Malignant Lymphoma

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Updated 7 March 2012

1. Diagnosis and Pathologic Classification

The diagnosis of malignant lymphoma requires the presence of malignant lymphocytes in a biopsy of lymph node or extra-lymphatic tissue. An excisional lymph node biopsy is essential for complete diagnostic assessment. If a whole lymph node is not obtainable, sufficient incised tissue from an extra-lymphatic site can be diagnostic but is less desirable. Fine needle aspiration biopsy (see Appendix I: Biopsy Procedures) is not sufficient for the initial diagnosis of malignant lymphoma. The following histologic sub-classification of the malignant lymphomas is an adaptation of the Working Formulation and the WHO/REAL classification (Jaffe E, Tumours of Hematopoietic and Lymphoid Tissues, World Health Organization Classification of Tumours, IARC Press, 2001) and is based on the light microscopic interpretation complemented by special stains, immunophenotyping, cytogenetics and other information as available. The specific lymphomas are divided into three major groups for treatment planning. Table 3.1 A clinically oriented classification of the lymphomas based on similar natural histories, modes of presentation and responses to treatment using the terminology of the REAL classification scheme: Grade Indolent B-cell Small lymphocytic* Lymphoplasmacytic** Follicular, grade 1, 2 or 3 A Marginal zone MALT*** nodal splenic T-cell Mycoses fungoides

Aggressive Follicular, grade 3 B Mantle cell Diffuse large cell+, any type Burkitt-like (small noncleaved cell)

Peripheral T cell, unspecified Peripheral T-cell, specified Angioimmunoblastic (AIL) Nasal T/NK cell Subcutaneous panniculitic Enteropathy associated Anaplastic large cell (CD30 positive) including null cell Lymphoblastic

Special

Burkitt

* Small lymphocytic lymphoma is biologically similar to chronic lymphocytic leukemia and is treated the same as CLL

** Includes Waldenstroms Macroglobulinemia *** Mucosa-associated lymphoid tissue + Includes primary mediastinal, T-cell rich B-cell, immunoblastic and intravascular variants of diffuse large B-cell lymphoma

2. Staging
The stage of disease is of major therapeutic and prognostic significance in the management of malignant lymphoma. The staging system used at the BC Cancer Agency is based on the Ann Arbor system with additional consideration of the bulk or size of individual tumours. The formal stage is assigned using the following system. Stage Involvement 1 2 3 4 Single lymph node region (1) or one extralymphatic site (1 E). Two or more lymph node regions, same side of the diaphragm (2) or local extralymphatic extension plus one or more lymph node regions same side of the diaphragm (2 E) Lymph node regions on both sides of diaphragm (3) which may be accompanied by local extralymphatic extension (3E) Diffuse involvement of one or more extralymphatic organs or sites

Symptoms A= B= no B symptoms presence of at least one of these: 1) 2) 3) Bulk Bulky = any tumour diameter > 10 cm Non bulky =all tumour diameters < 10 cm For treatment planning purposes patients with malignant lymphoma are divided into two groups by stage: Limited Stage: Stage 1 or Stage 2 confined to 3 or fewer adjacent lymph node regions No B symptoms and Non-bulky tumour (<10 cm) Advanced Stage: unexplained weight loss > 10% baseline during 6 months prior to staging unexplained fever > 38oC/font> night sweats

Stage 2 with disease beyond 3 adjacent lymph node regions or Stage 3 or 4 or B symptoms or Bulky tumour (> 10 cm)

Mandatory Staging Procedures

Pathology review 1. 2. 3. 4. 5. All patients should receive the immunizations recommended in Appendix III Complete history and physical examination including rectal and gynecological examinations Complete blood count Serum creatinine, alkaline phosphatase, LDH, AST (SGOT), bilirubin, protein electrophoresis, calcium Hepatitis B surface antigen (HBs-Ag) and hepatitis B core antibody (HBcoreAb) (positive results should be prominently noted in the patient's chart, usually in the Allergy/Alert section. Anti-viral agents should be given if any systemic anti-lymphoid cancer treatment is given see section 13 under Special Problems) Hepatitis C antibody (positive results should be prominently noted in the patient's chart, usually in the Allergy/Alert section) Human immunodeficiency virus (HIV) antibody Bone marrow aspiration and biopsy Chest radiograph, PA and lateral views CT scan of the abdomen and pelvis PET/CT scan should be performed at staging for patients with curable aggressive nonHodgkin lymphoma, including diffuse large B-cell lymphoma (all types, including primary mediastinal Bcell lymphoma) and peripheral T-cell lymphomas (specified and unspecified). Test

6. 7. 8. 9. 10. 11.

Presentation/ Condition

Primary lymphoma of brain, epidural lymphoma, any lymphoma Cerebrospinal fluid cytology with neurologic abnormalities referable to the brain or spinal cord Primary lymphoma of brain Supra-hyoid cervical lymph node or gastrointestinal tract involvement Waldeyer's ring involvement IgM associated lymphoid cancer including lymphoplasmacytic and other types often referred to as Waldenstroms macroglobulinemia Ophthalmologic examination ENT examination Upper gastrointestinal and small bowel follow-through contrast radiographs Serum viscosity