STEP 2 Heme/Onc Flashcards by Fritz Stabenau

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

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NF-2 features (inheritance, chromosome, clinical)

AD - central neurofibromatosis - MISME
Chromosome 22 merlin
Bilateral acoustic schwannomas, meningiomas, schwannomas

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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

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Non-beta-cell tumors?

Large mesenchymal tumors

Produce IGF-II (no c-peptide or insulin)

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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

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von Hippel Lindau features?

phakomatosis
FAMILY HISTORY (AD)
hemangioblastomas
RCC
pheos

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Sturge-Weber features?

phakomatosis - somatic mutation in GNAQ gene
NOT HERITABLE
port wine stain over trigeminal nerve territory
MR
seizures
visual impairment

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McCune-Albright features?

mosaic in cAMP-kinase gene
3 P's:
polyostotic fibrous dysplasia
precocious puberty
pigmentation (irregular border cafe au lait)

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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

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thal minor vs iron deficiency anemia

thal minor: nl RDW, nl RBCs, target cells

fe deficiency anemia: high RDW, low RBCs

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Smear findings: howell-jolly bodies?

Remnant of RBC nucleus.

Seen in cases of functional asplenia (eg sicklers) or splenectomy

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Helmet cells?

microangiopathy (DIC, HUS, TTP)

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Basophilic stippling?

Ribosomal precipitates (blue granules in cytoplasm)
seen in thalassemias and heavy metal poisoning

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Heinz bodies?

aggregates of denatured Hgb
seen in G6PD and thalassemia
accompanied by bite cells (when macrophages bite them off)

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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

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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

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trastuzumab tox?

cardiac, get an echo

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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)

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glucagonoma: what does NEC look like?

is there DM?

NEC - erythematous plaques with eroded borders and central clearing
DM is mild

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mediastinal masses - anterior, middle, posterior

anterior: 5T’s
- thyroid (retrosternal)
- thymus
- thoracic aorta
- terrible lymphoma
- teratoma
middle:
- bronchogenic cyst
posterior:
- esophageal leiomyoma
- neuroblastoma

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Waldenstroms vs MM ddx

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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

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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?

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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?

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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

STEP 2 Heme/Onc Flashcards

(50 cards)