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3rd Year (Step 2) > Heme/Onc 2 > Flashcards

Heme/Onc 2 Flashcards

1
Q

What is Polycythemia Vera?

A

Unregulated overproduction of all 3 cell lines
- RBC overproduction is most prominent
(Hematocrit > 60%)

  • elevated B12 w/ low MCV also seen
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2
Q

Polycythemia Vera – genetic mutation?

A

JAK2 protein mutation (most accurate Dx test)

- RBCs grow wildly despite low erythropoietin

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3
Q

Polycythemia Vera – presentation?

A

Sx of hyper viscosity from inc’d RBC mass:

  • Headache, blurred vision, tinnitus
  • HTN
  • Fatigue
  • Splenomegaly
  • Bleeding from engorged blood vessels
  • Thrombosis from hyper viscosity
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4
Q

How to differentiate Polycythemia Vera vs. other causes of inc’d hematocrit?

A
  • Renal Cell cancer has inc’d erythropoietin (PV is dec’d)

- Hypoxia has low oxygen (PV is nl oxygen)

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5
Q

Polycythemia Vera – Tx?

A
  • Phlebotomy & aspirin prevent thrombosis
  • Hydroxyurea helps lower cell count
  • Allopurinol or Rasburicase protects against uric acid rise
  • Antihistamines
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6
Q

Why does pruritis often follows warm showers in myeloproliferative diseases?

A

b/c of histamine release from increased numbers of basophils

  • Polycythemia Vera, CML, etc.
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7
Q

Essential Thrombocytosis – what is it?

A

Markedly elevated platelet count > 1 million

- leads to both Thrombosis & Bleeding

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8
Q

Essential Thrombocytosis – when do you treat?

A

If pt < 60yrs & asymptomatic – No Tx

if pt > 60 & symptomatic – Treat w/:

  • Hydroxyurea (best initial Tx)
  • Anagrelide used when Hydroxyurea causes RBC suppression
  • Aspirin used for erythromelalgia (painful, red hands)
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9
Q

Essential Thrombocytosis – best initial Tx?

A

Hydroxyurea = best initial Tx

  • Anagrelide used when Hydroxyurea causes RBC suppression
  • Aspirin used for erythromelalgia (painful, red hands)
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10
Q

Myelofibrosis – what is it?

A

Disease of older persons w/ pancytopenia ass’d w/ bone marrow showing marked fibrosis
- Blood production shifts to spleen & liver, which become markedly enlarged

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11
Q

Myelofibrosis – lab findings?

A

Blood smear shows:

  • Teardrop cells
  • Nucleated RBCs
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12
Q

Myelofibrosis – Tx?

A

Thalidomide & Lenalidomide are TNF-inhibitors that increase bone marrow production

  • if pt presents < 50-55yrs., allogeneic bone marrow transplantation is attempted
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13
Q

Acute Leukemia – presentation?

A

Signs of Pancytopenia (fatigue, infection, bleeding) even though WBC count is normal or inc’d

  • infections b/c leukemic cells (blasts) do not function properly, even though numbers are normal
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14
Q

Leukemia ass’d w/ DIC?

A

Acute Promyelocytic Leukemia (M3)

- also ass’d w/ Auer Rods (eosinophilic inclusions
translocation between chromosomes 15 & 17)

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15
Q

Acute Leukemia – best initial test?

A

Blood smear showing blasts

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16
Q

Acute Leukemia – most accurate test?

A

Flow cytometry

- detects specific CD subtypes ass’d w/ each type of leukemia

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17
Q

Myeloperoxidase is characteristic of which type of leukemia?

A

Acute Myelocytic Leukemia (AML)

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18
Q

Drug that prevents tumor lysis related rise in uric acid?

A

Rasburicase

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19
Q

M3 (AML) – Tx?

A

All-Trans-Retinoic Acid (ATRA)

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20
Q

Drug that prevents relapse of ALL in CNS?

A

Methotrexate

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21
Q

CML – presentation?

A

Persistently high WBC count that’s all neutrophils

  • Pruritis common after warm showers
  • Splenomegaly w/ early satiety, abdominal fullness, & LUQ pain
  • Vague Sx: fatigue, night sweats, fever (hyper metabolic)
  • High WBC on routine exam
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22
Q

How to differentiate CML vs. leukemoid rxn, when pt has high WBC count?

A

CML = low LAP

Reactive Leukocytosis (infection, stress) = high LAP

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23
Q

CML – Tx?

A

Best initial Tx = Tyrosine Kinase inhibitors – Imatinib, Dasatinib, Nilotinib

  • BMT can cure, but shouldn’t be 1st therapy
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24
Q

Acute Leukostasis Reaction – what is it?

A

WBC count > 100,000/microL

  • abnormal intravascular leukocyte aggregation and clumping
  • Emergency – must undergo Leukapheresis
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25
Q

Myelodysplastic Syndrome (MDS) – what is it?

A

Preleukemic disorder presenting in older pts (> 60)
- Pancytopenia despite a hyper-cellular bone marrow

  • Complications of infections & bleeding usually lead to death before leukemia occurs
26
Q

MDS – presentation?

A 
> 60 yrs. old patient w/ asymptomatic pancytopenia on routine CBC.
Sx that do occur are:
- Fatigue & weight loss
- Infection
- Bleeding
- Sometimes splenomegaly
27
Q

MDS – Dx tests?

A

CBC: anemia w/ inc’d MCV, Nucleated RBCs, & small # of blasts

  • Pelger Huet Cells (Bilobed nucleus – most distinct for MDS)
  • Marrow: hyper-cellular
  • Prussian blue stain shows ringed sideroblasts
  • Severity based on % of blasts
28
Q

CLL – what is it?

A

Clonal proliferation of normal, mature-appearing B lymphocytes that FUNCTION ABNORMALLY

(i. e. don’t make antibodies for infection when necessary, but do make them against self RBCs, platelets, etc.)
- 90% of pts > 50 yrs.

29
Q

CLL – Sx?

A

Many are asymptomatic @ presentation w/ only a markedly elevated WBC count (80-98% lymphocytes)

  • Fatigue (most common symptom)
  • Lymphadenopathy (80%)
  • Spleen or Liver enlargement (50%)
  • Infection from poor lymphocyte function
30
Q

PCP prophylaxis is indicated in which leukemia?

A

CLL

31
Q

Hairy Cell Leukemia (HCL) – presentation?

A

Middle aged men w/:

  • Pancytopenia
  • Massive splenomegaly
  • Monocytopenia
  • Inaspirable “dry” tap despite hypercellularity of the marrow
32
Q

HCL – best initial & most accurate test(s)?

A

Best initial = smear showing hairy cells (B-cells with filamentous projections)

Most accurate = Immunotyping by flow cytometry (e.g. CD11c)

33
Q

HCL – Tx?

A

Cladribine or Pentostatin

34
Q

Non-Hodgkin Lymphoma – what is it?

A

Proliferation of lymphocytes in the lymph nodes & spleen

  • extremely similar to CLL, except NHL is a solid mass & CLL is “liquid” or circulating
35
Q

NHL – presentation?

A

Painless Lymphadenopathy

  • May involve pelvic, retroperitoneal, or mesenteric structures
  • Nodes NOT red, warm, tender (would be infection)
  • “B” symptoms (fever, weight loss, night sweats)
36
Q

What are “B” symptoms?

A

Fever, weight loss, drenching night sweats

37
Q

MALT – what is it?

A

Mucosal Associated Lymphoid Tissue

- Lymphoma of the stomach in association w/ H. pylori

38
Q

MALT – Tx?

A

Treat the Helicobacter w/ Clarithromycin & Amoxicillin

39
Q

Hodgkin vs. Non-Hodgkin Lymphoma:

Severity on presentation?

A

HD = Local, stage 1, & stage 2 in 80-90%

NHL = Stage 3 & 4 in 80-90%

40
Q

Hodgkin Disease vs. Non-Hodgkin Lymphoma:

Location?

A

HD = Centers around cervical area

NHL = Disseminated

41
Q

Hodgkin Disease vs. Non-Hodgkin Lymphoma:

Pathology (cells)?

A

HD = Reed-Sternberg cells on pathology

NHL = No R-S cells on pathology

42
Q

Hodgkin Disease – which types have the best & worst prognosis?

A

Best = Lymphocyte predominant

Worst = Lymphocyte depleted

43
Q

NHL – which types have the worst prognosis?

A

Burkitt & Immunoblastic have the worst prognosis

44
Q

Doxorubicin – Toxicity?

A

Cardiomyopathy

45
Q

Vincristine – Toxicity?

A

Neuropathy

46
Q

Bleomycin – Toxicity?

A

Lung fibrosis

47
Q

Cyclophosphamide – Toxicity?

A

Hemorrhagic cystitis

48
Q

Cisplatin – Toxicity?

A

Renal & Ototoxicity

49
Q

Multiple Myeloma – what is it?

A

Abnormal proliferation of plasma cells that produce useless IgA & IgG

50
Q

Waldenstrom Macroglobulinemia – what is it?

A

Abnormal proliferation of plasma cells that produce non-functional IgM, which clog up the kidney

51
Q

Multiple Myeloma – presentation?

A
  • Bone pain from pathologic fractures (most common pres)
  • Hypercalcemia
  • Hyeruricemia (inc’d turnover of nuclear material of plasma cells)
  • Anemia (large #s of plasma cells infiltrate BM)
  • Renal failure (accumulation of Igs & Bence-Jones proteins + hypercalcemia/uricemia)
52
Q

Multiple Myeloma – Dx tests?

A
  • X-ray showing lytic lesions
  • SPEP shows IgG or IgA spike (“M” spike)

additional:

  • urine immunoelecrophoresis shows Bence Jones protein (NOT seen on dipstick)
  • smear w/ Rouleaux
  • BM biopsy w/ > 10% plasma cells defines myeloma
  • elevated total protein w/ normal albumin
53
Q

MM –Tx?

A

Best initial Tx = Dexamethasone + Lenalidomide, Bortezomib, or both

  • Melphalan is useful in older, fragile patients who cannot tolerate AEs
54
Q

MGUS – what is it?

A

Asymptomatic monoclonal expansion of plasma cells w/ M spike

- Precursor to Multiple Myeloma (1-2%/yr)

55
Q

CML Dx – most accurate test?

A

Bcr-Abl

via PCR or FISH on peripheral blood

56
Q

CLL treatment?

A

Fludarabine
( + Rituximab if offered)

Refractory: Cyclophosphamide
Mild: Chlorambucil
Severe infection: IVIG
Autoimmune thrombocytopenia or hemolysis: Prednisone

57
Q

CML & CLL age groups?

A

CML: 30 - 60

CLL: > 50

58
Q

NHL: diagnostic testing?

A

Excisional biopsy
(not needle aspiration b/c individual lymphocytes appear normal)

  • then do staging to determine intensity of Tx (CT chest/abdomen/pelvis & bone marrow biopsy)
  • CBC is normal in most cases
59
Q

NHL treatment?

A

Local stage 1 disease: local radiation & small dose chemo

Stage 3 & 4: combo V-PAC + Rituximab (or CARV-P)
(Vincristine, Prednisone, Adriamycin, Cyclophosphamide)

60
Q

Hodgkin Disease chemo regimen for stage 3 & 4, or if “B” symptoms are present?

A

ABVD

Adriamycin, Bleomycin, Vincristine, Dacarbazine

3rd Year (Step 2) (44 decks)

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