STEP 2 Heme/Onc Flashcards
NF-1 features (inheritance, chromosome, clinical)
AD - von Recklinghausen dz
Chromosome 17 neurofibromin
cafe-au-lait spots, iris hamartomas (Lisch nodules), neurofibromas (peripheral nerve sheath tumors - optic pathway), +brain tumor incidence
NF-2 features (inheritance, chromosome, clinical)
AD - central neurofibromatosis - MISME
Chromosome 22 merlin
Bilateral acoustic schwannomas, meningiomas, schwannomas
TS complex (inheritance, chromosome, clinical)
AD - TSC1/TSC2
hamartin/tuberin chr 9/16
Cortical {tubers, hamartomas, subependymal giant cell astrocytomas}, ash leaf spots, angiofibromas, renal angioleiomyomas, MR, seizures
Non-beta-cell tumors?
Large mesenchymal tumors
Produce IGF-II (no c-peptide or insulin)
Transfusion reactions: nonhemolytic - mechanism, onset, sx, tx
nonhemolytic febrile (most common): cytokine formation during storage
- 1-6hrs
- fevers, chills, etc
- stop transfusion give tylenol
TRALI: donor anti-leukocyte abx (usu. from preg.)
- 0-6hrs
- noncardiogenic pulmonary edema, bilateral infiltrates on CXR
- stop transfusion, resp support
allergic: reaction to donor proteins
- IgA deficient recipient -> +anaphylaxis risk
- seconds to minutes
- urticaria (minor), anaphylaxis (airway edema, hypotn - severe)
- antihistamines. if severe, stop transfusion, give IM epi, glucocorticoids, antihistamines, use washed RBCs and IgA free plasma next time
primary hypotensive: donor bradykinin
- incr risk if on ACEI
- within minutes
von Hippel Lindau features?
phakomatosis FAMILY HISTORY (AD) hemangioblastomas RCC pheos
Sturge-Weber features?
phakomatosis - somatic mutation in GNAQ gene
NOT HERITABLE
port wine stain over trigeminal nerve territory
MR
seizures
visual impairment
McCune-Albright features?
mosaic in cAMP-kinase gene 3 P's: polyostotic fibrous dysplasia precocious puberty pigmentation (irregular border cafe au lait)
HIT type 1 vs type 2
type I: drop in plt via direct action of heparin on plt. Normalizes w/in 2 days. type II: anti-PF4 complex abx vs plts plt drop > 50% nadir 30-60 5-10days arterial and venous thrombosis
thal minor vs iron deficiency anemia
thal minor: nl RDW, nl RBCs, target cells
fe deficiency anemia: high RDW, low RBCs
Smear findings: howell-jolly bodies?
Remnant of RBC nucleus.
Seen in cases of functional asplenia (eg sicklers) or splenectomy
Helmet cells?
microangiopathy (DIC, HUS, TTP)
Basophilic stippling?
Ribosomal precipitates (blue granules in cytoplasm) seen in thalassemias and heavy metal poisoning
Heinz bodies?
aggregates of denatured Hgb
seen in G6PD and thalassemia
accompanied by bite cells (when macrophages bite them off)
Transfusion reaction: hemolytic
acute hemolytic: ABO incompatibility, 1-hr timescale
-fever, chills, flank pain, renal failure, DIC
-pink plasma, +Coombs
delayed hemolytic: anamnestic response 2-10d
- +direct coombs
Thyroid cancers (4)
medullary: secretes calcitonin, MEN2A/B
papillary: orphan annie-eyes, psammoma bodies, unencapsulated, good prognosis, most common
follicular: early hematogenous spread, encapsulated, carcinoma invades
anaplastic: you’re screwed
trastuzumab tox?
cardiac, get an echo
classic triad for brain abscess
risk factors for brain abscess
fever, nocturnal or morning headaches, focal deficit, +- seizure
sinus, dental infx, otitis media/mastoiditis
cyanotic congenital heart dz (R->L shunt)
glucagonoma: what does NEC look like?
is there DM?
NEC - erythematous plaques with eroded borders and central clearing
DM is mild
mediastinal masses - anterior, middle, posterior
anterior: 5T’s
- thyroid (retrosternal)
- thymus
- thoracic aorta
- terrible lymphoma
- teratoma
middle:
- bronchogenic cyst
posterior:
- esophageal leiomyoma
- neuroblastoma
Waldenstroms vs MM ddx
Waldenstroms: hyperviscosity IgM - splenomegaly - retinal vein engorgement -> visual sx - headache and dizziness - pain and numbness in extremities MM: usually IgA or IgG - hypercalcemia - renal failure - anemia - bence-jones proteinuria - lytic bone lesions - infections
lung cancers and paraneoplastic syndromes: SCLC, squamous, adeno
SCLC: SIADH, ectopic 2ary Cushing’s (pigmentation!), Lambert-Eaton
squamous: hypercalcemia, cavitary lesion
adeno: (usu peripherally located lesion in lung): hypertrophic osteoarthropathy
cauda equina syndrome vs conus medullaris syndrome?
cauda equina - spinal nerve roots
- radicular distribution of pain (unilateral)
- dermatomal numbness (unilateral)
- lower motor neuron signs (hyporeflexia)
- saddle anesthesia
- late bowel bladder
conus medullaris - spinal cord
- back pain
- numbness perianal only
- upper and lower motor neuron signs (upgoing toes)
- early bowel bladder
hydroxychloroquine side effect?
retinopathy
AML + halo sign on chest CT?
aspergilloma
bilateral breast discharge green-brown?
galactorrhea
get TSH and prolactin
brain mets from lung ca mgmt?
NSCLC single met: surgery or stereotactic rads +- WBR
can’t operate: stereotactic rads
multiple mets or poor perf status: WBR
chemosensitive tumor (SCLC, choriocarcinoma, seminoma, lymphoma)
complications from sickle cell trait?
- painless hematuria
- hypo or isosthenuria = nocturia, polyuria
Esophageal manometry readings for scleroderma?
loss of distal peristalsis
Which organisms are hemochromatosis pts vulnerable to?
Vibrio vulnificus, Yersinia enterocolitica, Listeria
CML triad?
leukocytosis, basophilia, splenomegaly, BCR-ABL
decreased leukocyte alkaline phosphatase
no lymphadenopathy
lung ca hypercalcemia mechanism? Which type of ca?
PTHrP - squamous cell ca
risk factors for pancreatic ca?
first-degree relative with panc ca
BRCA1, BRCA2
Peutz-Jeghers
smoking
MGUS vs MM features?
serum monoclonal protein 3g cutoff
10% plasma cells in BM cutoff
presence of hypercalcemia, renal dysfunction, anemia, lytic lesions
-elevated beta-2 microglobulin in MM
causes of eosinophilia?
NAACP: neoplasm addison's (adrenal insufficiency) allergy collagen vascular dz / cholesterol emboli parasites
indications for low-dose chest CT screening?
age 55-80
no medical condition limiting life or willingness to surgerize
current smoker or quit = 30 PY smoking hx
causes of type IV RTA (hypoaldosteronism)?
diabetes, medications, inherited d/o
- calcineurin inhibitors
- NSAIDs
- ACEI/ARB
- heparin
ITP - history, smear/lab findings, tx
- antecedent viral infection
- smear: megakaryocytes
- isolated thrombocytopenia
- steroids and IVIG if platelets
workup of suspected acromegaly?
IGF-1 level (should be persistently elevated)
confirm with GH after glucose load
HNPCC II extracolonic cancer?
endometrial
tx for smoke inhalation
secure airway - airway edema
CO and HCN poisoning
tx: CO - oxygen (hyperbaric if HbCO > 40%)
HCN - hydroxocobalamin or sodium thiosulfate
-2nd line: induce methemoglobinemia: nitrites
indications for specialized RBC tx?
irradiated: immunodeficient, blood donated by 1st or 2nd degree relatives
leukoreduced:
-chronically transfused pts
-CMV seronegative with AIDS, transplant
-potential transplant recipients
-previous febrile nonhemolytic transfusion rx
washed:
-IgA deficient
-continued urticarial rxns despite antihistamine
-complement dependent AIHA
asbestos exposure + smoking = which cancer?
lung (aka “bronchogenic”) carcinoma
pleural mesothelioma less common
breast ca: expression of which gene influences tx?
HER2: can add trastuzumab
staging system for CLL?
stage 0 - smudge cells only stage I - smudge cells + lymphadenopathy stage II - splenomegaly stage III - anemia stage IV - thrombocytopenia - poor prognosis
complications of mono?
cold AIHA and thrombocytopenia due to serum cold agglutinins
splenic rupture
airway compression d/t tonsillar enlargement
can G6PD activity be normal in a G6PD patient?
inheritance of G6PD?
which cells?
yes - during an acute attack
X-linked
bite cells
osteoporosis risk factors?
advanced age thin body smoking alcohol steroids menopause malnutrition family history asian or caucasian ethnicity
in which 2 conditions can you see spherocytes?
AIHA - negative FHx, positive coombs
hereditary spherocytosis - +FHx, neg coombs
both have predominantly extravascular hemolysis
undifferentiated head & neck carcinoma risk facs?
smoking and EBV
