Predictive Factors for Early Relapse in Multiple Myeloma after Autologous Hematopoietic Stem Cell Transplant.

INTRODUCTION: Despite advances in therapy for multiple myeloma, patients have continued to experience relapse. We sought to better understand this. OBJECTIVE: To identify factors that predict early relapse in patients with multiple myeloma who receive autologous hematopoietic peripheral stem cell transplant (HSCT). METHODS: Retrospective analysis of Kaiser Permanente Southern California patients who received HSCTs between 2008 and 2012. RESULTS: A total of 141 patients were included. Factors found to be associated with inferior progression-free survival were disease status less than complete response at the time of HSCT, no use of maintenance therapy after HSCT, International Staging System stage III, and high Freiburg Comorbidity Index. Disease status less than complete response, stage III, higher Freiburg Comorbidity Index, no use of maintenance therapy, and male sex were the most predictive factors for early relapse (< 18 months). DISCUSSION: Our results identified a subgroup of high-risk individuals with multiple myeloma who will continue to do poorly after HSCT with the best available treatment using a combination of proteasome inhibitors and immunomodulatory drugs. These results highlight the need for consideration of alternative therapy in such instances.

An Unlikely Rapid Transformation of Myelodysplastic Syndrome to Acute Leukemia: A Case Report.

INTRODUCTION: Myelodysplastic syndrome is characterized by stem-cell-derived clonal myelopoiesis with an alteration in proliferation and differentiation. This condition carries a potential for transformation to acute leukemia, primarily in cases that are accompanied by high-risk features at diagnosis. CASE PRESENTATION: A 68-year-old man with recently diagnosed myelodysplastic syndrome and Sweet syndrome (acute febrile neutrophilic dermatosis) presented to our Emergency Department with shortness of breath. During his hospital course, he developed signs and symptoms, predominantly consisting of respiratory difficulties, that were not typically characteristic of transformation to acute leukemia. Several days into his hospitalization, it was determined that the patient's underlying hematologic process seemed to have rapidly evolved into an acute myeloid leukemia, which accounted for the progression of symptoms. This patient ultimately opted for comfort measures only and died shortly thereafter. DISCUSSION: Two important factors stood out as representing an atypical presentation. First, this patient lacked any of the high-risk features of myelodysplastic syndrome that typically portend transformation. In addition, his progression to acute leukemia in 28 days from the time of diagnosis was far more rapid than the 274-day median previously described in the literature. We theorize that the presence of Sweet syndrome may have served as a predisposing factor to transformation. This finding may offer benefit to physicians to potentially better predict this outcome and pursue more aggressive treatment measures earlier in the course of the disease in such a setting.

Characteristics of patients diagnosed with renal vein thrombosis and glomerulopathy: a case series.

BACKGROUND: Few contemporary studies have evaluated the clinical characteristics of patients with biopsy-proven glomerulopathy diagnosed with renal vein thrombosis (RVT). METHODS: Retrospective case series study within an integrated health system in a 12-year period (January 1, 2000 through December 31, 2011) investigating clinical characteristics of all adult patients who underwent native or transplant kidney biopsy and also had a diagnosis of RVT. Patient characteristics, diagnostic studies, and outcomes were evaluated. RESULTS: Among 3763 eligible patients, 17 had imaging confirmed RVT. Of these, 15 had membranous nephropathy (idiopathic or secondary to autoimmune disease). Although the biopsy population included primary and secondary glomerular disease patients, all 17 RVT patients had severe nephrotic syndrome and profound hypoalbuminemia with mean (SD) of albumin: 1.5 g/dL (0.66). CONCLUSION: Clinically significant RVT in patients with glomerulopathy appears to be a rather rare entity, occurring predominantly in patients with severe nephrotic syndrome due to idiopathic membranous nephropathy and membranous nephropathy secondary to autoimmune disease.

Impact of surgery, radiation and systemic therapy on the outcomes of patients with dendritic cell and histiocytic sarcomas.

BACKGROUND: Neoplasms of histiocytic and dendritic cell origin, including follicular dendritic cell sarcoma (FDCS), histiocytic sarcoma (HS) and interdigitating dendritic cell sarcoma (IDCS), are extremely rare, and data on their natural history and treatment outcomes are sparse. We evaluated the impact of surgery, radiation and systemic therapies on overall survival (OS). METHODS: We conducted a retrospective chart review of patients with FDCS, IDCS and HS treated at Memorial Sloan Kettering Cancer Center between 1995 and 2014. RESULTS: We identified 31, 15 and 7 patients with FDCS, HS and IDCS, respectively. Median age was 48.7, 42.3 and 58.8years for FDCS, HS and IDCS, respectively. Only a slight disparity in gender distribution existed for FDCS and HS; however, IDCS predominantly affected males (6:1). The most common sites of presentation were abdomen and pelvis (42%), extremities (33%) and head and neck (57%) for FDCS, HS and IDCS, respectively. At diagnosis, 74%, 40% and 86% of patients presented with localised disease in FDCS, HS and IDCS, respectively. Patients with localised disease had significantly improved OS than those with metastatic disease in FDCS (P=0.04) and IDCS (P=0.014) but not in HS (P=0.95). In FDCS and HS, adjuvant or neo-adjuvant therapy was not associated with improved OS compared with observation. In IDCS, surgery alone provided a 5-year overall survival rate of 71%. CONCLUSIONS: Adjuvant or neo-adjuvant treatment in FDCS and HS did not affect OS. Patients with IDCS had an excellent outcome with surgery. In the metastatic setting, chemotherapy and small molecule inhibitors may provide benefit.