Heme/Onc & Preventive Med Flashcards
How do you test for estrogen /progesterone receptors or HER 2/neu to dx breast cancer?
must do a core needle bx OR open bx, which allows for frozen section to be done while patient is in the OR and immediate resection of the cancer followed by sentinel node bx; CANNOT DO RECEPTOR TESTING FROM FNA
Mammography guidelines
start at age 50, do every 2 years, stop at 75
When is breast U/S the right answer?
when dealing with an indeterminate mass lesions (need to determine whether it is cystic or solid), it is painful, and varies in size/pain with menstruation
When is breast PET scar the right answer?
when you need to determine the content of abnormal LN’s that are not easily accessible to bx
BRCA is asso with which cancers?
breast, ovarian, and pancreatic cancer
Has BRCA been shown to add mortality benefit to usual mgmt?
No
What is the sentinel node?
first node identified in the operative field of a definitively identified breast cancer; a negative sentinel node eliminates the need for axillary LN dissection
Main tx for breast cancer
lumpectomy and radiation
Role of radiation in breast cancer tx
prevents recurrences
Tamoxifen, who gets it, what it does, s/e’s
give to PREmenopausal women who are hormone positive, use when multiple 1st-degree relatives have breast cancer as it lower risk (prophylactically); risk of endometrial cancer and forming clots
Aromatase inhibitors, who gets it, what it does, s/e’s
give to POSTmenopausal women who are hormone positive, most likely to benefit the patient (vs SERMs), very useful in preventing metastasis in those with proven breast cancer; risk of osteoporosis
Trastuzumab, who gets it, what it does, s/e’s
give to women who test HER 2/neu positive, decreases risk of recurrent disease, increases survival
When to give adjuvant chemo in breast cancer?
if there is suspicion of microscopic cancer cells too small to be detected; give when lesion >1 cm and positive axillary LNs
Complications of prostatectomy and radiation
Prostatectomy - erectile dysfunction, urinary incontinence
Radiation - diarrhea
Hormonal manipulation in prostate cancer, agents
flutamide (androgen antagonist), GNRH agonists, ketoconazole, orchiectomy to help control size; do no prevent recurrences
Role of chemo in prostate cancer
only used when hormonal thx does not work
Get transrectal U/S to screen for prostate cancer only if
elevated PSA and no palpable mass; once mass identified - bx
> 50 yo F with increasing abdominal girth but is still losing wt
ovarian cancer
CA 125
ovarian cancer
Tx of ovarian cancer
remove all visible tumor and pelvic organs and give chemo
alpha-feto protein in pt with testicular mass or concerns for testicular cancer
non-seminoma
testicular cancer that is sensitive to chemo
non-seminoma
testicular cancer that is sensitive to chemo and radiation
seminoma
tx of testicular cancer after orchiectomy
local disease –> radiation
metastasis –> chemo
contraindication to 5HT inhibitors (e.g. nausea medication)
QT prolongation on ECG
what do you give a patient with chemo-induced nausea who has QT prolongation on ECG?
NK receptor antagonists (aprepitant)
colon cancer epidemiology
lifetime risk: 6-8%, number of deaths per year: 50,000, percentage of deaths that would’ve been preventable with screening: 95%
who gets pneumococcal vaccine?
everyone above 65, cochlear implant, CSF leak, alcoholic, tobacco smokers
Hep A postexposure ppx
between 12 mo and 40 years - vaccine (single dose)
<12 mo, >40 yrs - immune globulin
immunological response to different pneumococcal vaccines
23 valent - capsular polysaccharide - T-cell-independent B cell response
13 valent - conjugated capsular polysaccharide - T-cell dependent response
who diagnoses alcoholism?
the patient
patient with signs of pancytopenia (fatigue, infection, bleeding) with normal WBC and blood smear showing blasts
Acute leukemia
leukemia asso with DIC
Acute promyelocytic leukemia (M3)
leukemia with chromosome 15 to 17 translocation
Acute promyelocytic leukemia (M3)
Myeloperoxidase + Auer rods
Acute myelocytic leukemia / Auer rods are more common in acute promyelocytic leukemia (M3)
best indicator of prognosis in acute leukemia is…
cytogenetics - assessing specific chromosomal characteristics found in each patien
good cytogenetics –> chemo
poor cytogenetics –> greater chance of relapse –> BMT
tx of M3
ATRA (all trans retinoid acid)
what do you have to watch out for in ALL?
CNS relapse - add intrathecal methotrexate to thx
prevents tumor lysis related rise in uric acid
rasburicase
patient with persistently high WBC that is all neutrophils, may have aquagenic pruritus, splenomegaly (with early satiety, abdominal fullness, LUQ pain), hypermetabolic syndrome (fatigue, fever, night sweats)
Chronic myelogenous leukemia (must differentiate from a leukemoid reaction; leukemoid reaction is FAP positive)
Dx of CML
BCR-ABL = 9:22 translocation = Philadelphia chromosome
if CML is untreated, 20% of patients will develop what disorder?
blast crisis (acute leukemia)
tx of CML
imatinib; only BMT can cure CML but should never be 1st thx
priority in leukostasis reaction (extremely elevated WBC typically in patient with AML or CML presenting with sxs of decreased tissue perfusion)
leukaphersis - removal of excessive WBCs
myelodysplastic syndrome definition
preleukemic disorder presenting in older patients with a pancytopenia despite a hyper cellular bone marrow; most patient never develop AML because complication of infection and bleeding lead to death before leukemia occurs
myelodysplastic syndrome diagnosis and tx
asymptomatic pancytopenia on CBC, hypercellular BM, Pelget-Huet cells, 5q deletion
tx with transfusion, EPO, azacitidine (decreases transfusion presence and increases survival), lenalidomide (esp in those with 5q deletion), BMT in <50
pt (typically asx or may present with fatigue) with increased WBC which are mostly lymphocytes; dx with flow cytometry
chronic lymphocytic leukemia
CLL sxs
fatigue, LAD, hepatosplenomegaly, infection, hemolysis
diagnosis of CLL
increased WBC (usually >20,000), 80-90% lymphocytes, hypogammaglobulinemia, may present with anemia and thrombocytopenia (d/t BM infiltration or autoimmune warm IgG antibodies)
what is Richter phenomenon?
conversion of CLL to high-grade lymphoma, happens in 5% of patients
staging and tx of CLL
stage 0 (high WBC), stage 1 (LAD), and stage 2 (hepatosplenomegaly) - no treatment
stage 3 (anemia) and stage 4 (thrombocytopenia) - FLUDarabine, cyclophosphamide, RITUXimab (anti CD 20)
if FLUDarabine fails, use ALEMTUZUMAB (anti CD 52)
mild case + elderly –> CHLORambucil
autoimmune hemolysis or thrombocytopenia –> steroids
give PCP ppx
Hairy cell leukemia story
All the old (mature/Hairy) B cells left the BM (“dry tap”) and got TRAPped (TRAP positive) in the spleen (massive splenomegaly) - tx with CLADRIBINE or PENTOSTATIN
Non-Hodgkin Lymphoma definition and presentation
a proliferation of lymphocytes in the lymph nodes and spleen, can affect any organ that has lymphoid tissue, presents with painless LAD, may involve pelvic/retroperitoneal/mesenteric structures, B sxs, most patients present with widespread disease (stage III and IV)
B sxs
fever, weight loss, drenching night sweats
Non-Hodgkin Lymphoma dx and staging
dx: excisional bx (take the whole LN out), LDH levels correlate with severity, staging determines intensity of thx, staging procedures involve CT scan of chest/abdomen/pelvis and BM bx
staging: stage 1 - LN group stage 2 - >/= 2 LN groups / same side of diaphragm stage 3 - both sides of diaphragm stage 4 - widespread disease
Non-Hodgkin Lymphoma tx
local disease (stage I and II) - local radiation and small dose/course of chemo advanced disease (stage III and IV, B sxs) - chemo with CHOP and rituximab
C - cyclophosphamide
H - adriamycin (doxorubicin)
O - vincristine
P - prednisone
CHOP
C - cyclophosphamide
H - adriamycin (doxorubicin)
O - vincristine
P - prednisone
tx of Non-Hodgkin Lymphoma
MALT tx
Tx Helicobacter with clarithromycin and amoxicillin
NHLs with worst prognosis
Burkitt and immunoblastic
Hodgkin disease features
almost the same as NHL, but Reed-Sternberg cells on pathology, not usually disseminated but centered around cervical area, most patients stage I and II
Tx of Hodgkin disease
stage III and IV, B sxs: ABVD
A - adriamycin (doxorubicin)
B - bleomycin
V - vinblastine
D - dacarbazine
ABVD
A - adriamycin (doxorubicin)
B - bleomycin
V - vinblastine
D - dacarbazine
tx of Hodgkin disease
Complications of radiation thx
solid tumors (breast, thyroid, lung), increased risk of premature CAD, risk of acute leukemia, MDS, and NHL
Contraindication to adramycin (doxorubicin)
EF <50%
Adverse side effect of Doxorubicin - Vincristine - Bleomycin - Cyclophosphamide - Cisplatin -
Adverse side effect of Doxorubicin -cardiomyopathy Vincristine - neuropathy Bleomycin - lung fibrosis Cyclophosphamide - hemorrhagic cystitis Cisplatin - renal and ototoxicity
abnormal proliferation of plasma cells
myeloma
most common causes of death in myeloma
renal failure and infection
serum protein electrophoresis shows IgG or IgA spike (monoclonal or “M” spike)
most likely monoclonal gammopathy of unknown signfiicance, but
serum protein electrophoresis shows IgM spike (monoclonal or “M” spike)
Waldenstrom macroglobulinemia
lab findings in multiple myeloma
IgA or IgG “M” spike
light chains or Bence-Jones protein on urine immunoelectrophoresis (not detected in urine dipstick)
hypercalcemia
smear with rouleaux
elevated BUN and creatinine
decreased anion gap
BM bx with > 10% plasma cells (nothing beside myeloma has this finding)
tx of multiple myeloma
combo of steroids and lenalidomide, bortezomib, or both
tx of Waldrenstrom macroglobulinemia
plasmapheresis; long term thx with rituximab, prednisone, cyclophosphamide
types of bleeding
A) superficial, epistaxis, purpura, mucosal surfaces =
B) deep, joints, muscle =
A) platelet bleeding
B) factor bleeding
megakaryocytes + thrombocytopenia + normal sized spleen =
immune thrombocytopenia purpura
tx of ITP A) no bleeding, count >30,000 B) mild bleeding, count <30,000 C) severe bleeding, count <10,000 D) recurrent episodes, steroid dependent E) refractory
tx of ITP
A) no bleeding, count >30,000 - observation (no tx)
B) mild bleeding, count <30,000 - steroids
C) severe bleeding, count <10,000 - IVIG, anti-Rho (anti D)
D) recurrent episodes, steroid dependent - splenectomy
E) refractory - romisplostim or eltrombopag, rituximab, steroid alt (azathioprine, cyclosporine, mycophenolate)
vWD tx
DDAVP (desmopressin)
reverses warfarin toxicity
prothrombin complex concentrate > FFP > vit K
cryoprecipitate replaces
fibrinogen; never first line treatment
tx of DIC
replace platelets and clotting factors (FFP), heparin?, replace fibrinogen (cryoprecipitate) if FFP does not control bleeding
replaces clotting factors
FFP
test for HIT
platelet factor 4 antibodies or serotonin release assay
when do you give platelets?
bleeding patient with count <50,000; contraindicated in TTP
FFP uses
replaces clotting factors in those with elevated PT, aPTT, or INR and bleeding. used as replacement with plasmapheresis. not a choice for those with hemophilia or vWD
sxs of anemia
dyspnea and fatigue, lightheadedness, angina, syncope, chest pain
less common causes of macrocytic anemia
liver disease, hypothyroidism, medications (zidovudine, phenytoin), myelodysplastic syndrome, cold agglutinins can falsely elevate MCV by clumping cells
very low hematocrit in the elderly or those with heart disease
25-30
things that will raise reticulocyte count
blood loss and hemolysis
only microcytic anemia with elevated reticulocyte count
alpha thalassemia with 3 gene deleted
stores iron; also acute phase reaction (elevated in acute infection)
ferritin
regulates iron absorption; low in anemia of chronic disease
hepcidin
alcohol causes what kind of anemia?
sideroblastic
anemia of inability of iron to be incorporated into heme
sideroblastic
initial test for microcytic anemia
iron study
low ferritin + low serum iron + high TIBC
iron deficiency anemia
high or normal ferritin + low serum iron + low TIBC
anemia of chronic disease
the only microcytic anemia where serum iron is elevated
sideroblastic
microcytic anemia with normal iron studies
thalassemia
single most acute test to diagnose iron deficiency anemia, rarely done
BM bx for stainable iron
staining for ringed sideroblasts
Prussian blue
initial test for macrocytic anemia
blood smear looking for hypersegmented neutrophils; if positive, then get B12 and folate levels, both have high homocysteine levels but only B12 gives high methylmalonic acid levels; will also have increased LDH and indirect bilirubin levels (–> ineffective erythropoiesis)
pancreatic insufficiency is asso with what type of anemia?
macrocytic anemia; pancreatic enzymes are need to remove B12 from R-protein so it can bind IF
metformin is asso with what type of anemia?
B12 deficiency –> macrocytic anemia
B12 may be normal in patients with B12 deficiency because
transcobalamine (the carrier protein) is an acute phase reactant
what is a major complication of B12 or folate replacement?
hypokalemia; rapid production of RBCs depletes K+
haptoglobin levels in hemolytic anemia
decreased
what lowers mortality in sickle cell disease?
hydroxyurea (prevents sickle cell crises) and antibiotics with fever
patient with sickle cell disease is admitted in acute pain crisis. hct drops from 34 –> 22% during stay. what’s the best initial test? what is the likely diagnosis? what is the best initial therapy?
patient with sickle cell disease is admitted in acute pain crisis. hct drops from 34 –> 22% during stay. what’s the best initial test? - reticulocyte count; if decreased –> aplastic crisis
what is the likely diagnosis? parvovirus B12 infection (confirm with PCR)
what is the best initial therapy? IVIG
increased MCHC
hereditary spherocytosis
alternative tx to diminish need for steroids
cyclophosphamide, cyclosporine, azathioprine, mycophenolate mofetil
cold agglutinin disease asso w
EBV, Mycoplasma pneumoniae, and Waldenstrom macroglobulinemia
tx of cold agglutinin disease
keep patient warm, rituximab, immunosuppressive agents
STEROIDS IS NEVER THE RIGHT ANSWER
G6PD level after a hemolytic event
will be normal
tx of HUS and TTP
plasmapheresis; if there is a delay to plasmapheresis –> infuse FFP; DO NOT TRANSFUSE PLATELETS
most common cause of death in paroxysmal nocturnal hemoglobinuria
thrombosis (large vessel or mesenteric/hepatic veins)
most accurate test to dx paroxysmal nocturnal hemoglobinuria
CD55 and CD59 levels
eculizumab
complement ib, use in PNH, give meningococcal vaccine prior
pancytopenia of unclear etiology
aplastic anemia
tx of aplastic anemia
supportive: transfusions, antibiotic for infection, platelets for bleeding
<50 years old: BMT
> 5O: antithymocyte globulin and cyclosporine, also altentuzumab (anti CD 52, suppresses T cells) *
aplastic anemia is a autoimmune disorder in which the T cells attack the patient’s own marrow, treatment is based on medications like cyclosporine that inhibit T cells, this brings the marrow back to life
labs in polycythemia vera
hct >60%, platelets and WBCs up as well, normal oxygen levels, low EPO, high vit B12 (unknown reason), low iron, low MCV, JAK2 mutation
JAK inhibitor
RUXOLItinib
meylofibrosis tx
teardrop shaped cells, tx with thalidomide and lenalidomide
erythromelagia
burning cyanosis in hands and feet, seen in p vera, tx w ASA
most common cause of iron deficiency anemia in a child
excessive consumption of cow’s milk (>24 oz or 700 ml/day)
RDW
red cell distribution width, elevated (>20%) in iron deficiency anemia and other nutritional deficiencies
duration of liver vit k supply
30 days, but in acutely ill person it can be depleted in 7-10 days
factor v leiden causes a hypercoagulable state due to
activated protein C resistance
most common bleeding disorders in children
hemophilias and vWD
hemophilia and other coagulation disorders commonly present with hemarthroses, soft tissue hematomas, and intramuscular hematomas; in constant platelet aggregation disorders such as vWD present predominantly with easy mucosal bleeding, ecchymoses, and petechiae
anemia of prematurity
most common cause of anemia in preterm infants, d/t low EPO levels, short RBC life span, and blood loss; exacerbated by frequent blood draws / phlebotomy in the NICU
autosomal recessive disorder of absent platelet glycoprotein Ib-IX-V, which acts as a receptor for vWF. patients usually have mild thrombocytopenia, circulating giant platelets, severe platelet dysfunction, and bleeding out of proportion to the degree of thrombocytopenia
Bernard-Soulier syndrome
HIT causes thrombocytopenia (due to reticuloendothelial sequestration of antibody-coated platelets) plus
increased risk of arterial and venous thrombus formation (due to HIT antibody activation of platelets, results in platelet aggregation and release of procoagulant factors)
effect of CYP450 inhibitors and inducers on warfarin
inhibitor - increase effect –> bleeding
inducers - decrease effect –> hypercoagulable
CYP450 inhibitors
acetaminophen, NSAID antimicrobials amiodarone cimetidine cranberry juice, ginkgo, vit E omeprazole TH SSRIs
CYP450 inducers
carbamazepine, phenytoin ginseng, St John's wort OCPs phenobarbital rifampin
difference btwn CML and leukemoid reaction
in CML, the leukocyte alkaline phosphatase score (a marker of neutrophil activity) is typically low
