosteroids, ASA, splenectomy
causes of DIC
sepsis, transfusion reaction, neoplasia, trauma, obstetric complications (amniotic embolus and septic abortion)
coag parameters affected in DIC
increased PT/PTT, and bleeding time
fibrinogen level in DIC
Howell-Jolly bodies
cytoplasmic remnants of nuclear chromatin normally removed by spleen seen in sickle cell disease
vaccines for patients with sickle cell
h flu and pneumococcal
penicillin prophylaxis for sickle cell patients under what age
defect in sickle cell
defect in beta globulin chain
defect in thalassemia
decreased quantity of alpha or beta globulin chain
defect in porphyria
defect in heme synthesis
what does electrophoresis show in thalassemia?
increased HbF, decreased HbA, and increased HbA2
treatment for alpha thal
periodic transfusions
treatment for B thal major
aggressive transfusions, splenectomy to enhance RBC survival; BMT; ddeferoxamine
treatment for B thal minor
avoid oxidative stress
secondary causes of polythycemia
hypxia - COPD, lung disease, smoking, high altitude, RCC
red face and pruritis after warm shower
epo level in polycythemia vera
what does p vera increase the risk of?
conversion to CML, myelofibrosis, or AML
most frequent cause of transfusion reaction
ABO incompatibility due to clerical error
what can hemoglobinuria associated with transfusion reaction lead to?
ATN, olguria, renal failure
what do patients with IgA deficiency have a reaction to with a transfusion
donor IgA
pathophys of acute transplant eaction
cytotoxic T cells react against foreign MHCs
how does GVHD often present
painful pruritic maculopapular rash, hepatic dysfunction, diarrhea
hypersegmented neutrophils
folate/B12 deficiency
basophilic stippling
lead poisoning
bite cells
G6PD; hemolytic anemia
teardrop-shaped RBCs
schistocytes, helmet cells, fragmented RBCs
intravascular hemolysis
acanthocytes and spur cells
from reticulocytosis; alerts to possibility of hemolysis
rouleax formation
multiple myeloma
iron inclusions inside RBCs of bone marrow
sideroblastic anemia
extramedullary hematopoeisis
crew cut skull
eophageal webs, iron deficiency anemia, glossitis
plummer vinson syndrome
anticonvulsant causing folate deficiency
classically phenytoin
neurologic deficiencies in B12 deficiency
loss of sensation and position sense, paresthesias, ataxia, spasticity, hyperreflexia, positive babinski, dementia
polychromatophilic stippling
sideroblastic anemia
increased MCHC
drugs known to cause aplastic anemia
chloramphenicol, carbamazepine, phenylbutazone, sulfa drugs, zidovudine, gold
what is myelophthisic anemia?
space-occupying lesion in bone marrow
blood product for IgA deficiency
washed red blood cells
what is the most common cancer in most organs?
metastatic CA
treatment for spinal cord compression?
steroids, MRI, radiation
edema and redness of neck, face and CNS symptoms
SVC syndrome
lung cancer associated with Cushing's
small cell carcinoma
lung cancer and PTHrP
squamous cell
lung cancer and SIADH
small cell
what is Eaton-Lambert syndrome?
myasthenia gravis-like disease from lung cancer that spares ocular muscles
lung cancer associated with Eaton-Lambert syndrome?
small cell carcinoma
most common histological type of breast cancer?
invasive ductal carcinoma
classic tumor marker for colon CA?
whipple's triad
hypoglycemia (<50), CNS symptoms due to hypoglycemia, and improvement with administration of glucose
what do glucagonomas cause
hyperglycemia with high glucagon and migratory necrotizing skin erythema
what do VIPomas cause?
watery diarrhea, hypokalemia, achlorhydria
most common type of ovarian CA
serous cystadenoma
ovarian tumor that causes virilization
sertoli-leydig cell
ovarian tumor that causes femininization and precocious puberty
granulosa/theca-cell (classically present <30)
what is Meigs' syndrome
ovarian fibroma that causes ascites and right hydrothorax
what cancers do OCPs reduce the risk of?
ovarian and endometrial
where does cervical cancer begin?
transformation zone
which is more common in alcoholics: folate or B12 deficiency
what is associated with a poor prognosis in CLL?
treatment for hairy cell leukemia
treatement for CLL
chlorambucin and prednisone
treatment for non-Hodgkins
testicular cancer associated wtih elevated AFP
embryonal cell CA
testicular cancer associated with elevated B-HCG
with what complication is HIT associated?
arterial thrombosis
type of anemia caused by lead poisoning
hypochromic, microcytic
drugs that can cause folic acid deficiency
phenytoin, TMP, MTX
treatment for patient with familial colonic polyposis
proctocolectomy at diagnosis
treatment for pregnant patient with antiphospholipid antibody
heparin and ASA
cancers most likely to metastasize to brain
lung, breast, melanoma
what tumor should you suspect in child wtih intracranial calcifications on skull radiograph
what tumor resembles a bunch of grapes coming out of the vagina
sarcoma botryoids, type of embryonal rhabdomyosarcoma usually seen in kids
who does nasopharyngeal cancer affect? what is is associated with?
asians; EBV
increased calcitonin and thyroid cancer
medullary thyroid cancer
most common primary tumor of the liver
benign liver tumor in women of reproductive age who take birth control pills
hepatic adenoma
liver cancer associated with IBD and liver flukes (clonorchis)
liver cancer and exposure to vinyl chloride
most common primary liver malignancy in children
rash that doesn't respond to multiple treatments in an HIV+ patient
Kaposi's sarcoma
vascular skin tumor that commonly begins as a papule or plaque on the upper body or in the oral cavity; highly associated with HHV-8
Kaposi's sarcoma
how can you differentiate Wilms' tumor from neuroblastoma?
neuroblastoma -adrenal gland, calcifications
wilms tumor- kidney, no calcifications
two major cytologic clues for histiocytosis
CD1 positive cells and birbeck granules
cytoplasmic inclusion bodies that look like tennis rackets
birbeck granules
expansile, lytic, well-demarcated benign lesion in proximal portion of the humerus in children and adolescents
unicameral bone cyst
how does retinoblastoma classically present?
child less than 3 with leukocoria (red reflex changes to white) and/or unilateral exophthalmos