Blurry Vision as a Presentation of Waldenström's Macroglobulinemia: A Case Report With Review of Current Management.

A patient was diagnosed with Waldenström's macroglobulinemia (WM) after the initial findings of anemia and ophthalmological findings of retinal hemorrhage. Upon further workup, the patient was found to have an IgM predominant monoclonal gammopathy on serum protein electrophoresis (SPEP) and urine protein electrophoresis (UPEP). This highlights the need for open communication between different specialties, streamlining rapid and accurate diagnosis. Also highlighted are the unique pathophysiological changes involved in the development of WM. A patient's primary complaint was blurry vision. After the patient was noted to have a monoclonal gammopathy on SPEP, bone marrow biopsy was performed. The bone marrow biopsy findings were consistent with lymphoplasmacytic lymphoma (LPL). The patient received plasmapheresis and chemotherapy. The disease course is described. The patient saw rapid improvement in all lab abnormalities after the beginning of the appropriate therapy of plasmapheresis and chemotherapy. Remission is common with WM. Regular follow-up with this patient is important.

Three-year survival, correlates and salvage therapies in patients receiving first-line pembrolizumab for advanced Merkel cell carcinoma.

BACKGROUND: Merkel cell carcinoma (MCC) is an aggressive skin cancer associated with poor survival. Programmed cell death-1 (PD-1) pathway inhibitors have shown high rates of durable tumor regression compared with chemotherapy for MCC. The current study was undertaken to assess baseline and on-treatment factors associated with MCC regression and 3-year survival, and to explore the effects of salvage therapies in patients experiencing initial non-response or tumor progression after response or stable disease following first-line pembrolizumab therapy on Cancer Immunotherapy Trials Network-09/KEYNOTE-017. METHODS: In this multicenter phase II trial, 50 patients with advanced unresectable MCC received pembrolizumab 2 mg/kg every 3 weeks for ≤2 years. Patients were followed for a median of 31.8 months. RESULTS: Overall response rate to pembrolizumab was 58% (complete response 30%+partial response 28%; 95% CI 43.2 to 71.8). Among 29 responders, the median response duration was not reached (NR) at 3 years (range 1.0+ to 51.8+ months). Median progression-free survival (PFS) was 16.8 months (95% CI 4.6 to 43.4) and the 3-year PFS was 39.1%. Median OS was NR; the 3-year OS was 59.4% for all patients and 89.5% for responders. Baseline Eastern Cooperative Oncology Group performance status of 0, greater per cent tumor reduction, completion of 2 years of treatment and low neutrophil-to-lymphocyte ratio were associated with response and longer survival. Among patients with initial disease progression or those who developed progression after response or stable disease, some had extended survival with subsequent treatments including chemotherapies and immunotherapies. CONCLUSIONS: This study represents the longest available follow-up from any first-line anti-programmed death-(ligand) 1 (anti-PD-(L)1) therapy in MCC, confirming durable PFS and OS in a proportion of patients. After initial tumor progression or relapse following response, some patients receiving salvage therapies survived. Improving the management of anti-PD-(L)1-refractory MCC remains a challenge and a high priority. TRIAL REGISTRATION NUMBER: NCT02267603.

Durable Tumor Regression and Overall Survival in Patients With Advanced Merkel Cell Carcinoma Receiving Pembrolizumab as First-Line Therapy.

PURPOSE: Merkel cell carcinoma (MCC) is an aggressive skin cancer often caused by the Merkel cell polyomavirus. Clinical trials of programmed cell death-1 pathway inhibitors for advanced MCC (aMCC) demonstrate increased progression-free survival (PFS) compared with historical chemotherapy data. However, response durability and overall survival (OS) data are limited. PATIENTS AND METHODS: In this multicenter phase II trial (Cancer Immunotherapy Trials Network-09/Keynote-017), 50 adults naïve to systemic therapy for aMCC received pembrolizumab (2 mg/kg every 3 weeks) for up to 2 years. Radiographic responses were assessed centrally per Response Evaluation Criteria in Solid Tumors (RECIST) v1.1. RESULTS: Among 50 patients, the median age was 70.5 years, and 64% had Merkel cell polyomavirus-positive tumors. The objective response rate (ORR) to pembrolizumab was 56% (complete response [24%] plus partial response [32%]; 95% CI, 41.3% to 70.0%), with ORRs of 59% in virus-positive and 53% in virus-negative tumors. Median follow-up time was 14.9 months (range, 0.4 to 36.4+ months). Among 28 responders, median response duration was not reached (range, 5.9 to 34.5+ months). The 24-month PFS rate was 48.3%, and median PFS time was 16.8 months (95% CI, 4.6 months to not estimable). The 24-month OS rate was 68.7%, and median OS time was not reached. Although tumor viral status did not correlate with ORR, PFS, or OS, there was a trend toward improved PFS and OS in patients with programmed death ligand-1-positive tumors. Grade 3 or greater treatment-related adverse events occurred in 14 (28%) of 50 patients and led to treatment discontinuation in seven (14%) of 50 patients, including one treatment-related death. CONCLUSION: Here, we present the longest observation to date of patients with aMCC receiving first-line anti-programmed cell death-1 therapy. Pembrolizumab demonstrated durable tumor control, a generally manageable safety profile, and favorable OS compared with historical data from patients treated with first-line chemotherapy.

PSA flare after initiation of abiraterone acetate.

Prostate cancer is the second-most common cause of male cancer-related death among US men. The American Cancer Society estimates that in 2014 there will be about 233,000 new cases of prostate cancer and about 29,500 deaths due to prostate cancer. Sixty percent of cases are diagnosed in men aged 65 years or older. Localized prostate cancers are managed with active surveillance or external beam radiation therapy or radical prostatectomy after risk stratification. Advanced prostate cancers are managed with hormonal therapy for castrate sensitive prostate cancer and with novel androgen blocking therapies, chemotherapy, or radio-immunotherapy for castrate resistant prostate cancers. Prostate-specific antigen (PSA) flare is characterized by a rise in the PSA level, followed by a decline to below baseline values after starting therapies such as androgen deprivation therapy, systemic chemotherapy, or local therapies such as brachytherapy or cryotherapy.

Hemoglobin SO-Arab and α-thalassemia diagnosed in an adult: A case-based review of the hemoglobinopathies.

Hemoglobin SO-Arab is a rare sickling disorder with a clinical course similar to that of hemoglobin SS. Hemoglobin C-Harlem is another rare condition that produces sickling disorders in affected individuals with a disease course and electrophoretic findings similar to that of hemoglobin SO-Arab. The authors report the case of a 38-year-old African American man with hemoglobin SO-Arab and the challenges that may arise in working up a rare hemoglobinopathy.

A paradigm shift in the treatment of advanced non-small cell lung cancer.

Lung cancer is the most common cause of cancer death for men and women worldwide. Nonsmall cell lung cancer (NSCLC) accounts for approximately 85% of all lung cancers. Systemic chemotherapy is a cornerstone of treatment in the management of stage IV NSCLC. First-line chemotherapy typically consists of a platinum-based treatment. The optimum approach to long-term treatment beyond 4 to 6 cycles of chemotherapy is still evolving. Second-line chemotherapy given at the time of disease progression has shown clear survival advantages when compared with best supportive care alone. With the recent increase in the number of active and more tolerable agents available to treat NSCLC, there is renewed interest in the role of continuation of antineoplastic agents (continuation maintenance) or switching to a different agent (switch maintenance) after first-line chemotherapy. This case-based review discusses the role of maintenance chemotherapy in the long-term management of advanced NSCLC.

Restoration of medical oncology services at LSU Interim Public Hospital in New Orleans after Hurricane Katrina: a two-year experience of LSUHSC.

BACKGROUND: Oncology services at Charity Hospital were discontinued following Hurricane Katrina in August 2005. Medical oncology and chemotherapy services resumed at the Louisiana State University Interim Public Hospital in 2007. Demographic, clinical, and displacement data of the re-established patient cohort were reviewed. METHODS: Patients evaluated in the Louisiana State University Health Sciences Center (LSUHSC) Oncology Clinics from September 1, 2007, to August 31, 2009, were identified and data collected included time from diagnosis of malignancy to initial oncology evaluation, insurance status, percentage displaced for six months or more due to Hurricane Katrina, ethnicity, referrals for radiation oncology, and the number of outpatient clinical encounters (OCE). RESULTS: 464 patients were evaluated in the study time period. Sixty-five percent of the patients had new cancer diagnoses and 35% re-established cancer care in the Charity System and a substantial proportion were either unfunded or had Medicaid coverage. Thirty-four percent were confirmed to be displaced from New Orleans for greater than six months and the majority of patients were black. The majority of new cancer diagnoses were breast, lung, and colon cancer. Human immunodeficiency virus (HIV) positive patients made up 7.5% of the patient cohort. There was a 70% decline in patient volumes following Hurricane Katrina. CONCLUSIONS: Oncology services for a minority-based, underinsured patient population were severely impacted by Hurricane Katrina. Following the storm, persistent systemwide resource limitations led to suboptimal timeliness of medical oncology evaluations. Health care systems serving underinsured patients require a disaster plan to minimize interruption of oncology care. Our experience illustrates the need for resources to ensure rapid re-establishment of care for economically disadvantaged patients following natural disasters.

Adjuvant high-dose interferon-{alpha} for resected melanoma in a patient with HIV infection.

Adjuvant interferon (IFN)-alpha remains the standard adjuvant therapy for intermediate and high-risk melanoma after definitive surgical resection. Data addressing the role and safety of adjuvant immunotherapy in HIV-infected patients with melanoma are lacking. We report on an HIV(+) patient who received IFN-alpha as adjuvant treatment for high-risk melanoma. To our knowledge, this is the first reported case of such an approach.

A 32-year-old woman with chest pain, hematemesis, and thrombocytopenia.

The diagnosis of TTP should be considered in any clinical scenario in which hemolysis and thrombocytopenia are present. If other secondary causes are ruled out, therapy should instituted as rapidly as possible. TPE remains the standard of care for TTP. In emergent situations where TPE is not immediately available, infusion of FFP at 15 mL/kg per day and initiation of glucocorticoids is acceptable.

Definitive altered fractionation radiotherapy and concomitant weekly cisplatin for locally advanced head and neck cancer.

BACKGROUND: The purpose of this study was to determine the efficacy and toxicities of single-agent weekly cisplatin for patients with squamous cell carcinoma of the head and neck treated definitively with radiation therapy (RT). METHODS: Thirty-five patients with American Joint Committee of Cancer stage II (3%), stage III (14%), or stage IV (83%) squamous cell carcinoma of the oropharynx, larynx, or hypopharynx treated from June 2000 to November 2003 at the University of Florida were retrospectively reviewed. Subjects received radiation therapy (RT; median, 74.4 Gy) and cisplatin, 30 mg/m2/wk. Altered fractionation was used in 34 of 35 (97%) patients. The endpoints were best response, percentage of grade III or IV toxicities, local-regional control, disease-free survival, cause-specific survival, and overall survival. RESULTS: The median number of cycles of cisplatin administered was 6. Grade III or IV toxicities were: anemia, 11%; thrombocytopenia, 6%; leukopenia, 26%; and mucositis, 23%. No patients had renal failure and 1 patient (3%) died because of therapy-related complications. Responses to therapy included 71% complete response, 17% partial response, and 6% stable disease. Median follow-up for all patients was 1.8 years (range, 0.1-7.8 years); median follow-up for living patients was 4.4 years (range, 2.6-7.8 years). The 3-year outcomes were: local-regional control, 85%; disease-free survival, 56%; cause-specific survival, 59%; and overall survival, 40%. CONCLUSION: Concomitant weekly CDDP with definitive RT is feasible, tolerable, highly active, and comparable with more complex, costly, and toxic regimens. Intercurrent disease was a significant contributor to mortality in our population. Our regimen is an attractive alternative to sequential chemoradiotherapy programs.