heme-onc Flashcards by jessica davis

what is the classic smear finding for myelofibrosis?

teardrop cells

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what might you think of in young person with diarrhea and iron deficiency anemia?

celiac dz

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how do you use methlymalonic acid and homocysteine in megaloblastic anemia workup?

If B12 is borderline (200-400) you can order
in folate def HC is up
in B12 def both are up

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what biliary complication do patients with hereditary spherocytosis get?

cholelithiasis from bilirubin stones

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how do you test for PNH? what are the major consequences of the disease?

flow for CD55 & CD 59
can p/w hemolysis, hemoglobinuria, Fe deficienc, arterial or venous thrombosis
(mechanism is genetic change that affects a surface protein that when missing makes cells susceptible to complement-mediated lysis)

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true/false: steroids are effective for both warm (IgG) and cold( IgM) antibody-mediated autoimmune hemolytic anemia.

false – used for IgG only. for IgM, just keep person warm and tx underlying cause (neoplas or infxn (mycoplasma, EBV))

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what might you suspect in someone who appears cyantotic with a normal O2 sat? causes? tx?

methemoglobinemia – o2 sat falsely high but PaO2 will be low
exposure to nitrites/nitrats, tylenol, dapsone, sulfas, ‘caines

tx with methylene blue

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what is tx for methemoglobinemia?

methylene blue

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give levels of transferrin saturation and ferritin for which people should be tested for hemochromatosis?

transferrin sat >45% (women) or >50% (men) or ferritin >1000

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what bleeding problems do people with primary hemostasis problems (platelets) present with? what about secondary hemostasis (factor deficiencies)?

platelets - gingival bleeding, petechiae, echymoses

factors - hemarthrosis, hematomas (more deep tissue)

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PT monitors which coag cascade? PTT?

PT – extrinsic
PTT - intrinsic

(PT is like physical therapy so it measure the “exercise” pathway)

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what disorders might you think of in someone with a normal PT, prolonged PTT and bleeding problems?

what about the same pt but with clotting problems?

bleeding – Factor VII, Factor IX, Factor IX or Factor XII deficiency

clotting – antiphospholipid Ab syndrome

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what is diagnosis in somene with clotting and prolonged PTT?

antiphospholipid Ab syndrome

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compare/contrast ITP and TTP.

ITP: autoimmune, can be idiopathic or from drugs or infxn, tx with steroids or IVIG; splenectomy is 2nd line, rarely bleed, NO schistocytes

TTP: FAT-RN (fever, anemia, thrombocytop, ARF, neuro sx), get thrombosis, atx with plasmaphresis or steroids, + schistocytes

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why do we avoid abx in kids who might have EHEC diarrhea?

giving abx increases the cahnce that they get HUS!

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which two disorders that present with thrombocytopenia have a high clotting risk?

HIT and TTP – so never give platelets

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T/F: THere are schistocytes in TTP but not in ITP.

true
ITP: autoimmune, no schistos, tx wtih steorids/IVIG
TTP: FAT-RN, get clots, +schistos

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what are tx options for bleeding in factor VIII deficiency?

DDAVP (causes release of vWF and Factor VIII) or recombinant Factor VIII

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how are factor VIII and Factor IX deficiences inherited? VWD?

factor VIII and IX are recessive

VMD is dominant

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which factor deficiency results in a long PTT but NO clinical bleeding, even with surgeries.

Factor XII

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what bleeding disorders can present with nl plt, PT, PTT and bleeding time?

mild VWD – can check levels of VWF and F VIII
mild hemophlia – can chek levels o F VIII and F IX
F XIII deficiency - can do “clot lysis” test (these peopl present with late post-surgical bleeding and poor wound healing)

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why is warfarin initially pro-thrombotic?

proteins C and S decrease before factors bc they have shortest half-life

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what is a common complication of patients iwth protein C deficiency who are started on warfarin?

warfarin skin necrosis

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what should you test for in pts with warfarin-related skin necrosis?

protein C deficieny (predisposes)

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what 3 antibpodies make up the antiphospholipid abs?

lupus anticoagulant Ab anticardiolipin Ab anti-beta2-glycoprotein 1 Ab

how does Factor V Leiden cause thrombosis? Do you have to be homozygote or heretero?

it causes resistance to activated protein C which normally acts to inactivate Factor V and VIII so with them unchecked you clot more homozygotes have 20x risk of clotting, heteros 7x

what is tx for follicular lymphoma (usually diffuse LAD in middle-aged with +CD20) in asymptomatic pts? symptomatic?

asymptomatic -- watchful waiting | symptomatic -- rituximab, chemo and prednisone

what type of RBC transfusion should you give someone who has a h/o severe anaphylaxis with trasnfusion? what disease should you suspect in someone with a h/o severe anaphylaxis with transfusion?

washed (b/c they are reacting against hte plasma proteins? IgA deficiency can p/w severe anaphylaxis to transfusion (bc people have anti-IgA Abs)

what immunoglobulin deficiency can p/w severe anaphylaxis to blood transfusion?

IgA deficiency

compare and contrast when you use tamoxifen, raloxifene and aromatase inhibitors in tx of hormone-receptor positive breast cancer.

premenopausal -- use tamoxifen postmenopausal -- use aromatase inhib raloxifene -- only used for tx of osteoporosis

true/false asymptomatic pts with hairy cell leukemia should be treated.

yes -- 1 round of cladribine cures >80%

what is treatment for hairy cell leukemia

cladribine

what leukemia might you suspect in an elderly man with pancytopenia, splenomegaly without lympadenopathy and a dry aspirate when BM is attempted? what is tx?

hairy cell leukemia tx with cladribine even if asymptomatic b/c cures >80% of pts

what can you do tx hromone receptor positive breast cancer in a pre-menopausal pt with h/o DVT?

ovarian ablation (tamoxifen CI 2/2 clot history)

contrast/compare the electrophoresis findings for thalasemmic pts with alpha-thal trai and beta-thal minor.

alpha-thal trait: have nl electrophoresis results | beta-thal: elevated heglobni A2 band.

what is diagnosis of african-american woman with MCV 70, low RDW, hgb 11 wiht normal hgb electrophoresis?

alpha-thal trait | beta-thal would show elevated hgb A2 band

what is diagnosis of african-american woman with MCV 70, low RDW, hgb 11 with elevated hgb A2 band on electrophoresis?

beta-thal | alpha-thal trait would have nl hgb electrophoresis

give the three cytogenetic changes assoc with favorable prognosis in AML.

t(8;21) (M2) t(15;17)(M3--APML) inv16(M4)

give thee cytogenetic changes assoc with negative prognosis in AML.

inv(3) del(5q) del7

``` for each of the following cytogenetic characteristics of AML, say if they give a favorable or unfavorable prognosis: inv(3) t(8;21) t(15;17) FLT3 mutation del(5q) del7 inv16 NPMI1 mutation ```

good: (t8;21),t(15;17), inv16, NPMI mutation bad: del5q, del7, inv(3), FLT3 mutation

which AML is assoc with DIC?

APML (M3)

what is major SE of ATRA given to pts with APML?

ATRA sydnrome -- fever, volume overload, effusions, respiratory distress and hypotension tx by holding ATRA, giving steroids

what is standard induction for AML? who should be offered BMT?

ara-c x 7 days with daunorubicin x3 days | can tx if t achieve remission after induction or those with poor cytogenetics (inv3, del5q, del7)

what % of blasta on bone marrow defines acute leukemia?

>20%

how is AML classified? ALL?

AML: M0 through M7 (M3 is APML) ALL: precursor B, precursor T, mature B (Burkitt's lyhphoma)

Is the t(9;22) translocation favorable or unfavorable in CML? In ALL?

CML: favorable ALL: unfavorable

what is typical chemotherapy for ALL?

prednisone, vincrstine, daunorubicin +/- cyclophosphamid + L-asaparginase

is del5q favorable or unfavorable in AML? in MDS?

unfavorable in AML, favorable in MDS

give two favorable cytogenetic markers for MDS.

del5q and del20q

how do patients with del5q MDS present? what is tx?

p/w refractory anemia and thrombocytopenia suusally respond to lenaliodmide

what drug is used to treat CML with t(9;22)(BCR_ABL)? what are common SE?

imatinib SE: cardiotoxicity, hepatotoxicity, cytopenia

when do you treat essential thrombocythemia? with what?

tx if significant erythromelalgia symptoms (HA, visual changes, pain) with hydroxyurea everyone (even asymptomatic) gets ASA to decreased clot risk

is epo high or low in PV?

LOW! would be high in secondary causes

is follicular lymphoma indolent or aggressive? what is classic cytologic marker? what is tx?

indolent t(14;18) translocation which overexpresses BCL-2 (antiapoptotic protein) can watch/wait if asympatomatic or

T/F: diffuse large B cell that is present after transformation from follicular lymphoma is more aggressive than de novo dz.

true

what cancer classically presents with a high white count and smudge cells on the smear?

CLL

name 2 good prognostic indicators in CLL and 2 bad ones.

good: 13q del, low ZAP-70 bad: 17p del, 11q del

what is standard tx for diffuse large B cell lymphoma?

``` R-CHOP rituximab cyclophosphamide doxorubicin vincristine prednisone if local dz, do 3 cycles + XRT if widespread, 6-8 cycles ``` if recurs in young person, do autoSCT

what cytogenetic dz is assoc with Burkett Lymphoma?

t(8;14)translocation, overexpresses C-MYC

what lymphoma is t(14;18) assoc with? t(8;14)? t(11;14)?

t(14;18) --> BCL-2 --> Follicular t(8;14) --> C-MYC --> Burkitts t(11;14)-->BCL-1-->Mantle Cell

what has a better prognosis, hodgkin or NHL?

hodgkin

what lymphoma is assoc with Reed-Sternberg cells?

hodgkin lymphoma

what is chemo for of hodgkin?

``` ABVD adriamycin (doxorubicin) bleomycin vinblastine dacarbazine ```

what virus is associated with HIV-associated primary effusion lymphomaa?

HHV-8

t/f: bone scan will show lytic lesions for multiple myeloma.

FALSE MM lesions are lytic but do not show up on bone scan b/c no new bone being produced do skeletal survey

what is tx for plasmacytoma?

local XRT

when do tx SVC syndrome emergently? how?

get tissue dx prior to tx unless increased ICP or airway compromise tx with XRT (steroids controversial)

what cancers can p/w lytic lesions?

MM, breast, renal

t/f: biopsy any palpable non-cystic breast mass even if not seen on ultrasound

true -- mammon on 80% sensitive

what does erlotinib target?

EGFR

what is tx for high-risk MDS (anemic, thrombocytopenic, 10-19% blasts)?

azacitadine

how do you minimize risk of erythrocyte alloimmunization?

phenotypically match units (matches C, E and K Ag) | irrad prevents GVHD in IS, washing prevents allergic rxn

t/f: washing RBC units reduces risk of alloimmunization.

false -- lessens allergies have to phenotypically match units (match C,E, K Ag) to reduce risk of alloimmunization

what does Bevacizumab target? what are common SE?

targets VEGF SE: HTN, vascular "catastrophes," poor wound healing, intestinal perforation

contrast/compare results of coombs test for: warm agglutinin Ab cold agglutinin Ab hereditary spherocytosis

warm (IgG abx): IgG+, C3 neg cold (IgM abx): IgG neg, C3+ spherocytosis: both neg

what is tx for DCIS?

local excision with adequate margins +/- XRT (XRT reduces recurrence but doesn't improve survival)

is her2/neu a good or bad prognostic marker in breast cancer?

bad (altghou can use trastuzumab)

what is trastuzumab against? what is its major SE?

her2/neu cardiac toxicity

true false - if do lumpectomy in breast cancer, you always do XRT.

true

what is used for adjuvant hormonal tx in premenopausal women? postmenopausal

pre --> tamoxifen | post--> aromatase inhibi (anastrozole, letrozole, exemestane)

t/f: pregnant women who have a history of idiopathic VTE should get enoxparin for ppx.

true

T/F: you have to get a bx if you have LGSIL on pap smear

true

which testicular cancer causes AFP elevation?

yolk sac (a nonseminomnous germ cell tumor)

which testicular cancers can have elevated HCG?

choriocarcinoma -- a nonseminous germ cell tumor -- 100% embyronal cell cancer -- a nonseminomonous germ cell tumor -- 50% seminoma - germ cell tumor -- 5-10%

what testicular cancer do you suspect if someone has an elevated AFP?

yolk sac tumor

describe staging for testicular cancers?

stage I - testis only stage II - RP nodes stage III - mets stage IV - there isn't one!

what stage is testicular cancer involving RP nodes?

what stage is metastatic testicular cancer?

III (there is no stage IV)

describe classification of testicular cancers.

GERM CELL (95%) or STROMAL (5%) stromal: leydig or sertoli GC: seminoma (good prognosis) or nonseminoma nonseminoma: embryonal, teratoma, choriocarcinoma (HCG+), yolk sac (AFP+)

T/F: Metastatic seminoma of the testicle is curable. What is tx?

true orchiectomy XRT to RP nodes if nodes >5 cm or mets --> chemo

describe mgmt of patients with: T1-T2N0M0 prostate CA T1-T3N0M0 prostate CA prostate CA wtih high (7-10) Gleason score

T1-T2: radical prostatectomy T1-T3: XRT high Gleason: hormonal deprivation tx

hwo do you tx a woman with adenocarcinoma of unknown primary in axillary nodes?

like stage II breast CA -- mastectomy

what is tx for localized carcinoid tumor? metastatic?

localized -- resection | metastatic - symptomatic tx

whihc MEN syndrome includes carcinoid tumors?

MENI

what does imatinib bind? what cancers is it used in?

binds tyrosine kinase preventing cell signaling used in CML and GIST tumors

what does traztuzumab bind? SE to know?

anti HER2/neu can cause reversible CHF

what chemotx is assoc iwth hemorrhagic cystitis?

cyclophosphamide

what are major toxicities of platinums (cisplatin, carboplatin, oxalitplatin

oto/neurotoxic nephrotoxic myelopsuppressive

what is major SE of busulfan?

interstitial pneumonitis/pulm fibrosis

what are the following monoclonals active against: - bevacizumab - cetuximab - erlotinib

bevacizumab: VEGFR | cetuximab/erlotinib: EGFR

what is major SE of bleomycin?

irresversible pulmonary fibrosis

what chemo causes magnesium-wasting?

cisplatin

describe how SCLC is staged and treated.

limited (1 XRT field) - chemo + chest XRT + brain XRT | extended - chemo only

T/F: Kidney cancer is the only malignancy in which removing the primary tumor in the setting of metastatic disease can improve overall outcome rather than just reduce local symptoms.

true!

t/f: if a breast cancer has a bony met, you need to bx it.

yes! b/c hormone and Her2 statuses are sometimes diff in bony mets so tx can change

what is the platelet goal for transfusion for major surgeries? CNS surgeries?

CNS is 100K | everything else 50K

what do pts with alloAb get with transfusion? IgA deficiency

IgA deficiency --> anaphylaxis (they have anti-IgA ab that activate against donor serum) alloAb (freq transfusion recipients) --> 5-10 days later get jaundice, hemolytic anemia

how do you screen for hereditary angioedema? diagnose?

screen: look for low C4 level dx: check C1-INH functional assay

true/false. herediatry angioedema, unlikely angioedema from an immediate hypersensitivity reaction, does NOT cause itching or urticaria.

true!

what infection do patients with the following complement deficiencies get?: - C1, C2 or C4 - C3 - C5-C9

C1,2,4: recurrent sinopulmonary infxns, increased early-onset rheum dzs C3: severe pyogenic bacterial infxns C5-C9: increased neisseria infxns

deficiency of which complement is assoc with neisserial infxns/

C5:C9 (diagnse by checkign CH50)

deficiency of which complement is assoc with severe pyogenic bacterial infxns?

what infxns do people with congenital agammaglobulinemia get?

pyogenic/encapsulated organisms so recurrent sinopulmonary infxns nl protection against fungi do okay against most GNs and viruses (have T cell)

what presents with eczema, low IgM, high IgA and IgE and thrombocytopenia? tx?

Wiskott-Aldrich (Wisk through the EXIT (eosinophilia, X-lined, immunodeficiency, thrombocytopenia) tx wth BMT