Hegerova, Livia

8581 Background: Autologous stem cell transplant (ASCT) improves survival in patients with multiple myeloma (MM). Recent studies have elucidated the relationship in ASCT between absolute lymphocyte count (ALC) recovery and improved survival, highlighting the importance of immune recovery. We conducted a retrospective analysis of patients with multiple myeloma to observe the impact of different measures of immune recovery at day 100 post-ASCT on outcome. Methods: Retrospective analysis of data from the existing clinical databases identified 1184 patients with multiple myeloma that underwent ASCT at Mayo Clinic between 1987-2011. Markers of immune recovery analyzed on day 100 status-post ASCT included ALC, monocyte count, and immunoglobulin levels. Kaplan Meier analysis was performed to determine progression free survival (PFS) and overall survival (OS). Results: Among the 1184 patients, median age at diagnosis of multiple myeloma was 57 (range, 23-75 yr), and median age at time of transplantation was 59 (range, 24-75 yr); 59% were male. The median OS and PFS post-transplant were 80 months and 34 months respectively. 62% of patients were alive 5-years post-transplant. The median OS was not improved with normal vs. abnormal IgG levels at day 100 (p=.298). In contrast, monocyte recovery was a significant predictor of OS. Patients with normal monocyte counts of greater than 0.3 x 10⁹ cells/L at day 100 showed superior survival (65 vs. 37 months, p= .001). Similarly, patients with normal monocyte recovery at day 15 post-transplant showed a significant survival benefit (68 vs. 50 months, p=.007). Early ALC recovery, which has been shown to be a positive prognostic indicator at day 15 post-transplant, was not prognostic at day 100 (p= .960). Conclusions: The present study further elucidates prognostic indicators after ASCT for MM highlighting the importance of various markers of immune recovery. Immunoglobulin recovery was not associated with superior survival, while monocyte recovery at day 15 and day 100 post-transplant translated to an improved survival and requires further study. It may be hypothesized that early immune system reconstitution may have a positive effect against disease post-transplant.

Abstract Abstract 5152 Introduction: Cold agglutinin disease (CAD) is a rare and poorly understood disorder accounting for 15% of patients with autoimmune hemolytic anemia. Treatment has been largely controversial with few studies addressing safety and efficacy of various treatment regimens. This study reports a single institution's experience with cold agglutinin disease, defines the clinical features, prognosis and management. Methods: A retrospective analysis of Mayo Clinic medical records since 1960 was performed to identify all cases of CAD. Initial appraisal identified 89 patients after which an in depth review of clinical notes, laboratory evaluations, and treatment regimens was performed. Statistical analysis was performed via descriptive statistics and Kaplan-Meier survival. Results: The median age at onset of symptoms was 65 years (range: 40, 82), while the median age at diagnosis was 72 (42, 91). The most common sign at presentation was anemia (38. 4%). The most common symptom during the course of disease was similarly finger discoloration (43. 8%). Over half of patients had symptoms that were triggered by a cold environment (52. 8%) and a minority (22. 5%) had exacerbations precipitated by other factors. The median time from onset of symptoms to time of disease diagnosis was 37 months (0, 374). The majority of patients had an underlying hematologic disorder (48. 3%) of which the most common was monoclonal gammopathy of undetermined significance. At diagnosis the average hemoglobin was 10. 2 (6. 2, 17. 7) with positive coombs testing in 75% of patients. All patients had documented positive cold agglutinin titers. Approximately 40% of patients received transfusions at some point during their disease course and 83% needed drug therapy at some point. The most common reason to initiate drug treatment was progressive anemia (44. 9%). In 15% of patients, CAD was able to be managed with watchful waiting. The median duration between initial symptoms to initiation of treatment was 60 months. 56. 2% and 33. 7% of patients required second and third line therapy respectively. Treatment characteristics and responses are displayed in Table 1. Rituximab showed the longest duration of response and had the lowest percentage of patients needing further treatment, while 4 out of 5 patients on prednisone required additional therapies. Median survival in all patients with CAD was 127. 5 months and 76. 9% of patients were alive at 5-years after diagnosis (Figure 1). Conclusions: This is the largest study of patients with cold agglutinin disease to date. Symptoms were frequently ill-defined resulting in delay of diagnosis. Although drug therapy was frequently indicated, many patients were successfully observed. New treatment agents including Rituximab demonstrate promising response rates compared to traditional regimens, especially in patients with underlying hematologic abnormalities. These results support consideration of CAD as part of the differential diagnosis in the setting of new onset anemia and re-enforces the importance of evaluation for underlying B Cell abnormality in this patient population. Disclosures: Off Label Use: Rituximab, Cyclophosphomide, Chlorambucil, and Prednisone will be discussed as therapy modalatities for the treatment of cold agglutinin hemolytic anemia.

Central nervous system metastases as the presentation of primary cardiac sarcoma are a very rare entity, with only a few previously reported cases. Sarcomas specifically make up 10 to 20% of all primary cardiac tumors. Patients with primary cardiac tumors typically present with cardiac symptomatology that may include arrhythmias, obstruction to blood flow and valve function, or symptoms of heart failure. We report a unique case of a patient with a primary cardiac sarcoma who presented with progressive neurologic dysfunction secondary to brain metastases without any preceding cardiac symptoms. We describe our novel management of these unique cases and discuss the current medical and surgical approaches to treating neurologic metastases from cardiac sarcoma.

Abstract Cold agglutinin disease is a rare and poorly understood disorder affecting 15% of patients with autoimmune hemolytic anemia. We reviewed the clinical and pathologic features, prognosis, and management in the literature and describe our institutional experience to improve strategies for accurate diagnosis and treatment. Retrospective analysis identified 89 patients from our institution with cold agglutinin disease from 1970 through 2012. Median age at symptom onset was 65 years (range, 41 to 83 years), whereas the median age at diagnosis was 72 years (range, 43 to 91 years). Median survival of all patients was 10.6 years, and 68 patients (76%) were alive 5 years after the diagnosis. The most common symptom was acrocyanosis (n = 39 [44%]), and many had symptoms triggered by cold (n = 35 [39%]) or other factors (n = 20 [22%]). An underlying hematologic disorder was detected in 69 patients (78%). Thirty-six patients (40%) received transfusions during their disease course, and 82% received drug therapy. Rituximab was associated with the longest response duration (median, 24 months) and the lowest proportion of patients needing further treatment (55%). Our institution’s experience and review of the literature confirms that early diagnostic evaluation and treatment improves outcomes in cold agglutinin disease.