MMSC 433 Exam 3 PDF

MMSC 433 exam 3 Study online at https://quizlet.com/_g3etre 1. Chromosomal Philadelphia chromosome (95% of cases) abnormality in -translocation of AB: proto-oncogene from band q34 of CML chromosome 9 to BCR of band q11 of chromosome 22 2....

MMSC 433 exam 3 Study online at https://quizlet.com/_g3etre 1. Chromosomal Philadelphia chromosome (95% of cases) abnormality in -translocation of AB: proto-oncogene from band q34 of CML chromosome 9 to BCR of band q11 of chromosome 22 2. Chromosomal JAK2V617F (most cases) abnormality in - point mutation replaces guanine with thymine at exon PV 4 changing amino acid at position 617 from valine to phenylanine 3. CML (chron- -over rpoduction of white blood cells in the Bm causes ic myelogenous cancer-a single genetic translocation in a pluripotential leukemia) hematopoietic stem cell producing a clonal overproduction of the myeloid cell line, resulting in a preponderance of immature cells in the neutophilic line - infection, bleeding, anemia, splenomegaly 4. Normal ABL functions as a kinase and can be active or inactive. They gene add phosphate groups to other proteins and are involved in cell growth and proliferation 5. Normal BCR -Act as a GTPase activating protein (GAP) gene -Cell signaling -Active when bound to GTP 6. BCR-ABL 1 fu- identified with Philadelphia chromosome, appears in 20% sion gene of adults and 2-5% of children 7. CML morpholog- Increased: total WBCs, neutrophils, ba- ical changes in sophils,eosinophils, myelocytes, platelets the PB Decreased: erythrocytes, LAP (normal 15-170) 8. CML accelerated Myeloblasts increase in bone marrow. Basophils increase phase in blood. Spleen size increases and leukocyte numbers are high. Characterized by more genetic lesions-unrelated to therapy 9. Blastic phase of Blasts consititue >20% of total BM cellularity CML 10. CML treatment gleevec 1 / 13  MMSC 433 exam 3 Study online at https://quizlet.com/_g3etre 11. PV (poly- neoplastic clonal myeloproliferative disorder that common- cythemia vera) ly manifests with panmyelosis in the BM and increases in erythrocytes, granulocytes, and platelets in the PB-over production of RBCs in bone marrow-> thick blood-> poor circulation 12. PV PB morpho- Increased: Hgb, Hct, RCV, total WBCs, granulocytes, logical changes PLTs, LAP - increased RBC masss 35 males 31 females - triad of BM fibrosis, splenomegaly, and anemia with dacrocytes 13. PV treatment Phlebotomy; hydroxyurea 14. ET (essen- -Clonal MPN tial thrombo- - over production of platelets in the BM-Primary thrombo- cythemia) cytosis, idiopathic thrombocytosis, hemorrhagic thrombo- cytopenia 15. ET clinical pre- -vascular occulsion sentation -hemmorhage -increased PLT count -splenomegaly -erythromelagia 16. Common Morph Decreased: hgb, hct, plt fxn changes in PB increasded: total wbcs, neutrophils, plts for ET 17. ET Treatment -hydroxyurea -ruxolitin lestourtibin (JAK inhibitors) -aspirin 18. PMF (primary splenomegaly and ineffective hematopoiesis associated myelofibrosis) with areas of marrow hypercellularity, fibrosis, increased megakaryocytes 19. PMF clinical find- fatigue, pruitus, bone pain, palpitations, night sweats, ings splenomegaly, hepatomegaly 2 / 13  MMSC 433 exam 3 Study online at https://quizlet.com/_g3etre 20. PMF morphology immature granulocytes, normoblasts, dacrocytes 21. PMF treatment -hydroxyurea -ruxolitin lestourtibin (JAK inhibitors) -CYT387 and TG101348 (JAK inhibitors) 22. WHO major crite- 1. hgb >16.5 men, >16 women, or hct >49% men, 48% ria PV women, or increased RCM 2. BM biopsy with hypercellularity for age with panmyelo- sis 3. presence of JAK261VF or JAK2 exon 12 mutation 23. WHO minor crite- subnormal serum EPO level ria PV 24. WHO criteria for - PB leukocytosis due to increased numbers of neu- accelerated CML trophils and their precursors (promyelocytes, myelocytes, metamyelocytes) comprising e 10% of leukocytes - dysgranulopoiesis, which may include abnormal chro- matin clumping - no or minimal absolute basophilia; basophils usually < 2% of leukocytes - no or minimal absolute monocytosis; monocytes < 10% of leukocytes - hypercellular BM with granulocytic proliferation and gran- ulocytic dysplasia, with or without dysplasia in the ery- throid and megakaryocytic lineages- < 20% blasts in the blood and BM - no evidence of PDGFRA, PDGFRB or FGFR1 re- arrangement, or PCM1-JAK2- not meeting WHO criteria for BCR-ABL+CML, PMF, PV, or ET 25. PrePMF - WHO 1.Megakaryocytic proliferation without reticulin fibrosis criteria (major) >grade 1, accompanied by increased age-adjusted BM cellularity, granulocytic proliferation, and often decreased erythropoiesis 2. Not meeting the WHO criteria for BCR-ABL1, CML, PV, ET, MDS, or other myeloid neoplasms 3.Presence of JAK2, CALR, or MPL mutation or in the 3 / 13  MMSC 433 exam 3 Study online at https://quizlet.com/_g3etre absence of these mutations, presence of another clonal marker, or absence of minor reactive BM reticulin fibrosis 26. PrePMF - WHO - Anemia not attributed to a comorbid condition criteria (minor) - Leukocytosis e11 x 109/L - Palpable splenomegaly - LDH increased to above upper normal limit of institutional reference range - Dx of prePMF requires meeting all 3 Major Criteria and at least 1 minor criterion 27. Overt PMF - WHO 1.Presence of megakaryocytic proliferation accompanied criteria (major) by either reticulin and/or collagen fibrosis grades 2 or 3 2. Not meeting WHO criteria for ET, PV, BCR-ABL1, CML, MDS, or other myeloid neoplasms 3. Presence of JAK2, CALR, or MPL mutation or in the absence of these mutations, presence of another clonal marker, or absence of reactive myelofibrosis 28. Overt PMF - WHO - Anemia not attributed to a comorbid condition criteria (minor) - Leukocytosis e11 x 109/L - Palpable splenomegaly - LDH increased to above upper normal limit of institutional reference range - Leukoerythroblastosis (immature erythroid AND myeloid cells in PB) - Dx of overt PMF requires meeting all 3 Major criteria and at least 1 minor criterion 29. ET WHO criteria 1. platelet count > 450 x 109/L Major 2.BM biopsy showing proliferation mainly of the megakary- ocytic lineage with increased numbers of enlarged, mature megakaryocytes with hyperlobulated nuclei; no significant increase or left shift in neutrophil granulopoiesis or ery- thropoiesis and very rarely minor (grade 1) increase in retiulin fibers 3.not meeting WHO criteria for BCR-ABL1, CML, PV, PMF, MDS, or other myeloid neoplasms 4.presence of JAK2, CALR, or MPL mutation 4 / 13  MMSC 433 exam 3 Study online at https://quizlet.com/_g3etre 30. ET WHO criteria - presence of a clonal marker or absence of evidence for minor reactive thrombocytosis - Dx requires meeting all 4 major criteria or the first three major and the minor criterion 31. MDS - treatment - Improve quality of life and prolong survival - Supportive care - Lenalidomide, azacitidine, decitabine—FDA approved - Growth factors (EPO, TPO, G-CSF) - Immunosuppressive therapy - Farnesyltransferase inhibitors~interfere NRAS - Hematopoietic stem cell transplantation~cure 32. Myelodysplastic - MDS, NOS with single lineage dysplasia (MDS-SLD) syndromes - MDS, NOS with multilineage dysplasia (MDS-MLD) (MDS) - WHO - MDS with isolated del(5q) classification - MDS with Excess Blasts (MDS-EB) - MDS with mutated SF3B1 - MDS, NOS without dysplasia- MDS/AML 33. MDS - etiology - chemical insult - radiation - hematopoietic stem cells: pathological clone of progeny; interferes w/ normal hematopoiesis; "crowding out"; dys- poiesis 34. MDS a group of acquired clonal hematologic disorders charac- terized by progressive cytopenias in the peripheral blood reflecting defects in erythroid, myeloid, and/or megakary- ocytic maturation 35. Therapy related - Patients who have been treated with "chemotherapy" MDS and/or "radiotherapy" - 4 to 7 years after therapy - More aggressive than de novo MDS - Transitions into AML 36. MDS w/ isolated Dysplastic lineages: e1 del(5q) - lab find- Cytopenias: e1 ings Thrombocytosis may be present 5 / 13  MMSC 433 exam 3 Study online at https://quizlet.com/_g3etre BM Blasts: 40 y/o, M>F mutations in MYD88 (chrom 3) and 6q- normally involved in cell signaling 72. waldenstroms hyperviscosity macroglobuline- amyloidosis mia clinical cryoglobulins (cryoglobulinemic purpura) features anemia PT, PTT, BT prolongs Raynauds phenomenon 73. Lab finds in WM N/N anemia thrombocytopenia lymphocytosis Dutcher bodies rouleuax increased ESR bence jones proteins proteinuria plasmacytoid lymphocytes 74. electrophoresis spikes in gamma region, IgM in WM 75. WM tx chemotherapy plasmapheresis 76. plasma cell most common in African Americans, aggressive disease leukemia bone pain- lytic bone lesions hypercalcemia 10 / 13  MMSC 433 exam 3 Study online at https://quizlet.com/_g3etre fatigue kidney damage hepatosplenomegaly 77. lab finds of plas- hypercalcemia ma cell leukemia abnormal plasma cell makeup >20% of total WBC in PB smear (>2 x 10^9/L) migration to spleen, liver, body cavity fluids including CSF either kappa or lambda light chains in malignant clone, but NOT both increased ESR 78. plasma cell CD CD38, CD138, CD20 markers 79. tx of plasma cell chemo: Bortezomib, Lenalidomide leukemia 80. Burkitts Lym- medium sized highly proliferating mature B phoma lymphoid cells w basophilic vacuolated cytoplasm avg age dx: 3-12 y/o 81. etiology of EBV Burkitts Lym- chromosomal translations t(8;14), t(8;22), t(2;8 phoma 82. sporadic Burkitts children/young adults Lymphoma abdominal mass GI tract, gonads, breast 83. immunodeficient occurs in HIV ppl/organ transplantation nodal disease Burkitts Lymphoma 84. tx and prognosis chemotherapy: cyclophosphamide, cytarabine, doxoru- of BL bicin, methotrexate immunodeficient type worst prognosis 85. lab finds: Burkitt low magnification "starry sky" appearancemacrophages Lymphoma phagocytize apoptopic debris 11 / 13  MMSC 433 exam 3 Study online at https://quizlet.com/_g3etre 86. burkitt cell lym- diffuse proliferation of lymphoid cells phoma morphol- medium size w round nuclei ogy finely distributed chromatin small nucleoli cytoplasm is vacuolated 87. most common t(8;14) translocation in Burkitt Lym- phoma 88. immunopheno- CD19+, CD20+, CD10+ type/CDs of Burkitt Lymphoma 89. Non Hodgkins lymphoma: solid tumor neoplasm of lymphoid tissue, can- lymphoma cer cells may develop in thymus, lymph nodes, bone mar- row, spleen 90. sxs of lymphoma swollen lymph nodes unexplained fever weight loss drenching night sweats cough/breathlessness fatigue 91. martle cell lym- diffuse, nodular or mantle zone pattern phoma medium size lymphocytes w irregular nuclei CD20+, CD19+, CD5+, FMC7+ t(11;14) 92. follicular lym- t(14;18), germinal center cell, follicular pattern, CD20+, phoma CD19+, CD10+, BCL6+, BCL2+ 93. Extranodal mar- marginal zone cell, clear abundant cytoplasm ginal zone lym- phoma of mu- 12 / 13  MMSC 433 exam 3 Study online at https://quizlet.com/_g3etre cosa associated lymphoid tissue 94. plasma cell sheets/large aggregates of plasma cells myeloma 95. CLL lympho- "soccer ball" appearance in PB cytes are known as 96. B-CLL with hy- having fewer than normal 46 chromosomes poploidy blasts contain 50 chromosomes without translocations favorable prognosis affects 25-35% children age 3-5 immunophenotyping: CD19+, CD10+, CD34+, CD45 neg 13 / 13