Hematological Malignancies

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giddyupp
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67160
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Hematological Malignancies
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2011-02-17 20:55:18
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Hematological Malignancies PHPR523 Test3
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Hematological Malignancies
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  1. How are Leukemias classified?
    • Disease acuity (acute vs chronic)
    • cell of origin (lymphoid vs myeloid)
  2. What types of Leukemia mainly affect older adults?
    • AML
    • CML
  3. What type of leukemia is associated with Auer rods?
    AML
  4. What type of leukemia is associated with hypertrophy of the gums?
    AML (M4 and M5 especially)
  5. What type of leukemia is associated with infiltration of the gingivae, skin, soft tissues and meninges?
    AML (M4 and M5 especially)
  6. What is the most important aspect of treatment to ensure best results?
    start chemotherapy within 72h
  7. What is the induction tx for AML in pts < 60yo?
    • 7 + 3:
    • Cytarabine + Anthracycline (Daunorubicin or Idarubicin)
    • HiDAC:
    • High-dose Cytarabine + Anthracycline (2-3g)
  8. What is the induction tx for AML pts 60yo and older?
    • 7 + 3:
    • Cytarabine + Anthracycline (Daunorubicin, Idarubicin, or Mitoxantrone)
    • Low-intensity:
    • SC Cytarabine
    • 5-Azacytidine
    • Decitabine
    • Intermediate-intensity:
    • Clofarabine
    • Best Supportive Care:
    • Hydroxyurea
    • Transfusions
  9. What are the SE of cytarabine?
    • Myelosuppression
    • N/V
    • Mucositis
    • Cerebellar toxicity (slurred speech, nystagmus, dysmetria) (HIGH DOSE)
    • Conjunctivitis/keratitis (HIGH DOSE)
  10. What are the SE of Anthracyclines?
    • Myelosuppression
    • N/V
    • Mucositis
    • Cardiac toxicity
    • Urine discoloration
  11. What is the appropriate supportive care for AML?
    • Transfusion for Hb < 8g/dL or any sx of anemia
    • Transfusion for PLT < 10,000/mm3 or any signs of bleeding
    • CSF for leukopenia after induction and consolidation tx (esp in > 55yo)
    • Rasburicase or Allopurinol with hydration for Tumor Lysis Syndrome
    • Fluconazole, SMZ/TMP, or Acyclovir prophylaxis if 0 WBC
    • Saline or steroid eye drops for conjunctivitis/keratitis
  12. What is the tx for CNS AML?
    • Methotrexate +/- Cytarabine intrathecally +/- RT
    • start concurrently with induction tx if CT/MRI or LP is positive at diagnosis
  13. What type of leukemia can affect any age?
    APL
  14. What is the most diagnostic feature of APL?
    presence of PML-RAR fusion protein
  15. What is the key sx of APL?
    severe coagulopathy
  16. What is the induction tx for APL?
    • ATRA (all-trans-retinoic acid; Tretinoin) +/- Cytarabine
    • ATRA + As2O3
  17. What are the SE of ATRA (all-trans-retinoic acid; Tretinoin)?
    • HA
    • Skin and membrane rxns
    • Bone pain
    • Nausea
    • APL differentiation syndrome (fever, rapidly rising WBC, SOB, hypoxemia, fluid retention, wt gain, pulmonary infiltrates, and pleural/pericardial effusions)
  18. What are the SE of As2O3?
    • QT prolongation
    • APL differentiation syndrome (fever, rapidly rising WBC, SOB, hypoxemia, fluid retention, wt gain, pulmonary infiltrates, and pleural/pericardial effusions)
    • Peripheral neuropathy
    • Fatigue
    • Wt gain
  19. What is the supportive care for APL?
    • Transfusion for PLT < 50,000/mm3
    • Dexamethasone prophylaxis or treatment for APL differentiation syndrome
    • NO CSF!!!!!!!
  20. What type of leukemia affects mainly children?
    ALL
  21. What type of leukemia is associated with the Philadelphia Chromosome?
    • CML
    • ALL (in adults)
  22. Why is adult ALL not treated as intensively/aggressively as it is in kids?
    adults are not able to tolerate tx as well
  23. What type of leukemia is associated with enlarged testicles?
    ALL
  24. What is the induction tx for ALL?
    • Corticosteroid (Dexamethasone, Prednisone, or Prednisolone)
    • Vincristine
    • Anthracycline
    • Aspariginase
    • Cytarabine
    • Cyclophosphamide
    • CNS prophylaxis always req'd (intrathecal Methotrexate and/or Cytarabine, or triple tx with hydrocortisone; or High-dose systemic Methotrexate or Cytarabine)
    • Ph+ ALL: include TKI
    • T-Cell ALL: include Nelarabine
  25. What is the supportive care for ALL?
    • Transfusion for Hb < 8 g/dL or any sx of anemia
    • Transfusion for PLT < 10,000/mm3
    • CSF after induction for leukopenia
    • Allopurinol or Rasburicase and hydration for Tumor Lysis Syndrome
    • Acyclovir or SMZ/TMP for infection prophylaxis if 0 WBC
    • Leucovorin + NaCl urine alkalinzationfor Methotrexate rescue
    • MESNA to prevent hemorrhagic cystitis from Cyclophosphamide
  26. What type of leukemia transitions from <10% blasts to >30% blasts in the bone marrow?
    CML
  27. What is the normal level of blasts in the bone marrow?
    < 5%
  28. What type of leukemia is associated with thrombocytosis (increased platelet count)?
    CML
  29. What type of leukemia is associated with blood hyperviscosity?
    CML d/t thrombocytosis
  30. What does the Philly chromosome cause?
    mutant tyrosine kinase which leads to uncontrolled cell proliferation
  31. What is the tx for CML?
    • Chronic Phase:
    • Imatinib
    • Dasatinib
    • Nilotinib
    • Accelerated Phase:
    • Dasatinib
    • Nilotinib
    • Blast Phase:
    • Cytarabine + Anthracycline +TKI
    • Corticosteroid + Vincristine + Anthracycline + TKI
  32. What should be done if Imatinib resistance develops?
    • Primary resistance (lack of efficacy at start): switch to Nilotinib or Desatinib
    • Secondary resistance (loss of efficacy over time): increase Imatinib dose
  33. What is the supportive tx for CML?
    • Neutropenia: hold drug until ANC at least 1000-1500/mm3 then resume or reduce dose; CSF
    • Thrombocytopenia: hold drug until PLT at least 50,000 - 75,000/mm3 then resume or reduce dose
    • Anemia (grade 3-4): RBC transfusions
    • Diarrhea: supportive care
    • GI upset: take with meal and large glass of water, divide dose
    • Rash: topical/systemic steroids, dose reduction, interruption, or d/c
  34. What type of lymphoma is associated with Reed-Sternberg cells?
    Hodgkin's lymphoma
  35. What are the sx of Hodgkin's Lymphoma?
    • Lymphadenopathy (usually painless)
    • Systemc B sx (fever, night sweats, wt loss)
    • Chronic pruritis
    • Extranodal involvement (spleen, lungs, liver, and bone marrow)
  36. What is the tx for classical Hodgkin's Lymphoma?
    • Early stage: RT + chemo
    • Advanced state: chemo
    • Chemotherapy:
    • ABVD (Doxorubicin, Bleomycin, Vinblastine, and Dacarbazine)
    • Stanford V (Doxorubicin, Vinblastine, Mechlorethamine, Etoposide, Vincristine, Bleomycin, Prednisone)
    • BEACOPP (Bleomycin, Etoposide, Doxorubicin, Cyclophosphomide, Vincristine, Procarbazine, Prednisone) - reserved for high-risk disease d/t greater long-term toxicity
  37. When does Hodgkin's Lymphoma primarily strike?
    15-30 and >55 yo (BIMODAL)
  38. When does Non-Hodgkin's Lymphoma strike?
    any age
  39. What is the tx for asymptomatic early-stage CLL (non-Hodgkin's Lymphoma)?
    observation and supportive care
  40. What is the tx for symptomatic or high-risk CLL (non-Hodgkin's Lymphoma)?
    • Chlorambucil
    • Cyclophosphamide
    • Bendamustine
    • Fludarabine
    • Cladribine
    • Pentostatin
    • Rituximab
    • Alemtuzumab
  41. What type of lymphoma is associated with Autoimmune disease?
    CLL (non-Hodgkin's)
  42. What type of lymphoma is associated with secondary malignancy?
    CLL (non-Hodgkin's)
  43. What type of lymphoma is associated with Richter transformation?
    CLL (non-Hodgkin's)
  44. What is the supportive tx for CLL (non-Hodgkin's Lymphoma)?
    • Blood product support
    • Rasburicase or Allopurinol with hydration for Tumor Lysis Syndrome
    • Corticosteroids, Rituximab, IVIG, Cyclosporine, Splenectomy, Eltrombopag, or Romiplostim for Autoimmune cytopenias
    • SMZ/TMP, Acyclovir, or Gancyclovir prophylaxis for infections
    • Vaccinations (annual influenza, pneumococcal q 5yrs) (Avoid live vaccines)

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