Lenalidomide and Dexamethasone in Scleromyxedema: A Case Report of Long-Term Efficacy and Challenges in a 54-Year-Old Patient.

Lichen myxedematosus (LM) is characterized by dermal mucin buildup, fibroblast proliferation, and variable presentation. The diffuse variant, known as scleromyxedema, is identified by monoclonal paraprotein presence and lack of thyroid issues, with considerations for infections and underlying conditions. Despite lacking FDA-approved treatment, intravenous immunoglobulin stands as effective, although resource-intensive, while targeting the clonal source of pathogenic immunoglobulin offers an alternate therapeutic route.

Phase 2 study of venetoclax plus carfilzomib and dexamethasone in patients with relapsed/refractory multiple myeloma.

Proteins in the antiapoptotic B-cell lymphoma 2 (BCL-2) family play a role in the pathophysiology of multiple myeloma (MM). Venetoclax is a highly selective, potent, oral BCL-2 inhibitor that induces apoptosis of MM cells, and its efficacy may be potentiated through combination with agents that increase BCL-2 dependency or have complementary mechanisms of action. The safety, tolerability, pharmacokinetics, and antitumor activity of venetoclax in combination with carfilzomib and dexamethasone (VenKd) in adults with relapsed/refractory MM (RRMM) were investigated in this phase 2 dose-escalation study.

Autologous hematopoietic transplantation following COVID-19 infection.

Autologous hematopoietic cell transplantation following induction therapy is standard of care for most patients with newly diagnosed multiple myeloma ( 2017, ; 1311). Though active COVID-19 infection is typically a contraindication to aggressive therapy, little is known about the safety of autologous transplantation after resolution of acute symptoms and undetectable pathogen by nasopharyngeal PCR.

Venetoclax and Decitabine for T/Myeloid Mixed-Phenotype Acute Leukemia Not Otherwise Specified (MPAL NOS).

T/myeloid mixed-phenotype acute leukemia not otherwise specified (MPAL NOS) is an uncommon and aggressive leukemia without well-established treatment guidelines, particularly when relapsed. Venetoclax plus a hypomethylating agent offers a promising option in this situation since studies support its use in both acute myeloid and, albeit with fewer data to date, acute T-cell-lymphoblastic leukemias. We report the successful eradication of T/myeloid MPAL NOS relapsed after allogeneic stem cell transplant with venetoclax plus decitabine.

Mantle cell lymphoma relapsed after autologous stem cell transplantation: a single-center experience.

Background: Autologous stem cell transplantation (autoSCT) can extend remission of mantle cell lymphoma (MCL), but the management of subsequent relapse is challenging.

Methods: We examined consecutive patients with MCL who underwent autoSCT at Veterans Affairs Puget Sound Health Care System between 2009 and 2017 (N=37).

Results: Ten patients experienced disease progression after autoSCT and were included in this analysis.

DLGAP1 directs megakaryocytic growth and differentiation in an MPL dependent manner in hematopoietic cells.

Background: The MPL protein is a major regulator of megakaryopoiesis and platelet formation as well as stem cell regulation. Aberrant MPL and downstream Jak/STAT signaling results in the development of the Myeloproliferative Neoplasms (MPN). The pathogenetic and phenotypic features of the classical MPNs cannot be explained by the known mutations and genetic variants associated with the disease.

Association of Monoclonal Gammopathy with Progression to ESKD among US Veterans.

Background And Objectives: Whether patients with monoclonal protein are at a higher risk for progression of kidney disease is not known. The goal of this study was to measure the association of monoclonal protein with progression to ESKD.

Design, Setting, Participants, & Measurements: This was a retrospective cohort study of 2,156,317 patients who underwent serum creatinine testing between October 1, 2000 and September 30, 2001 at a Department of Veterans Affairs medical center, among whom 21,898 had paraprotein testing within 1 year before or after cohort entry.

Glucocorticoids in multiple myeloma: past, present, and future.

Glucocorticoids are a backbone of treatment for multiple myeloma in both the upfront and relapsed/refractory setting. While glucocorticoids have single agent activity in multiple myeloma, in the modern era, they are paired with novel agents to induce high clinical response rates. On the other hand, toxicities of steroid therapy limit high dose delivery and impact patient quality of life.

Monoclonal gammopathies: Electronic subspecialty consultation.

Importance: Electronic consultation (e-consult) is an important component of care for patients in the Veterans Health Administration who require subspecialty consultation but not urgent face-to-face evaluation. Monoclonal gammopathy of undetermined significance (MGUS) is a common reason for e-consult. While often benign, MGUS requires careful evaluation and persistent surveillance over time.

The eIF2-alpha kinase HRI: a potential target beyond the red blood cell.

The eIF2α kinase heme-regulated inhibitor (HRI) is one of four well-described kinases that phosphorylate eIF2α in response to various cell stressors, resulting in reduced ternary complex formation and attenuation of mRNA translation. Although HRI is well known for its role as a heme sensor in erythroid progenitors, pharmacologic activation of HRI has been demonstrated to have anti-cancer activity across a wide range of tumor sub-types. Here, the potential of HRI activators as novel cancer therapeutics is explored.

Vitamin D and plasma cell dyscrasias: reviewing the significance.

Monoclonal gammopathy of undetermined significance (MGUS) is a clonal plasma cell disorder and precursor disease to multiple myeloma and other related cancers. While MGUS is considered a benign disorder, with a low risk of disease progression, patients have altered bone microarchitecture and an increased risk of bone fracture. In addition, alterations in immune function are regularly found to correlate with disease activity.

Lymphomatoid granulomatosis associated with azathioprine use for immune-mediated neuropathy.

Lymphomatoid granulomatosis (LG) is a rare Epstein-Barr virus-driven lymphoproliferative disorder that generally arises in immunosuppressed patients and which can be life-threatening. Here we describe the development of pulmonary LG in a patient on long-term azathioprine for immune-mediated neuropathy. Although azathioprine carries a boxed warning for malignancy, its association specifically with LG, an otherwise rare entity, is poorly recognised.

AL Amyloidosis Complicated by Persistent Oral Bleeding.

A case of amyloid light chain (AL) amyloidosis is presented here with uncontrolled bleeding after a nonsurgical dental procedure, most likely multifactorial in nature, and consequently treated with a multidisciplinary approach.

Utilization of stored autologous PBSCs to support second autologous transplantation in multiple myeloma patients in the era of novel agent therapy.

Outcomes in multiple myeloma (MM) have improved significantly with novel agent therapy and autologous stem cell transplantation (ASCT). ASCTs are typically planned as either tandem or a single transplant with additional stored PBSCs available for a second salvage transplant. To accommodate these strategies, many centers routinely collect and store adequate PBSCs for two ASCTs.

Panobinostat: a review of trial results and future prospects in multiple myeloma.

Multiple myeloma is an incurable often devastating disease that is responsible for 1-2% of all cancers. Multiple myeloma is the second most common hematologic malignancy. Over the past two decades, advances in therapy have doubled life expectancy.

CXCR7-dependent angiogenic mononuclear cell trafficking regulates tumor progression in multiple myeloma.

The CXCR4/stromal cell-derived factor-1 (SDF-1) axis is essential for cell trafficking and has been shown to regulate tumor progression and metastasis in many tumors including multiple myeloma (MM). A second chemokine receptor for SDF-1, CXCR7 was discovered recently and found on activated endothelial cells. We examined the role of CXCR7 in angiogenic mononuclear cells (AMCs) trafficking in MM.

A prospective study of lenalidomide monotherapy for relapse after Allo-SCT for multiple myeloma.

Allo-SCT can result in long-term remission in patients with multiple myeloma (MM), although its overall role in disease management remains controversial. We evaluated lenalidomide monotherapy response and tolerability among 18 patients with MM who progressed or relapsed after Allo-SCT, who were enrolled a median of 12 months (range 3-104) following transplant. Treatment duration of lenalidomide was 8 months (range 1-57).