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Leukaemias and Malignant Lymphomas

Acute leukaemias. Presentation: Bleeding Infection Fatigue Leukaemic blood smear or

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Leukaemias and Malignant Lymphomas

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    1. Basic Principles of Internal Medicine Leukaemias and Malignant Lymphomas Szabolcs Modok Dentists, 4th year, Internal medicine

    2. Acute leukaemias Presentation: Bleeding Infection Fatigue Leukaemic blood smear or “hiatus leukaemicus” Pancytopenia Basic Principles of Internal Medicine

    3. Acute myeloid vs lymphoid leukaemia Myeloid Elderly MPO, CD34, etc Sudan black stain DIC Karyotypes (good): t(8:12) t(15:17) inv(16) Lymphoid Younger age T cell (CD3, CD4) B cell (CD10,19,20) Lymphadenomegaly Karyotypes (bad): t(9:22) Basic Principles of Internal Medicine

    4. AML clinical subtypes De novo AML Secondary: after MDS or myeloproliferative disease Treatment related (~ 5 years after chemo- or radiotherapy) Basic Principles of Internal Medicine

    5. Acute Myeloid Leukaemia: treatment Remission INDUCTION: 7+3, i.e. cytarabine + anthracycline CONSOLIDATION: high dose cytarabine (3 courses) AML M3/promyelocytic: ATRA, AsO3 New agents: anti-CD33 antibody, clofarabine Allogeneic stem cell transplantation Basic Principles of Internal Medicine

    6. Acute lymphoid leukaemia: treatment Induction + consolidation + reinduction + maintenance = 2 years Drugs: vincristine, anthracyclines, steroid, cytarabine CNS prophylaxis t(9:22): imatinib + early SCT Basic Principles of Internal Medicine

    7. WHO classification of myeloproliferatív diseases Classic Molecularly defined: CML (Bcr/abl+) Clinico-pathological definition: (Bcr/abl- és gyakori a JAK2V617F): Polycythaemia vera (100%) Essential thrombocythaemia (50%) Myelofibrosis (50%)

    8. Bcr/abl és imatinib

    9. Imatinib: milestone in the treatment of CML

    10. Basic Principles of Internal Medicine Malignant Lymphomas Definition: uncontrolled proliferation of lymphoid cells Classification: Hodgkin’s Lymphomas Non-Hodgkin Lymphomas

    11. Basic Principles of Internal Medicine Facts about lymphomas (ACC, 2006 estimates) Hodgkin’s lymphoma stable incidence over last 20 years Survival 1 year 93 % 5 years 85 % 10 years 80 % Non-Hodgkin’s lymphoma incidence doubled since early 1970s Survival 1 year 78 % 5 years 60 % 10 years 49%

    12. Basic Principles of Internal Medicine Hodgkin’s lymphoma Nodular lymphocyte-predominant Hodgkin’s lymphoma (LPHD) Classical Hodgkin’s lymphoma 65 % Nodular sclerosis Hodgkin’s lymphoma 5 %Lymphocyte-rich classical Hodgkin’s lymphoma 25 % Mixed-cellularity Hodgkin’s lymphoma 5 % Lymphocyte-depleted Hodgkin’s lymphoma

    13. Basic Principles of Internal Medicine Diagnosis lymph node excision, i.e. histology Staging & prognosis Chest and abdominal CT scans Bone marrow biopsy Full blood count, ESR, CRP, Alb, ALP, LDH Clinical staging (CS) according to Ann Arbor (bulky disease, spleen and extranodal involvement) Hodgkin’s lymphoma

    14. Basic Principles of Internal Medicine Ann Arbor clinical staging One lymph node region Two or more region on the same side of the diaphragm Multiple lymph node regions on both sides of the diaphragm Extra-lymphatic organ involvement

    15. Basic Principles of Internal Medicine B symptoms & Risk assessment

    16. Basic Principles of Internal Medicine Hodgkin’s lymphoma - LPHD Stage I – involved field irradiation (30 Gy) Recurrent disease – avoid aggressive treatment, because it is indolent Rituximab

    17. Basic Principles of Internal Medicine Limited stage: 2 - 4 cycles of ABVD with involved field irradiation (30 –36 Gy) Intermediate stage: 4 cycles of ABVD with involved field irradiation (30 –36 Gy) Advanced stage: 8 cycles of ABVD (or BEACOPP) with involved field irradiation to bulky tumours (> 7.5 cm; 30 – 36 Gy) or to residual tumour mass after chemotherapy. Hodgkin’s lymphoma - Classic

    18. Basic Principles of Internal Medicine Physical examination, blood tests and CT scans after the 4th and the last cycle of chemo/radiotherapy Biopsy Repeated radiology scans PET CT (negative predictive value) Hodgkin’s lymphoma – Response evaluation

    19. Basic Principles of Internal Medicine History and physical examination every 3 months for a year, every 6 months for 3 years, then once a year Laboratory analysis and chest X-ray at 6, 12 and 24 months CT scans once to confirm remission status Thyroid function after neck irradiation after 1, 2 and 5 years After chest irradiation for premenopausal, and especially at an age below 25 years, women should be screened for secondary breast cancer clinically, and after the age of 40 -50, by mammography Hodgkin’s lymphoma – Follow up

    20. Basic Principles of Internal Medicine DHAP, Dexa-BEAM, EPOCH & SCT for chemosensitive patients with good performance status Experimental treatments, low intensity chemo or local radiotherapy for others Hodgkin’s lymphoma – Relapse

    21. Basic Principles of Internal Medicine Non-Hodgkin’s lymphomas (WHO classification) B-cell neoplasms Precursor B-cell neoplasm: precursor B-acute lymphoblastic leukemia/lymphoblastic lymphoma (LBL) Peripheral B-cell neoplasms: B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma B-cell prolymphocytic leukemia Lymphoplasmacytic lymphoma/immunocytoma Mantle cell lymphoma Follicular lymphoma Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphatic tissue (MALT) type Nodal marginal zone B-cell lymphoma (± monocytoid B-cells) Splenic marginal zone lymphoma (± villous lymphocytes) Hairy cell leukemia Plasmacytoma/plasma cell myeloma Diffuse large B-cell lymphoma Burkitt's lymphoma T-cell and putative NK-cell neoplasms Precursor T-cell neoplasm: precursor T-acute lymphoblastic leukemia/LBL Peripheral T-cell and NK-cell neoplasms: T-cell chronic lymphocytic leukemia/prolymphocytic leukemia T-cell granular lymphocytic leukemia Mycosis fungoides/Sézary syndrome Peripheral T-cell lymphoma, not otherwise characterized Hepatosplenic gamma/delta T-cell lymphoma Subcutaneous panniculitis-like T-cell lymphoma Angioimmunoblastic T-cell lymphoma Extranodal T-/NK-cell lymphoma, nasal type Enteropathy-type intestinal T-cell lymphoma Adult T-cell lymphoma/leukemia (human T-lymphotrophic virus [HTLV] 1+) Anaplastic large cell lymphoma, primary systemic type Anaplastic large cell lymphoma, primary cutaneous type Aggressive NK-cell leukemia

    22. Basic Principles of Internal Medicine Chronic Lymphocytic Leukaemia (CLL) Incidence: 3/100000 Diagnosis: > 5*109/L lymphocytes in blood CD19 & CD5/23, CD20 + bone marrow infiltration > 30 % lymph node histology Immunodeficiency (infection/second tumour) Autoimmune phenotypes

    23. Basic Principles of Internal Medicine CLL – Modified Rai Staging

    24. Basic Principles of Internal Medicine CLL - Treatment Watch & wait in stable limited disease Curative intent in selected cases (< 60 years, allogen SCT) Palliative therapy in most cases chlorambucil cyclophosphamide fludarabine alemtuzumab Glucocorticosteroids/azathioprin Cyclosporin A Immunoglobulins Pneumovax 23

    25. Basic Principles of Internal Medicine Diffuse Large B-Cell Lymphoma (DLBCL) Incidence: 3 - 4/100000 Subtypes: 1. primary mediastinal 2. intravascular 3. T-cel/histiocyte rich 4. lymphomatoid granulosis like 5. primary effusion

    26. Basic Principles of Internal Medicine ECOG performance score 0 no symptoms 1 symptomatic, out patient 2 symptomatic, < 50 % in bed 3 symptomatic, > 50 % in bed 4 bedridden, inpatient care is necessary

    27. Basic Principles of Internal Medicine Age adjusted international prognostic index (aaIPI) Stage III & IV serum LDH ? ECOG = 2

    28. Basic Principles of Internal Medicine DLBCL - Treatment < 60 years of age Low risk: 6 x R-CHOP (21) High risk: 8 x R-CHOP (14) HDCT + SCT > 60 years of age 8 x R-CHOP (21)

    29. Basic Principles of Internal Medicine Follicular lymphoma (FL) Incidence: 5 - 7/100000 (rising) Bcl2 overexpression due to t(14:18) or t(18:22) CD20/CD19/CD10 + CD5 –

    30. Basic Principles of Internal Medicine I Centrocytes & < 5 centroblast per large viewfield II Centrocytes & 6 -15 centroblast per large viewfield III/A < 15 centroblast per large viewfield with centrocytes III/B > 15 centroblast per large viewfield without centrocytes FL – WHO grades

    31. Basic Principles of Internal Medicine FL + prognosis = FLIPI Risk factors > 60 years of age Stage III & IV (75 – 80 %) > 4 lymph node areas Serum LDH ?

    32. Basic Principles of Internal Medicine Stage I: extended field irradiation with curative intent Stage II – IV: 15 – 20 % spontaneous regression treatment is only indicated for progressive disease fludarabine chlorambucil rituximab CHOP remission maintenance or consolidation IFN – a rituximab radioimmunotherapy, HDCT + SCT FL – Treatment

    33. Basic Principles of Internal Medicine Multiple myeloma (MM) Incidence: 6/100000 Diagnosis: M – protein in urine and/or plasma immunofixation plasma cells in the bone marrow (%) lytic bone lesions

    34. Basic Principles of Internal Medicine MM – Durie – Salmon staging

    35. Basic Principles of Internal Medicine MM – MRI/PET staging

    36. Basic Principles of Internal Medicine MM – International Prognostic Index (IPI)

    37. Basic Principles of Internal Medicine MM - Treatment Watch & wait in indolent disease Standard: melphalan (9mg/m2/day; 4 days) and prednisone (30mg/m2/day; 4 days) repeated every 4 – 6 weeks until stable disease INF - ? prolongs plateau phase (3 MU/m2 sc; 3 x weekly) Bisphosphonates HD melphalan (200 mg/m2 iv) + APBSCT (< 65 years, no renal impairment) after VAD induction

    38. Indications for autologous SCT Agressive lymphoma (chemosensitive relapse) Follicular lymphoma (transformation to agressive lymphoma) Multiple myeloma (chemosensitive disease) Hodgkin lymphoma (chemosensitive relapse)

    39. Allogeneic SCT AML (intermediate or poor prognosis in remission) Adult ALL (remission) Aplastic anemia CML (special situations)

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