ALK- negative Anaplastic Large Cell Lymphoma of Bone
نویسنده
چکیده
Introduction Primary bone lymphoma (PBL) is a rare disease that was first described by Oberling in 1928.1 The vast majority of PBL cases are non-Hodgkin lymphoma (NHL), whereas primary Hodgkin lymphoma (HL) of bone is extremely rare. PBL constitutes 7% of all malignant bone tumors, 4-5% of all extranodal NHL, and less than 1% of all malignant lymphomas.2-5 Patients with primary NHL of bone commonly present with local bone pain, soft tissue swelling, and a mass or pathological fracture. Osteomyelitis may imitate primary bone lymphomas.6 PBL can occur at any age, although there is particular tendency to affect older adults who are over 45-50 years of age. There is also a slight male predominance. Primary NHL of bone can arise in any part of the skeleton, but long bones (femur, tibia) are the most common sites of presentation.2,7 According to the World Health Organization (WHO) classification, lymphomas involving bone are classified into four groups including Group I, lymphoma with a single bone involvement with or without any regional lymph node involvement; Group II, lymphoma with multiple bone sites, but no visceral or lymph node involvement; Group III, bone tumor with involvement of other visceral sites or lymph nodes at multiple sites; and Group IV, lymphoma involving any other sites and found on bone biopsy done to rule out possible involvement.3 Most cases of primary bone lymphomas (PBLs) are classified as diffuse large B-cell lymphomas (DLBCLs) in the WHO classification of hematological malignancies. Bone scintigraphy is a valuable tool in staging of PBL. It detects multifocal involvement, which may alter prognosis and possibly the treatment. Moreover, clinically silent involvement of weight-bearing sites may be detected allowing for prophylactic treatment.9 Pediatric PBL-DLBCL has a favorable prognosis but remains poorly characterized.
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تاریخ انتشار 2011