Leukemia

Question 1

• Neoplastic proliferation of blasts often leads to?
• Normal blast range in bone marrow? Leukemia?
• Signs and symptoms acute?
• See what on smear? Nucleoli? Cytoplasm?
  1. ) Scheme of AML? (2)
  2. ) Signs its a myeloproliferative neoplasm? (2) Transform to AML?
  3. ) Signs it’s a myelodysplastic syndrome (MDS)? (4) Transform to AML?

Answer 1

• Crowding out other lineages
• 1-2%; >20%
• Yes
• Large, immature cells; punched out; not much
  1. ) >/= 20% immature myeloid cells in blood or marrow; presence of cyto markers
  2. ) Persistant increase of 1 or more myeloid lineages; /= 1 lineage; blasts

Question 2

MPN Schematic:

• MPN to CML signs? (3)
• Not CML to PV signs? (4)
• Not CML to Not PV sign?
• Not PV to ET signs? (3)
• Not PV to PMF signs? (3)

Answer 2

• BCR-ABL positive, high neutro’s and baso’s; maybe increased ptt’s
• High RBC; JAK2 mutation; high neutro’s and ptts
• Normal RBC
• High ptt’s with normal neutro’s; megakaryocytosis; +/- JAK2 mutation
• High ptts and high neutro; bizare big cells; +/- JAK2 mutation

Question 3

• Hematologic malignancies often arise from?
• Acute leukemia: Progressive? Cells typically what? Often present with? (3)
• Chronic leukemia: Increased what? Why? Symptoms at diagnosis? Chance of going to acute?
• High grade vs low grade lymphoma?
• Cyto markers in many leukemias? What else can play role? (3)
• Epi of leukemia in kids? Lymphoma? Adults?
• WHO scheme uses?
• MDS: Marrow often taken over by?
• MPN: Marrow taken over by?
• Hodgkin lymphoma?
• Non? Cell is more common?

Answer 3

• Clonal malignant population of cells
• Yes; blasts; low N’s, ptts, RBC’s
• WBC count; accumulation of mature normal cells; sometimes none; rare
• Rapidly enlarging (usually with acute) vs. not
• translocations are common; enviro, virus, chemo
• Leukemia = 1 Lymph = 3; Leu = 6 Lym = 7
• multi parameter
• Clone that makes abnormal cell
• Clone makes too many normal cells
• Derived from B cells with unique cell
• Malignancy of mature lymphocytes; B cell

Question 4

• AML/ALL presentation? (3)
  1. ) ALL: 75% of cases? Require a percentage blast? Immunophenotyping helps distinguish what?
• Markers: Lympho/myelo blasts have what? Only on immature lympho? B cell? (2) T cell? (2)
  a. ) B-ALL: Percent of ALL? Usually lack what marker? Common in who? Common trnaslocation? 3 common cyto findings and related prognosis?
  b. ) T-ALL: % of ALL? HIgh frequency in? Males or females? May also have?
• Treatment: Who has better prognosis? B lymphoblastic hyperdiploidy? Hypodiploid?

Answer 4

• Anemia, thrombocytopenia, netropenia

- Kids

Question 5

• AML: More common in who? Defined how? Common markers? (2) Lympho/myelo blast differentiation? But?
• What is used to diagnose?
  1. ) RUNx1-RUNX1T1: Trans? Prognosis? % cases?
  2. ) CBFB: Trans? Prognosis? % cases? Abnormal? (3)
  3. ) RARA: Trans? Prognosis? % cases? Called? Morph? Risk? Treatment?
  4. ) Often seen with downs trans? Prognosis?
  5. ) MLL: Trans? Prognosis? What lineage?
• Treatment options? (2) Risk?
• Other markers? FLT3? NPM1? CEBPA? Trumps?

Answer 5

• Adults; >20% blasts; CD34 and CD117; tough to tell; may contain AUER rods
• Cytogenetic abnormalities
  1. ) t8:21; 5%; good
  2. ) t16:16; good; 5-10%; baso,eisino granules
  3. ) t15:17; good if treated; 5-10%; APL; hypergranular; DIC, retinoic acid
  4. ) t1:22; megakaryblastic; good
  5. ) t11:23; poor; monocytic
• Alkylating agents; topoisomerase inhibitors; problems later
• Bad prognosis; good; good; FLT3

Question 6

• MDS: Marrow replaced with? Ineffective what? Increased risk of? How many types?
  1. ) Primary (idiopathic): Common age?
  2. ) Secondary: Due to? Usually deletions where?
• Diagnosis: Morph evidence of? Increased? Common cyto abnormalities? (3)
• Potential pitfalls with morphology? (4)
• Low grade: Blasts in BM and blood? 2 types? Prognosis?
• High grade: Blasts in BM and blood? 2 types? Blasts? Prognosis?
• MPN vs. MDS: Multiple lineages with? Normal cells? Increased marrow cells? Insiduous onset? AML more common in? Spleno/hepatomegaly with?

Answer 6

• Malignant clone; hematopoieses; AML; 2
  1. ) over 50
  2. ) Therapy; chrom 5 or 7
• dysplasia; myoblasts (

Question 7

MPN’s:

1. ) CML: Translocation? Gene? WBC? Lots of? Basophils? Multiple breakpoints due to? Treatment? May need to add? Prognosis?
2. ) Polycythmia Vera: RBC mass? Mutation? Pitfalls? (3) Common complications? Treatment?
3. ) Primary myelofibrosis: Granulocytes? Megakaryocytes? Mutation? Leads to? RBC shape? Most deaths due to?
4. ) Essential thrombocytopenia: Granulocytic hyperplasia? Mutation? Megakaryocytes? Splenomegaly? Symptoms? High risk of?

Answer 7

1. ) t9:22; BCR:ABL; 12k-1 mil; neutrophils; high as well; alt. splicing; Imatinib; 2nd/3rd gen therapy; good
2. ) High; JAK2; smokers, chronic hypoxia, Hb disorders; clots in BIG arteries; blood letting
3. ) High; high; JAK 2 50%; extramedullary hemato.; darco teardrop; bone marrow failure
4. ) No; 50% JAK; even bigger; no, often non; ischemia