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Medicine Boards > Heme/Onc > Flashcards

Heme/Onc Flashcards

1
Q

Name triggers/associations for ITP (immune thrombocytopenic purpura)

A
  • idiopathic
  • triggered by medications
  • associated with SLE, CLL, Lymphoma, HIV, HCV, H. pylori
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2
Q

What characterizes PNH? Flow cytometry?

Treatment?

A
  • Chronic Hemolytic Anemia (abdominal pain during times of increased hemolysis)
  • Iron deficiency through urinary losses
  • Venous thrombosis (**Budd Chiari syndrome)
  • Pancytopenia
  • CD55 and CD59

Tx: eculizumab (reduces intravascular hemolysis, hemoglobinuria, and need for transfusion) ALL must be on prophylactic anticoagulation and iron + folic acid

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3
Q

What 3 conditions are acquired defects of hematopoietic stem cells? (present with pancytopenia)

A
  1. Aplastic anemia
  2. PNH
  3. MDS

clinic overlap, and all cause pancytopenia

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4
Q

Treatment of Apastic Anemia

associated condition?

A
  1. withdraw any offending agents (meds, radiation, toxins, fix nutritional deficiencies)
  2. Immunosuppression, 1st line = Cyclosporine and antithymocyte globulin (70% get disease control)
  3. If <50yo t/c allogenic HSCT potentially curative

Associated w/ Thymoma (so is pure red cell aplasia, and myasthenia gravis)

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5
Q

Etiology of pure red cell aplasia? What does Bone Marrow show? Treatment?

A

T cell autoimmunity = pregnancy, thymoma, malignancy
or
Direct toxicity to erythrocyte precursors = viral, drug tox

BM = erythroid hypoplasia, clonal CD57+ T cells = large granular lymphocytosis

Tx:

  • transfusion, immunosuppression (prednisone, cyclosporine, antithymocyte globulin)
  • IVIG for pt with AIDS and chronic parvo B19
  • Methotrexate or cyclosporine for large granular lymphocytosis
  • Thymectomy for thymoma
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6
Q

Evaluation for Pancytopenia

A
  • BM aspirate + biopsy (hypocellular with inc fat content)
  • Cytogenic analysis to exclude other BM disorders (ie. MDS)
  • PNH screening flow cytometry with cell surface markers CD55 and CD59 absent
  • Vit B12 + folate, hepatitis, HIV
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7
Q

Myelodysplatic Syndromes

  • Describe BM
  • Describe peripheral smear
  • Which subtype has a specific treatment? what is that treatment?
A
  1. hypercellular marrow with dysplastic erythroid precursors
  2. cytopenias in at least 2 cell lines + morphological abnormalities of erythrocytes (macrocytosis with nucleated erythrocytes and tear drop cells)
  3. -5q syndrome > lenalidomide to treat (2/3rds will respond)
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8
Q

Treatment of high or very high risk MDS (by IPSS-R criteria)? What are you trying to prevent?

A
  • Preventing transformation to AML
  • Fit + Young – allogenic HSCT
  • not bm transplant candidate – azacytidine and decitabine
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9
Q

CML

  • clinical picture + CBC findings
  • diagnosis
  • Treatment + SE
A
  1. Asx, splenomegaly, leukocytosis with inc #granulocytic cells in all phases of maturation in peripheral blood smear, if blasts 10-20% = accelerated or if >20% = blast phase (to AML)
  2. molecular = philadelphia chormosome (t 9:22) + BCR-ABL gene in peripheral blood or bone marrow
  3. Tx:
    - - Tyrosine Kinase Inhibitors (imitanib, dasitinib, nilotinib) –> SE QTc prolongation
    - - Allogenic HSCT if have accelerated or blast crisis
    - - Hydroxyurea - palliative to alleviate leukocytosis and splenomegaly
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10
Q

Essential Thrombocythemia

  • Sx
  • mutation?
  • Treatment (low vs. high risk)
A
  1. usu Asx, or vasomotor (erythromelalgia), livido reticularis, HA, vision sx, arterial/venous thrombosis, 50% splenomegaly
  2. JAK2 mutation (50%)
  3. Tx:
    - low risk - ASA 81 (<60yo, no thrombosis, WBC<11)
    - high risk (nonpreg) - ASA + Hydroxyurea
    - high risk + TIA/CVA/MI/GIB = plateletpheresis
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11
Q

Secondary causes of polycythemia

A
  • Hypoxemia ** most common
  • Volume contraction 2/2 diuretics
  • Androgens
  • Secretion of Erythropoietin 2/2 kidney or liver carcinoma
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12
Q

How do diagnose Polycythemia Vera? Treatment?

A
  • Hb >16 (female) or >16.5 (male) and low EPO – epo-indepenent prolif of erthrocytes
  • therapeutic phlebot to keep hct<45%
  • ASA 81mg for all unless c/i
  • Hydroxyurea if high risk thrombosis (>60-yo, prev thrombosis, leukocytosis)
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13
Q

Hypereosinophiic Syndrome

  • Eo count?
  • pathophys?
  • Treatment
A
  • Eo count >1500/mcl
  • molec activ of PDGFR a or b (platelet derived growth factor receptor)
  • Imitinib otherwsie use glucocorticoid
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14
Q

AML

  • CBC + peripheral smear
  • how to dx?
  • When to consider alternate dx?
A
  • high WBC, low Hgb, LOW PLATELETS - bleeding/bruising/infection. Smear - blasts, auer rods
  • BM with >20% myeloblasts
  • if lymphadenopathy or hepatosplenomegaly b/c does not happen in AML
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15
Q

APML translocation + clinical picture + treatment (SE? tx?)

A
  • t (15;17) translocation- disturbs retinoic acid receptor
  • significant bleeding 2/2 fibrinolysis + DIC (clotting)
  • ATRA (all trans retinoic acid) - start ASAP b/c high mortality of APML
  • SE ATRA - differentiation syndrome (fever/hypoxemia/pulm infiltrate/hyperleukocytosis) - treat w/ dexamethasone
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16
Q

Name 4 plasma cell dyscrasias

A
  1. MM
  2. MGUS
  3. Waldenstrom macroglobulinemia
  4. AL amyloidosis (light-chain assoc amyloidosis)
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17
Q

difference btwn MGUS vs. Smoldering MM vs. MM

Whats the interval of screening?

A
  1. MGUS - monoclonal protein <3g/dL, BM clonal plasma cells <10%, no end organ damage&raquo_space; ANNUAL LABS + H&P
  2. Smoldering MM - monoclonal prot =/>3g/dL, BM =/>10% plasma cells, no end organ damage&raquo_space; must first get whole body MRI to assess for lytic lesions (if skeletal survey negative)&raquo_space; then Q3 MONTH LABS
  3. MM - above + end organ damage (CRAB)
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18
Q

AL Amyloidosis

- how to diagnose?

A

** abdominal fat pad aspirate or BM bx ** showing apple green birefringence under polarized light with congo red staining

  • kappa/lambda chain detection + typing
  • serum or urine M protein OR clonal plasma cells in bone marrow

(AL amyloidosis found in 10% of pts with MM, but may be diagnosed in pts who lack other myeloma findings)

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19
Q

Waldenstrom Macroglobulinemia

  • What is it
  • How to diagnose
  • What concerning syndrome can pts get? Tx?
A
  • Indolent B-cell lymphoma with clonal lymphoplasmacytic infiltration of the bone marrow that secretes IgM in the blood (IgM Spike)
  • B symptoms (fever, sweats, wt loss) + anemia, HSM
  • Dx by BM bx
  • 1/3rd get Hyperviscosity Syndrome – Medical Emergency&raquo_space; Plasmapheresis
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20
Q

Name the 4 Myeloproliferative Disorders

A

CML
PCV
Essential Thrombocytosis
Myelofibrosis (MF)

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21
Q

Smudge cells

A

CLL

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22
Q

How do you urgently reverse Warfarin coagulopathy?

A

Four factor prothrombin complex concentrate (PCC) - contains factors II, VII, IX, and X as a lyophilized powder and can be administered quickly in a small reconstituted volume. It provides effective hemostasis 90% of the time.

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23
Q

Treatment of patient with hypogammaglobulinemia + recurrent infections

A

IVIG

ie. multiple myeloma with recurrent sinusitis

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24
Q

Hereditary Spherocytosis

  • Exam
  • Dx
A
  • 2nd and 3rd MCP osteophytes
  • cirrhosis, cardiomyopathy, DM, skin pigmentation, fatigue, arthralgias, loss of libido
  • transferrin sat >45% + high ferritin
  • confirm with genetic testing for mutation in HFE gene
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25
Q

What type of transfusion/blood do you give in autoimmune hemolytic anemia?

A

ABO matched
Rh matched
but doesn’t have to be cross-matched b/c may be impossible to find 2/2 DAT detecting IgG or complement coating that body forma autoantibodies against

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26
Q

Treatment of iron overload from chronic transfusion

A

Chelating agents

  • PO deferasirox
  • IV deferoxamine
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27
Q

Dabigitran reversal agent, when to use?

A

Idarucizumab – give before emergent surgery! (elevated aPTT, dabigitran elimination half-life is 12-17hr with normal renal function)

“ida-dabigitran”

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28
Q

Pre-op Hb goal for sickle cell patient undergoing low to medium risk surgeries?

A

Hb 10 (by simple transfusion)

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29
Q

Patient starts Warfarin, they then get “retiform purpura” with areas of skin necrosis – whats the diagnosis?

A

Protein C or S deficiency (+when warfarin is started has transient hypercoag state b/c reduces prot C activity by 50%) the rash shows up when heparin is discontinued

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30
Q

What are the PERC Criteria? (pulm embolism rule out criteria)

A
  • <50yo
  • HR <100
  • SpO2 >95%
  • No hemoptysis
  • No estrogen use
  • No previous DVT or PE
  • No unilateral leg swelling
  • No surgery or trauma requiring hospitalization in the last 4 weeks
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31
Q

Type of transfusion for low fibrinogen?

A

cryoprecipitate

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32
Q

How to distinguish DIC vs. Liver coagulopathy

A

both have low fibrinogen but in liver disease – normal/increased Factor VII – which is consumed/low in DIC

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33
Q

Alpha thal trait

  • electropheresis
  • Hb, MCV
  • Treatment?
A
  • normal electropheresis! normal Hb A2 and Hb F
  • Hb 10, chronic microcytic anemia
  • Can supplement Folic Acid (NOT iron b/c inc ability to absorb iron&raquo_space; overload)
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34
Q

Beta Thal minor

  • Hb, MCV
  • elecropheresis
A
  • chronic microcytic anemia
  • electrophoresis – excess alpha chains link with delta (HbA2) and lambda chains (HbF) to produces increased amounts of Hb A2 + Hb F
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35
Q

If patient has asymptomatic INR elevation, at what point do you have to treat?

A

INR 4.5 - 10 = watch

INR >10 = PO Vit K 2.5mg

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36
Q

Describe electropheresis of Hemoglobin S B-thalassemia

A
  • HbA 5-30% (inversely proportional to sx of pain crisis)

- HbS ~60% (vs. sickle cell trait have HbS«50% and more HbA vs. sickle cell disease HbS <90% and no HbA)

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37
Q

How do you diagnose presumptive TTP? Treatment?

A

Dx - blood smear with schistocytes in a patient with hemolytic anemia and thrombocytopenia, fever, change in mental status

Schistocytes = microangiopathic hemolytic anemia (MAHA)

Tx = emergent plasma exchange

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38
Q

pancytopenia with macrocytosis + peripheral blood smear with macrocytic erythrocytes and rare nucleated erythrocytes + 6-lobed neutrophils with negative Coombs test

A

B12 (cobalamin) deficiency, intermedullary hemolysis (with elevated LDH and bili)

**occurs with other autoimmune conditions (ie. hashimoto thyroiditis)

**B12<200, but if not can get MMA + homocystine levels which will be elevated in 98% of B12 deficiency

**B12 def can present with subacute combined degeneration of spinal colum (weakness, paresthesias, ataxia) without anemia or macrocytosis

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39
Q

what in an iron panel confers definite iron deficiency?

A

Ferritin <14 ng/mL

40
Q

Other than alcohol, name 3 other drugs that cause macrocytic anemia

A

zidovudine
hydroxyurea
methotrexate

41
Q

Sphreocytes (name 2 conditions)

A

Autoimmune hemolytic anemia

Hereditary sphreocytosis

42
Q

Target Cells (name 3 conditions)

A

thalassemia
liver disease
“other hemoglobinopathy”

43
Q

Bite Cells

A

G6PD (eccentrically located Hb confined to one side of cell)

44
Q

What causes cold agglutinin hemolysis? What do you see on peripheral smear? Treatment?

A

smear with erythrocyte agglutination

Myocplasma
CLL
lymphoproliferative disease

Tx: cold avoidance or rituximab if persitent sx (steroids and splenectomy ineffective)

45
Q

Whats the test for autoimmune hemolytic anemia?

A

DAT (coombs test)

46
Q

Indications for exchange transfusion in sickle cell

A

Acute CVA
Fat embolism
ACS requiring ICU/intubation

prophylactic - with history of ischemic CVA

47
Q

Betal thal major treatment

A

transfusion
iron chelation (if Ferritin >1000)
splenectomy
HSCT (severe)

48
Q

Prolonged PT

Normal aPTT

A

Factor VII deficiency or inhib
Vit K def
Warfarin ingestion
DIC Liver disease

49
Q

Normal PT

Prolonged aPTT

A

Factor VIII, IX, XI, or XII
vWD (if severe and VIII low)
Heparin exposure

50
Q

Prolonged PT

Prolonged aPTT

A

Deficiency of II, V, X
severe liver dx, DIC, Vit K def, Warfarin tox
Heparin overdose

51
Q

what can you give mild hemophilia A patients for acute bleeding or prior to minimally invasive procedure?

A

Desmopressin

**also 1/3rd pt can have F VIII inhibitor which you can measure with bethesda assay, if low <5 units can overcome with factor replacement

52
Q

What can you give to prevent new skeletal-related events + improve survival in MM pts requiring therapy?

A

IV bisphosphonate – Zolendronic Acid or Pamindronate (indefinite)

53
Q

Heparin resistance, as evidenced by normal aPTT despite heparin administration. Causes?

A

Antithrombin Deficiency**
increase acute phase reactant protein (ie. Factor VIII) which will bind and neutralize heparin, others increase clearance of heparin,

54
Q

Cause of prolonged aPTT that does not fully correct with mixing study? Dx + Tx

A

presence of inhibitor
Usually think of autoantibody to factor VIII aka acquired hemophilia A (women>men)

Treatment - recombinant Factor VII

55
Q

Treating ITP in pregnancy

  • when
  • how
A

Plt <30,000

IVIG

56
Q

Tx of small cell lung CA with large centrally located tumors and no mets

A

Chemo + Radiation (cure 20-30%)

57
Q

Tx of high risk prostate CA? what makes them high risk?

A
  • GNRH agonist + radiation

- PSA >20, or Gleason score 8-10, or extraprostatic extension

58
Q

Tx of locally advanced head and neck CA

A

Cisplastin or Cetuximab + radiation

Cisplatin is c/i in kidney disease

59
Q

Treatment of early stage hormone receptor positive breast cancer in premenopausal woman

A

10yr of Tamoxifen (even if goes into post menopausal)

if already post menopausal then use aromatase inhibitors (lestrozole, anastrozole)

60
Q

Tx for classic Hodgkin Lymphoma

A

Chemotherapy (doxorubicin, bleomycin, vinblastine, dacarbazine)

61
Q

Name 4 classic and 1 non-classic type of Hodgkin Lymphoma

A
  1. nodular sclerosing
  2. mixed cellularity
  3. lymphocyte predominant
  4. lymphocyte depleted

non-classic = nodular lymphocyte-predominant subtype with CD20 cell surface Ag

62
Q

Treatment of advanced ovarian CA with germline BRCA1 and BRCA2 mutations previously treated with 3 or more lines of chemo

A

Olaparib - oral poly ADP polymerase inhibitor (PARP)

63
Q

Newly diagnosed metastatic nonsquamous, non-small cell, lung cancer – what should you first evaluate for before tx? why?

A

Activating Mutations

  • if EGFR mutation –> tx with erlotinib
  • if ALK and ROS1 mutations –> crizotinib

can start chemotherapy while awaiting results, but obtain tests b4 starting chemo

64
Q

What do you have to monitor while on aromatase inhibitors?

A

DEXA q2 years, if T score < - 2.5 start bisphosphonate

(anastrozole, letrozole, exmestane) - postmenopausal breast cancer adjuvant treatment

**aromatase inhibitors are c/i in premenopausal women

65
Q

When do you use adjuvant chemo in breast cancer?

A

triple negative (hormone receptor neg), HER2-positive, high grade, extensive lymphovascular invasion, and, positive nodes

66
Q

When is trastuzumab used? what screening do you have to get before starting?

A

Monoclonal antibody used for HER-2 positive breast cancer – used as adjuvant to chemotherapy

get eval of LV function (echo) before starting and monitor during

67
Q

Key treatment differences in SCLC vs. NSCLC

A

SCLC - usually systemic. For both get CT c/a/p + PET to stage.

SCLC - adding radiation to chemo only when limited stage disease. If extensive - offer chemo b/c can increase survival.

NSCLC - radiation in Stage I-III in addition to cisplastin or platinum based chemo. If stage IV (mets beyond contralat mediastinal LN) – chemo + immunotherapy with antiPDI checkpoint inhibitor. Offer chemo only if good performance status, otherwise no.

68
Q

What is a sister Mary Joseph node? Virchow Node?

A 
SMJN = periumbilical node 
VN = left supraclavicular node

both can be found in gastric cancer

69
Q

Treatment of

  • colon cancer
  • rectal cancer
A

COLON
- surgery – if single metastatic lesion to single organ, remove primary and mets lesion. Also of course if confined to colon (Stage I) or local invasion (Stage II)
- resection + chemo – mets to regional LN (stage III)
- chemo – distant mets (Stage IV)
adjuvant chemo == FOLFOX or CAPOX
for metastatic = FOLFOX and FOLFIRI – addition of bevacizumb mab against VEGF, increases efficacy of chemo, anti-EGFR monoclonal ab cetuximab or panitumumab (alone or in combo with chemo) - but cant use if have k-ras and n-ras mutation

RECTAL
- stage II-III preoperative radiation+chemo and postoperative chemo

70
Q

Treatment of anal cancer

A

Radiation + mitomycin + 5-FU

avoid surgery

71
Q

Treatment for advanced ovarian cancer

A

IV and intraperitoneal chemo (after debulking) - cisplatin and paclitaxel

16mo improvement in medial overall survival in stage III ovarian cancer if no residual mass >1cm after debulking surgery

72
Q

Neuroendocrine tumors
Pancreatic vs. Gastrointestinal NET (carcinoid)
- Treatment differences
- How to eval for somatostatin receptor?

A
  • appear same, treated different
  • Pancreatic NET - can tx with chemo, sunitinib (anti-VEGF ), everolimus (mTOR inhib)
  • GI NET - usu asx, just follow CT 3-6mo. no chemo.
  • surgery if symptomatic local tumors
  • can use Idium 111 pentetreotide scan to evaluate for somatostatin receptor and can treat with analogs (octreotide or lanreotide)
73
Q

Prostate Cancer Treatment

  • local vs. hormonal
  • castration sensitive vs. castration resistant in metastatic disease
A

Early stage gleeson <10 - active surveillance if life expectancy >5yr and not many commodities

Local = external beam radiotherapy/brachytherapy/radical prostatectomy

If high risk disease, need adjuvant ADT (androgen deprivation therapy) for 2-3 yrs (GNRH agonist - luprolide, goserelin)

Metastatic disease

(1) Castration sensitive disease
- — docetaxel + ADT

(2) Castration resistant (progresses despite ADT)
- – 1st line chemo= docetaxel + prednisone
- – ketoconazole, megesterol, glucocritcoids, estrogens
- – new anti-androgens - abiraterone & enzalutamide

74
Q

What does elevated AFP in testicular cancer indicate?

A

NONseminomatous tumor or tumor component –> must treat at such

(hCG can be elevated in seminomatous or nonseminomatous)

75
Q

Treatment for seminoma (testicular cancer)

  • stage I (low risk)
  • Stage II-III
  • PET scan positive residual masses
A

in general cisplatin-based chemo recommended over radiation

  • observation, if need tx give 1-2 doses of carboplatin chemo
  • II=III = cisplatin based chemo for intermediate, advanced, and nonpulmonary visceral mets
  • Residual masses PET scan positive should be resected
76
Q

Treatment for nonseminoma testicular CA

  • Stage I
  • Stage II-III
  • Bulky RP LN
  • Elevated Tumor markers but negative imaing
A
  • active surveillance, 1 cycle of cisplatin chemo, or retroperitoneal LN dissection
  • All the rest === cisplatin chemo
77
Q

Name 4 paraneoplastic syndromes related to Renal Cell Carcinoma

A

erythrocytosis
AA amyloidosis
polymyalgia rheumatica
hepatic dysfunction (unrelated to mets)

78
Q

Treatment for RCC

  • surgical
  • targeted
A
  • early/local - partial or radical nephrectomy
  • mets - debulking nephrectomy

Targeted

(1) VEGF inhib (bevacizuab) or VEGF tyr kinase inhib (sunitinib, sorafenib, pazopanib, axitinib)
(2) mTOR inhib (everolimus, temsirolimus)
(3) Immunotherapy w/ PD1 inhib (pembrolizumab, nivolumab)

79
Q

MEN 1
MEN 2A
MEN 2B

A

MEN 1 - pituitary, parathyroid, pancreatic NET

MEN 2 – Medullary thyroid CA (high calcitonin) + pheochromocytoma
2A - parathyroid
2B - mucosal neuromas (marfinoid too)

80
Q

What gene mutation should you test for in FNAB of thyroid nodule? why?

A

BRAF gene mutation - papillary carcinoma = more aggressive forms of cancer

81
Q

What germ line mutation is associated with medullary thyroid cancer? so what?

A

RET proto-oncogene

screen family mem bers for disease if positive

82
Q

Most common thyroid cancers? treatment?

A 
PFMA 
papillary (85%)
follicular (10%)
medullary (3%)
anaplastic (1%)
  • papillary and follicular = total thyroidectomy followed by radioiodine therapy
  • medullary = total thyroidectomy and neck dissection (no radioiodine b/c not taken up by c-cells)
83
Q

When to stop antibiotics for febrile neutropenia?

A

when ANC =/>500

84
Q

Neutropenia + RLQ abdominal pain – whats your concern?

A

Typhlitis (necrotizing enterocolitis) –> get CT abdomen

85
Q

When to start antifungal therapy in febrile neutropenia?

A

persistent fever despite 4-7 day of empiric broad spectrum antibiotics

86
Q

Treatment of Hairy Cell Leukemia

A

Cladribine (nucleoside analog, other is pentostatin)– complete and durable remission

(“dry tap” 2/2 marrow cell fibrosis + pancytopenia + progressive splenomegaly with NO lymphadenopathy

87
Q

What is Idelalisib?

A

P13K kinase inhibitor - oral agent for treatment of relapsed follicular lympohoma (once transformation is excluded)

88
Q

When to start breast cancer screening for patients who’ve had chest wall radiation?

A

If had chest wall radiation before the age of 30 years – screen @ 25yo or 8 yrs post radiation (whichever is latest)

89
Q

How do you treat high-grade or recurrent low-grade cancer?

A
  1. TURBT ..followed by..
  2. Intravesical chemo with BCG or mitomycin and periodic cystoscopies

If mets/muscle invasion - then cisplastin chemo

90
Q

How to treat metastatic recurrent breast cancer with E/P positive her HER2 negative? (previous adjuvant aromatase inhibitor tx)

A

Letrozole + palbociclib ( oral CDK4 and CDK6 inhib) synergistic

91
Q

You’ve diagnosed metastatic NSCLC, what do you now have to test for?

A

PD-L1 expression

b.c Pembrolizumab (MAD vs. PDL1) is superior to chemotherapy

92
Q

Steps to diagnose testicular cancer

A
  1. US to determine testicular mass
  2. bhCG and AFP levels
  3. orchiectomy to get tissue diagnosis
93
Q

BRAF

A

melanoma

94
Q

HER2

A

breast cancer

upper GI tumors

95
Q

RAS

A

colorectal cancer (EGFR monoclonal antibodies)

Medicine Boards (11 decks)

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