A Rare Case of Renal Thrombotic Microangiopathy and Focal Segmental Glomerulosclerosis Secondary to Plasma Cell Leukemia.

Plasma cell dyscrasias are a subset of hematological malignancies involving the production of monoclonal immunoglobulins. This spectrum of disorders includes asymptomatic conditions such as monoclonal gammopathy of unknown significance as well as extremely aggressive malignancies such as plasma cell leukemia. Monoclonal gammopathies are occasionally associated with renal failure, which can occur via many pathophysiological processes.

Stakeholder engagement and participation in the design, delivery, and dissemination of the ostomy self-management telehealth (OSMT) program.

Purpose: Stakeholder engagement is increasingly integrated into clinical research processes. We conducted a mixed methods analysis to describe stakeholders' (peer ostomates, ostomy nurses, telehealth engineers) perceptions of their engagement and participation in a multisite, randomized trial of a telehealth-delivered curriculum for cancer survivors with ostomies.

Methods: Stakeholder notes were analyzed using narrative analysis.

Light chain deposition disease masquerading as smoking-associated nodular glomerulosclerosis with deposits identified by electron microscopy only.

Light chain deposition disease (LCDD) is a form of monoclonal gammopathy of renal significance. The diagnosis is based on the immunofluorescence (IF) findings of linear monoclonal light chain staining of basement membranes throughout the kidney, which appear as non-organized, granular punctate to powdery electron dense deposits by electron microscopy (EM). Although "LCDD by IF only" without EM deposits has been well-described, LCDD identified by EM with negative IF is very rare and hardly mentioned in the literature.

An Unusual Case of Multiple Myeloma with Light-Chain Cast Nephropathy Secondary to a Very Large Plasmacytoma without Bone Marrow Involvement.

Here, we report a case of a patient who presented to Strong Memorial Hospital with new-onset renal failure and anemia and was found to have multiple myeloma with lambda light-chain cast nephropathy secondary to a very large (14 cm × 14 cm × 12 cm) plasmacytoma without bone marrow involvement. This case is notable as solitary plasmacytomas are almost never seen with concomitant myeloma-defining CRAB criteria or significantly elevated serum free light-chain ratios. Although solitary plasmacytomas are typically definitively treated with radiation, this case highlights that systemic treatment may be helpful in certain clinical scenarios.

Cancer survivors' greatest challenges of living with an ostomy: findings from the Ostomy Self-Management Telehealth (OSMT) randomized trial.

Objectives: An ostomy results in lifelong quality of life changes for a cancer survivor. We describe the greatest challenges reported from a randomized trial of cancer survivors with stomas (ostomies).

Methods: Cancer survivors with ostomies participating in a multi-site randomized prospective trial of an Ostomy Self-Management Telehealth (OSMT) program versus usual care (UC) were surveyed at six months post accrual.

Interaction kinetics with transcriptomic and secretory responses of CD19-CAR natural killer-cell therapy in CD20 resistant non-hodgkin lymphoma.

We investigated the cytolytic and mechanistic activity of anti-CD19 chimeric antigen receptor natural killer (CD19.CAR.NK92) therapy in lymphoma cell lines (diffuse large B-cell, follicular, and Burkitt lymphoma), including rituximab- and obinutuzumab-resistant cells, patient-derived cells, and a human xenograft model.

Combinatorial ixazomib and belinostat therapy induces NFE2L2-dependent apoptosis in Hodgkin and T-cell lymphoma.

Ixazomib activity and transcriptomic analyses previously established in T cell (TCL) and Hodgkin (HL) lymphoma models predicted synergistic activity for histone deacetylase (HDAC) inhibitory combination. In this present study, we determined the mechanistic basis for ixazomib combination with the HDAC inhibitor, belinostat, in HL and TCL cells lines (ixazomib-sensitive/resistant clones) and primary tumour cells. In ixazomib-treated TCL and HL cells, transient inhibition followed by full recovery of proteasomal activity observed was accompanied by induction of proteasomal gene expression with NFE2L2 (also termed NRF2) as a prominent upstream regulator.

A review of thrombotic microangiopathies in multiple myeloma.

Patients with multiple myeloma (MM) are susceptible to developing thrombotic microangiopathies (TMAs), an etiologically diverse group of syndromes which include atypical hemolytic uremic syndrome (aHUS) and thrombotic thrombocytopenic purpura (TTP). The TMAs are characterized by thrombocytopenia and microangiopathic hemolytic anemia (MAHA), and are associated with a high mortality risk and irreversible end-organ damage when treatment is delayed. In MM patients, TMAs may be triggered by specific chemotherapies, bone marrow transplantation (BMT), and progression of underlying disease.

Intracellular and extracellular effector activity of mouse neutrophils in response to cutaneous and visceral Leishmania parasites.

Neutrophils are short-lived phagocytic cells equipped with several receptors for pathogen recognition and phagocytosis and have intracellular and extracellular effector mechanisms that can inactivate pathogens. Leishmaniases are diseases caused by different species of Leishmania that mainly afflicts poorer populations of tropical and subtropical regions and immunocompromised individuals. Thus, the present study aims to investigate the effector response of murine neutrophils to species of Leishmania causing American cutaneous leishmaniasis and zoonotic visceral leishmaniasis by evaluating pattern recognition receptors (PRR) and intracellular and extracellular effector microbicide activity.

Canine neutrophils activate effector mechanisms in response to Leishmania infantum.

Canine leishmaniosis caused by L. infantum is a severe zoonotic disease. Although macrophages are the definitive host cells, neutrophils are the first cells to encounter the parasite soon after its inoculation in the dermis by the phlebotomine vector.

Ostomy telehealth for cancer survivors: Design of the Ostomy Self-management Training (OSMT) randomized trial.

Purpose: An ostomy adversely affects health-related quality of life (HRQOL) in a diverse population of cancer survivors and their caregivers. Hit-or-miss ostomy care, nurse counseling, and community referral have been the primary modes of self-management education and support in the peri-operative setting. Few evidence-based, systematic ostomy self-management programs are available to ensure optimal post-operative care.

Second line treatment options for pancreatic cancer.

Patients with advanced pancreatic cancer (APC) refractory to first-line therapy have a dismal prognosis and limited therapeutic options, with only one option consisting of nanoliposomal irinotecan in combination with fluorouracil and folinic acid which was approved by FDA based upon results of the phase III NAPOLI-1 study. Areas covered: We performed a literature search for relevant published clinical trials, abstracts of trials in progress and ongoing or planned trials for the second line treatment of APC using Pubmed.com, ClinicalTrials.

The safety and efficacy of Onivyde (irinotecan liposome injection) for the treatment of metastatic pancreatic cancer following gemcitabine-based therapy.

Introduction: Patients with advanced and metastatic pancreatic cancer refractory to gemcitabine based therapy have a dismal prognosis and limited therapeutic options. Recently, the FDA approved nanoliposomal irinotecan combined with fluorouracil/folinic acid for such patients based upon results of the NAPOLI-1 study which showed this regimen compared to fluorouracil/folinic acid significantly prolonged progression free survival (3.1 vs.

Proteasomal Inhibition by Ixazomib Induces CHK1 and MYC-Dependent Cell Death in T-cell and Hodgkin Lymphoma.

Proteasome-regulated NF-κB has been shown to be important for cell survival in T-cell lymphoma and Hodgkin lymphoma models. Several new small-molecule proteasome inhibitors are under various stages of active preclinical and clinical development. We completed a comprehensive preclinical examination of the efficacy and associated biologic effects of a second-generation proteasome inhibitor, ixazomib, in T-cell lymphoma and Hodgkin lymphoma cells and in vivo SCID mouse models.

Otic mesenchyme expression of Cre recombinase directed by the inner ear enhancer of the Brn4/Pou3f4 gene.

Brn4/Pou3f4 is a POU-domain transcription factor expressed in the otic mesenchyme that is required for the normal development of the inner ear. In this report, we describe the isolation of an otic mesenchyme enhancer in the Brn4 gene. Subsequently, this enhancer was used to drive the expression of Cre recombinase in the otic mesenchyme of transgenic mice.

Mexiletine for refractory chronic daily headache: a report of nine cases.

Objective: To describe the use of mexiletine in the treatment of chronic daily headache in a refractory headache population.

Background: Intravenous lidocaine is a novel treatment for chronic daily headache with medication overuse and SUNCT syndrome. Mexiletine is a similar but orally active anti-arrhythmic that has been demonstrated to be an effective analgesic in various types of neuropathic pain.

Serum amylase and macroamylase in patients with systemic lupus erythematosus.

We measured serum amylase and macroamylase activity in 25 patients with systemic lupus erythematosus (SLE) and 15 controls. The mean (+/- SD) for SLE was greater than for the controls (161 +/- 71.8 versus 116 +/- 38.

Scleritis, pericarditis, and aortic insufficiency in a patient with rheumatoid arthritis.

A 55-year-old black man developed anterior nodular scleritis in his left eye in November 1981. He had no symptoms of systemic disease, and initial laboratory tests revealed only a positive rheumatoid factor. Fourteen months later he presented with pericarditis and aortic insufficiency requiring aortic valve replacement.

Granulocyte-associated IgG in neutropenic disorders.

We applied a radiolabeled antiglobulin test to a study of patients with a variety of neutropenic disorders. After defining the nature of the interaction of radiolabeled anti-IgG with the neutrophil, we studied 16 patients with neutropenia of uncertain etiology and adequate bone marrow granulocyte precursors. Twelve of these 16 patients had increased neutrophil-associated IgG (PMN-IgG).

Decreased numbers of monocytes in inflammatory exudates in systemic lupus erythematosus.

Rebuck skin windows and delayed hypersensitivity testing were performed on patients with systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA). Patients with SLE had a decreased appearance of mononuclear/macrophage cells in the inflammatory exudate compared to patients with RA and normal controls. This abnormal response was unrelated to corticosteroid therapy, but appeared to correlate with activity of disease.

Hemopneumothorax in systemic lupus erythematosus.

Neither hemothorax nor pneumothorax are well recognized manifestations of systemic lupus erythematosus (SLE). Two patients with SLE who developed hemopneumothorax during the course of their illness are described. The clinical, laboratory, roentgenographic, and pathological findings are presented.