Waldenstrom's macroglobulinemia may undergo histologic transformation to a more aggressive hematologic neoplasm. This is analogous to Richter's syndrome in patients with CLL.

Patient selection: Waldenstrom's macroglobulinemia


Frequency of histologic transformation: around 2% (up to 10%)


Features of patient's showing a histologic transformation:

(1) development of a high-grade hematologic neoplasm, often a diffuse large B-cell lymphoma (DLBCL)

(2) presence of high-risk features: extranodal disease, high IPI score

(3) inferior survival


Sites of extranodal disease: CNS, ascites, testis, skin

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