Solitary plasmacytoma (SP) is a rare plasma cell dyscrasia characterized by the presence of bone or extramedullary plasma cell tumors. The treatment of choice is local radiotherapy (R/T) 6 surgical excision. The role of adjuvant chemotherapy (C/T) or novel agents (NA) is uncertain. Data related to prognostic factors are inconclusive. Herein, we describe the clinical features, survival and prognosis of 97 consecutive patients, 65 with bone SP (SBP), and 32 with extramedullary SP (SEP), diagnosed and treated in 12 Greek Myeloma Centers. Objective response rate (PR) and complete response (CR) was 91.8% and 61.9%, respectively, and did not differ between the 2 groups. Overall, 38 patients relapsed or progressed to multiple myeloma (MM). After a median follow-up of 60 months, 5 and 10-year overall survival (OS) probability was 92% and 89% in SEP and 86% and 69% in SBP, respectively (P 5 0.2). The 5-and 10-year MMfree survival (MMFS) probability was 90% and 70% for patients with SEP vs. 59% and 50% for patients with SBP, respectively (P 5 0.054). Overall, the 5-and 10-year OS probability, plasmacytoma relapse-free survival (PRFS), progression-free survival and MMFS was 84% and 78%, 72% and 58%, 58% and 43%, and 70% and 59%, respectively. In the multivariate analysis, prolonged PRFS and young age were positive predictors of OS. Achievement of CR was the only positive predictor of PRFS. Immunoparesis was the only negative predictor of progression to MM. The addition of C/T or NA-based treatment increased toxicity without offering any survival advantage over R/T.
Hypercalcemia remains a poor prognostic feature in the era of novel agents despite the improvement in the outcomes of patients who present with elevated calcium.
The current study was undertaken to investigate the effect of alendronate on bone mineral density (BMD), bone metabolism markers, and serum bone-resorbing cytokines in patients with chronic idiopathic neutropenia (CIN)-associated osteopenia/osteoporosis. Sixteen randomly selected women, 7 with CIN-associated osteoporosis and 9 with CIN-associated osteopenia, and 14 age- and menopausal status-matched healthy volunteers, were enrolled in the study. Patients received 10 mg alendronate daily per os for 360 days and studies were done before treatment (day 0) and at varying time points during the study. We found that patients' BMD measurements increased by 5.32% after treatment, and that the elevated serum osteocalcin (OC), a bone formation marker, decreased by day 30, normalized by day 90, and increased again by day 270 of treatment. Elevated values of patients' urine deoxypyridinoline (Dpd) and N-telopeptide of type I of collagen (NTx), two bone resorption markers, returned to the control range by day 30 and decreased thereafter. Increased levels of patients' serum tumor necrosis factor-alpha (TNFalpha) and interleukin-1beta (IL-1beta), two bone resorbing cytokines, returned to the control range by day 30 and decreased thereafter. Peripheral blood neutrophil counts increased by day 30 and continued to rise thereafter, reaching a mean value higher than 2650 neutrophils per microl of blood on day 360. Interestingly, alendronate-induced changes in the levels of both cytokines correlated inversely with the respective changes in neutrophil counts and BMD measurements, and positively with the changes in the respective means of urine NTx and Dpd values. All these findings indicate that alendronate is effective in treating CIN-associated osteopenia/osteoporosis, and that the beneficial effect of the compound may lie, at least in part, in its property to inhibit the production of TNFalpha and IL-1beta by cells of the monocyte/macrophage system, in which osteoclasts are included.
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License. Systemic Lupus Erythematosus (SLE) is a systemic autoimmune disorder that is frequently complicated by hematological manifestations such as hemolytic anemia, leukopenia and thrombocytopenia. 1,2 These disorders are included in the diagnostic criteria of SLE both in the earlier (1982), as well as in the revised criteria of the American College of Rheumatology (2003), and the 2012 Systemic Lupus International Collaborating Clinics (SLCC) criteria. 3-5 Thrombocytopenia (<100x10 9 /L) has been reported in 20% to 40% of patients with SLE 2,6-8 and is usually attributed to an autoimmune mechanism similar to that of idiopathic immune thrombocytopenia (ITP). It may be the first manifestation of lupus in up to 16% of patients, presenting months or as early as 10 years before diagnosis. 9,10 It can also be a complication of therapeutic agents such as azathioprine, methotrexate and rarely hydroxychloroquine. Severe thrombocytopenia (<20 to 50x10 9 /L according to study definition) is relatively rare, occurring in about 3-10% of patients. 11,12 Occasionally, thrombo-Cite this article as: Galanopoulos N, Christoforidou A, Bezirgiannidou Z. Lupus thrombocytopenia: pathogenesis and therapeutic implications.
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