Transformed lymphoma refers to a more aggressive or fast growing lymphoma that arises from a indolent disease.
The most common type of transformed Lymphoma is a follicular that evolves into a diffuse large B – cell lymphoma (DLCBL), or, less commonly high-grade B-cell lymphoma with double hit biology after the indolent disease acquires a MYC rearrangement.
Marginal zone lymphoma and lymphoplasmacytic lymphoma can also transforming to DLBCL.
Chronic lymphocytic leukemia and small lymphocytic lymphoma can transform into Richter‘s transformation.
Slow growing lymphomas can acquire mutations or genomics hits that alter the process into a very aggressive phenotype.
The overall incidence of transformation from indolent lymphomas is low, at approximately 2-3% per year.
Factors associated with transformed lymphoma include early progression of follicular lymphoma within 24 months.
75% of patients with follicular lymphoma who experience disease progression within 24 months have a transformed disease.
Patients with follicular lymphoma with a high baseline FLIPI score of three – five have a higher risk of transformation.
Other factors associated with transformation include: poor performance status, high levels of LDH, B symptoms, follicular lymphoma grade 3A or higher, high FLIPI, loss of beta-2-microglobulin, increased T cells, and mutations in genes such as TP53, PIM1, or beta-2-microglobulin.
Progressive disease relates to an increase in the size and number of lymph nodes or lymphomatous areas of involvement.
When more aggressive histology is noted, typically DLBCL or high-grade B- cell lymphoma more aggressive treatment is warranted. and it is possible to cure more aggressive types of informer, whereas indolent lymphoma will essentially always recur.
Therapy for transform disease include stem cell transplant in chimeric antigen receptor T-cell therapy.