Myeloma: A malignant disorder of bone and soft tissue
TL;DR: Despite many efforts over the years and the large number of treatment agents introduced, it is apparent that the authors do not have sufficient ability or knowledge to control this very troublesome disorder.
Abstract: The purpose of this brief report is to review a large series of cases of myeloma, a highly malignant tumor. The disease was first described over 150 years ago, but not named myeloma until 1873. The lesion appears to be caused by the production of plasma cells in the bone marrow occasionally associated with amyloid. Patients present with pain in bones, principally spine, pelvis, ribs, calvarium and long bones. The sedimentation rate is elevated and marrow shows 10% plasma cells and the sedimentation rate is always elevated. There is no identified genetic cause and the disease is not familial. The disease has a highly malignant potential. We treated 181 mature adult patients with tumor in multiple sites. 63% were dead of disease at an average of 4.5 years after diagnosis. The use of some new drugs have been helpful, chiefly lalanlidomide or bortezomib. Radiation appears to be useful for some lesions and bisphosphonates sometimes help support the bone structure. Despite many efforts over the years and the large number of treatment agents introduced, it is apparent that we do not have sufficient ability or knowledge to control this very troublesome disorder.
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TL;DR: In patients with an apparently benign monoclonal gammopathy, follow-up must be continued indefinitely because multiple myeloma, amyloidosis, macroglobulinemia, or related disorders occur in approximately a fourth of them.
327 citations
TL;DR: Reasons for the reduced transplant‐related mortality appeared to be fewer deaths owing to bacterial and fungal infections and interstitial pneumonitis, in turn a result of earlier transplantation and less prior chemotherapy, which may also play a role.
Abstract: Out of 690 allogeneic matched sibling donor transplants for multiple myeloma reported to the European Group for Blood and Marrow Transplantation (EBMT) registry, 334 were performed during the period 1983-93 (all with bone marrow) and 356 during 1994-98 [223 with bone marrow and 133 with peripheral blood stem cells (PBSCs)]. The median overall survival was 10 months for patients transplanted during the earlier time period and 50 months for patients transplanted with hone marrow during the later period. The use of PBSCs was associated with earlier engraftment but no significant survival benefit compared to bone marrow transplants during the same time period. The improvement in survival since 1994 with the result of a significant reduction in transplant-related mortality, which was 38%, 21% and 25% at 6 months and 46%, 30% and 37% at 2 years during the earlier period, and the later period with bone marrow and PBSCs respectively. Reasons for the reduced transplant-related mortality appeared to be fewer deaths owing to bacterial and fungal infections and interstitial pneumonitis, in turn a result of earlier transplantation and less prior chemotherapy. Better supportive treatment and more frequent use of cytokines may also play a role. The improvement in survival was not directly related to the increased use of PBSCs.
321 citations
TL;DR: It is concluded that isolated, strong CD56 expression is common in MM, but not in MGUS or reactive plasma cells, and the potential biologic importance of CD56 positivity in myeloma is reviewed.
284 citations
TL;DR: The radiotherapy experience at M. D. Anderson Hospital is reviewed to confirm the better prognosis of EMPC and support the theory that SPB and EMPC are two different entities.
Abstract: Solitary plasmacytoma of bone (SPB) and extramedullary plasmacytoma (EMPC) are unusual solitary tumors of plasma cell origin. The clinical differences between these tumors and multiple myeloma are the subject of continued study. This review examines the radiotherapy experience at M. D. Anderson Hospital. Between 1948 and 1977, 12 patients with SPB and 12 patients with EMPC were treated with radiotherapy. Radiotherapeutic doses were most often (22/24) greater than 4,000 rads, at 200 rads per day. In order to qualify as solitary plasmacytoma a disease-free interval of 3 years was required. In the group of SPB, 5 patients of 9 were alive with no evidence of disease (NED) at 3 years, 4 of 5 at 5 years, and none of 4 at 10 years. In the EMPC group, 8 patients of 11 were NED at 3 and 5 years, and 6 of 9 at 10 years. Six patients with SPB developed multiple myeloma compared with only two with EMPC. The results confirm the better prognosis of EMPC and support the theory that SPB and EMPC are two different entities.
254 citations
TL;DR: The quantity of plasma cell burden in the biopsy proved to be a useful criterion for histologic staging of MM, supplementing any clinical staging system in use, and provided information required for decisions on treatment modalities.
Abstract: Bone marrow biopsies of 674 patients with multiple myeloma (MM) were processed for diagnostic evaluation. Histologic variables were correlated with the clinical features to determine factors of value in predicting prognosis. Four of these were used to classify MM into six histologic types: Marschalko type; small cell type; cleaved type; polymorphous type; asynchronous type; and blastic type. These six types were subsequently combined into three prognostic grades: low, intermediate, and high, analogous to the malignant lymphomas. The quantity of plasma cell burden in the biopsy proved to be a useful criterion for histologic staging of MM, supplementing any clinical staging system in use. Both these parameters, grade and stage, provide information required for decisions on treatment modalities, while the effects of therapy can be monitored by sequential biopsies.
244 citations
"Myeloma: A malignant disorder of bo..." refers background in this paper
...In the latter disorder, known as multiple myeloma, production of plasma cells in the bone marrow not only causes weakness of bone structure, pathologic fractures and spinal cord compression, but in addition may result in leucopenia, anemia and thrombocytopenia [6, 8, 9, 10, 15, 16, 28, 29] (Figure 1, 2)....
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...At the turn of the century, the increased serum proteins and rapid sedimentation rate were identified as characteristic and the origin of the tumor was defined as arising from plasma cells [6-10] ( Figure 1)....
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...Bence-Jones protein is often present in the urine [6, 8, 9, 12] with corresponding kappa or lambda light chains in the serum....
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