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Lymphomas (Non Hodgkin’s (Standard therapy (Agressive NHL:
30% standard…
Lymphomas
Non Hodgkin’s
- B cell cancers by cell development *
Classification
- Indolent (low grade)
- Agressive (intermediate grade)
- Very agressive (high grade)
- Special types:
advanced follicular NHL (incurable)
Mantle cell NHL (MCL) (poor prognosis, median OS 3 Y)
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Standard therapy
- Agressive NHL:
30% standard chemotherapy + Rituximab
Treatment failures = HDC/aut PHSCT
- Advanced low/intermediate grade:
incurable
Treatment options: CVP, FND, FCR, CHOP, Rituximab, Radioimmunotherapy, Ibrutinib, Thalidomide, Lenalidomide
New therapies
- Monoclonal antibodies (galiximab anti-CD80)
- mTOR inhibitors (temsirolimus)
- Oligonucleotides (G3139)
- Vaccines
- Bendamustin (HN2 + benzimidazol group)
- Proteasom inhibitors (bortezomib)
- Bcl-2 inhibitors
symptoms
- Lymph node enlargement (supraclavicular, axillary, neck, painless lump, usually without inflammatory signs)
- general symptoms: Fever, Weight loss, Drenching sweats, Itching
- B symptoms: fever, night sweet, weight loss
CNS NHL
- Most commonly deep structures of frontal and or occipital lobes involvement, in 75% ependymal infiltration, characteristic – no calcification, no bleeding
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Prognostic factors
early disease
1 - bulky mediastinum
2 - extranodal involvement
3 - ESR >50 without general symptoms, or >30 if general symptoms present
4 - ≥ 3 lymph node regions involved
advanced disease
- Hypoalbuminemia < 4g%
- Anemia Hb < 10.5g%
- Sex: Male
- Age ≥ 45 years
- Leukocytosis >15000
- Lymphopenia < 8%
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Etiology
- Immunosuppresion
- Diet, oncogens, herbicydes, alcohol, smoking
- response to the microbial exposure:
Mycobacterium, Helicobacter, Viruses (EBV (LMP1 i 2, EBNA), HCV, HTLV 1 i 2, Measle virus)
Pathogenesis
- Somatic mutations
- Viral oncogenesis
- Immunologic dysregulation
Histopathology
- Mononuclear Hodgkin’s cells and polynuclear Reed-Sternberg cells constitue 0.1 to 1.0% of all cells in involved lymph nodes
- Immunohistochemistry - positive expression of CD30, CD15, CD25, CD20 (LP), CD70, Ki- 27
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Clinical staging
- History and physical examination
- Chest X-ray- CT of the chest and abdomen
- CT of the pelvis only in cases of positve abdominal or inguinal lymph nodes
- Bone marrow examination
- Histopathological examination of the unusual fluids
- Histopathological examination of all suspicious extranodal foci
- Laparotomy, Scyntygraphy, PET (18-FDG), Ann Arbor (Cotswold’s modification) classification
Treatment
HL
Early stages
- radiotherapy (36 Gy)
- chemotherapy
Active drugs: doxorubicin, bleomycin, vinblastin, dacarbazine, etoposide, cyclophosphamide, prednison, vincristin
(ABVD 4 cycles, or +2 after CR)
- combined treatment
2 - 4 cycles of chemotherapy (ABVD) plus involved field radiotherapy (26-36 Gy)
advanced stages
- 6-8 cycles of chemotherapy (ABVD, BEACOPP, Stanford V)
- No benefit from mantle, or total nodal irradiation (TNI) added to chemotherapy
- Modest benefit from radiotherapy to the residual disease
treatment of relapses
- High-dose chemotherapy + autologous hematopoetic stem cells transplantation
- Further relapses: brentuximab vedotin = an antibody-drug conjugate (ADC) directed to the protein CD30, which is expressed in classical Hodgkin lymphoma (HL) and systemic anaplastic large cell lymphoma (sALCL).
Causes of death
- Hodgkin’s lymphoma
- Secondary malignancy, secondary leukemia
- Cardiac, Pulmonary
- Bimodal distribution (young adults and >60 years of age)
- Male : Female = 1,4:1,0 (exception NS type)
- Race: white > non-white