Nishanth Vallumsetla

Waldenström macroglobulinemia: What a hematologist needs to know.

Waldenström macroglobulinemia (WM) is a distinct hematologic malignancy characterized by a lymphoplasmacytic bone marrow infiltration and the presence of immunoglobulin (Ig)M monoclonal protein. Patients typically present at an advanced age, and a substantial proportion are asymptomatic at diagnosis. A unifying diagnosis of WM may be missed by an unsuspecting hematologist, as symptomatic patients present with a multitude of non-specific manifestations. Although constitutional and neuropathy-related symptoms predominate, concomitant IgM-induced hyperviscosity-associated features can provide useful diagnostic clues. There are specific indications for initiation of therapy. This review focuses on the most up-to-date management strategies of WM, in addition to highlighting the recent discoveries of MYD88 and CXCR4 mutations that have shed unprecedented light on the complex signaling pathways, and opened avenues for novel therapeutic targeting. Although WM remains incurable, with the rapid emergence and integration of effective novel therapies, its clinical course appears poised to improve in the foreseeable future.

Use of Blenderized Tube Feeding in Adult and Pediatric Home Enteral Nutrition Patients.

Background: Long-term use of enteral nutrition (EN) continues to increase due to significant noted benefits. Patients also continue to express significant desire to pursue holistic and organic diets. Despite this, many nutrition providers are not well versed in assisting patients with blenderized tube feeding (BTF), and prevalence of its use is unknown. Methods: A validated survey was administered to Oley Foundation members or individuals with access to the Oley website to assess the prevalence of BTF. Results: A total of 216 participants took the survey, of whom 125 (57.8%) were pediatric patients with a mean age of 5.4 ± 3.5 years and 91 (42.2%) were adults with a mean age of 51.7 ± 19.5 years. Of pediatric patients, 112 (89.6%) used BTF for an average of 71% of their total daily nutrition intake; 93 (83%) reported that BTF comprised >50% of their daily EN, 12 (10.7%) reported it comprised 25%–50% of their daily enteral intake, and 7 (6.3%) reported BTF comprised < 25% of their daily intake. In the adult population, 60 (65.9%) used BTF for an average of 56% of total daily nutrition intake; 41 (68.4%) reported BTF comprised >50% of their daily nutrition intake, 11 (18.3%) reported it compromised 25%–50%, and 8 (13.3%) reported BTF comprised <25% of their daily intake. Conclusions: Most of the pediatric and adult patients surveyed use BTF as some portion of their enteral intake, making it essential that clinicians expand their knowledge related to BTF to appropriately care for this patient population.

Use of Home Parenteral Nutrition in Post–Bariatric Surgery–Related Malnutrition

Background: Bariatric surgery is one of the most effective techniques for achieving sustained weight loss but can be associated with surgical complications or malabsorption so significant that it leads to malnutrition. Parenteral nutrition (PN) may be necessary to help treat surgical complications or malnutrition from these procedures. There are limited data describing this patient population and role for home PN (HPN). Methods: A retrospective review of our HPN database was conducted to identify patients who were initiated on HPN between January 1, 2003, and August 31, 2015, and had a history of bariatric surgery. Results: A total of 54 HPN patients (6.3%) had a history of bariatric surgery. Average age was 52.1 ± 12.8 years, and 80% were female. The most common surgical procedure was Roux-en-Y gastric bypass (72%), with malnutrition or failure to thrive being the most common HPN indication (57%). Weight at the time of HPN initiation was 71.9 ± 20.4 kg and significantly increased to 78.9 ± 24.4 kg by the end of treatment (P = .0001). Serum albumin levels rose from 2.8 ± 0.77 g/dL to 3.7 ± 0.58 g/dL by the end of HPN (P < .0001). Forty-five of 54 patients (83.3%) went on to revision surgery. Conclusion: The results of this retrospective review support initiation of HPN in the malnourished post–bariatric surgery patient both nutritionally and as a bridge to revision surgery.

Accuracy of Intravenous Electrocardiography Confirmation of Peripherally Inserted Central Catheter for Parenteral Nutrition

BACKGROUND Parenteral nutrition (PN) is a life-saving therapy for patients with intestinal failure. Safe delivery of hyperosmotic solution requires a central venous catheter (CVC) with tip in the lower superior vena cava (SVC) or at the SVC-right atrium (RA) junction. To reduce cost and delay in use of CVC, new techniques such as intravascular electrocardiogram (ECG) are being used for tip confirmation in place of chest x-ray (CXR). The present study assessed for accuracy of ECG confirmation in home PN (HPN). METHODS Records for all patients consulted for HPN from December 17, 2014, to June 16, 2015, were reviewed for patient demographics, diagnosis leading to HPN initiation, and ECG and CXR confirmation. CXRs were subsequently reviewed by a radiologist to reassess location of the CVC tip and identify those that should be adjusted. RESULTS Seventy-three patients were eligible, and after assessment for research authorization and postplacement CXR, 17 patients (30% male) with an age of 54 ± 14 years were reviewed. In all patients, postplacement intravascular ECG reading stated tip in the SVC. However, based on CXR, the location of the catheter tip was satisfactory (low SVC or SVC-RA junction) in 10 of 17 patients (59%). CONCLUSION Due to the high osmolality of PN, CVC tip location is of paramount importance. After radiology review of CXR, we noted that 7 of 17 (41%) peripherally inserted central catheter lines were in an unsatisfactory position despite ECG confirmation. With current data available, intravenous ECG confirmation should not be used as the sole source of tip confirmation in patients receiving HPN.

Effect of Home Enteral Nutrition on Diabetes and Its Management

BACKGROUND Diabetes mellitus (DM) is estimated to affect 9% of Americans and is associated with significant morbidity, mortality, and increased healthcare costs. METHODS A retrospective review of a home enteral nutrition (HEN) database of patients seen between March 1, 2004, and April 31, 2014, at our institution was conducted to identify HEN patients who had a diagnosis of DM or were diagnosed within the acute period (4 months) of starting HEN therapy. RESULTS 174 (3.7%) of 4682 patients in the HEN program had DM. HEN was provided through a gastrostomy tube in 82 patients and through a jejunostomy tube in 92 patients. At 3 months, data were available for 42 gastrostomy patients; 44% had a change in DM management, with 60% undergoing a change to insulin. Similarly, 34 patients with jejunostomy had data available, with 41% undergoing change in medication and 93% being changed to insulin therapy. For patients with available glycated hemogloblin values, at 3 months the gastrostomy patients noted a decrease of 0.5% (7.3% ± 1.1% to 6.8% ± 0.7%, not significant) and the jejunostomy patients noted a decrease of 0.4% (6.9% ± 0.9% to 6.5% ± 1.1%, P = .06). CONCLUSIONS Overall, our results suggest that closer follow-up and laboratory assessment are necessary for management of HEN patients with DM. In those with data available, glycemic control can be maintained in patients receiving HEN with appropriate adjustment of DM management and focus on ensuring that patients are not overfed.

Pilot Study Comparing 2 Oral Rehydration Solutions in Patients With Short Bowel Syndrome Receiving Home Parenteral Nutrition: A Prospective Double-Blind Randomized Controlled Trial

Background: Short bowel syndrome (SBS) is a common indication for home parenteral nutrition (HPN). Oral rehydration solutions (ORSs) have the ability to supplement or reduce HPN dependence. However, ORSs have suffered from poor taste profiles, making long-term consumption and compliance unlikely. The goal of the current study was to assess the taste and compliance of 2 ORSs among patients with SBS requiring HPN. Methods: All participants with SBS receiving HPN with anticipated duration >3 months were offered enrollment: 31 participants met inclusion criteria; 3 declined enrollment; and 28 were randomized to receive a modified World Health Organization ORS (group A) or a commercially available ORS (DripDrop; group B). Results: Six participants dropped out shortly after randomization (3 in each group) due to poor taste or intolerance. An additional 3 (1 in group A and 2 in group B) discontinued the ORS before the end of the study at 6 months. At the end of the study, 19 remained. The mean taste rating given by the participants was, on a scale of 1–10, 7.3 ± 1.9 for group A and 7.6 ± 1.6 for group B (P = .61). The mean number of days that ORSs were consumed each week was 6.0 ± 1.3 for group A and 6.6 ± 1 days for group B (P = .06). Conclusion: Taste rating was not different for both ORSs; however, a significant number of participants did not complete the study.

Bortezomib in mantle cell lymphoma: comparative therapeutic outcomes.

Mantle cell lymphoma (MCL) is an incurable, typically aggressive subtype of non-Hodgkin lymphoma, accounting for 4%–7% of newly diagnosed non-Hodgkin lymphoma cases. Chemoresistance commonly ensues in MCL, and patients with this heterogeneous disease invariably relapse, underscoring the unmet need for better therapies. Over the past few years, several novel agents with promising activity and unique mechanisms of action have been deemed effective in MCL. Bortezomib is a reversible proteasome inhibitor, approved as a single agent for patients with relapsed/refractory MCL who have received at least one prior line of therapy. Addition of bortezomib to chemoimmunotherapies has demonstrated good tolerability and superior efficacy, both in the upfront and salvage settings, and recently one such combination of bortezomib plus rituximab, cyclophosphamide, doxorubicin, and prednisone was approved as a frontline regimen in untreated patients with MCL. This review examines the role of bortezomib in a multitude of clinical settings and ongoing clinical trials designed to optimize its integration in the current treatment paradigms of MCL.