RESUMEN
Systemic immunoglobulin light chain (AL) amyloidosis is a rare but fatal disease. It results from clonal proliferation of plasma cells with excessive production of insoluble misfolded proteins that aggregate in the extracellular matrix, causing damage to the normal architecture and function of various organs. For decades, treatment for AL amyloidosis was based mainly on therapeutic agents previously studied for its more common counterpart, multiple myeloma. As the prevalence and incidence of AL amyloidosis have increased, ongoing research has been conducted with treatments typically used in myeloma with varying success. In this review, we focus on current treatment strategies and updates to clinical guidelines and therapeutics for AL amyloidosis.
RESUMEN
BACKGROUND: Pembrolizumab is an anti-programmed death receptor 1 (PD-1) that was shown to have a tolerable safety profile with 17% of grade 3-4 drug-related adverse events, notable response rate of 16% with median duration of response of 8 mo, and median overall survival of 8 mo. Severe mucositis is a very rare complication with only two cases of grade 4 mucositis reported, and both cases had good response to intravenous methylprednisolone and subsequent oral prednisone tapering. We report the first case of pembrolizumab-induced severe mucositis that was refractory to steroid treatment. CASE SUMMARY: An 80-year-old woman with a past medical history of recurrent right cheek nodular melanoma status post resection and new right lung metastatic melanoma on immunotherapy presented with dysphagia and odynophagia for 2 mo. She initially received 2 doses of ipilimumab 1 year ago with good outcome, but treatment was discontinued after developing severe diarrhea and rash. Pembrolizumab was then initiated 4 mo after disease progression. Significant improvement was noted after 3 doses. However, after 6 cycles of pembrolizumab, patient developed odynophagia and malnutrition. Improvement of symptoms was noted after discontinuation of pembrolizumab and initiation of steroids. 3 mo later, patient developed pharyngeal swelling with hoarseness and new oxygen requirement due to impending airway obstruction while being on prednisone tapering regimen, finally ended up with intubation and tracheostomy. Histologic analysis of left laryngeal and epiglottis tissue showed granulation tissue with acute on chronic inflammation, negative for malignancy and infection. Patient achieved marked improvement after 2 doses of infliximab of 5 mg/kg every 2 wk while continuing on prednisone tapering course. CONCLUSION: We report the first case of pembrolizumab-induced grade 4 mucositis that had limited recovery with prolonged steroid course but had rapid response with addition of infliximab. The patient had recurrent mucositis symptoms whenever steroids was tapered but achieved complete response after receiving two doses of infliximab while continuing to be on tapering steroids. The success of infliximab in this patient with pembrolizumab-induced severe mucositis presents a potentially safe approach to reduce prolonged steroid course and accelerate recovery in managing this rare complication.
RESUMEN
Bladder cancer (BC) is a relatively common tumor, with a male preponderance. High-grade muscle invasive bladder cancer (MIBC) has a very high incidence of pelvic lymph node metastasis at presentation. Involvement of the retro-crural lymph nodes, although has been described in other pelvic tumors, is very uncommon for BC. Cryoablation in the retro-crural region is extremely challenging due to the proximity to the critical structures like inferior venacava and aorta and has not been extensively reported. We describe a 56-year old male patient with MIBC who underwent extensive treatments including radical cystoprostatectomy, chemoradiation and immunotherapy, ultimately with localized disease in the retro-crural region. Single session cryoablation of these lymph nodes was performed with a curative intent yielding a positive response that has persisted for more than 2 years.
RESUMEN
BACKGROUND: Transfusion-associated hyperkalemic cardiac arrest is a serious complication in patients receiving packed red blood cell (PRBC) transfusions. Mortality from hyperkalemia increases with large volumes of PRBC transfusion, increased rate of transfusion, and the use of stored PRBCs. Theoretically, hyperkalemia may be complicated by low cardiac output, acidosis, hyperglycemia, hypocalcemia, and hypothermia. In this study, we focus on transfusion-related hyperkalemia involving only medical intensive care unit (MICU) patients. METHOD: This prospective observational study focuses on PRBC transfusions among MICU patients greater than 18 years of age. Factors considered during each transfusion included patient's diagnosis, indication for transfusion, medical co-morbidities, acid-base disorders, K(+) levels before and after each PRBC transfusion, age of stored blood, volume and rate of transfusion, and other adverse events. We used Pearson correlation and multivariate analysis for each factor listed above and performed a logistic regression analysis. RESULTS: Between June 2011 and December 2011, 125 patients received a total of 160 units of PRBCs. Median age was 63 years (22 - 92 years). Seventy-one (57%) were females. Sixty-three patients (50%) had metabolic acidosis, 75 (60%) had acute renal failure (ARF), and 12 (10%) had end-stage renal disease (ESRD). Indications for transfusion included septic shock (n = 65, 52%), acute blood loss (n = 25, 20%), non-ST elevation myocardial infarction (NSTEMI) (n = 25, 20%) and preparation for procedures (n = 14, 11%). Baseline K(+) value was 3.9 ± 1.1 mEq/L compared to 4.3 ± 1.2 mEq/L post-transfusion respectively (P = 0.9). During this study period, 4% of patients developed hyperkalemia (K(+) 5.5 mEq/L or above). The mean change of serum potassium in patients receiving transfusion ≥ 12 days old blood was 4.1 ± 0.4 mEq/L compared to 4.8 ± 0.3 mEq/L (mean ± SD) in patients receiving blood 12 days or less old. Sixty-two patients (77.5%) that were transfused stored blood (for more than 12 days) had increased serum K(+); eight (17.7%) patients received blood that was stored for less than 12 days. In both univariate (P = 0.02) and multivariate (P = 0.04) analysis, findings showed that among all factors, transfusion of stored blood was the only factor that affected serum potassium levels (95% CI: 0.32 - 0.91). No difference was found between central and peripheral intravenous access (P = 0.12), acidosis (P = 0.12), ARF (P = 0.6), ESRD (P = 0.5), and multiple transfusions (P = 0.09). One subject developed a sustained cardiac arrest after developing severe hyperkalemia (K(+) = 9.0) following transfusion of seven units of PRBCs. Multivariate logistic regression showed linear correlation between duration of stored blood and serum K(+) (R(2) = 0.889). CONCLUSION: This study assesses factors that affect K(+) in patients admitted to MICU. Results from the study show that rise in serum K(+) level is more pronounced in patients who receive stored blood (> 12 days). Future studies should focus on the use of altered storage solution, inclusion of potassium absorption filters during transfusion and cautious use of blood warmer in patients requiring massive blood transfusions.
