Non-Hodgkin's Lymphoma
Staging and prognostic factors

 

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    Non-Hodgkin's Lymphoma
 
   

Staging techniques -

Supradiaphragmatic involvement

Chest x-ray

(mediastinal or hilar adenopathy, pleural effusions, parenchymal lesions) the chest radiograph is abnormal in fewer than 50% of patients, identification of hilar or mediastinal adenopathy, parenchymal lesions, or pleural effusions is important and provides an easy method for reevaluation.

CT scan of the chest

(mediastinal, hilar, or parenchymal pulmonary disease)

Subdiaphragmatic involvement

CT scan

CT scan of the abdomen and pelvis is essential in staging of lymphoma (enlarged lymph nodes, splenomegaly, filling defects in liver and spleen)

  • CT scan has a sensitivity of 80% and a specificity of 70% in detection of lymphoma.

PET

PET (FDG-glucose) scanning is gaining wider acceptance as a potential diagnostic approach for staging at diagnosis, response assessment, and relapse.

Hematologic involvement

CBC

CBC with differential and platelet count (peripheral blood lymphocytosis with circulating malignant cells is common in low-grade and mantle cell lymphomas). Bone marrow and peripheral blood involvement may be present, and the distinction between leukemia and lymphoma is difficult to make in some cases.

Bilateral bone marrow biopsy

Unilateral percutaneous bone marrow biopsies must be performed, because the likelihood of lymphomatous involvement of the marrow is relatively high, especially in most indolent lymphomas, in which marrow involvement occurs in as high as 40% to 90% of cases.

Extranodal evaluation

Endoscopy

Upper GI endoscopy and/or GI series with small bowel follow-through in patients with head and neck involvement (tonsil, base of tongue, nasopharynx) and those with a GI primary; mantle- cell lymphoma is associated with a high incidence of occult GI involvement.

Ultrasonography

Ultrasonography of opposite testis in patients with a testicular primary.

CNS involvement

CSF examination

Perform examination of CSF and strongly consider CNS prophylaxis in patients with

  1. Diffuse aggressive NHL with bone marrow, epidural, testicular, paranasal sinus, or nasopharyngeal involvement
  2. Highgrade lymphoblastic lymphoma and Burkitt lymphoma and its variants
  3. HIV-related lymphoma
  4. Primary CNS lymphoma if no evidence of increased intracranial pressure
  5. Lymphoma related to immunosuppression after solid-organ transplant (ie, liver, lungs, and kidneys)

General chemistry panel (lactic dehydrogenase [LDH] level determination) is mandatory; β2 -microglobulin (β2M) is recommended.

Spinal MRI scan

Spinal MRI scan for epidural disease when clinically indicated (useful in the evaluation of suspected spinal cord compression).

HIV serology

HIV serology in at-risk patients with diffuse large cell, and other aggressive and Burkitt histologies; HTLV-1 serology in select patients with cutaneous T-cell lymphoma, especially if they have hypercalcemia.

Staging of Non-Hodgkin's Lymphoma

Prognostic factors

The NHLs can be divided into 2 prognostic groups: the indolent lymphomas and the aggressive lymphomas.

Indolent NHL

Indolent NHL types have a relatively good prognosis, with median survival as long as 10 years, but they usually are not curable in advanced clinical stages.

An international index for follicular lymphoma (i.e., the Follicular Lymphoma International Prognostic Index [FLIPI]) identified 5 significant risk factors prognostic of overall survival:

  • Age (≤ 60 years vs. > 60 years).
  • Serum lactate dehydrogenase (normal vs. elevated).
  • Stage (stage I or stage II vs. stage III or stage IV).
  • Hemoglobin level (≥ 120 g/L vs. < 120 g/L).
  • Number of nodal areas (≤ 4 vs. > 4).

Patients with 0 to 1 risk factors have an 85% 10-year survival rate, while 3 or more risk factors confer a 40% 10-year survival rate.

There are several other prognostic index models for the low- grade lymphomas. Unfavorable prognostic factors include extent of bone marrow involvement (> 20%), bulky disease (≥ 5-7 cm), more than one extranodal site, LDH level > 1 times normal values, elevated β2M, and nonambulatory performance status.

Aggressive type NHL

The aggressive type of NHL has a relatively poor prognosis and shorter natural history, but a significant number of these patients can be cured with intensive combination chemotherapy regimens.

An International Prognostic Index (IPI) for aggressive NHL (diffuse large cell lymphoma) identifies 5 significant risk factors prognostic of overall survival:

  • Age (≤60 years of age vs. >60 years of age).
  • Serum lactate dehydrogenase (normal vs. elevated).
  • Stage (stage I or stage II vs. stage III or stage IV).
  • Performance status (0 or 1 vs. 2-4).
  • Extranodal site involvement (0 or 1 vs. 2-4).

Patients with 2 or more risk factors have a <50% chance of relapse-free and overall survival at 5 years. This study also identifies patients at high risk of relapse based on specific sites of involvement, including bone marrow, central nervous system (CNS), liver, lung, and spleen. Age-adjusted and stage-adjusted modifications of this IPI are used for younger patients with localized disease.

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