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General Pathology

General Pathology. Histogenetic Classification of Neoplasms Lymphomas & Leukemias (Hemoblastomas & Hemoblastoses). Jaroslava Dušková Inst. Pathol. ,1st Med. Faculty, Charles Univ. Prague. Leukemia. def. - diffuse neoplastic proliferation of the hemopoietic marrow cell granulocytic

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General Pathology

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  1. General Pathology Histogenetic Classification of Neoplasms Lymphomas & Leukemias (Hemoblastomas & Hemoblastoses) Jaroslava Dušková Inst. Pathol. ,1st Med. Faculty, Charles Univ. Prague

  2. Leukemia def. -diffuse neoplastic proliferation of the hemopoietic marrow cellgranulocytic lymphocytic

  3. Lymphoma def. malignant neoplasms of cells native to lymphoid tissue (lymphocytes, histiocytes) and their precursors and derivatives. non Hodgkin (B,T) Hodgkin

  4. NEOPLASIA – classification HISTOGENETIC (cell of origin) • mesenchymal • epithelial • neuroectodermal • mixed, teratoma • choriocarcinoma • mesotelioma

  5. Main functions of the bone marrow and lymph nodes: hematopoetry immune response Hematopoetry gr. haima- blood, poiétria - art of composition characterized by great beauty of expression

  6. LYMPHOMA painless lymphadenomegaly infiltrated organs hepatosplenomegaly bone marrow involvement (& leukemia) LEUKEMIA anaemia / fatigue immunodepression –infections, fever haemorrhagic diathesis epistaxis, ecchymoses bone pain hepatosplenomegaly CNS –meningeal (ALL) Lymphomas and Leukemias - clinical symptomathology

  7. Pathology & Genetics Tumours of Haemopoietic and Lymphoid Tissues WHO 2001

  8. Principles of Classification • primarily according to lineage • myeloid • lymphoid • histiocytic/dendritic cell • mast cell • within each category • morphology • immunophenotype • genetic features • clinical syndromes

  9. NEOPLASMS OF HEMATOPOIETIC AND LYMPHOID TISSUE(WHO 2001, abbreviated) Myeloid neoplasms Chronic myeloproliferative disorders Myeloproliferative / myelodysplastic diseases Myelodysplastic syndromes Acute myeloid leukemias Lymphoid neoplasms Precursor B and T cell neoplasms Mature B cell neoplasms Mature T and NK cell neoplasms Hodgkin lymphoma Histiocytic and dendritic cell neoplasms Histiocytic sarcoma Dendritic cell neoplasms Mastocytosis Most of them originate / may originate in lymph nodes

  10. LYMPH NODES enlargement (painless) BONE MARROW pyoid (yellowish) or gray infiltration activation of reserve zones Lymphomas and Leukemias - general macroscopy features • HEMOPOETRY HISTOHOMOLOGOUS ORGANS • enlargement • OTHER ORGANS • diffuse or tumorous infiltration

  11. LYMPH NODES architecture partly / fully obscurred with the neoplastic population subcapsullar sinus defunct nodular / diffuse transcapsullar spread BONE MARROW hypercellular architecture partly / fully obscurred with the neoplastic population haemopoetry activation in the formerly fatty (reserve) marrow Lymphomas and Leukemias - general histology features HISTOHOMOLOGOUS ORGANS colonised – liver, spleen, nodes

  12. NEOPLASMS OF HEMATOPOIETIC AND LYMPHOID TISSUE(WHO 2001, abbreviated) Myeloid neoplasms Chronic myeloproliferative disorders Myeloproliferative / myelodysplastic diseases Myelodysplastic syndromes Acute myeloid leukemias Lymphoid neoplasms Precursor B and T cell neoplasms Mature B cell neoplasms Mature T and NK cell neoplasms Hodgkin lymphoma Histiocytic and dendritic cell neoplasms Histiocytic sarcoma Dendritic cell neoplasms Mastocytosis Most of them originate / may originate in lymph nodes

  13. Myeloid Diseases • Chronic myeloproliferative diseases –CMPD • Myelodysplastic/myeloproliferative diseases –MDS/MPD • Myelodysplastic syndromes - MDS • Acute myeloid leukaemias - AML

  14. Chronic Myeloproliferative Diseases –CMPD def.: clonal proliferation of one or more of the myeloid lineages (granulocytic, erythroid, megakaryocytic) hemopoietic stem cells in the bone marrow • hepatomegaly, splenomegaly • developmentinto myelofibrosis or acute blast phase

  15. Myeloid Diseases • Chronic myeloproliferative diseases – CMPD • CML (Philadelphia chromosome –t(9,22)(q34;q11) • Chronic neutrophilic leukaemia • Chronic eosinophilic leukaemia • Polycythemia vera • Chronic idiopathic myelofibrosis • Essential thrombocythemia

  16. Myeloid Diseases • Myelodysplastic/myeloproliferative diseases – MDS/MPD • Chronic myelomonocytic leukaemia • Atypical chronic myeloid leukaemia • Juvenile myelomonocytic leukaemia • MDS/MPD - unclassifiable

  17. Myelodysplastic syndromes - MDS def.: bone marrow failure and dysplasia in one or more myeloid cell lineages • the number of blasts is in the blood or marrow < 20% (xAML) • development to acute leukaemia or death of bone marrow failure

  18. Myeloid Diseases • Myelodysplastic syndromes - MDS • Refractory anaemia • Refractory anaemia with ringed sideroblasts • Refractory anaemia with multilineage dysplasia • Refractory anaemia with excess blasts • MDS associated with isolated del(5q) chromosome abnormality • MDS - unclassifiable

  19. Myeloid Diseases Acute myeloid leukaemias - AML clonal expansion of myeloid blasts in bone marrow blood or other tissue

  20. Leukemia Acute morphology: aplastic anemia agranulocytosis, thrombocytopenia AML - adults ALL - children

  21. Myeloid Diseases Acute myeloid leukaemias - AML • AML with recurrent cytogenetic abnormalities • AML with multilineage dysplasia • AML and MDS therapy- related • AML not otherwise categorised 19 nosology units

  22. NEOPLASMS OF HEMATOPOIETIC AND LYMPHOID TISSUE(WHO 2001, abbreviated) Myeloid neoplasms Chronic myeloproliferative disorders Myeloproliferative / myelodysplastic diseases Myelodysplastic syndromes Acute myeloid leukemias Lymphoid neoplasms Precursor B and T cell neoplasms Mature B cell neoplasms Mature T and NK cell neoplasms Hodgkin lymphoma Histiocytic and dendritic cell neoplasms Histiocytic sarcoma Dendritic cell neoplasms Mastocytosis Most of them originate / may originate in lymph nodes

  23. Lymphoma def. malignant neoplasms of cells native to lymphoid tissue (lymphocytes, histiocytes) and their precursors and derivatives. non Hodgkin (B,T) Hodgkin

  24. Lymphoid Neoplasms • B cell (lymphomas & leukaemias) • T and NK cell -“- • Hodgkin lymphomas

  25. Lymphoid Neoplasms B cell lymphomas & leukaemias (17 nosology units in 2001 WHO classif.) • Precursor B-cell neoplasm • Mature B-cell neoplasms (85% nH ML) • B-cell proliferations of uncertain malignant potential

  26. Small Lymphocytic Lymhpoma (SLL) / Chronic Lymhocytic Leukemia (CLL) Small B-lymphocytes, proliferating cells (prolymphocyte,paraimmunoblast) • peripheral blood lymphocytes>10x109/ l, • bone marrow lymphocytic infiltration • splenomegaly, hepatomegaly, lymphadenopathy • immunodeficiency, bleeding, disordered healing Clinical behaviour • INDOLENT • leukemisation common • occasional transformation to aggressive lymphoma / leukemia Dg: Morphology confusing - immunophenotyping necessary CD23+, CD5+, cyclin D1 –

  27. Leukemia Chronic morphology:bone marrow infiltration splenomegaly hepatomegaly enlarged lymph nodes clinic: may remain silent for a long time CML – related to myeloproliferative disorders CLL – close to nH ML (95%B) Hairy cell leukemia tricholeukemia – small B lymphoid cells

  28. Lymphoplasmocytic lymphoma LPL /Waldenström macroglobulinemia • small B lymhocytes lymphoma • bone marrow, LN, spleen • older adults • monoclonal IgM serum paraprotein • hyperviscosity symptoms

  29. Burkitt´s Lymphoma • highly malignant small B cell lymphoma EBV (DNA) related • endemic in Africa, sporadic elsewhere • high mitotic rate • „starry sky“ appearance (due to non neoplastic macrophages admixture)

  30. Lymphoid Neoplasms • B cell (lymphomas & leukaemias) • T and NK cell -“- • Hodgkin lymphomas

  31. Lymphoid Neoplasms T and NK cell lymphomas & leukaemias (16 nosology units in 2001 WHO classif.) • Precursor T-cell neoplasm • Mature T-cell neoplasms(EB virus HTLV-1) • T-cell proliferations of uncertain malignant potential

  32. Mycosis Fungoides and Sezary Syndrome Def.: MF:mature T- cell lymphoma presenting in the skin with patches/plaques and characterized by epidermal and dermal infiltration of small to medium size T-cells with cerebriform nuclei SS:generalized mature T- cell lymphoma characterized by the presence of erythroderma , lymphadenopathy and T-cells with cerebriform nuclei – aggresive form of MF

  33. Mycosis fungoides and Sezary Syndrome • adults M:F 2:1 • years lasting course • trunk erruptions • rarely generalization

  34. Lymphoid Neoplasms • B cell (lymphomas & leukaemias) • T and NK cell -“- • Hodgkin lymphomas

  35. Lymphogranuloma Malignum Hodgkin def. malignant lymphoma containing diagnostic RSor Hodgkin tumorous cells in a rich cellular background

  36. Lymphoid Neoplasms WHO 2001 • Hodgkin lymphomas HL • Nodular lymphocyte predominant HL • Classical HL • Nodular sclerosis classical HL • Lymphocyte-rich classical HL • Mixed cellularity classical HL • Lymphocyte-depleted classical HL

  37. Classical HL (CHL) • 85% of HL • Nodular sclerosis CHL (NSHL) • Most frequent, young adults • Very good prognosis with treatment • Lymphocyte-rich CHL (LRCHL) • Rare • Very good prognosis with treatment • Mixed cellularity CHL (MCHL) • Frequent, adults • Medium prognosis • Lymphocyte –depleted CHL (LDHL) • Very rare, immunocompromised patients • Poor prognosis

  38. Nodular Lymphocyte Predominant HL 15% of HL B-lymphoma Differential diagnosis may be very difficult Highly atypical CD30-/CD15-/CD20+/CD45+ L/H cells = popcorn cells Reactive cells Lymphocytes, histiocytes, plasma cells, no eosinophils Nodular growth No fibrosis Very good prognosis with treatment even in relapsing disease

  39. NEOPLASMS OF HEMATOPOIETIC AND LYMPHOID TISSUE(WHO 2001, abbreviated) Myeloid neoplasms Chronic myeloproliferative disorders Myeloproliferative / myelodysplastic diseases Myelodysplastic syndromes Acute myeloid leukemias Lymphoid neoplasms Precursor B and T cell neoplasms Mature B cell neoplasms Mature T and NK cell neoplasms Hodgkin lymphoma Histiocytic and dendritic cell neoplasms Histiocytic sarcoma Dendritic cell neoplasms Mastocytosis Most of them originate / may originate in lymph nodes

  40. Histiocytic and Dendritic Cell Neoplasms • Macrophage/histiocytic neoplasm (CD68, CD 1) • Langerhans ´cells: • m. Hand –Schüller- Christian HSCH triad : calva defects, diab.insip., exophtalmos • eosinophilic granuloma (bone) • m. Letterer Sive (skin , hepatosplenomegaly, lymph nodes) • Dendritic cell neoplasms • Mastocytosis

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