Replaces the normal tissues of the marrow e. Cell size Results in anemia, granulocytopenia , f. None of the above thrombocytopenia. 6. ALL: Malaise, fatigue and pallor Burkitt type Vulnerable to infection, easily bruising, 3-5% petechiae, epistaxis , fever chills Blasts are large and primitive appearing and Bone pains, swelling of joints, cranial nerve homogenous paralysis, inc. ICP, Finely stippled chromatin Superficial lymph node enlargement, One or more vesicular nucleoli splenomegaly and hepatomegaly Cytoplasm is intensely basophilic Cytoplasmic vacuolation frequent He had a bleeding, his Leukocyte count from Vacuoles stain oil red O 10,000 100,000 cu/mm Poor prognosis responds poorly to Leukemic lymphoid blast are predominant chemoTx Aleukemic leukemia blasts are not found in PS a. L3 but abundant in BM. b. L2 BM always hypercellular 7. Cytochemical classification used for ALL: Heavily infiltrated or replaced by lymphoid cells Sudan black B BM fibrosis 10-15% Myeloperoxidase Thrombocytopenia and anemia is always Acif phosphatase present Non specific esterase He has what type of leukemia? Tdt- marker for primitive lymphoid cells a. Acute lymphoblastic leukemia ALL negative for Sudan black and b. Acute myelogenous leukemia myeloperoxidase c. Acute lymphocytic leukemia some ALL cells are weakly + to sudan black B 2. Classification of leukemia is done by? 8. PAS reaction for ALL: a. FAB L1 strongly + b. GAB L2- low c. AABB L3- negative 3. Classification of Leukemia wherein: 9. CD assoc with ALL a. CD5 Blasts are small about twice the size of small b. Cd7 lymphocyte c. Cd10 Uniform , lack variation in sizes d. Cd13 Chromatin finely dispersed but appear e. Cd19 more clump in smaller cells f. Cd33 Nuclear shape is regular some degree of g. Hla-dr folding , clefting and indentation 10. Most common CD associated with CLL Nucleoli usually not visible a. CALLA Cytoplasm scanty , slightly basophilic b. CD10 Vacuoles not present c. CD22 Best prognosis during therapy d. CD2 a. L1 e. A AND B b. L2 11. cells of burkitts leukemia are positive for 4. Classification of leukemia whrein: HLA-DR, CD19, sCD22, CD24 Most cells are more than the twice the diameter weak expression of CD10 - 1/3 of a small lymphocyte less than 5% of ALL cases Marked difference in cell size cytoplasmic Immunoglobulin (cIg) and Chromatin ranges from dispersed to coarse and TdT are not present condensed -= mixed corresponds to L3 Nucleoli always visible A. B CELL ALL Nuclei cleaving , indentation and folding are B. PRO-B CELL characteristic C. PRE B CELL Nuclear size are irregular 12. presence of HLA DR, TdT, CD19, CD20 and a. L2 CD24. b. L1 CALLA (CD10) may be present 5. All but one are main differences of l1 and l2, capable of synthesizing m-heavy chain except: cytoplasmic Ig (cIg) m is present a. Nuclear cytoplasmic ratio sIg is absent c. Presence, prominence and frequency of 10-15% found in ALL in children and adult nucleoli majority are classified as L1 chromosomal abnormality t(1;19) are found in this a. prednisone group b. methotrexate A. PRE-B CELL 22. folic acid antagonist, inhibits dna synthesis B. PRO B CELL a. methotrexate C. B CELL b. 6-mercaptopurne 13. Presence of HLA DR , TdT, CD10, Cd19, 23. purine antagonist, interfere purine syn. CD24, and sometimes CD20 Inhibit DNA and RNA syn. cells lack sIg, cIg, and m heavy chain a. 6-mercaptopurine 85% in child b. Cyclophosphamide 75% in adult highest remission rate 24. syn . Alkalyting agent , in. DNA syn 5% children t(9;22) phil chromosome a. cyclophosphamide 20% adults - t(9;22) phil chromosome b. daunorubicin Pre early B - +HLA DR,TdT,CD19,CD34 CD34 is found in hematopoetic stem cells 25. antibiotic , in. DNA and RNA syn 5% in children , 11% adult a. daunorubicin Chromosomal t(4;11) nonresponsive to b. l-asparaginase therapy 26. enzyme (E.coli) , lysis of lymphoblasts A. PRO B CELL ( deprive cells of L-asparagine) B. PRE B CELL a. L-asparaginase C. B CELL b. Cytarabine 14. includes CD7, CD5, CD2 27. Pyrimidine antimetabolite, inhibits dna synthesis cytoplasmic CD3and CD6 maybe present a. Cytarabine 15% in childhood and adults b. Vincristine higher in males 5:1 ratio 28 proliferation and accumulation of half of patients have mediastinal (thymic) lymphocytes (usu. B-cells) that are relatively mass unresponsive to antigenic stimuli. very high leukocytes counts 29 Dormant and accumulate in the blood, BM, higher incidence of CNS involvement LN, spleen carries poorer prognosis 30 Rarely caused by T cell proliferation A. T CELL ALL 31 Males, older age (50 -60yo) , inherited or B. B CELL ALL acquired 15. used to predict therapeutic outcomes 32 Ionizing radiation, HTLV virus, genetic A. KARYOTYPING 33. More common in men B. PHENOTYPING Fatigue, reduced exercise tolerance, 16. CHROMOSOMAL TRANSLOCATIONS FOR bruising, pallor, jaundice asso with anemia, PRE B CELL fever due to infection, bone tenderness, a. t(1;9) weight loss, edema from LN obstruction, b. t (8;14) erythroderma pruritus c. t(9;22) Benign to severe 17. CHROMOSOMAL TRANSLOCATIONS FOR Some may live 10-15 years without TX PRo B CELL Aggressive ones about a 3-5years d. t(1;9) Splenomegaly , hepatomegaly may involve e. t (8;14) other organs like gonads, kidneys, skin, f. t(9;22) prostate, walls of GI tracts. 34. Absolute lymphocyte count 10k -150k 18. CHROMOSOMAL TRANSLOCATIONS FOR b cumm. 1M cu mm found in more aggressive cell burkitt CELL conditions g. t(1;9) Lymphocytes appear normal but larger h. t (8;14) Nuclei with clumped or condensed chromatin i. t(9;22) Nucleoli are prominent Cytoplasm are abundant, non-granular, 19. conditions to do bm transplantion moderately basophilic a. Considered in patient who relapse and Lymphocyte are more fragile than normal achieve a second remission smudge cells b. Response rate in ALL are high Neutrophils are decreased c. Allogenic transplant is preferred Aggressive dse. Have granulocytopenia, d. All of the above thrombocytopenia and anemia 20. plant alkaloid ,inhibits RNA Autoimmune hemolysis - + DAT triggered by a. vincristine viral infxn, drugs, dse. Progression b. prednisone Lymphocytes contain glycogen PAS+ 21. steroid, lysis of blasts Plasma Igs dec. BM aspiration not necessary Leukemic reticuloendotheliosis (EWALD 30% more lymphocytes in BM with 1923) lymphocytosis DX Dse. is described as large spleen with Erythroid cells in BM megaloblastic with EH circulating mononulear cells with numerous 35. Hans has Absolute lymphocyte count cytoplasmic projections 10k -150k cumm Lymphocytes appear normal Anemia , bleeding , infection, paraneoplastic but larger complications, autoimmune syndrome, Nuclei with clumped or condensed chromatin paraproteinemias Lymphocyte are more fragile than normal weakness, bleeding, fatigue and infection smudge cells scant to abundant , agranular , light grayish Neutrophils are decreased blue cytoplasm Have granulocytopenia, thrombocytopenia loose and lacy and anemia 90% - splenomegaly Autoimmune hemolysis - + DAT triggered by Occ. Liver enlargement viral infxn, drugs, dse. Progression a. Hairy cell leukemia Lymphocytes contain glycogen PAS+ b. Pll Plasma Igs dec. c. CLL BM aspiration not necessary 53. Most consistent feature of HCL 36. More prominent dx a. Pancytopenia a. 30% more lymphocytes in BM with b. Leukopenia lymphocytosis 54. Most common causes of leukopenia b. 40% lymphocyte in bm a. Granulocytopenia c. 60% b. Monocytopenia 37. Clymphocytic is associated with what anemia? c. Lymphocytopenia a. Megaloblastic d. A and B b. Aplastic 55. induces partial to complete remission 38. (14q) , t(11;14) is most common a. pentostatin 39. Alteration in chromosome 11,6,18,3,17 and 8 b. 2-chlorodeoxyadenosine 40. B-CLL express low amts. Of sIg, but inc. cIg c. Cladribine 41. Goals of therapy are d. All of the above a. the relief of symptoms 56. Most common malignant dse of plasma cells b. prevention of complications generally affects older individuals (50-75yrs). c. treat disease itself d. a and b a. Multiple myeloma 42. Galton 1974 b. Plasma cell myeloma 43. More on men age 60yo c. A and b 44. Prolif. of abnormal cells in spleen , BM, d. None sometimes liver 57. Transformation occurs at the hematopoietic 45. Low Ig stem cell 46. T lymphocytes are below normal a. CALLA (CD10) Ling is a 60 year old man with the ff b. CALLA CD11 manifestations fatigue, weight loss, impressive 58. leading to bone resorption and lytic bone dis. splenomegaly, leucocyte count of 1M, with a a. Osteoclast activating factor large mononuclear with an oval to round b. Osteoblast nucleus, coarse chromatn strand with 1-2 59. Leukemia with Anemia , infection and vesicular nucleoli w/ perinuclear condensations, bleeding cytoplasm is agranular and basophilic, Cells also infiltrate LN, spleen, and other Glycosuria and hyperglycemia organs Increased risk of infection decreased a. Prolymphocytic leukemia b. Lymphoblastic leukemia production of normal Igs sp. asso. in primary 47. PLL chromosome immune response a. Chromosome 1, 6 M proteins- may cause the clinical b. Chromosome 2,6 manifestations 48. Pll is with what trisomy L chains ( bence jones proteins) a. 12 filtered out by the kidney glomeruli b. 11 with massive reabsorption in the proximal c. 13 tubules. In time entire nephron will be 49. T (6;12), T (11;14) damaged. renal failure 50. CD103 positive 60. Sentinel prime Skeletal pains is the chief 51. Low CD5 expression 52. 2% of all leukemias complaint Back pains, as his pathologic fracture a. PCL Anemia b. MM Renal insufficiency 68. NN anemia Hge, hemodilution from Weakness and fatigue hypovolemia, dec. red cell survival Bleeding episode Retic count N or dec Susceptibility to infection Platelets and leukocytes are in ref range a. MM Inc ESR rouleaux formation b. BM BM shows variation of cell types (small c. CM lymphocytes, plasmacytoid lymphocytes, plasma 61. NN anemia, Hb 7-12g/dl cells) Rouleaux formation Mast cells maybe inc. Blood film bluish tinge macroscopically a. Waldenstroms macroglobulinemia Slight neutropenia b. MM No plasma cells seen in early stage 69. Retinal abnormalities preceding retinal Hge and Abnormal plasma cells late stage but rare ESR increased due elevated Se. globulins neurologic changes - hyperviscosity Cellular inclusions like Dutcher bodies Mast cells may be increased; electrophoresis crystalline features of abn. Igs monoclonal peak in beta gamma region Russell bodies rounded accumulations of Ig in Prolonged APTT and TT 70. the most common infiltration of plasmacytoid the cisternae of rough ER of the cytoplasm Few IgA myeloma flame cells abundant lymphocytes into duodenal or jejunal wall , producing malabsorption and abdominal distress cytoplasm with reddish tinge of ribosomal .respiratory tract IgA involved in few cases proteins a. a- HCD b. beta-HCD d. MM 71. common in young people e. BM a. alpha- HCD f. CM b. beta- HCD 62. Chemistry- Protein electrophoresis monoclonal 72. mostly in older men peak a. gamma HCD 63. Tx: splenomegaly b. beta HCD 64. Radiologic evidence osteoporosis and lytic 73. rarest form relate to CLL bone dse. a. u HCD 65. BJP worst prognosis b. gamma HCD 66. Urine protein studies , CBC, Xray, may be for 74. Atypical lymphocyte and plasma cells seen PB monitoring dse process generally composed of immunoblast, 67. when circulating plasma cells exceed lymphocytes and plasma cells like WM 2000/cumm. 75. spike in beta gamma region Can occur on late manifestation of MM but is a 76. gamma H chain in IEP no L chain more discreet entity 77. mature plasma and vacuolated PC Occur in younger 78. seen in BM in 2/3 of cases Complain of bone pain and show less osteolysis 79. vacuoles dont contain IG > incidence of LAD and hepatosplenomegaly 80. Only HCD that secrete L chain in urine usually a. pcl kappa b. dcl 81. Abnormality in H &L chain assembly 82. closely related to plasma cells dis. But few may 68. PS - > 2000/cu.mm of plasma cells have polyclonal forms 83. Amyloid is composed of L chain fragments Pancytopenia 84. from the variable regions of Ig 85. l- chain often involved Inc. ESR 86. Secondary Amyloidosis related to chronic illness. Amyloid is not related to Ig Abnormal plasma cells are small with little 87. Cause is unknown cytoplasm Environmental , EBV 7th mc cause of cancer deaths in US BM infiltration are diffuse and proliferating cells Common in 20s decline at 40s but rises during are about 45% advance stage More common in men 3:2 Serum and protein studies will differentiate this Clinical feature rapid cervical enlarge- ment of disorder from more common lymphocytic cervical LN, mediastinal mass Xray,fever , <night leukemias sweats, weight loss> (B-symptoms) A symptoms no symptoms Dse spreads in orderly fashion Advanced cases involved blood a. HODGKIN b. NONHODGKIN 88. NN anemia 10% Retic ct N or low BM seldom involved except in advanced stage Laboratory se iron and TIBC reduced, DAT+, inc ESR, inc. Se. Globulins, ceruloplasmin, leukocyte alk.phos,LDH and transaminase Hyperuricemia renal failure a. HODGKIN b. NONHODGKIN 89. Known before as lymphosarcoma 90. Rudolf Virchow 91. 3x more common than HD 92. Cong. Immunodefeciency , Ataxia telangiectasia, wiskott-aldrich syn, IgA def., SCId 93. Rheu. Arthritis, sjogrens syn, SLE 94. Immunosuppresive drugs 95. AIDS 96. EBV, HTLV 97. Painless LN enlargement 98. Cervical LN 99. Fever , night sweats, weight loss 30% esp with advanced stages 100. Blood counts are normal even with BM involvements 101. Chemistries like inc alk.phos,LDH,uric a.,may also occur like in HD