Background DICER1 syndrome is an autosomal dominant tumour predisposition syndrome associated with a wide variety of cancerous and noncancerous conditions, including ovarian sex cord–stromal tumours and thyroid conditions, including multinodular goiter. The most common ovarian sex cord–stromal tumour associated with DICER1 syndrome is Sertoli–Leydig cell tumour, with germline DICER1 mutations present in more than 50% of cases. We present a case in which a patient in her late 30s was diagnosed with a Sertoli–Leydig cell tumour in the background of a strong family history of multinodular goiter and Sertoli–Leydig cell tumour with a germline mutation in DICER1.Case Presentation A 38-year-old woman with history of multinodular goiter was found to have stage iiic ovarian Sertoli–Leydig cell cancer after presenting with abdominal pain. She underwent multiple surgeries and chemotherapy. The patient developed rapid disease progression and died 7 months after diagnosis. Seven years earlier, a daughter had experienced the same disease and was found to have a germline DICER1 mutation. The mother had not undergone testing before her own diagnosis.Summary The co-occurrence of Sertoli–Leydig cell tumour and multinodular goiter is highly suggestive of DICER1 syndrome. The recognition of DICER1 syndrome within a family is essential for increased awareness and potential early recognition of complications. Most conditions associated with DICER1 syndrome occur in childhood, and most of the current screening recommendations are specific for childhood and young adulthood. Cancer risks and findings for the adult population are not as well defined. Clinicians who encounter DICER1 syndrome should review recommendations for genetic testing and surveillance and enrol patients in the DICER1 registry.

No abstract

Hepatosplenic T cell lymphoma (HSTCL) is a rare subtype of peripheral non-Hodgkin lymphoma, of which 20% of cases are associated with chronic immunosuppression. It often occurs in patients with inflammatory bowel disease treated with immunomodulating medications such a thiopurines or TNF-alpha inhibitors. Cytopenias are commonly seen but autoimmune hemolytic anemia (AIHA) is rare. Here we present a young male with longstanding ulcerative colitis on chronic azathioprine, exhibiting several rare features of HSTCL. He initially presented with refractory AIHA, thrombocytopenia, and massive splenomegaly, requiring splenectomy. Histologic examination of his spleen confirmed diagnosis of HSTCL. Approximately 3 months after diagnosis, he was found to have leukemic transformation, representing a secondary malignancy.

1510 Background: Despite curative or disease-controlling roles in AML/MDS and MM, access to allogeneic (allo) and autologous (auto) hematopoietic stem cell transplantation (SCT) remains far from universal. Socioeconomic status (SES) and geographic distance from SCT centers have been shown to be barriers to SCT access. In 2016, Hartford HealthCare (HHC) and the Memorial Sloan Kettering Cancer Center (MSK) pioneered a Shared-Care Model (SCM) to streamline access to allo and auto SCT at MSK, featuring a dedicated nurse SCT coordinator, shared hematology tumor boards, MSK-led didactics for HHC providers, and an electronic health record sharing pipeline. We sought to determine if this has improved access to SCT for HHC patients. Methods: A retrospective chart review was conducted of HHC patients aged 18-70 with new diagnoses of AML, MDS, and MM between 2016 and 2020. Socioeconomic status (SES) was estimated by 9-digit zip-code using the Area Deprivation Index (ADI), shown to be a surrogate for healthcare access. Referral or not to a SCT center, referral to MSK through the SCM, and reasons for non-referral were abstracted from the medical record. For patients referred for SCT at MSK, we also captured the number of peri-SCT days in New York City (NYC) and number of subsequent MSK and HHC clinic visits/hospitalizations within 1-year post-SCT. Results: A total of 126 patients was included, with 81 (64%) treated for AML/MDS and 45 (36%) for MM. The median age was 60 years (interquartile range [IQR]: 53-66). The majority were white (n = 101, 80%) followed by 10% (n = 13) Black/African American; 10% (n = 12) were of Hispanic ethnicity. The median ADI percentile was 38 (IQR: 20-51; higher percentiles reflect decreased SES). The median ADI for MSK SCT referrals from New York, New Jersey, and Connecticut 2016-2020 for the same indications was 19 (IQR: 10-30, p < 0.001). A total of 90 patients (71%) were referred to SCT centers. Leading reasons for no referral were favorable-risk disease (n = 10), goals of care (n = 9), and death prior to referral (n = 5); 3 patients were not referred due to comorbidities/performance status. No differences were found between patients referred to MSK vs. other centers. Thirty-four HHC patients were referred to MSK (21 AML/MDS, 13 MM), vs. 3 between 2010 and 2015. Twelve patients underwent allo SCT, with median 97 days in NYC (range: 68-247); 8 underwent auto SCT, with median 21 days in NYC (range: 15-48). Conclusions: Our findings show the feasibility of a shared-care model between a non-SCT-providing large regional hospital system and a major academic transplantation center. Close collaboration between institutions may minimize time patients spend away from home. The SES of HHC referrals was lower than the general MSK population, suggesting that a shared-care model may facilitate access to SCT for patients with previous barriers for this potentially curative therapy.

A527patients with a recent ischemic stroke/TIA. The purpose of this study was to estimate the cost-effectiveness of two screening methods for detection of silent AF, brief intermittent long-term ECG recordings at regular time intervals and shortterm 24-hours continuous ECG (Holter-ECG) and to compare them to a no screening alternative in patients with a recent ischemic stroke. Methods: The long-term (20 year) costs and effects of brief intermittent long-term ECG recordings at regular time intervals and short term continuous ECG are estimated with a decision analytic model combining the result of a clinical study and epidemiological data. The structure of a cost-effectiveness analysis was used in this study. The short term decision tree model analyzed the screening procedure until the onset of anticoagulant treatment. The second part of the decision model follows a Markov design simulating the patients for 20 years. Results: Continuous 24 h ECG recording was dominated by intermittent ECG due to lower sensitivity and higher costs. The base case analysis compared intermittent-ECG screening with no screening of patients with recent stroke. The implementation of the screening program on 1000 patients resulted in 10,9 avoided strokes and the gain of 29,2 life years or 22,7 QALYs and cost savings of € 55 000. ConClusions: Screening of silent AF by intermittent ECG recordings in patients with a recent ischaemic stroke is cost-effective use of health care resources saving costs, lives and quality of life.