Neoplastic proliferation arising from white blod cells

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24/2/2013leuke.ppt*Neoplastic proliferation arising from white blood cellsLymphoproliferative and myeloproliferative diseases and syndromes  Oliver Rácz, 2012/13

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24/2/2013leuke.ppt*Introductory remarks„Leukemia“ and „lymphoma“are old descriptive terms On the other side the new classification is also descriptive and not fully rational. High number of subtypes in most group. Typisation is based on new knowledge – histology, surface antigens (imunochemistry, flow cytometry) and genetics Not without purpose – different treatment regimes

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24/2/2013leuke.ppt*Classification„LYMPHOID NEOPLASMS Hodgkin, non-Hodgkin ALL, CLL CHAOS TILL 1994 – REAL, 1997 WHO Immunochemical and genetic characteristics MYELOID NEOPLASMS Acute AML Chronic CML Myelodysplastic syndromes

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24/2/2013leuke.ppt*LYMPHOID NEOPLASMS, B;T; HODGKINPrecursor B cell neoplasms (1) Peripheral B cell neoplasms (12) why? Hairy cell leukemia, plasmocytoma, Burkitt lymphoma Precursor T cell neoplasms (1) Peripheral T and NK cell neoplasms (12) why? Hodgkin lymphoma (5 subtypes)

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24/2/2013leuke.ppt*LYMPHOID NEOPLASMS, B;T; HODGKINPrecursor B cell neoplasms (1) Peripheral B cell neoplasms (12) why? Hairy cell leukemia, plasmocytoma, Burkitt lymphoma Precursor T cell neoplasms (1) Peripheral T and NK cell neoplasms (12) why? WHY 1:12? There is only one precursor but many development stages

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24/2/2013leuke.ppt*

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24/2/2013leuke.ppt*LYMPHOID NEOPLASMS, B;T; HODGKINPrecursor B cell neoplasms (1) NHL Peripheral B cell neoplasms (12) NHL Precursor T cell neoplasms (1) NHL Peripheral T and NK cell neoplasms (12) NHL Hodgkin lymphoma (5 subtypes) HL CLINICAL PICTURE NODAL ENLARGEMENT IN 80% NHL, ALL HL SUPRESSION OF NORMAL HEMATOPOIESIS SPLEEN AND LIVER INFILTRATION IMMUNE SYSTEM DYSFUNCTION SPECIFIC OR also NOTHING

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24/2/2013leuke.ppt*LYMPHOID NEOPLASMS, B;T; HODGKINPrecursor B cell neoplasms (1) NHL Peripheral B cell neoplasms (12) NHL Precursor T cell neoplasms (1) NHL Peripheral T and NK cell neoplasms (12) NHL Hodgkin lymphoma (5 subtypes) HL LABORATORY PICTURE PERIPHERAL BLOOD NEOPLASM BIOPSY ANTIGEN RECEPTORS AND OTHER SURFACE PROTEINS (CD1 – 79) PRESENT ALSO ON NORMAL CELLS – FLOW CYTOMETRY CHROMOSOME AND GENE ANALYSIS

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24/2/2013leuke.ppt*Hodgkin: Reed-Sternberg cells

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24/2/2013leuke.ppt*Hodgkin: Reed-Sternberg cellsSize between 20 - 50 microns; Amphofilic, finely granular/homogenous cytoplasm; Two mirror-image nuclei ("owl eyes") each with an eosinophilic nucleolus and a thick nuclear membrane (chromatin is distributed on the inner surface of the nuclear membrane, generating a halo image around the nucleolus).

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24/2/2013leuke.ppt*Hodgkin: Clinical picture5 subtypes according Ly morphology and tumor histology, 1 – 4 is the same, 5 (nodular lymphocyte predominant Hodgkin disease; NLPHD) is a separate disease Enlargement of one ore more lymphatic nodes, without pain, mostly in the cervical or mediastinal region Fever, sweat, quiver, weight loss (or no) Later dissemination to other organs

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24/2/2013leuke.ppt*The Reed-Sternberg cells affect the immune system !

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24/2/2013leuke.ppt*Non-Hodgkins: More frequentB >> T (80-85 vs 15-20 %) Associated with (cause ???) Epstein – Barr virus (Africa, Burkitt) HTLV – human T-cell Lymphotropic (Japan) HIV Posttransplantation (immunosupression?) Helicobacter – MALT lymphoma?

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24/2/2013leuke.ppt*Non-HodgkinsB >> T (80-85 vs 15-20 %) V(D)J recombinations and regulated somatic hypermutations – higher possibility of mistakes

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24/2/2013leuke.ppt*Non-Hodgkins: Symptoms, prognosisMore generalized as HL Enlarged L.N., no pain Many types, for general medicine is enough Low grade Aggressive Very aggressive (but radio-chemosenzitive) During disease the aggresivity can increase Therapy with monoclonal antibodies – rituximab against CD20 (B) with 90Y Transplantation of stem cells or bone marrow

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24/2/2013leuke.ppt*Lymphoid and myeloid leukemiasABNORMAL PROLIFERATION OF CELLS, CIRCULATING IN BLOOD, INFILTRATING ORGANS IN AGE 1 – 14 YEARS LEADING CAUSE OFMORTALITY, DESPITE THE FACT THAT LEUKÉMIA IS 10-TIMES MORE REQUENT IN ADULTS ALL children, 2 – 4 y. CLL older, m > w AML adults CML 30 – 50, and also children

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24/2/2013leuke.ppt*Etiology in generalRadiation – Hiroshima, Tchernobyl and ? Aromatic substances Antitumor drugs !!! Down (RR  10), Recklinghausen, Fanconi, Bloom > 500 different translocations and other chromosomal aberrations (Philadelphia)

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24/2/2013leuke.ppt*Philadelphia chromosome, chronic myeloid leukemia (CML)ablbcrabl-bcr hybrid genep210 tyrosinkinase with increased activity9 22

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24/2/2013leuke.ppt*Neurofibromatosis 1 Recklinghausen (and Genersich) Tumor supressor gene on 17th ch. (NF1) Risk of other malignities, too AD, 1/2500 - 4000

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24/2/2013leuke.ppt*Acute lymphatic leukemiaChildren, boys > girls Whites > nonwhites (2x) preB > > preT, B earlier, T later Blood cell morphology makes possible differential dg ofALL and AML, further immunophenotypisation Chromosomes: hyperploidia (> 50), polyploidia, Philadelphia and other translocations Microchip technology – specific patterns of abnormal gene expression

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24/2/2013leuke.ppt*Symptoms (common for ALL, AML)Rapid onset of symptoms (weeks) Anaemia, fatigue, fever, bleeding Bone pain Generalised lymphadenopatia, splenomegaly, hepatomegaly ALL > AML, sometimes testes (ALL) Headache, cramps (ALL) THE PROGNOSIS IS IMPROVING

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24/2/2013leuke.ppt*Chronic lymphatic leukemia, CLLMost frequent from all Manifestation in age over 50, M > W Ly > 4000/ml, sometimes 20 000/ml Prolymphocytes in LN, high number of mitoses In blood small Ly, small amount of cytoplazma, fragile „smudge“ cells. Ly in bone marrow and other tissues Special immunophenotype Low number of chromosomal abnormalities Often asymptomatic Danger of increase in degree of malignancy

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24/2/2013leuke.ppt*Myeloid neoplasma types in generalFrom developmemtal stages of these cells Less infiltrate spleen, liver and L.N. AML – cells are nondifferentiated, haematopoesis blocked CML – differentiated cells Myelodysplastic syndromes Danger of transformation towards more agressive forms

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24/2/2013leuke.ppt*AMLMostly in young adults The maturation is blocked !!! „Blasts“ 10 000 – 100 000/ml Frequent chromosomal aberrations (up to 90 % with modern technology) The prognosis is not very good

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24/2/2013leuke.ppt*AMLClassification M0 – M7, mostly 1 and 2, AML without and with maturation, but also erytro... (M6) a megakaryo... (M7) M3 often associated with DIC (acute promyelocyte L) WHO classification according to chromosomal aberrations

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24/2/2013leuke.ppt*Symptoms (common for ALL, AML)Rapid onset of symptoms (weeks) Anaemia, fatigue, fever, bleeding Bone pain Generalised lymphadenopatia, splenomegaly, hepatomegaly ALL > AML, sometimes testes (ALL) Headache, cramps (ALL) THE PROGNOSIS IS IMPROVING

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Last Updated: 8th March 2018