LYMPHOMA FOR THE GENERALISTLee Berkowitz, MD
GOALS AND OBJECTIVES
1. Understand the importance of pathology and staging in the approach to management of patients with lymphoma.
2. Recognize that a lymph node biopsy is the correct procedure to diagnose lymphoma.
3. Appreciate that the approach to the management of patients with lymphoma is varied and idividualized.
NON-HODGKINS
EPIDEMIOLOGY
5th most common cancer in adults Incidence is increasing 2-3% per year
PATHOGENESIS
1. Immune suppression/dysregulation – HIV, organ transplant, RA, SCID
2. H. pylori – MALT 3. EBV – Burkitts, ? Hodgkins 4. HHV 8 – Castlemans 5. HTLV 1 – T cell leukemia, lymphoma 6. t(14;18) – follicular 7. t(11;14) – Mantle cell 8. t(8;14) - Burkitts
Pathology Staging
NON-HODGKINS The indolent lymphomas B-cell neoplasms Small
lymphocytic lymphoma/B-cell chronic lymphocytic leukemia Lymphoplasmacytic lymphoma (± Waldenstrom's macroglobulinemia) Plasma cell myeloma/plasmacytoma Hairy cell leukemia Follicular lymphoma (grade I and II) Marginal zone B-cell lymphoma Mantle cell lymphoma T-cell neoplasms T-cell large granular lymphocyte leukemia Mycosis fungoides T-cell prolymphocytic leukemia Natural killer cell neoplasms Natural killer cell large granular lymphocyte leukemia
The aggressive lymphomas B-cell neoplasms Follicular lymphoma (grade III) Diffuse large B-cell lymphoma Mantle cell lymphoma T-cell neoplasms Peripheral T-cell lymphoma Anaplastic large cell lymphoma, T/null cell
The highly aggressive lymphomas B-cell neoplasms Burkitt's lymphoma Precursor B lymphoblastic leukemia/lymphoma T-cell neoplasms Adult T-cell lymphoma/leukemia Precursor T lymphoblastic
PATHOLOGY
Key aspects – follicular vs diffuse size of the cells in their normal environment
FOLLICULAR PATTERN
DIFFUSE PATTERN
PATHOLOGY
Nodal architecture The ONLY way to get this information
is to biopsy or excise a node. A fine needle aspiration will not be adequate.
PATHOLOGY
Indolent Aggressive follicular grades I,II follicular grade III marginal zone diffuse large cell MALT mantle cell Burkitts
NATURAL HISTORY OF LYMPHOMAS
Indolent(Follicular) – mean survival of 8 years Aggressive(Diffuse)– mean survival of 12
months Highly aggressive – mean survival of 8- 10
weeks
STAGING
I. 1 nodal group II. 2 nodal groups on the same side of the
diaphragm III. Disease above and below the diaphragm IV. Disease in other organs
STAGING
Physical examination CT scans Bone marrow biopsies
STAGING NON-HODGKINS
Low –Grade I-II III- IV
STAGING NON-HODGKINS
Low-Grade I-II 5% III-IV 95%
STAGINGNON-HODGKINS
Intermediate Grade I-II 30% III-IV 70%
INTERNATIONAL PROGNOSTIC INDEX(IPI) DIFFUSE LYMPHOMAS
Age>60 LDH> normal Performance status Stage III or IV Two or more extra nodal sites
IPI
Risk Risk sum 5 yr survival % Low 0-1 73 Low –Interm 2 51 High –Interm 3 43 High 4-5 26
FOLLICULAR LYMPHOMA IPI Age>60 Stage III/IV LDH>normal Anemia 5 or more nodal sites
FLIPI
Risk Risk score 10 yr survival Low 0-1 70% Interm 2 50% High 3 36%
TREATMENT LOW-GRADE
1. These cells over express bcl 2 2. The median survival for these patients
untreated is 8 years
TREATMENTNON-HODGKINS LOW-GRADE
1. Observation 2. Standard chemo 3. Monoclonal antibodies – rituximab 4. Stem-cell transplants
TREATMENTNON-HODGKINS
Intermediate and High-grade – Cure with chemotherapy
HODGKINS
EPIDEMIOLOGY
8000 new patients per year Bimodal distribution – one peak at 30 years one peak at 50 years
PATHOGENESIS - HODGKINS
1. EBV 2 NF- kB ( nuclear factor kappa B)
HODGKINS
1. Lymphocyte Predominant2. Nodular Sclerosing3. Mixed Cellularity4. Lymphocyte Depleted
STAGINGHODGKINS
I. 15% II. 35% III.35% IV.15%
TREATMENTHODGKINS
Stage I – Cure with radiation therapy Stage II, III, IV – Cure with chemotherapy
WHEN TO SUSPECT LYMPHOMA
1. Patients with impaired immune systems HIV, Transplant, Autoimmune diseases 2. Patients with unexplained fever, night
sweats, weight loss 3. Patients with lymphadenopathy
LYMPHADENOPATHY
Medicine 79:338 – 47, 2000 Biopsy or not
Neg Positive Tenderness Generalized Pruritus Size < 1cm Supraclavicular Hard Size > 2 cm
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