Ch. 8 Pathology of Lymph nodes

XIII. Clinical features- non-Hodgkin's lymphoma

A. Clinical presentation
- 1. Enlarging mass(es), typically painless, at sites of nodal tissue
- 2. Obstruction, ulceration of hollow organs- MALT- pain
- 3. Interference with normal organ function- solid organ infiltration- kidneys, liver, bone marrow
B. Clinical staging of lymphomas
- 1. Defines extent of disease; determines therapy and prognosis
- 2. Based on physical, radiologic examination, bone marrow biopsy and aspiration
- 3. Ann Arbor Staging system
- 4. B symptoms- fever, weight loss > 10% body weight, night sweats

Ann Arbor Staging System

Stage	Distribution of Disease
I	Involvement of a single lymph node region (I) or involvement of a single extralymphatic organ or site (IE)
II	Involvement of two or more lymph node regions on the same side of the diaphragm alone (II) or with involvement of limited contiguous extralymphatic organ or tissue (IIE)
III	Involvement of lymph node regions on both sides of the diaphragm (III), which may include the spleen (IIIs) and/or limited contiguous
IV	Multiple or disseminated foci of involvement of one or more extralymphatic organs or tissues with or without lymphatic involvement.

C. Therapy
- 1. Seminar cases will also discuss
- 2. Indolent lymphomas, limited stage (5-10% cases) treated with radiation therapy- can be curative
- 3. Indolent lymphomas, disseminated (90%) treated by
  - a. Low morbidity limited chemotherapy
    - 1) No expectation of cure
    - 2) Older patients
    - 3) Most will respond totally or partially, with months to years of disease free survival, but will relapse
    - 4) Many will respond to additional rounds of similar or alternative regimens
    - 5) Pts will eventually die of their disease, or another disease of elderly
    - 6) Death from disease due to
      - a.) Immune suppression- infections
      - b.) Progression to aggressive lymphoma
  - b. Bone marrow transplant (allo/auto/peripheral stem cell)
    - 1) Effort at cure-
    - 2) Younger patients
- 4. Aggressive lymphomas treated with multiagent (>/= 4 drugs) chemotherapy
  - a. Complete remission rates 60-80%
  - b.30-40% cured
  - c. Therapy determined by prognostic index- see below
- 5. Newer therapies and their roles still being established
  - a. Autologous bone marrow transplantation/high dose chemotherapy with peripheral stem cell harvest
  - b. Immunotherapy
D. Prognosis
- 1. Primarily based on inherent biology of tumor and stage
- 2. Are however additional factors that can be used for prognosis
  - a. International prognostic index- word recognition
    - 1). Five clinical and laboratory features proven by regression analysis to best stratify patients with large cell lymphoma into four risk groups with statistically different survival rates
    - 2). Used to decide on aggressiveness of therapy
    - 3). Used in evaluation of new therapies
    - 4). Although developed for large cell lymphoma, also has utility in stratifying indolent lymphomas
  - b. Cytogenetics/Oncogenes- know they exist
    - 1) Are cytogenetic/molecular abnormalities that predict more aggressive clinical course
    - 2) Others can predict acceleration/transformation from low to higher grade lymphoma
    - 3) Not routinely used beyond tertiary care centers; practicality/cost effectiveness still being established
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