Analysis of Updates in Multiple Myeloma Treatment and Management.

Introduction: During the past two decades, new therapeutic agents have greatly improved the treatment landscape in multiple myeloma (MM). Treatments such as proteasome inhibitors, immunomodulatory agents, targeted monoclonal antibody therapy, and chimeric antigen receptor (CAR) T-cell therapy have improved outcomes with less toxicity. Advances in laboratory testing have accompanied this change, performing faster and more accurate assessments of treatment response. Despite these advances, however, disparities in MM outcomes persist. Objective: The purpose of this study was to review epidemiological trends in MM over the past two decades and to identify disparities that may impact MM identification and survival. Methods: Retrospective analysis was conducted on adult patients diagnosed with MM between the years 2000-2019 using the November 2021 Surveillance, Epidemiology, and End Results (SEER) program database. Joinpoint models were used to calculate annual percent changes (APCs) and average annual percent change (AAPC). Results: There were a total of 111,328 diagnoses of MM extracted from the SEER database. Most patients were male (55.17%) and white (76.7%). Age-adjusted rate analysis found a significantly higher incidence among black patients compared to white patients. The APC between 2000-2015 was 1.46, and the APC between 2015-2019 was -1.34. Relative survival also increased from 2000 to 2014. The 5-year cancer survival in MM also increased at an average of 1.8% for every year after diagnosis. The annual probability of MM-related mortality at the 1-year mark also decreased from 28.5% in 2000 to 16.7% in 2018. Conclusion: Novel advances in MM therapeutic agents and diagnostic testing have paved the way for significant improvements in patient survival outcomes. Disparities persist along racial lines. Further research is needed to evaluate responses to specific MM treatment in the age of newly developed targeted therapies to overcome these disparities.

Kidney and urinary tract involvement in systemic mastocytosis.

Systemic mastocytosis (SM) is a disorder of excessive mast cell accumulation in tissues due to a somatic gain-of-function mutation, commonly in the KIT gene, which prevents apoptosis of mast cells. Whereas bone marrow, skin, lymph nodes, spleen and gastrointestinal tract are commonly involved, kidneys are rarely involved directly by SM. However, there are increasing reports of indirect kidney involvement in patients with SM. Novel anti-neoplastic agents to treat advanced forms of SM include non-specific tyrosine kinase inhibitors, which are reported to be associated with kidney dysfunction in some patients. SM is also associated with immune-mediated glomerulonephritis (GN) such as mesangioproliferative GN, membranous nephropathy and diffuse proliferative GN. Kidney injury, in the form of monoclonal deposition disease and primary light chain amyloidosis, is reported in SM associated with plasma cell dyscrasia. In this narrative review we discuss the various ways kidneys (and the urinary tract) are involved in patients with SM.

Cytogenetically normal acute myeloid leukemia with a novel KIT mutation in exon 11 G565V developing a sole trisomy 13 at relapse: a clinical dilemma.

We describe a patient with acute myeloid leukemia (AML) who had a normal karyotype at diagnosis and was negative for NPM1 and FLT3 mutations, but had a KIT G565V mutation in exon 11. This has not been described previously in AML. The patient received induction and consolidation chemotherapy and was in hematologic remission for 351 days when deletion 7q was cytogenetically detected in 8% of the bone marrow cells. After an initial treatment of azacitidine followed by decitabine, an unrelated trisomy 13 clone was identified, followed by subclonal rearrangement of ETV6. The patient underwent reinduction with high-dose cytarabine and mitoxantrone followed by voluntary-unrelated-donor allogeneic stem cell transplantation with a reduced-intensity conditioning. As of writing, the patient is in complete hematologic and cytogenetic remission with 100% donor cell engraftment.