Non-Secretory Multiple Myeloma Associated With High-Risk Phenotype and Complex Cytogenetics Including t(8;22).

Multiple myeloma (MM) is a plasma cell dyscrasia which is typically characterized by identifiable paraprotein in the blood or urine. However, the minority of patients in whom paraprotein cannot be identified are designated non-secretory MM (NSM). Evaluation of treatment response is more difficult in these patients as paraprotein levels cannot be followed. A dearth of clinical trials including these patients exists because of an inability to measure response by classical serum and urine measurement mechanisms as well as seemingly decreased overall survival compared to secretory MM. NSM is subdivided into four subgroups: "non-producers", "true non-secretors", "oligosecretors" and "false non-secretors". The "non-producers" phenotype is associated with more aggressive disease course. Translocations such as those involving the proto-oncogene c-MYC (chromosome 8) and the lambda light chain gene IGL (chromosome 22) - more commonly associated with Burkitt lymphoma - are rare in MM. We describe a 60-year-old male with NSM who was identified as having multiple high-risk features including complex cytogenetics and a non-producer phenotype, which are features not considered in conventional MM staging and risk stratification. This case highlights the need for awareness of phenotypes and cytogenetics associated with higher clinical risk that are not included in the revised International Staging System.

How did we reform our out of control massive transfusion protocol program?

BACKGROUND: The massive transfusion protocol (MTP) is designed to quickly provide blood products at a fixed ratio for the exsanguinating patient. At our academic medical center, the frequency of MTP activation increased over 10-fold between 2008 and 2015, putting inordinate stress on our transfusion service. STUDY DESIGN AND METHODS: Gathering a large number of relevant stakeholders, we performed a multidisciplinary root cause analysis (RCA) in response to the acute clinical need to reform our MTP. RESULTS: Through the RCA, we identified four principal opportunities for improvement (OFI) associated with our MTP: education, stewardship, process improvement, and communication. Through the deployment of new approaches to each of these OFI, we reduced MTP activations, blood product waste, and transfusion service technologist stress. CONCLUSION: The MTP is amenable to improvement, and, although time intensive, the RCA process yields significant favorable effects: improving communication with colleagues, reducing stress within the transfusion service, and improving resource utilization. Activation of the MTP at our institution is now more aligned with its primary purpose: rapidly providing large quantities of blood products to exsanguinating patients.

Recent Advances in Imaging of Hypertensive Heart Disease.

PURPOSE OF REVIEW: To review recent advances in the imaging of hypertensive heart disease (HHD) with an emphasis on developments in the imaging of diffuse myocardial fibrosis using cardiac magnetic resonance (CMR). RECENT FINDINGS: HHD results from long-standing hypertension and is characterized by the development of left ventricular hypertrophy and diffuse interstitial fibrosis. Diffuse fibrosis traditionally required endomyocardial biopsy to diagnose, but recent developments using T1 mapping in CMR allow for noninvasive assessment. Studies using T1 mapping have shown an increase in extracellular volume fraction (ECV) in patients with HHD compared to normal controls, suggesting ECV can be used as a noninvasive marker for fibrosis in HHD. In addition to T1 mapping, other recent advances in HHD imaging include improvements in three-dimensional echocardiography, allowing for accurate real-time volumetric measurements, and the use of speckle tracking echocardiography to detect subclinical systolic dysfunction. Measurement of ECV using T1 mapping in CMR can be used as a noninvasive marker of diffuse myocardial fibrosis in HHD. While further studies are needed to validate this approach with larger patient cohorts, ECV can potentially be used to both monitor disease progression and assess therapeutic interventions in HHD.