Mantle cell lymphoma natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Natural History
Complications
Prognosis
- Prognosis of MCL is problematic and indexes do not work as well due to patients presenting with advanced stage disease. Staging is used but is not very informative, since the malignant B-cells can travel freely though the lymphatic system and therefore most patients are at stage II or IV at diagnosis. Prognosis is not strongly affected by staging in MCL and the concept of metastasis does not really apply.
- (The letter A after the Roman numeral indicates that normal external symptoms are not present. The Letter B indicates the symptoms are present and hence they are called “B Symptoms”.)
- MCL is one of the few NHLs that can cross the boundary into the brain, yet it can be treated in that event.
- There are a number of prognostic indicators that have been studied. There is not universal agreement on their importance or usefulness in prognosis.
- Ki-67 is an indicator of how fast cells mature and is expressed in a range from about 10% to 90%. The lower the percentage, the lower the speed of maturity, and the more indolent the disease. Katzenberger et al Blood 2006;107:3407 graphs survival versus time for subsets of patients with varying Ki-67 indices. He shows median survival times of about one year for 61-90% Ki-67 and nearly 4 years for 5-20% Ki-67 index.
- MCL cell types can aid in prognosis in a subjective way. Blastic is a larger cell type. Diffuse is spread through the node. Nodular are small groups of collected cells spread through the node. Diffuse and nodular are similar in behavior. Blastic is faster growing and it is harder to get long remissions. Some thought is that given a long time, some non-blastic MCL transforms to blastic. Although survival of most blastic patients is shorter, some data shows that 25% of blastic MCL patients survive to 5 years. That is longer than diffuse type and almost as long as nodular (almost 7 yrs).
- Beta-2 microglobulin is another risk factor in MCL used primarily for transplant patients. Values less than 3 have yielded 95% overall survival to 6 yrs for auto SCT where over 3 yields a median of 44 mos overall survival for auto SCT (Khouri 03). This is not yet fully validated.
- Testing for high levels of LDH in NHL patients is useful because LDH is released when body tissues break down forany reason. While it cannot be used as a sole means of diagnosing NHL, it is a surrogate for tracking tumor burden in those diagnosed by other means. The normal range is approximately 100-190.