Heme onc Flashcards

1
Q

Autoimmune hemolytic anemia

A
  • from antibodies usually from warm agglutinin (IgG) disease (less commonly cold agglutinin IgM)
  • labs: spherocytosis (increased MCHC), anemia/low haptoglobin, hemolytic anemia
  • dx positive coombs (direct antiglobulin test)
  • steroids (for Warm only! for cold warm them up and maybe rituximab) , splenectomy

in cold disease, cold exposure gives livedo reticularis and cyanosis, avoid cold temps and tx with rituximab

NO schistocytes

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2
Q

Testing when normal PT, elevated PTT

A

mixing study. If it corrects: factor deficiency (i.e. hemophilia with factor 8 or 9 def)

Otherwise factor inhibitor

Note: Lupus anticoagulant causes isolated elevated PTT, will have thrombosis rather than bleeding

Note: Factor 12 deficiency causes elevated PTT without bleeding

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3
Q

which factors are deficient in chronic liver disease or vitamin K deficiency i.e. abx or etoh

A

vitamin K dependent: 2, 7, 9, 10.

Factor 7 is the first one to go, so will have more significantly prolonged PT

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4
Q

T/F: b12 deficiency always causes macrocytosis and anemia

A

False. can have low normal range of b12 and still have sxs of def, need to check MMA

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5
Q

Most common types of thyroid cancer

A

PAPILLARY (BRAF mut)
Follicular
*medullary (RET mut) less common but is part of MEN2A/2B syndromes

Thyroid Cancer: Pray For MEN2

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6
Q

T/F: thyroglobulin is a good marker for recurrence/persistence of thyroid cancer

A

true, it’s usually secreted by Papillary and Follicular

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7
Q

Palpitations, hypertension, kidney stones, neck mass

A

Probably MEN2 syndrome (pheo, hyperparathyroidism) with medullary thyroid cancer. can also have marfanoid habitus/ganglioneuromas on tongue/lips/eyelids in Men 2b

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8
Q

mutations in thyroid cancer

A

BRAF: Papillary
RET: Medullary

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9
Q

Tx of thyroid cancer

A

Papillary, Follicular: Total thyroidectomy + radioiodine therapy

Medullary: Total thyroidectomy, neck dissection for LN. no role for radioiodone therapy

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10
Q

types of lung cancer

A

Small Cell

vs

Nonsmall Cell: AdenoCA, Squamous cell, Large cell

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11
Q

poor performance status in extensive NSLC and SCLC

A

NSLC: if poor performance and advanced dz, no good response to therapy and need hospice referral

SCLC: even with aggressive dz and poor performance status, can respond well to chemotherapy. “can still have a small chance when it looks over”

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12
Q

MDS vs myelofibrosis

A

MDS: Older patient with cytopenias, often megaloblastic. Progresses to acute leukemia or bone marrow failure. no hepatosplenomegaly.

Primary myelofibrosis: massive splenomegaly is characteristic, teardrop cells. Supportive. avoid splenectomy b/c hemorrhagic and thrombotic complications

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13
Q

Myelodysplastic syndrome with -5q syndrome

A

use lenalidomide

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14
Q

for breast cancer, if you need lumpectomy what else must they get

A

radiation.

Radiation and Lumpectomy is as good as mastectomy

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15
Q

ER or PR positive breast cancer

A

pre menopause: Tamoxifen (need annual gyn exams)

post menopause: Aromatase inhibitor (letrozole, anastrozole, exemestane) (monitor bone density)

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16
Q

Workup for breast mass

A
  1. Imaging
  2. Biopsy
  3. Receptors
  4. Stage and Tx
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17
Q

If someone gets aromatase inhibitor, what screening?

A

Bone density/DEXA every 2 years

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18
Q

side effect to watch out for osteoclast inhibitors (bisphophonates, denosumab)

A

osteonecrosis of the jaw

so all patients on them need good routine dental care

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19
Q

MMA and Homocysteine in folate, b12 deficiency

A

Folate: Homocysteine elevated, MMA normal
B12: Homocysteine elevated, MMA elevated

“You must b 12 to move up to MMA with all the homos”

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20
Q

Dx of Hemochromatosis

A

Fe/TIBC >45%

Ferritin >200 females >300 males

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21
Q

febrile transfusion rxn

A

Hemolytic: immune mediated, VERY BAD. check hemolysis labs, stop transfusion and support. ABO incompatibility

Nonhemolytic: labs negative. just from cytokines/wbc. give tylenol and continue transfusion

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22
Q

trali vs taco

A

both dyspnea 1-6 hours post transfusion.

TRALI: immune mediated. very bad. ARDS like, noncardiogenic pulmonary edema. BNP normal.

TACO: circulatory overload, CHF type picture. Diuretics and transfuse slower.

Eating TACOs is better than pushing TRALI’s

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23
Q

High WBC count

A
  • Mostly mature lymphocytes, older, not too sick. smudge cells –> CLL
  • increase in all myeloid precursors (meta, myelo, promyelo, bands), philadelphia chrom –> CLL
  • increase blasts, very sick and severe cytopenia = Acute Leukemia…if Auer rods = AML, if CNS involvement + LN + hepatosplenomegaly = ALL
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24
Q

Panyctopenia

A
  • Older patient, hypercellular bone marrow, macrocytic anemia: MDS
  • younger patient with hypocellular bone marrow: Aplastic anemia
  • splenomegaly with dry tap on BM: Hairy cell leukemia
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25
Q

Imaging for bony lesions for MM

A

needs a Skeletal Survey, not bone scans

MM needs SS, bone scan is BS

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26
Q

test when normal platelet count + mucocutaneous bleeding

A

PFA-100 to eval for acquired platelet dysfunction (has replaced bleeding time)

27
Q

Pulmonary Embolism Severity Score index

A

<65 = very low risk 30 day mortality

<85 = low risk

28
Q

T/F: Fat pad biopsy for amyloidosis

A

true

29
Q

what to use to reverse effects of warfarin in patients with bleeding/urgent surgery?

A

Four-factor prothrombin complex (4f-PCC). It has factors 2, 7, 9, 10

30
Q

reversal of dabigatran

A

idarucizumab

31
Q

T/F: APML presents with reduced total leukocyte count and DIC features

A

true. Transfuion with FFP and cryoprecipitate (fibrinogen) + platelets for the thrombocytopenia . Aspirate and bx the bone marrow urgently and treat with ATRA

32
Q

hemolytic anemia, pancytopenia, unprovoked atypical thrombosis, hematuria

A

PNH, dx with flow cytometry (absent CD 55, 59)

33
Q

isolated low hgb in sickle cell (pure red cell. aplasia)

A

parvo b19

34
Q

t/f: macrocytosis in myelodysplastic syndrome

A

true

35
Q

How to tx waldenstroms (LAD, splenomegaly, hyperviscosity syndrome). produce monoclonal IgM

A

plasmapharesis

36
Q

initial tx of aplastic anemia

A

Immunsuppression: cyclosporine + antithymocyte globulin. consider allogeneic HSCT

If sx: Eculizumab

All patients: prophylactic anticoag + Iron, Folic acid supp

37
Q

T/F: Workup for pure red cell aplasia same to panctyopenia but includes CT chest to eval for thymoma

A

true

38
Q

Main causes of isolated neutropenia

A
  • acute HIV, CMV, EBV
  • Rickettsial infection
  • chemo
  • SLE, RA (felty syndrome)
  • Nsaids, phenytoin, cephalosporins, bactrim, PTU
39
Q

older person with cytopenias, macrocytosis and morphologic abnormalities

A

Myelodysplastic syndrome

40
Q

Hydroxyurea alleviates

A

leukocytosis and splenomegaly

41
Q

CML patient is treated with tyrosine kinase inhibitor, whats the link to cardiology?

A

Prolongs QT

42
Q

Myeloproliferative Neoplasms

A

-can all progress to AML.
-look for unusual thromboses, massive splenomegaly, maybe systemic signs
-most common =
essential thrombocythemia
-others are CML, PV, PMF

43
Q

tx of essential thrombocythemia

A

Aspirin
Add hydroxyurea

if life threatening, then plateletpharesis

44
Q

thrombocythemia

A
  • most common causes = iron deficiency anemia and infection…plts will improve within a few weeks
  • look for essential thrombocythemia (splenomegaly, red/painful hands and feet, livedo reticularis, clots)
45
Q

tx of polycythemia vera

A

plasmapharesis to reduce hct <45%

aspirin, hydroxyurea

46
Q

Portal vein or hepatic vein (Budd-chiari) clots should prompt consideration of

A

Polycythemia Vera

47
Q

T/F: Do splenectomy for the massive splenomegaly seen in Primary Myelofibrosis

A

False, associated with hemmorage and thrombosis. THey have huge splenomegaly, giant platelets, teardrop erythrocytes.

Tx is supportive, consider allogeneic HSCT

48
Q

most common cancers associated with eosinophlia

A

lymphoma

49
Q

bulky lymphadenopathy in a young sick patient, splenomegaly and random CNS involvement

A

ALL

30% have CNS involvement

So tx = induction chemotherapy + CNS prophylaxis (intrathecal chemo +/- radiation)

50
Q

T/F: AML patients usually don’t have lympadenopathy or hepatosplenomegaly

A

true. will have significant thrombocytopenia with bleeding, burising, petechiae, infection

51
Q

T/F: AML usually have a really high wbc count, anemia, thrombocytopenia, blasts. gingival hypertorphy, nontender cutaneous plaques

A

True, can see auer rods on peripheral smear. >20% blasts.

*APML is an exception and even acute leukemia can sometimes present with pancytopenia, but BM exam has hypercellular marrow with >20% blasts

52
Q

differentiation syndrome

A

rxn to ATRA for APML. fever, pulm infiltrates, hypoxemia. tx dexamethasone

53
Q

t/f: amyloid as prlonged pt. and ptt

A

true often, factor x def

54
Q

ferritin > _____ rules out IDA

A

100

55
Q

bite and target cells

A

bite: G6PD
target: thalassemia, liver dz

56
Q

tx of warm vs cold hemolytic anemia

A

warm: steroids, splenectomy
cold: warm patient, rituximab

57
Q

which way does retic count go during sickle cell aplastic crisis?

A

low. vs in SS (hemolytic anemia) its high normally

58
Q

what kind of transfusion for sickle cell patient with acute stroke, chest syndrome or fat embolism?

A

exchange transfusion. otherwise can use simple transfusion. don’t tranfuse sickle cell patients unless needed though b/c transfusion complications

59
Q

very low mcv, mild anemia

A

thalassemia

60
Q

platelet clumps

A

repeat count using a citrated or heparinized tube

61
Q

T/F: Use eculizimab in PNH patients symptomatic

A

true, reduces intravascular hemolysis, hemoglobinuria and need for transfusion. allogeneic HSCT for long term survival anticoag, iron, folate supp.

This med also used for atypical HUS of pregnancy

62
Q

beta thal:

A

increased A2 and F

63
Q

SS disease

A

SS: >90% HbS, no HbA

SS trait: HbS <50% (average 30%), more amount HbA (no painful crises, Hgb normal). Amount HbA inversely related to severity.

SBeta-thal: HbS around 60%, can have painful crises