Clinical Characteristics and Long-term Prognosis Analysis of Patients with Primary Bone Lymphoma / 中国实验血液学杂志

OBJECTIVE@#To analyze the clinical characteristics and long-term prognosis of patients with primary bone lymphoma (PBL).@*METHODS@#The clinical data of 21 patients with PBL treated in our center from 2005 to 2018 were analyzed retrospectively, the clinical characteristics and the factors affecting prognosis of the patients were analyzed.@*RESULTS@#The median age of all the 21 newly diagnosed PBL patients was 40(12-71) years old. Ostealgia was the initial symptom in most of the patients (19/21,90.5%). 42.9%(9/21) of the patients showed single bone lesion only. 571% (12/21) of the patients showed diffuse large B cell lymphoma. 28.6% (6/21) of the patients showed anaplastic large cell lymphoma and 9.5% (2/21) of the patients showed T cell lymphoblastic lymphoma. All the patients received chemotherapy (CHOP or CHOP like regimen, 33.3% plus rituximab) with or without radiotherapy and/or autologous hematopoietic stem cell transplantation (ASCT). 18 patients achieved clinical remission (including 15 for CR and 3 for PR). The median follow-up time was 48 months. The 5-year overall survival rate and progression-free survival rate of the patients were was 67.5% and 63.7%, respectively. The single factors analysis showed that ASCT was the important prognostic factor of PFS, while the single or multiple bone lesion was the factors affecting OS of the patients. There were no statistical differences with the effects of age, sex, stage, ECOG score, LDH level, B symptoms and radiotherapy for the prognosis of patients.@*CONCLUSION@#Diffuse large B cell lymphoma is the most common pathological type of PBL. Chemotherapy is the main treatment, which can be combined with radiotherapy and/or ASCT. The ASCT and the number of bone lesion are the factors for long time survival of the patients.

Clinical characteristics of primary extranodal versus nodal diffuse large B-Cell Lymphoma: A retrospective cohort study in a cancer center

Background The outcome of patients with primary extranodal diffuse large B-cell lymphoma (PE-DLBCL) varies according to the primary site involved. Primary gastrointestinal, breast, bone, craniofacial, and testicular DLBCL are rare extranodal manifestations of DLBCL. Objective The objective of the study was to describe the clinical course of patients with PE-DLBCL disease in a referral cancer center. Results From 637 patients, 51 (8.77%) were considered as having PE-DLBCL (25 gastrointestinal, 12 craniofacial, 6 breast, 5 bone, and 3 with primary testicular DLBCL). Complete remission was higher in all PE-DLBCL sites (100% in testicular, 92.6% craniofacial, 83.3% breast, 80% bone, and 80% gastrointestinal) compared with 73.3% in nodal DLBCL. Although 2 cases with breast PE-DLBC relapsed, they achieved a complete response with chemotherapy. The overall survival at 5 years was 100%, 80%, 78%, 58%, 58%, and 62% for patients with primary breast, primary bone, gastrointestinal, primary craniofacial, primary testicular, and nodal DLBCL, respectively. Conclusions PE-DLBCLs constitute rare, primary sites of lymphoproliferative disorders in most cases, with localized disease and good prognosis. They require a combined chemoimmunotherapy with radiotherapy in most cases to improve local and systemic disease.

ALK-positive anaplastic large-cell lymphoma with primary bone involvement: A rare case and review of the literature

@#Primary bone lymphoma (PBL) is an uncommon type of extranodal lymphoma involvement. An anaplastic large-cell lymphoma (ALCL) is an extremely rare type of PBL, and it remains unclear whether ALCLs that primarily involve the bone exhibit favourable or unfavourable biological behaviour, and whether they are similar to ALCLs in general, or not. We reported a case of ALKpositive ALCL with primary bone involvement, and reviewed the clinicopathological features of 22 previously reported cases. An ALCL with primary bone involvement mostly affects younger patients with a preponderant towards the involvement of axial-bone. The prognosis of an ALCL that primarily involves bone is unfavourable, compared with PBL generally. The ALK-positive ALCLs in PBLs had less decedents than the ALK-negative ALCLs with a statistical non-significance (p=0.198).

Primary bone marrow B-cell non-hodgkin's lymphoma successfully treated with R-CHOP / Linfoma primario no hodgkin de células B en la médula ósea tratado con R-CHOP

Primary isolated bone marrow disease as a presenting feature of lymphoma is very rare. We describe the case of a Chinese with isolated bone marrow small B-cell lymphoma as a first manifestation. A 55-year old woman was admitted to our hospital with fever. Her peripheral blood smear and laboratory findings were suggestive of bicytopenia. Bone marrow specimen showed diffusely distributed small-sized lymphocytes. Combined with immunophenotypic and chromosomal analysis, a diagnosis of primary bone marrow B-cell non-Hodgkin's lymphoma was made. The patient was treated with R-CHOP (rituximab and cyclophosphamide, epirubicin, vindesine, and prednisone) regimen for six cycles. She had complete remission and is still alive without relapse. We concluded that primary bone marrow mature small B-cell lymphoma is a rare but distinctive subtype of lymphoma. The prognosis for this entity is poor but rituximab-based treatment is promising for improving its outcomes.

La enfermedad aislada primaria de la médula ósea como rasgo de manifestación del linfoma es muy rara. Describimos el caso de una paciente china con linfoma aislado de células B pequeñas en la médula como una primera manifestación. Se trata de una mujer de 55 años que ingresara a nuestro hospital con fiebre. El frotis de sangre periférica y los hallazgos de laboratorio apuntaban a una bicitopenia. El espécimen de la médula ósea mostró la presencia de linfocitos de pequeño tamaño distribuidos de manera difusa. En combinación con un análisis inmunofenotípico y un análisis cromosómico, se realizó un diagnóstico de linfoma primario no Hodgkin de células B de la médula ósea. La paciente recibió como tratamiento un régimen de seis ciclos de R-CHOP (rituximab, ciclofosfamida, epirubicina, vindesina, y prednisona). Esto le permitió alcanzar una remisión completa, y todavía está viva sin que se haya producido recaída alguna. Concluimos que el linfoma primario de células B pequeñas maduras de la médula ósea es un subtipo raro pero particular de linfoma. La prognosis para esta entidad es pobre, pero el tratamiento a base de rituximab re-basado resulta promisorio en cuanto a mejorar su evolución clínica.

Linfoma ósseo primário simultâneo a osteocondroma simulando degeneração sarcomatosa: relato de caso / Primary bone lymphoma simultaneous to osteochondroma simulating sarcomatous degeneration: case report

Não há evidências relatadas na literatura de associação entre linfoma ósseo primário e osteocondroma ou da coexistência deles em uma mesma região óssea. Este relato de caso descreve um caso raro de linfoma ósseo primário ocorrendo juntamente com um osteocondroma no terço proximal de tíbia. Os sinais de imagem na ressonância magnética neste caso simulam uma degeneração sarcomatosa do osteocondroma.

In the literature, there is no evidence of relationship between primary bone lymphoma and osteochondroma or of coexistence of both of them in a single bone. The present report describes an uncommon case of primary bone lymphoma occurring simultaneously with osteochondroma in the proximal third of the tibia. In the present case, magnetic resonance imaging signs simulated the presence of sarcomatous degeneration.

Linfoma primario de hueso con afectación multicéntrica / Primary bone lymphoma with multicentric involvement

El linfoma primario de hueso es una enfermedad infrecuente, que tiene una presentación y evolución diferente a los linfomas de otras localizaciones. Se presenta un caso de linfoma primario de hueso de localización craneana y esternal de rápido crecimiento. En su evolución, realizada la exéresis de la lesión primaria de calota, presentó aparición de nuevas lesiones de rápido crecimiento a nivel craneano y fémur y progresión de lesión preesternal que, con anatomía patológica de linfoma no Hodgkin difuso de células grandes B, inició R-CHOPP (ciclofosfamida, doxorrubicina, vincristina, prednisona y rituximab) con rápida disminución de todas las lesiones sin evidencia de progresión al cabo de los seis ciclos.

Primary bone lymphoma is a rare disease, which usually has a different presentation and evolution than lymphomas of other locations. We are presenting a case of primary bone lymphoma of rapid growth, in cranial and sternal locations. In its evolution, once the excision of the primary lesion of the skull was performed, the patient presented new lesions of rapid growth at the skull and femur level, and progression of pre-sternal lesion. With large B-cell diffuse non-Hodgkin lymphoma pathology, the patient initiated R-CHOPP (cyclophosphamide, doxorubicin, vincristine, prednisone and rituximab) with rapid reduction of all lesions without evidence of progression after the six cycles.

Clinical characteristics and outcomes of primary bone lymphoma in Korea

BACKGROUND: This study evaluates the effectiveness of immunochemotherapy and radiation therapy in the treatment of patients with primary bone lymphoma (PBL). METHODS: We retrospectively reviewed the medical records of 33 patients with PBL who were treated at 6 medical centers in Korea from 1992 to 2010. Clinicopathological features and treatment outcomes were analyzed. RESULTS: The median age of the patients participating in our study was 40 years. The most common sites of involvement were the pelvis (12.36%) and femur (11.33%). CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisolone) or CHOP-like regimens were administered to 20 patients (61%), and R-CHOP (rituximab plus CHOP) was administered to the remaining 13 patients (39%). The overall response rate was 89% (complete response, 76%; partial response, 12%). The overall survival (OS) of patients with solitary bone lesions was longer than that of patients with multiple bone lesions (median OS: not reached vs. 166 months, respectively; P=0.089). Addition of rituximab to CHOP did not significantly affect either OS or progression-free survival (P=0.53 and P=0.23, respectively). Combining radiation therapy with chemotherapy also did not improve the OS or progression-free survival of patients with solitary bone lesions. CONCLUSION: Conventional cytotoxic chemotherapy remains an effective treatment option for patients with PBL. Additional benefits of supplementing chemotherapy with either rituximab or radiation therapy were not observed in this study. Further investigation is needed to characterize the role of immunochemotherapy in treating patients with PBL.

Positron Emission Tomography with 18F Fluorodeoxyglucose for Primary Lymphoma of Bone / 대한핵의학회잡지

PURPOSE: Accurate assessment of the lesion after treatment of patients with bone lymphoma is difficult. In this patient who demonstrated complete remission after chemotherapy, the regions of fluorine-18 fluorodeoxyglucose ( (18) FFDG) PET uptake diminished more rapidly following therapy, indicating a complete response at much earlier stage than did Magnetic Resonance Imaging (MRI) or CT based findings. With the conventional methods, such as MRI and CT, it was difficult to assess whether the residual tumor tissue was viable or not. Decision to complete response is very important in patients with lymphoma to plan the further treatment. We experienced a patient with primary lymphoma of bone who revealed complete response to chemotherapy on (18) FFDGPET while CT showed persistent destructive bone lesion. Thus, (18) FFDGPET study after therapy may be superior to CT in the evaluation of response to treatment in primary lymphoma of bone.

A Case of Primary Bone Lymphoma with Multifocal Bone Involvement / 대한혈액학회지

Primary bone lymphoma is a rare tumor accounting for only 5% of all primary osseous malignancies, defined as lymphoma which arises in the medullary cavity and there is no concurrent regional lymph node or visceral involvement. We experienced a case of primary bone lymphoma presented with multifocal bone involvemnt in a 30-year-old woman. She was admitted with 2-month-history of pain on the lower back and the right hip. On laboratory examination, pancytopenia was demonstrated. Radiologic studies showed multiple soft tissue masses that had destroyed the involved bones. The tissue section from bone marrow biopsy was positive for leukocyte common antigen and pan T-cell marker (UCHL-1) in the large malignant cells. The diagnosis of malignant lymphoma, diffuse large cell (T-cell phenotype) was made. On physical examination, pheripheral lymph nodes could not be palpable. Involvement of either regional lymph nodes or visceral organs could not be detected by radiological examinations including CT and Ga-67 scan. After the patient recieved 2 cycles of combination chemotheraphy with CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) regimen, complete remission was achieved. Eight cycles of the chemotherapy were successfully completed.

A case of primary bone lymphoma associated with acquired immunodeficiency syndrome

A 33-year old man with acquired immunodeficiency syndrome was admitted to Severance hospital following 1 year of diarrhea and 2 to 3 months of low sternal pain. The patient had progressive generalized lymphadenopathy for the previous 3 years. Whole body bone scan for evaluation of bone pain showed multiple abnormal hot uptakes at the low sternal body and T8 and T10 vertebra. Chest CT showed multifocal cortical erosion of the bone with soft tissue mass at the low sternal body and spine MRI showed multiple low-signal density in T1WI and high-signal density in T2WI at the T8 and T10 vertebral body. Biopsy was performed at the sternochondral junction and it showed high-grade malignant lymphoma of the large cell immunoblastic type. Immunostaining showed positive for the B-cell markers (CD79a and L26) and negative for the T-cell marker (UCHL1). Radiotherapy of 3,000 cGy was delivered to the sternum and vertebra. Since then, systemic chemotherapy with m-BACOD regimen (except dexamethasone) and anti-retroviral therapy with a combination of 3 drugs (didanosine, lamivudine, indinavir) has been performed. This is the first case report of primary bone lymphoma associated with acquired immunodeficiency syndrome in Korea.

Ki-1 Positive T-Cell Lymphoma of Bone in a Child

Ki-1 monoclonal antibody is a well known marker for Reed-Sternberg cells in Hodgkin's disease, but also occasionally reacts with activated lymphoid cells of either benign or malignant nature. Recently, Ki-1 antibody positive Non-Hodgkin's lymphoma, usually of large cell and/or polymorphous type, has been reported in the lymph nodes, skin, soft tissue, and stomach, but not in the bone. We report a case of multifocal primary bone lymphoma in a seven-year old body involving the left shoulder and right frontal bone, which proved to be a large cell, polymorphous lymphoma, helper T-cell type expressing Ki-1 antigen.