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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
Myelodysplastic Syndrome (MDS) -- what is it?
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
MDS -- presentation?
``` > 60 yrs. old patient w/ asymptomatic pancytopenia on routine CBC. Sx that do occur are: - Fatigue & weight loss - Infection - Bleeding - Sometimes splenomegaly ```
27
MDS -- Dx tests?
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
CLL -- what is it?
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
CLL -- Sx?
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
PCP prophylaxis is indicated in which leukemia?
CLL
31
Hairy Cell Leukemia (HCL) -- presentation?
Middle aged men w/: - Pancytopenia - Massive splenomegaly - Monocytopenia - Inaspirable "dry" tap despite hypercellularity of the marrow
32
HCL -- best initial & most accurate test(s)?
Best initial = smear showing hairy cells (B-cells with filamentous projections) Most accurate = Immunotyping by flow cytometry (e.g. CD11c)
33
HCL -- Tx?
Cladribine or Pentostatin
34
Non-Hodgkin Lymphoma -- what is it?
Proliferation of lymphocytes in the lymph nodes & spleen - extremely similar to CLL, except NHL is a solid mass & CLL is "liquid" or circulating
35
NHL -- presentation?
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
What are "B" symptoms?
Fever, weight loss, drenching night sweats
37
MALT -- what is it?
Mucosal Associated Lymphoid Tissue | - Lymphoma of the stomach in association w/ H. pylori
38
MALT -- Tx?
Treat the Helicobacter w/ Clarithromycin & Amoxicillin
39
Hodgkin vs. Non-Hodgkin Lymphoma: | Severity on presentation?
HD = Local, stage 1, & stage 2 in 80-90% NHL = Stage 3 & 4 in 80-90%
40
Hodgkin Disease vs. Non-Hodgkin Lymphoma: | Location?
HD = Centers around cervical area NHL = Disseminated
41
Hodgkin Disease vs. Non-Hodgkin Lymphoma: | Pathology (cells)?
HD = Reed-Sternberg cells on pathology NHL = No R-S cells on pathology
42
Hodgkin Disease -- which types have the best & worst prognosis?
Best = Lymphocyte predominant Worst = Lymphocyte depleted
43
NHL -- which types have the worst prognosis?
Burkitt & Immunoblastic have the worst prognosis
44
Doxorubicin -- Toxicity?
Cardiomyopathy
45
Vincristine -- Toxicity?
Neuropathy
46
Bleomycin -- Toxicity?
Lung fibrosis
47
Cyclophosphamide -- Toxicity?
Hemorrhagic cystitis
48
Cisplatin -- Toxicity?
Renal & Ototoxicity
49
Multiple Myeloma -- what is it?
Abnormal proliferation of plasma cells that produce useless IgA & IgG
50
Waldenstrom Macroglobulinemia -- what is it?
Abnormal proliferation of plasma cells that produce non-functional IgM, which clog up the kidney
51
Multiple Myeloma -- presentation?
- 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
Multiple Myeloma -- Dx tests?
- 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
MM --Tx?
Best initial Tx = Dexamethasone + Lenalidomide, Bortezomib, or both - Melphalan is useful in older, fragile patients who cannot tolerate AEs
54
MGUS -- what is it?
Asymptomatic monoclonal expansion of plasma cells w/ M spike | - Precursor to Multiple Myeloma (1-2%/yr)
55
CML Dx -- most accurate test?
Bcr-Abl | via PCR or FISH on peripheral blood
56
CLL treatment?
Fludarabine ( + Rituximab if offered) Refractory: Cyclophosphamide Mild: Chlorambucil Severe infection: IVIG Autoimmune thrombocytopenia or hemolysis: Prednisone
57
CML & CLL age groups?
CML: 30 - 60 CLL: > 50
58
NHL: diagnostic testing?
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
NHL treatment?
Local stage 1 disease: local radiation & small dose chemo Stage 3 & 4: combo V-PAC + Rituximab (or CARV-P) (Vincristine, Prednisone, Adriamycin, Cyclophosphamide)
60
Hodgkin Disease chemo regimen for stage 3 & 4, or if "B" symptoms are present?
ABVD Adriamycin, Bleomycin, Vincristine, Dacarbazine

3rd Year (Step 2) (44 decks)

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