Heme onc

Question 1

Autoimmune hemolytic anemia

Answer 1

• 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

Question 2

Testing when normal PT, elevated PTT

Answer 2

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

Question 3

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

Answer 3

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

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

Question 4

T/F: b12 deficiency always causes macrocytosis and anemia

Answer 4

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

Question 5

Most common types of thyroid cancer

Answer 5

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

Thyroid Cancer: Pray For MEN2

Question 6

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

Answer 6

true, it’s usually secreted by Papillary and Follicular

Question 7

Palpitations, hypertension, kidney stones, neck mass

Answer 7

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

Question 8

mutations in thyroid cancer

Answer 8

BRAF: Papillary
RET: Medullary

Question 9

Tx of thyroid cancer

Answer 9

Papillary, Follicular: Total thyroidectomy + radioiodine therapy

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

Question 10

types of lung cancer

Answer 10

Small Cell

vs

Nonsmall Cell: AdenoCA, Squamous cell, Large cell

Question 11

poor performance status in extensive NSLC and SCLC

Answer 11

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”

Question 12

MDS vs myelofibrosis

Answer 12

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

Question 13

Myelodysplastic syndrome with -5q syndrome

Answer 13

use lenalidomide

Question 14

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

Answer 14

radiation.

Radiation and Lumpectomy is as good as mastectomy

Question 15

ER or PR positive breast cancer

Answer 15

pre menopause: Tamoxifen (need annual gyn exams)

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

Question 16

Workup for breast mass

Answer 16

1. Imaging
2. Biopsy
3. Receptors
4. Stage and Tx

Question 17

If someone gets aromatase inhibitor, what screening?

Answer 17

Bone density/DEXA every 2 years

Question 18

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

Answer 18

osteonecrosis of the jaw

so all patients on them need good routine dental care

Question 19

MMA and Homocysteine in folate, b12 deficiency

Answer 19

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

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

Question 20

Dx of Hemochromatosis

Answer 20

Fe/TIBC >45%

Ferritin >200 females >300 males

Question 21

febrile transfusion rxn

Answer 21

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

Question 22

trali vs taco

Answer 22

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

Question 23

High WBC count

Answer 23

• 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

Question 24

Panyctopenia

Answer 24

• 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

Question 25

Imaging for bony lesions for MM

Answer 25

needs a Skeletal Survey, not bone scans MM needs SS, bone scan is BS

Question 26

test when normal platelet count + mucocutaneous bleeding

Answer 26

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

Question 27

Pulmonary Embolism Severity Score index

Answer 27

<65 = very low risk 30 day mortality <85 = low risk

Question 28

T/F: Fat pad biopsy for amyloidosis

Answer 28

true

Question 29

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

Answer 29

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

Question 30

reversal of dabigatran

Answer 30

idarucizumab

Question 31

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

Answer 31

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

Question 32

hemolytic anemia, pancytopenia, unprovoked atypical thrombosis, hematuria

Answer 32

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

Question 33

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

Answer 33

parvo b19

Question 34

t/f: macrocytosis in myelodysplastic syndrome

Answer 34

true

Question 35

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

Answer 35

plasmapharesis

Question 36

initial tx of aplastic anemia

Answer 36

Immunsuppression: cyclosporine + antithymocyte globulin. consider allogeneic HSCT If sx: Eculizumab All patients: prophylactic anticoag + Iron, Folic acid supp

Question 37

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

Answer 37

true

Question 38

Main causes of isolated neutropenia

Answer 38

- acute HIV, CMV, EBV - Rickettsial infection - chemo - SLE, RA (felty syndrome) - Nsaids, phenytoin, cephalosporins, bactrim, PTU

Question 39

older person with cytopenias, macrocytosis and morphologic abnormalities

Answer 39

Myelodysplastic syndrome

Question 40

Hydroxyurea alleviates

Answer 40

leukocytosis and splenomegaly

Question 41

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

Answer 41

Prolongs QT

Question 42

Myeloproliferative Neoplasms

Answer 42

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

Question 43

tx of essential thrombocythemia

Answer 43

Aspirin Add hydroxyurea if life threatening, then plateletpharesis

Question 44

thrombocythemia

Answer 44

- 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)

Question 45

tx of polycythemia vera

Answer 45

plasmapharesis to reduce hct <45% aspirin, hydroxyurea

Question 46

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

Answer 46

Polycythemia Vera

Question 47

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

Answer 47

False, associated with hemmorage and thrombosis. THey have huge splenomegaly, giant platelets, teardrop erythrocytes. Tx is supportive, consider allogeneic HSCT

Question 48

most common cancers associated with eosinophlia

Answer 48

lymphoma

Question 49

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

Answer 49

ALL 30% have CNS involvement So tx = induction chemotherapy + CNS prophylaxis (intrathecal chemo +/- radiation)

Question 50

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

Answer 50

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

Question 51

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

Answer 51

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

Question 52

differentiation syndrome

Answer 52

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

Question 53

t/f: amyloid as prlonged pt. and ptt

Answer 53

true often, factor x def

Question 54

ferritin > _____ rules out IDA

Answer 54

100

Question 55

bite and target cells

Answer 55

bite: G6PD target: thalassemia, liver dz

Question 56

tx of warm vs cold hemolytic anemia

Answer 56

warm: steroids, splenectomy cold: warm patient, rituximab

Question 57

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

Answer 57

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

Question 58

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

Answer 58

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

Question 59

very low mcv, mild anemia

Answer 59

thalassemia

Question 60

platelet clumps

Answer 60

repeat count using a citrated or heparinized tube

Question 61

T/F: Use eculizimab in PNH patients symptomatic

Answer 61

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

Question 62

beta thal:

Answer 62

increased A2 and F

Question 63

SS disease

Answer 63

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