Rare transformation to double hit lymphoma in Waldenstrom's macroglobulinemia

Waldenström macroglobulinemia (WM) is a lymphoproliferative lymphoma that is characterized by monoclonal immunoglobulin M (IgM) protein and bone marrow infiltration. Its incidence is rare and rarer still is its ability to transform to a B-cell lymphoma, particularly the aggressive diffuse large B-ce...

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Bibliographic Details
Published in:Immunotherapy 2017-08, Vol.9 (9), p.709-714
Main Authors: Okolo, Onyemaechi N, Johnson, Ariel C, Yun, Seongseok, Arnold, Stacy J, Anwer, Faiz
Format: Article
Language:English
Subjects:
Anemia
Bcl-2 protein
Bcl-6 protein
Blood
Bone marrow
Case Report
Cell cycle
Cell differentiation
Coronary vessels
Flow cytometry
Genetic transformation
Histology
Immunoglobulin M
Immunoglobulins
Leukemia
Lymphatic system
Lymphocytes
Lymphocytes B
Lymphoma
Macroglobulinemia
Medical prognosis
Mutation
Myc protein
Patients
Prognosis
Proteins
Tumors
Waldenstrom's macroglobulinemia
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Summary:Waldenström macroglobulinemia (WM) is a lymphoproliferative lymphoma that is characterized by monoclonal immunoglobulin M (IgM) protein and bone marrow infiltration. Its incidence is rare and rarer still is its ability to transform to a B-cell lymphoma, particularly the aggressive diffuse large B-cell lymphoma, which bodes a poor prognosis. When transformation includes mutations of MYC, BCL-2 and/or BCL-6, it is known as a 'double hit' or 'triple hit' lymphoma respectively. This paper presents a rare case of WM with mutations positive for MYC and BCL2, making it a case of double hit B-cell lymphoplasmacytic lymphoma with plasmatic differentiation without morphological transformation to aggressive histology like DLBCL. The paper also broadens to include discussions on current topics in the classification, diagnosis, possible causes of transformation, and treatment of WM, including transformation to double hit lymphoma. The significance of this case lies in that the presence of double hit lymphoma-like genetic mutations in WM have not been previously described in the literature and potentially such changes are harbinger of extra-nodal presentation, aggressive growth, and possibly poor prognosis, if data from other double-hit lymphoma are extrapolated.
ISSN:1750-743X
1750-7448
DOI:10.2217/imt-2017-0027