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      A Primary Bone Diffuse Large B-Cell Lymphoma with Ocular Adnexal Involvement Translated title: Oküler Adneks Tutulumu Olan Kemiğin Primer Diffüz Büyük B Hücreli Lenfoması

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          Abstract

          To the Editor, Primary bone lymphomas (PBLs) [1,2] and ocular adnexal (OA) lymphomas [3,4] are rare types of extranodal lymphomas. Coexistence of these two rare entities without lymph node infiltration has not been reported previously. A 55-year-old man presented with left hip pain without a history of trauma. His medical history and physical examination did not reveal any remarkable findings. The X-ray radiographs of the pelvis and left hip showed multiple lytic lesions. Body 18fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) demonstrated multiple osteolytic bone lesions in the left zygomatic bone, vertebral column, bilateral iliac bones, left caput femoris, and trochanter major of the femur with increased 18F-FDG uptake (SUVmax: 31) (Figure 1a). Tru-Cut biopsy of the caput femoris showed atypical lymphoid cells that were pancreatin (-), s-100 (-), CD138 (+), CD30 (-), CD20 (+), CD3 (-), CD5 (-), CD10 (-), bcl-6 (+), and MUM-1 (+), consistent with diffuse large B-cell lymphoma. Laboratory data were as follows: erythrocyte sedimentation rate, 37 mm/h; lactate dehydrogenase level, 405 U/L; hemoglobin, 12 g/dL; white blood cell count, 6.74x109/L; platelet count, 250x109/L; normal liver and renal function tests. Bone marrow aspirate and core biopsy were normal. A rituximab, cyclophosphamide, doxorubicine, vincristine, and prednisolone (R-CHOP) regimen was planned. However, prior to the first chemotherapy day, the patient was admitted with swelling of the eyelids, exophthalmos, proptosis, and vision loss in his left eye that developed progressively over 3 days. Magnetic resonance imaging (MRI), which was performed two months after the initial 18F-FDG PET/CT, showed an orbital mass with diameters of 36x21x38 mm eroding the superior wall of the orbita and adjacent soft tissue (Figure 1b). After the detection of orbital involvement, investigations for central nervous system (CNS) involvement were negative. Chemotherapy treatment was commenced immediately without doing a biopsy because of the patient’s vision loss. After 4 cycles of R-CHOP chemotherapy, the patient’s left hip pain, left eye swelling, exophthalmos, and proptosis resolved completely, but his vision did not improve. Control 18F-FDG PET/CT showed marked regression of bone lesions with decreased 18F-FDG uptake (SUVmax: 4.6). Orbital MRI also showed that the mass had regressed to 14x12 mm in size. After obtaining this response, 2 cycles of R-CHOP, radiotherapy to the left orbita, and two cycles of high-dose methotrexate for CNS prophylaxis were planned. Considering the extensive lytic bone lesions and recent emergence of the OA tumor, the primary site of the disease must have been the bones in the presented case. Thus, the diagnosis can be categorized as PBL with OA involvement. An orbital mass was detected two months after diagnosis by means of MRI, but not by 18F-FDG PET/CT performed at diagnosis. It would be speculative to claim that such a large mass had arisen in a two month period. Taking into account that MRI is the gold standard imaging technique in evaluation of OA tumors [3], the orbital mass, which was probably small at the beginning, could not have been noticed on 18F-FDG PET/CT. This case emphasizes that a high suspicion index of OA involvement in PBL cases with any symptoms regarding the eyes and prompt assessment of the patients with MRI might prevent undesirable consequences. Ethics Ethics Committee Approval: Not applicable; Informed Consent: Not applicable.

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          Primary lymphoma of the ocular adnexa (orbital lymphoma) and primary intraocular lymphoma.

          Lymphomas of the orbit and eye are rare conditions that should be treated as separate entities due to the differences in presumed aetiology, investigations, management and outcomes. Orbital lymphoma is most often of low-grade histology; thyroid eye disease may predispose and chlamydial infection has been suggested as a trigger. Commonly, stage IE, in most cases, can be managed with radiotherapy alone using either a kilovoltage portal for conjunctival disease or a wedged pair of megavoltage beams for more infiltrative disease to a dose of 30 Gy in 15 fractions over 3 weeks. However, medical therapy is being investigated, including a rituximab-only approach for conjunctival-only presentations. The cure rate for stage IE disease is very high. In contrast, primary ocular lymphoma is often of high-grade histology, in particular diffuse large B-cell lymphoma, and can be regarded as one end of primary central nervous system lymphoma - both eyes and brain being at risk. Immunosuppression predisposes to the disease. Management consists of an initial high-dose chemotherapy regimen with methotrexate. In most cases, this should be followed by radiotherapy to the whole brain and globes to a dose of 30-36 Gy with a boost to bulk/presenting disease. Cure rates are rarely above 50%.
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            Primary lymphomas of bone.

            Primary lymphomas of bone are uncommon malignancies. The vast majority of them are non-Hodgkin lymphoma (NHL), whereas primary Hodgkin lymphoma (HL) of bone is extremely rare. Patients with primary NHL of bone commonly present with local bone pain, soft tissue swelling, and a mass or a pathological fracture. There is a slight male preponderance, and most patients are over 45-50 years of age. Primary NHL of bone can arise in any part of the skeleton, but long bones (femurs, tibia) are the most common sites of presentation. Comprehensive immunohistochemical studies are required to establish an accurate histological diagnosis of primary NHL of bone. Most cases of primary NHL of bone are classified as diffuse large B-cell lymphomas (DLBCL) in the World Health Organisation (WHO) classification of hematological malignancies. On full staging evaluation, most patients have disease of stage IE or IIE according to the Ann Arbor system. Several studies indicate that patients with primary NHL of bone have a favorable outcome, especially when treated by combined modality therapy. A number of studies reported that clinical stage is the most important prognostic variable in predicting overall survival. Interestingly, the rare occurrence of primary lymphoma of bone is in contrast with the frequency of plasma cell tumors in bone. This could be due to the fact that, during normal B-cell differentiation, the bone marrow is the normal site of homing of plasma cells which are terminally-differentiated, immunoglobulin-secreting post-germinal center B-cells. In this respect, there is circumstancial evidence that primary NHL of bone may represent tumors of post-germinal center B-cells. The present review summarizes data on the histogenesis of primary NHL of bone in view of the recent histogenetic classification of DLBCL on the basis of the B-cell differentiation gene expression profiles (germinal center vs. post-germinal center B-cell differentiation).
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              Author and article information

              Journal
              Turk J Haematol
              Turk J Haematol
              TJH
              Turkish Journal of Hematology
              Galenos Publishing
              1300-7777
              1308-5263
              September 2016
              19 August 2016
              : 33
              : 3
              : 254-255
              Affiliations
              [1 ] İstanbul Training and Research Hospital, Clinic of Hematology, İstanbul, Turkey
              [2 ] İstanbul Training and Research Hospital, Clinic of Nuclear Medicine, İstanbul, Turkey
              Author notes
              * Address for Correspondence: İstanbul Training and Research Hospital, Clinic of Hematology, İstanbul, Turkey Phone: +90 212 459 63 04 E-mail: elifsuyani@ 123456hotmail.com
              Article
              1769
              10.4274/tjh.2015.0424
              5111474
              27095383
              f89faada-a18d-4f60-aede-c05317aeeb9a
              © Turkish Journal of Hematology, Published by Galenos Publishing.

              This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

              History
              : 12 December 2015
              : 25 March 2016
              Categories
              Letters to the Editor

              primary bone lymphoma,ocular adnexal lymphoma,diffuse large b-cell lymphoma

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