Sclerosing mesenteritis affecting the small and the large intestine in a male patient with non-Hodgkin lymphoma: a case presentation and review of the literature

<p>Abstract</p> <p>Introduction</p> <p>Sclerosing mesenteritis is a rare disease resembling a mesenteric tumour. We present here a case of sclerosing mesenteritis that affected both the large and the small intestine of the patient. Therapeutic and diagnostic issues are discussed.</p> <p>Case presentation</p> <p>A 62-year-old man with a history of non-Hodgkin lymphoma presented with fatigue, a palpable tender abdominal mass and clinical signs of progressing intestinal obstruction. The preoperative evaluation failed to prove recurrence of the lymphoma or any other definite diagnosis. A laparotomy was performed through a midline incision. The mesentery resembled a tumour-like thickened and fibrotic mass. Abundant, rigid intestinal loop adhesions were observed. Diffuse fibrotic infiltration of the ileum and of the sigmoid colon, which obviously affected the intestinal vascular supply, were identified. A right colectomy and partial sigmoidectomy were performed. Pathological evaluation revealed extensive myofibroblastic reaction of the mesentery with accompanying loci of fat necrosis and areas of inflammation. A diffuse fibrotic infiltration that focally showed a ground-glass appearance was observed. The post-operative course was complicated by respiratory insufficiency and infections and the patient died 2 months after the operation.</p> <p>Conclusion</p> <p>Sclerosing mesenteritis that affects both the small and the large intestine is extremely rare. The disease is characterized by myofibroblastic reaction, fat necrosis and diffuse fibrosis of the mesentery. Pathological confirmation may be required for definite diagnosis. If the disease is characterized by severe and diffuse fibrosis, then the application of surgical therapy may be problematic.</p

POEMS Syndrome: Real World Experience in Diagnosis and Systemic Therapy - 108 Patients Multicenter Analysis

POEMS syndrome, a rare plasma cell disorder, is challenging both in the diagnostic and therapeutic management. We present real word retrospective analysis of 108 cases analyzing clinical features and therapeutic modes. We compare our results with the available literature. This is the first description with such wide use of proteasome inhibitors in first line treatment. POEMS (Polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes) syndrome is a rare and challenging plasma cell disorder, both in the diagnostic and therapeutic management of the disease. Currently, the literature on POEMS is sparse with most evidence being case reports and small case studies. We present a retrospective real world experience of 108 patients with POEMS. We analyzed the clinical features and therapeutic interventions. Regarding clinical features, our findings demonstrated that skin lesions, thrombocythemia and polycythemia were present less frequently than reported previously. Regarding clinical interventions, this is one of the largest analyses of front line treatment in POEMS and the first one to include frequent utilization of proteasome inhibitors (37%). Bortezomib monotherapy was the most effective therapy achieving complete remission/very good partial remissions (CR/VGPR) in 69% of patients. Thirty percent of patients proceeded to planned autologous stem cell transplant (ASCT) as part of the front-line treatment resulting in statistically superior progression-free (PFS) and overall survival (OS) compared to non-ASCT treated patients (P= .003). In multivariate analysis, anemia, thrombocytopenia, and as age over 60 were associated with a negative impact on patient outcomes

Similar survival outcomes in patients with biclonal versus monoclonal myeloma: a multi-institutional matched case-control study

Multiple myeloma is a plasma cell malignancy characterized by clonal proliferation of plasma cells in the bone marrow and associated organ damage. Usually, patients with myeloma present with a single monoclonal protein in serum and/or urine constituted by one heavy chain and one light chain. In less than 5% of the patients, more than one monoclonal protein can be identified. The aim of our retrospective multicenter matched case-control study was to describe the characteristics of cases with biclonal myeloma and compare them against a control group of monoclonal myeloma patients matched by age, sex, and year of diagnosis. A total of 50 previously untreated cases with biclonal myeloma and 50 matched controls with monoclonal myeloma were included in this study. The controls were matched (1:1) for age, sex, year of diagnosis, and participating center. There were no differences in the rates of anemia (52 vs. 59%; p = 0.52), renal dysfunction (36 vs. 34%; p = 0.83), hypercalcemia (9 vs. 16%; p = 0.28), or presence of lytic lesions (23 vs. 16%; p = 0.38) between groups. Similarly, there was no difference in the rates of overall response to therapy (85 vs. 90%; p = 0.88) or survival rates of cases with biclonal myeloma and controls with monoclonal myeloma (4-year survival 72 vs. 76%; p = 0.23). Results of our study suggest that patients with biclonal myeloma have similar response and survival rates than patients with monoclonal myeloma

A multicenter retrospective study of 223 patients with t(14;16) in multiple myeloma

The t(14;16) translocation, found in 3%-5% of newly diagnosed (ND) multiple myeloma (MM), has been associated with adverse outcomes. However, the studies establishing the characteristics of t(14;16) included solely small cohorts. The goal of the current international, multicenter (n = 25 centers), retrospective study was to describe the characteristics and outcomes of t(14;16) patients in a large, real-world cohort (n = 223). A substantial fraction of patients had renal impairment (24%) and hemoglobin &lt;10 g/dL (56%) on initial presentation. Combined therapy of both immunomodulatory drug and proteasome inhibitor (PI) in the first line was used in 35% of patients. Autologous stem cell transplantation was performed in 42% of patients. With a median follow up of 4.1 years (95% CI 3.7-18.7), the median progression-free survival (PFS) and overall survival (OS) from first line therapy were 2.1 years (95% CI 1.5-2.4) and 4.1 years (95% CI 3.3-5.5), respectively. Worse OS was predicted by age &gt; 60 years (HR = 1.65, 95% CI [1.05-2.58]), as well as revised International Scoring System (R-ISS) 3 (vs R-ISS 2; HR = 2.59, 95% CI [1.59-4.24]). In conclusion, based on the largest reported cohort of t(14;16) patients, quarter of this subset of MM patients initially presents with renal failure, while older age and the R-ISS 3 predict poor survival