MMSC 433 Exam 3 PDF

MMSC 433 Exam 3 Study online at https://quizlet.com/_g1f5rb 1. chronic myeloid Philadelphia chromosome (~95% of cases) leukemia (CML) chro- mosomal abnormality 2. polycythemia vera (PV) JAK2V617F mutation (most cases) chromosomal abnor- mality 3. chronic myelo...

MMSC 433 Exam 3 Study online at https://quizlet.com/_g1f5rb 1. chronic myeloid Philadelphia chromosome (~95% of cases) leukemia (CML) chro- mosomal abnormality 2. polycythemia vera (PV) JAK2V617F mutation (most cases) chromosomal abnor- mality 3. chronic myeloprolifer- -chronic myeloid leukemia (CML) ative disorders -polycythemia vera (PV) -essential thrombocythemia (ET) -primary myelofibrosis (PMF) -chronic neutrophilic leukemia (CNL) -chronic myelomonocytic leukemia (CMML) -juvenile myelomonocytic leukemia (JMML) 4. polycythemia vera ma- -hemoglobin >16.5 g/dL (men), >16 g/dL (women) jor criteria (WHO) -Hct >49% (men), >48% (women) -increased RCM >35 mL/kg (men), >31 mL/kg (women) -BM biopsy with hypercellularity and panmyelosis -presence of JAK2V617F or JAK2 Exon 12 muta- tions 5. polycythemia vera mi- subnormal serum EPO levels nor criteria (WHO) 6. What is the etiolo- A single genetic translocation in a pluripotent gy of chronic myeloid hematopoietic stem cell causes clonal overproduc- leukemia (CML)? tion of immature neutrophils, usually diagnosed be- tween age 45-55. 7. What chromosomal Philadelphia chromosome. abnormality is asso- ciated with chron- ic myeloid leukemia (CML)? 8. What morphological Myeloblasts, promyelocytes, bands, segmented features are seen in neutrophils (all stages of neutrophil development), 1 / 15  MMSC 433 Exam 3 Study online at https://quizlet.com/_g1f5rb the peripheral blood nRBCs, gaucher-like macrophages, and very small of patients with chron- megakaryocytes. ic myeloid leukemia (CML)? 9. What are common Infections with Strep pneumoniae, anemia, bleed- symptoms of chron- ing, and splenomegaly. ic myeloid leukemia (CML)? 10. What are the periph- RBCs normal to increased, reticulocytes nor- eral blood findings mal, total WBCs increased, granulocytes increased in chronic myeloid (neutrophils, basophils, eosinophils), platelets nor- leukemia (CML)? mal to increased, LAP stain decreased. 11. What are the bone mar- Hypercellular, increased granulopoiesis, de- row findings in chron- creased erythropoiesis, increased megakary- ic myeloid leukemia opoiesis, and increased reticulin fibers. (CML)? 12. chronic myeloid chronic phase (3-4 years) ’ accelerated phase ’ blas- leukemia (CML) stages tic phase ’ possible transition to acute leukemia (poor prognosis) 13. chronic myeloid -BM transplant or SCT= CURE leukemia (CML) treat- -*gleevec* (imatnib mesylate) ments -sprycel, dasigna, bosulib, iclusig 14. What is the etiology Neoplastic clonal stem cells mature independently of polycythemia vera of EPO stimulation, causing panmyelosis with a (PV)? predominant increase in erythrocytes. 15. What chromosomal JAK2V617F mutation. abnormality is associ- ated with polycythemia vera (PV)? 16. What is the morpholo- Normocytic/normochromic RBCs and teardrop gy of red blood cells cells (dacrocytes) present. 2 / 15  MMSC 433 Exam 3 Study online at https://quizlet.com/_g1f5rb in polycythemia vera (PV)? 17. What are the symp- Triad of bone marrow fibrosis, splenomegaly, and toms of polycythemia anemia with teardrop cells (dacrocytes). vera (PV)? 18. What are the lab find- Increased hemoglobin, hematocrit, and red cell ings in polycythemia mass; low serum EPO levels. vera (PV)? 19. What are the periph- Increased RBCs, total WBCs, granulocytes, and eral blood findings platelets; LAP stain normal to increased. in polycythemia vera (PV)? 20. What are the bone mar- Hypercellular bone marrow with increased nor- row findings in poly- moblasts, granulocytes, megakaryocytes, and reti- cythemia vera (PV)? culin fibers. 21. polycythemia vera (PV) -therapeutic phlebotomy: bring Hct d45% treatments -hydroxyurea -busulfan -JAK inhibitors: Ruxolitinib, Lestaurtinib 22. What is the etiology Clonal myeloproliferative neoplasm leading to in- of essential thrombo- creased megakaryopoiesis and thrombocytosis. cythemia (ET)? 23. What is the most com- JAK2V617F. mon chromosomal ab- normality associated with essential throm- bocythemia (ET)? 24. What are other com- CALR and MPL mutations. mon mutations as- sociated with essen- tial thrombocythemia (ET)? 3 / 15  MMSC 433 Exam 3 Study online at https://quizlet.com/_g1f5rb 25. What are the charac- Giant, agranular platelets and platelet clusters. teristic morphological features of platelets in essential thrombo- cythemia (ET)? 26. What are common Vascular occlusion in digits, veins & arteries, symptoms of essen- splenomegaly, erythromyelalgia. tial thrombocythemia (ET)? 27. What is the typi- Increased platelet count (e450*10y/L), slightly de- cal peripheral blood creased Hgb/Hct, normal RBC count, normal to (PB) finding in essen- increased total WBCs, increased segmented neu- tial thrombocythemia trophils, decreased platelet function. (ET)? 28. What are the bone Hypercellular, increased megakaryocytes, marrow (BM) findings large/variably sized megakaryocytes with hyperlob- in essential thrombo- ulated/dense nuclei, clusters of megakaryocytes, cythemia (ET)? increased erythropoiesis, normal to mildly in- creased reticulin fibers. 29. essential thrombo- -hydroxyurea cythemia (ET) treat- -JAK2 inhibitors: Ruxolitinib, Lestaurtinib ments -low dose aspirin (prevents thromboses) 30. What is the etiology of Splenomegaly and ineffective hematopoiesis primary myelofibrosis caused by hypercellularity, fibrosis, and increased (PMF)? megakaryocytosis of the bone marrow. 31. What is the most com- JAK2V617F mon chromosomal ab- normality associated with primary myelofi- brosis (PMF)? 32. What are other possi- MPL W515L/K, CBL, TET2, ASXL1, LNK, EZH2, ble chromosomal ab- IDH1/2 4 / 15  MMSC 433 Exam 3 Study online at https://quizlet.com/_g1f5rb normalities in primary myelofibrosis (PMF)? 33. What morphological Tear drop cells (dacrocytes), nRBCs, anisocytosis, features are present in poikilocytosis, polychromasia, abnormal platelets, primary myelofibrosis and micromegakaryocytes. (PMF)? 34. What are common Fatigue, pruritis (itching), bone pain, palpitations, symptoms of primary night sweats, splenomegaly, hepatomegaly. myelofibrosis (PMF)? 35. What are the lab Hemoglobin normal to decreased. findings in primary myelofibrosis (PMF)? 36. What are the periph- Increased normoblasts (immature RBCs), in- eral blood findings in creased immature granulocytes, variable total primary myelofibrosis WBCs, variable PLT count, megakaryocytes pre- (PMF)? sent, LAP stain variable. 37. What are the bone mar- Intense fibrosis (lots of reticulin and collagen row findings in primary fibers), hypercellular, increased granulocytes and myelofibrosis (PMF)? megakaryocytes, increased erythropoiesis and myelofibrosis, increased sinuses, dysmegakary- opoiesis and dysgranulopoiesis present. 38. What is a notable Dry tap due to the extent of fibrosis. finding during bone marrow aspiration in primary myelofibrosis (PMF)? 39. primary myelofibrosis -hydroxyurea (PMF) treatments -JAK2 inhibitors: Ruxolitinib, Lestaurtinib -CYT387 -TG101348 40. 5 / 15  MMSC 433 Exam 3 Study online at https://quizlet.com/_g1f5rb What is the etiology CNL is a rare clonal disorder that causes hyperpro- of chronic neutrophilic liferation of neutrophilic cells in the bone marrow leukemia (CNL)? and may progress to AML. 41. What chromosomal +8, +9, +21, Del(20q), Del(11q), Del(12p); Philadel- abnormalities are as- phia chromosome is NOT implicated. sociated with chronic neutrophilic leukemia (CNL)? 42. What is the mor- Extreme neutrophilia, increased neutrophil precur- phology of chronic sors (segmented neutrophils, bands), neutrophils neutrophilic leukemia containing toxic granules, decreased monocytes; (CNL)? RBCs and PLTs are normal. 43. What are the symp- Hepatosplenomegaly, mucocutaneous bleeds, toms of chronic gout, pruritis (itching). neutrophilic leukemia (CNL)? 44. What are the periph- Extreme neutrophilia (25x10y/L, monocytes decreased < in chronic neutrophilic 1x10y/L. leukemia (CNL)? 45. What are the bone mar- Hypercellular, increased neutrophils, myeloblasts row findings in chronic >5% of nucleated cells. neutrophilic leukemia (CNL)? 46. What is the eti- Malignant hematopoietic stem cells lead to bone ology of chron- marrow dysplasia and monocytosis, may transition ic myelomonocytic to AML. leukemia (CMML)? 47. What are the chromo- Deletion of chromosome 7 and trisomy 8. somal abnormalities associated with chron- ic myelomonocytic leukemia (CMML)? 6 / 15  MMSC 433 Exam 3 Study online at https://quizlet.com/_g1f5rb 48. What is the mor- Monocytes make up e10% of total WBCs in periph- phology character- eral blood. istic of chron- ic myelomonocytic leukemia (CMML)? 49. What are common Hepatomegaly and splenomegaly. symptoms of chron- ic myelomonocytic leukemia (CMML)? 50. What are the pe- e20%blasts, persistent monocytosis >1*10y/L, in- ripheral blood find- creased monocytes. ings in chron- ic myelomonocytic leukemia (CMML)? 51. What are the bone mar- e20%blasts and bone marrow dysplasia. row findings in chron- ic myelomonocytic leukemia (CMML)? 52. What is juve- An aggressive hematopoietic disorder that pre- nile myelomonocytic sents in childhood (~2 years old). leukemia (JMML)? 53. What chromosomal PTPN-11, K-RAS, N-RAS, CBL or NF1 mutations abnormalities are as- seen in ~90% of cases; Philadelphia chromosome sociated with juve- is *NOT* implicated. nile myelomonocytic leukemia (JMML)? 54. What is the morphol- Dysplastic/abnormal monocytes. ogy of cells in ju- venile myelomonocyt- ic leukemia (JMML)? 55. What are common Splenomegaly and anemia. symptoms of juve- 7 / 15  MMSC 433 Exam 3 Study online at https://quizlet.com/_g1f5rb nile myelomonocytic leukemia (JMML)? 56. What are the peripher- Leukocytosis, monocytosis (e 1*10y/L), thrombocy- al blood findings in ju- topenia, and >20% blasts. venile myelomonocyt- ic leukemia (JMML)? 57. What are the bone Increase of myeloid and monocytic cells (hypercel- marrow findings in ju- lular) and >20% blasts. venile myelomonocyt- ic leukemia (JMML)? 58. What is a criterion for Poor response to therapy the accelerated phase of chronic myeloid leukemia (CML) ac- cording to WHO? 59. What happens to he- Anemia is increased (hemoglobin is decreased) moglobin levels in the accelerated phase of chronic myeloid leukemia (CML)? 60. What is the sta- Mature WBCs decreased tus of mature white blood cells (WBCs) in the accelerated phase of chronic myeloid leukemia (CML)? 61. What happens to Basophils increased basophil levels in the accelerated phase of chronic myeloid leukemia (CML)? 62. What is the status of Platelets decreased platelets in the accel- 8 / 15  MMSC 433 Exam 3 Study online at https://quizlet.com/_g1f5rb erated phase of chron- ic myeloid leukemia (CML)? 63. What types of cells are Micromegakaryocytes & megakaryocyte fragments present in the acceler- present ated phase of chron- ic myeloid leukemia (CML)? 64. What is the WBC WBC count increased >10*10y and unresponsive to count in the acceler- therapy ated phase of chron- ic myeloid leukemia (CML)? 65. What is the status Splenomegaly unresponsive to therapy of splenomegaly in the accelerated phase of chronic myeloid leukemia (CML)? 66. What are the platelet Thrombocytosis OR thrombocytopenia conditions in the accelerated phase of chronic myeloid leukemia (CML)? 67. What percentage of e20%blasts in PB blasts is found in peripheral blood (PB) during the accelerat- ed phase of chron- ic myeloid leukemia (CML)? 68. What percentage of 10-19% blasts in BM blasts is found in bone marrow (BM) during the accelerated phase 9 / 15  MMSC 433 Exam 3 Study online at https://quizlet.com/_g1f5rb of chronic myeloid leukemia (CML)? 69. What additional chro- Additional clonal chromosomal abnormalities (oth- mosomal abnormali- er than Philadelphia chromosome) appear ties may appear in the accelerated phase of chronic myeloid leukemia (CML)? 70. fibrosis of bone mar- increased Platelet Derived Growth Factor (PDGF) row (PMF) leads to increased collagen and reticulin fiber syn- thesis -increased collagen and reticulin fibers in the bone marrow= fibrosis of the bone marrow 71. pre-PMF criteria -Major: increased megakaryopoiesis, increased (WHO) granulopoiesis, decreased erythropoiesis, NO reti- culin fibrosis, hypercellular BM, JAK2, CALR, or MPL mutation present -minor: anemia not attributed to another condition, leukocytosis 11*10y/L, palpable splenomegaly, LDH increased 72. overt PMF criteria -major: increased megakaryopoiesis with either (WHO) reticulin or collagen fibrosis, presence of JAK2, CALR or MPL mutation present, no other WHO diagnosis availible -minor: anemia not due to another disease, leuko- cytosis < 11x10y/L, palpable splenomegaly, LDH increased, leukoerythroblastosis (immature Grans and RBCs in PB) 73. essential thrombo- -major: platelet count e 450x10y/ L, BM biopsy show- cythemia (ET) criteria ing mainly enlarged megakaryocytes with hyper- (WHO) lobulated nuclei, no significant increase in reticulin fibers, presence of JAK2, CALR or MPL mutations, does not meet requirements for different diagnosis -minor: presence of clonal marker OR absence of reactive thrombocytosis 10 / 15  MMSC 433 Exam 3 Study online at https://quizlet.com/_g1f5rb 74. therapy-related MDS medical treatment (chemotherapy, radiation thera- (T-MDS) py) leads to myelodysplastic syndromes 4-7 years after treatment -therapies introduce mutations into cell lines and apply selective pressure on cells of the BM to allow mutations to accumulate -more aggressive than De Novo MDS -transitions into AML 75. MDS-SLD (MDS with -*1 dysplastic lineage* single lineage dyspla- -1-2 cytopenias sia) -