Rare forms of non-Hodgkin lymphomas: experience in treatment for primary bone lymphomas

Abstract

Primary bone lymphoma is a rare presentation of non-Hodgkin lymphoma. It accounts for a maximum of 1–2% of all non-Hodgkin lymphomas in adults. Primary bone lymphoma is diagnosed in focal lesions of one or more bones; soft tissue and regional lymph nodes may be involved too. The exclusion criteria are only bone marrow damage and involvement of distant lymph nodes. The first symptoms include intractable bone pain often accompanied by local edema, the formation of a tumor mass in the affected area; B symptoms occasionally join. Local lesions of long tubular bones in the diaphysis and metadiaphysis regions are more common (80%), while multifocal lesions are less frequent (20%). Diagnosis of lesions of the bone tissue in its primary and secondary involvement is based on the use of all available research methods (radiography; computed, magnetic resonance and positron emission tomography). Differential diagnosis requires an immunohistochemical study with determination of the expression of total leukocyte antigen, B-cell and T-cell markers, and clonality in one of immunoglobulin light chains κ or λ, bcl 2 and bcl 6, ALK, proliferative activity of Ki-67. Evaluation of the effectiveness of various treatments for primary bone lymphoma is complicated by a small number of observations and the absence of a uniform treatment strategy. CHOP-like chemotherapy cycles are often used as first-line therapy. Personalized therapy involves immunochemotherapy, radiation therapy and surgical treatment — endoprosthetics.