Systems - Heme - Malignancies

  1. What Ab are you looking for in inflammatory joint diseases?
    ANA - anti-nuclear Ab
  2. What is gaucher's disease and how would you diagnose?
    • -lysosomal storage disease
    • - "gaucher's cells" in BM, macrophages with too many lipids
  3. An example of why you would see lymphadenopathy in:
    inguinal, axillary, R supra, L supra, ant cervical, post cervical
    • inguinal: cervical cancer
    • axillary: breast cancer
    • R supra: malig of esophagus
    • L supra: gastric ca
    • ant cervical: infectious
    • post cerv: malignant
  4. What are B symptoms (literally B-cell Sx)
    • fever
    • night sweats
    • weight loss
    • fatigue
  5. List some causes of generalized lymphadenopathy
    • Infectious: HIV, TB, mono
    • Inflammation: SLE
    • malignancy
    • drugs
    • amyloidosis
  6. What is on the DDx for reactionary lymphocytosis?
    • infection
    • physiological stress
    • hypersensitivity to drugs
    • autoimmune

    HAPI mnemonic
  7. What is on the DDx for clonal lymphocytosis?
    • monoclonal B-cell lymphocytosis (new disease)
    • chronic lymphocytic leukemia
    • other B or T cell lymphomas
  8. When does Hodgkin's lymphoma happen?
    bimodal peaks: one at 20, the other at 65
  9. What is the clinical triad for MM
    • high M-protein
    • osteolytic bone lesions
    • ++ myeloma cells in BM
  10. What are the big 3 to watch out for in heme malignancies
    anemia, leukocytosis, thrombocytopenia
  11. What are the CRAB criteria for MM?
    • Ca++ elevated
    • Renal problems
    • Anemia
    • Bone disease (lesions)
  12. 6 major complications of all MPDs (MPNs)
    • clonal evolution (e.g. AML)
    • thrombosis
    • bleeding
    • B symptoms
    • Pruritis
    • bone pain
  13. Where do you see auer rods and granules?
  14. Clinical presentation of acute leukemia
    • -B symptoms
    • -SHORT (weeks to a few months) duration of symptoms
    • -Big 3 (anemia, leukopenia (especially neutropenia), thrombocytopenia)
    • -sometimes tissue infiltration and leukostasis
  15. When is acute leukemia an especially emergent condition?
    When there is DIC or tumor lysis syndrome
  16. incidence peaks of ALL and AML in kids
    • ALL: 2-4
    • AML: teens
  17. Describe the chemo therapy in ALL
    • 1) early intensive chemo to induce remission
    • 2) long term chemo to prevent resistance and relapse
  18. List three important aspects of palliative care
    • -all patients with a progressive incurable illness could benefit from PC
    • -PC applies to all ages
    • -PC constitutes active care guided by family's expectations
  19. List some reactive and clonal causes of lymphocytosis
    • Reactive:
    •   -Infection
    •   -physiologic stress response
    •   -hypersensitivity
    •   -autoimmune
    • Clonal:
    •   -monoclonal B-cell lymphocytosis (<5)
    •   -CLL
    •   -Other B or T cell leukemias
  20. In which hematological malignancy do you often test positive for Philadelphia chromosome?
    Philadelphia *C*rea*ML* cheese

  21. When diagnosing polycythemia vera what are two good tests?
    • 1) ABG to rule out hypoxia
    • 2) test for a JAK2 mutation
  22. What hematological malignancy has Auer rods?
  23. What is the most common childhood malignancy? Prognosis?

    "smALL people"

    Good prognosis (85% remission with chemo)
  24. How do you treat CLL?
    No chemo until they are symptomatic, then chemo is used only in a palliative manner
  25. If you see a patient come in with a VERY high WBC count (>500) with blasts, what should you be thinking?
  26. Describe tumor lysis syndrome
    During Tmt of hematological malignancy, rapic cellular death releases intracellular contents, causing a ton of electrolyte disturbances
  27. What is the diagnostic test of choice for NHL and HL?
    excisional LN biopsy!
  28. Which leukemia would you expect to see in the following ages?
    < 13 years (but can present at any age group)
    40-60 years
    60+ years
    < 13 years (but can present at any age group): ALL

    • 40-60 years: CML
    • 60+ years: CLL, AML
  29. How do you treat indolent NHL? aggressive NHL?
    • indolent: palliative chemo, symptomatic
    • aggressive: aggresively with a curative approach
  30. Describe the ages you most likely will see Hodgkin's lymphoma
    bimodal age dist

    3rd decade and around 60
  31. What is the classic triad of diagnostic criteria for Multiple Myeloma
    • >10% plasma cells in the bone marrow
    • M protein in serum or urine (not enough to dx on its own)
    • Bone lesions
  32. ALL is associated with which syndrome?

    We ALL go DOWN together
  33. What is the definition of acute leukemia?
    greater than 20% blasts in the BM at presentation
  34. What type of anemia does MDS cause?
  35. What mutation is common to PV, IMF, and ET? What is the incidence in each?
    • Jak2
    • PV: 95%
    • IMF: 50%
    • ET: 50%
  36. What are 3 things you see in idiopathic myelofibrosis?
    • -teardrop cells
    • -dry BM aspirate
    • -leukoerythroblastic blood film (RBC and granulocyte precursors)
  37. treatment of ALL vs AML? ALL treatment
    ALL tmt: induction->consolidation->maintenance->CNS prophylaxis

    • ALL vs AML
    • 1) no maintenance in AML
    • 2) no CNS prophylaxis in ALL
  38. how does HL present? (6 chars)
    painless, non-tender, firm, rubbery, enlargement of superficial cervical LN's
  39. What cells are used to pathognomonically differentiate NHL from HL?
    Reed-Sternburg cells are present in HL
  40. What is the goal of NHL treatment in indolent NHL? aggresive NHL? tmt for each?
    • indolent NHL: palliative/watchful waiting, R-CVP
    • aggressive NHL: curative, R-CHOP
  41. What does tmt of HL depend on? NHL?
    HL depends on staging, NHL depends on hisological subtype (indolent or aggressive)
  42. What is the natural history of CLL? What is it?
    malignancy of mature B-cells

    indolent, but incurable with a slow progression
  43. What is the triad that you are looking for in the serum and the bones in multiple myeloma
    • serum: spike in serum protein (M-protein)
    • Bone marrow: >10% plasma cells
    • osteolytic bone lesions
  44. How is MGUS different from MM? same? how many undergo malignant transformation per year?
    both have spike in M protein

    MGUS doesn't have CRAB (same with SMM), <10% plasma cells in marrow

    1% undergo transformation per year
  45. How much does one unit of pRBCs raise Hct by? Hb?
    • Hct: 4%
    • Hb: 10 g/L
  46. List the three categories of NHL-associated conditions with an example of each.
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Systems - Heme - Malignancies
Systems - Heme - Malignancies