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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Specialty: Hematology Oncology
