L YMPHOMA FOR THE G ENERALIST Lee Berkowitz, MD. G OALS AND O BJECTIVES 1. Understand the importance...

33
LYMPHOMA FOR THE GENERALIST Lee Berkowitz, MD

Transcript of L YMPHOMA FOR THE G ENERALIST Lee Berkowitz, MD. G OALS AND O BJECTIVES 1. Understand the importance...

Page 1: L YMPHOMA FOR THE G ENERALIST Lee Berkowitz, MD. G OALS AND O BJECTIVES 1. Understand the importance of pathology and staging in the approach to management.

LYMPHOMA FOR THE GENERALISTLee Berkowitz, MD

Page 2: L YMPHOMA FOR THE G ENERALIST Lee Berkowitz, MD. G OALS AND O BJECTIVES 1. Understand the importance of pathology and staging in the approach to management.

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.

Page 3: L YMPHOMA FOR THE G ENERALIST Lee Berkowitz, MD. G OALS AND O BJECTIVES 1. Understand the importance of pathology and staging in the approach to management.

NON-HODGKINS

Page 4: L YMPHOMA FOR THE G ENERALIST Lee Berkowitz, MD. G OALS AND O BJECTIVES 1. Understand the importance of pathology and staging in the approach to management.

EPIDEMIOLOGY

5th most common cancer in adults Incidence is increasing 2-3% per year

Page 5: L YMPHOMA FOR THE G ENERALIST Lee Berkowitz, MD. G OALS AND O BJECTIVES 1. Understand the importance of pathology and staging in the approach to management.

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

Page 6: L YMPHOMA FOR THE G ENERALIST Lee Berkowitz, MD. G OALS AND O BJECTIVES 1. Understand the importance of pathology and staging in the approach to management.

Pathology Staging

Page 7: L YMPHOMA FOR THE G ENERALIST Lee Berkowitz, MD. G OALS AND O BJECTIVES 1. Understand the importance of pathology and staging in the approach to management.

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

Page 8: L YMPHOMA FOR THE G ENERALIST Lee Berkowitz, MD. G OALS AND O BJECTIVES 1. Understand the importance of pathology and staging in the approach to management.

PATHOLOGY

Key aspects – follicular vs diffuse size of the cells in their normal environment

Page 9: L YMPHOMA FOR THE G ENERALIST Lee Berkowitz, MD. G OALS AND O BJECTIVES 1. Understand the importance of pathology and staging in the approach to management.

FOLLICULAR PATTERN

Page 10: L YMPHOMA FOR THE G ENERALIST Lee Berkowitz, MD. G OALS AND O BJECTIVES 1. Understand the importance of pathology and staging in the approach to management.

DIFFUSE PATTERN

Page 11: L YMPHOMA FOR THE G ENERALIST Lee Berkowitz, MD. G OALS AND O BJECTIVES 1. Understand the importance of pathology and staging in the approach to management.

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.

Page 12: L YMPHOMA FOR THE G ENERALIST Lee Berkowitz, MD. G OALS AND O BJECTIVES 1. Understand the importance of pathology and staging in the approach to management.

PATHOLOGY

Indolent Aggressive follicular grades I,II follicular grade III marginal zone diffuse large cell MALT mantle cell Burkitts

Page 13: L YMPHOMA FOR THE G ENERALIST Lee Berkowitz, MD. G OALS AND O BJECTIVES 1. Understand the importance of pathology and staging in the approach to management.

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

Page 14: L YMPHOMA FOR THE G ENERALIST Lee Berkowitz, MD. G OALS AND O BJECTIVES 1. Understand the importance of pathology and staging in the approach to management.

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

Page 15: L YMPHOMA FOR THE G ENERALIST Lee Berkowitz, MD. G OALS AND O BJECTIVES 1. Understand the importance of pathology and staging in the approach to management.

STAGING

Physical examination CT scans Bone marrow biopsies

Page 16: L YMPHOMA FOR THE G ENERALIST Lee Berkowitz, MD. G OALS AND O BJECTIVES 1. Understand the importance of pathology and staging in the approach to management.

STAGING NON-HODGKINS

Low –Grade I-II III- IV

Page 17: L YMPHOMA FOR THE G ENERALIST Lee Berkowitz, MD. G OALS AND O BJECTIVES 1. Understand the importance of pathology and staging in the approach to management.

STAGING NON-HODGKINS

Low-Grade I-II 5% III-IV 95%

Page 18: L YMPHOMA FOR THE G ENERALIST Lee Berkowitz, MD. G OALS AND O BJECTIVES 1. Understand the importance of pathology and staging in the approach to management.

STAGINGNON-HODGKINS

Intermediate Grade I-II 30% III-IV 70%

Page 19: L YMPHOMA FOR THE G ENERALIST Lee Berkowitz, MD. G OALS AND O BJECTIVES 1. Understand the importance of pathology and staging in the approach to management.

INTERNATIONAL PROGNOSTIC INDEX(IPI) DIFFUSE LYMPHOMAS

Age>60 LDH> normal Performance status Stage III or IV Two or more extra nodal sites

Page 20: L YMPHOMA FOR THE G ENERALIST Lee Berkowitz, MD. G OALS AND O BJECTIVES 1. Understand the importance of pathology and staging in the approach to management.

IPI

Risk Risk sum 5 yr survival % Low 0-1 73 Low –Interm 2 51 High –Interm 3 43 High 4-5 26

Page 21: L YMPHOMA FOR THE G ENERALIST Lee Berkowitz, MD. G OALS AND O BJECTIVES 1. Understand the importance of pathology and staging in the approach to management.

FOLLICULAR LYMPHOMA IPI Age>60 Stage III/IV LDH>normal Anemia 5 or more nodal sites

Page 22: L YMPHOMA FOR THE G ENERALIST Lee Berkowitz, MD. G OALS AND O BJECTIVES 1. Understand the importance of pathology and staging in the approach to management.

FLIPI

Risk Risk score 10 yr survival Low 0-1 70% Interm 2 50% High 3 36%

Page 23: L YMPHOMA FOR THE G ENERALIST Lee Berkowitz, MD. G OALS AND O BJECTIVES 1. Understand the importance of pathology and staging in the approach to management.

TREATMENT LOW-GRADE

1. These cells over express bcl 2 2. The median survival for these patients

untreated is 8 years

Page 24: L YMPHOMA FOR THE G ENERALIST Lee Berkowitz, MD. G OALS AND O BJECTIVES 1. Understand the importance of pathology and staging in the approach to management.

TREATMENTNON-HODGKINS LOW-GRADE

1. Observation 2. Standard chemo 3. Monoclonal antibodies – rituximab 4. Stem-cell transplants

Page 25: L YMPHOMA FOR THE G ENERALIST Lee Berkowitz, MD. G OALS AND O BJECTIVES 1. Understand the importance of pathology and staging in the approach to management.

TREATMENTNON-HODGKINS

Intermediate and High-grade – Cure with chemotherapy

Page 26: L YMPHOMA FOR THE G ENERALIST Lee Berkowitz, MD. G OALS AND O BJECTIVES 1. Understand the importance of pathology and staging in the approach to management.

HODGKINS

Page 27: L YMPHOMA FOR THE G ENERALIST Lee Berkowitz, MD. G OALS AND O BJECTIVES 1. Understand the importance of pathology and staging in the approach to management.

EPIDEMIOLOGY

8000 new patients per year Bimodal distribution – one peak at 30 years one peak at 50 years

Page 28: L YMPHOMA FOR THE G ENERALIST Lee Berkowitz, MD. G OALS AND O BJECTIVES 1. Understand the importance of pathology and staging in the approach to management.

PATHOGENESIS - HODGKINS

1. EBV 2 NF- kB ( nuclear factor kappa B)

Page 29: L YMPHOMA FOR THE G ENERALIST Lee Berkowitz, MD. G OALS AND O BJECTIVES 1. Understand the importance of pathology and staging in the approach to management.

HODGKINS

1. Lymphocyte Predominant2. Nodular Sclerosing3. Mixed Cellularity4. Lymphocyte Depleted

Page 30: L YMPHOMA FOR THE G ENERALIST Lee Berkowitz, MD. G OALS AND O BJECTIVES 1. Understand the importance of pathology and staging in the approach to management.

STAGINGHODGKINS

I. 15% II. 35% III.35% IV.15%

Page 31: L YMPHOMA FOR THE G ENERALIST Lee Berkowitz, MD. G OALS AND O BJECTIVES 1. Understand the importance of pathology and staging in the approach to management.

TREATMENTHODGKINS

Stage I – Cure with radiation therapy Stage II, III, IV – Cure with chemotherapy

Page 32: L YMPHOMA FOR THE G ENERALIST Lee Berkowitz, MD. G OALS AND O BJECTIVES 1. Understand the importance of pathology and staging in the approach to management.

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

Page 33: L YMPHOMA FOR THE G ENERALIST Lee Berkowitz, MD. G OALS AND O BJECTIVES 1. Understand the importance of pathology and staging in the approach to management.

LYMPHADENOPATHY

Medicine 79:338 – 47, 2000 Biopsy or not

Neg Positive Tenderness Generalized Pruritus Size < 1cm Supraclavicular Hard Size > 2 cm