Abstract
Waldenström macroglobulinemia/lymphoplasmatocytic lymphoma (WM/LPL) is a B-cell neoplasia characterized by the presence of clonally related IgM-secreting lymphoplasmatocytic cells primary involving the bone marrow. WM/LPL is usually an indolent disease and evolution to high grade lymphoma is uncommon.
A 56-year-old male with a history of early stage (IIA) testicular seminoma, treated with orchiectomy and adjuvant infradiaphragmatic radiotherapy, subsequently developed IgM-kappa monoclonal gammopathy of undetermined significance (MGUS) and pancreatic cancer (T1aN0M0, complete remission after Whipple resection). After 6 years, IgM-kappa MGUS evolved to WM/LPL, presenting with symptomatic hyperviscosity, treated with regular plasma exchanges, 1 course of fludarabine/cyclophosphamide and 4 courses of rituximab/cyclophophamide without achieving a response. After completion of 2 cycles of bortezomib/dexamethasone he presented with severe constitutional symptoms, abdominal lymphadenopathy and splenomegaly. Laboratory analyses revealed circulating immunoblasts, rising paraprotein, increased lactate dehydrogenase and renal impairment. Bone marrow biopsy demonstrated a diffuse infiltration by large B-cells expressing CD20 and IgM-kappa. Marrow cytogenetics disclosed complex karyotype abnormalities. The patient was diagnosed with transformed diffuse large B-cell lymphoma (DLBCL) from WM/LPL and treated with rituximab- hyper-CVAD combination, followed by a course of methotrexate and cytarabine. He died due to the complications of toxic epidermal necrosis.
We report WM/LPL transformation to DLBCL occuring in a seminomatous testicular and pancreatic cancer survivor. DLBCL presented with rapid extramedullary involvement and behaved aggressively in accordance with complex karyotype abnormalities.
Although the contribution of radiotherapy to this aggressive transformation can not be ruled out, genetic predisposition must be considered in this particular case.
A 56-year-old male with a history of early stage (IIA) testicular seminoma, treated with orchiectomy and adjuvant infradiaphragmatic radiotherapy, subsequently developed IgM-kappa monoclonal gammopathy of undetermined significance (MGUS) and pancreatic cancer (T1aN0M0, complete remission after Whipple resection). After 6 years, IgM-kappa MGUS evolved to WM/LPL, presenting with symptomatic hyperviscosity, treated with regular plasma exchanges, 1 course of fludarabine/cyclophosphamide and 4 courses of rituximab/cyclophophamide without achieving a response. After completion of 2 cycles of bortezomib/dexamethasone he presented with severe constitutional symptoms, abdominal lymphadenopathy and splenomegaly. Laboratory analyses revealed circulating immunoblasts, rising paraprotein, increased lactate dehydrogenase and renal impairment. Bone marrow biopsy demonstrated a diffuse infiltration by large B-cells expressing CD20 and IgM-kappa. Marrow cytogenetics disclosed complex karyotype abnormalities. The patient was diagnosed with transformed diffuse large B-cell lymphoma (DLBCL) from WM/LPL and treated with rituximab- hyper-CVAD combination, followed by a course of methotrexate and cytarabine. He died due to the complications of toxic epidermal necrosis.
We report WM/LPL transformation to DLBCL occuring in a seminomatous testicular and pancreatic cancer survivor. DLBCL presented with rapid extramedullary involvement and behaved aggressively in accordance with complex karyotype abnormalities.
Although the contribution of radiotherapy to this aggressive transformation can not be ruled out, genetic predisposition must be considered in this particular case.
| Original language | English |
|---|---|
| Title of host publication | 25th General Meeting of the Belgian Hematological Society - Abstract Posters Clinical Hematology p 28 |
| Publisher | Belgian Hematological Society |
| Publication status | Published - 29 Jan 2010 |
Keywords
- Waldenström
- diffuse large B-cell lymphoma
- MGUS
- CD20