Clinical characteristics and outcomes of black patients with mycosis fungoides and Sézary syndrome: a subgroup analysis of the phase III MAVORIC trial.

Treatment-specific responses and comprehensive disease characteristics are limited in black patients with cutaneous T-cell lymphoma (CTCL). These shortcomings prompted us to perform a subgroup analysis of black patients enrolled in the MAVORIC trial - an international, randomized, phase 3 trial comparing mogamulizumab vs. vorinostat in relapsed/refractory mycosis fungoides (MF) and Sézary syndrome (SS). Ten percent (N = 37) of the entire MAVORIC population (N = 372) identified as black. Significant clinical differences in black patients when compared to non-black patients included a younger median age at enrollment (53 vs. 66 years; p < 0.001), an increased frequency of MF as opposed to SS (73% vs. 52.8%; p < 0.001), and higher rates of earlier-stage disease (IB-IIA) at enrollment (37.8% vs. 21.2%; p = 0.022). Mogamulizumab offered similar response rates and progression-free survival in black patients (7.57 months) compared to the entire MAVORIC population (7.7 months) and was associated with a similar safety profile.

Implementation of an Outpatient HD-MTX Initiative.

INTRODUCTION: Methotrexate (MTX) a folate antagonist is often given in high doses (≥500 mg/m2) to treat a variety of disease processes. While inpatient administration has been the norm, outpatient administration, has been shown to be safe, effective, and patient centered. Here in we describe development of an outpatient HDMTX protocol and our initial experience. METHODS: All patients were to receive their first cycle of HDMTX in the hospital to ensure they tolerate it well and also to use this time to assist in training for home administration. The outpatient protocol involved continuous IV sodium bicarbonate, along with oral leucovorin and acetazolamide. Patients were required to visit the infusion center daily for labs and methotrexate levels. Clear criteria for admission were developed in the case of delayed clearance or methotrexate toxicity. RESULTS: Two patients completed the safety run-in phase. Both patients tolerated treatment well. There were no associated toxicity. Methotrexate cleared within 3 days for all cycles. Both patients were able to follow the preadmission instructions for sodium bicarbonate and acetazolamide. The patients reported adequate teaching on the protocol and were able to maintain frequency of urine dipstick checks. CONCLUSION: We developed and implemented an outpatient protocol for high dose methotrexate. This study largely details the development of this protocol and its initial safety evaluation. More work needs to be done to assess its feasibility on a larger number of patients who receive more cycles in the outpatient setting.

Extreme Peripheral Blood Plasmacytosis Mimicking Plasma Cell Leukemia as a Presenting Feature of Angioimmunoblastic T-Cell Lymphoma (AITL).

Angioimmunoblastic T-cell lymphoma (AITL) is one of four major subtypes of nodal peripheral T cell lymphoma, characterized by its cell of origin, the follicular helper T-cell (TFH). Patients typically present with prominent constitutional (B) symptoms, generalized lymphadenopathy, hepatosplenomegaly, cytopenias, and rash. Here we present a case of a 62-year-old male with progressive cervical adenopathy, fevers and weight loss presenting with extreme polyclonal plasmacytosis and high plasma EBV viral load. While the initial presentation appeared to mimic plasma cell leukemia or severe infection, lymph node biopsy and bone marrow biopsy confirmed a diagnosis of AITL. This case highlights the heterogeneity of the clinical presentation of AITL to enable physicians to more promptly recognize, diagnose and initiate treatment.

Systemic therapy of cutaneous T-cell lymphoma (CTCL).

Cutaneous T-cell lymphomas (CTCLs) are a heterogeneous and relatively rare family of extranodal non-Hodgkin's lymphomas (NHL) that are primarily localized to the skin. Most patients present with cutaneous patches, plaques, tumors, more rarely with erythroderma. The type of skin lesions and the surface extent of skin involvement, as well as the presence of extracutaneous disease, are the most important prognostic factors in patients with CTCL. Patients with early-stage disease can be effectively treated with skin-directed therapies only. As the disease progresses, systemic therapy often becomes necessary. In this review, we will provide an overview of the systemic treatment options for CTCL, including mechanism of action, efficacy, side effects, and discuss current principles for rational combination therapy and sequential use. Systemic therapy strategies have been evolving with the recent FDA approval of new monoclonal antibodies such as brentuximab vedotin and mogamulizumab to established drugs, such as retinoids, interferons (IFNs), and HDAC inhibitors. Numerous new agents are currently being studied in clinical trials. Identifying the genetic, molecular, and immunologic features of CTCL that are associated with disease progression, drug-resistance, and immune impairment, will optimize the utilization of existing therapies, and facilitate the development of new ones.

The spectrum of CD30+ T cell lymphoproliferative disorders in the skin.

Primary cutaneous CD30+ T cell lymphoproliferative disorders (pcCD30+ T cell LPDs) are a spectrum of pre-malignant to frankly neoplastic lymphoproliferations that comprise lymphomatoid papulosis (LyP), primary cutaneous anaplastic large cell lymphoma (pcALCL), and borderline lesions. Although the atypical T cells that are the hallmark of these disorders share the expression of CD30, as the identifying marker, the clinical presentation, histological features and clinical course are vastly different. Furthermore, histopathologic features of pcCD30+ T cell LPDs may overlap with other cutaneous and systemic lymphomas. While most pcCD30+ T cell LPDs have an excellent prognosis, systemic lymphomas typically have a poorer outcome. CD30 has now been shown to be a reliable therapeutic target in pcCD30+ T cell LPDs. This review will emphasize the structure and function of CD30, along with the clinical and pathological spectrum of pcCD30+ T cell LPDs. It will also highlight other CD30+ lymphoproliferations that must be differentiated by careful clinical and pathological correlation.

Implications of Mutation Profiling in Myeloid Malignancies-PART 2: Myeloproliferative Neoplasms and Other Myeloid Malignancies.

Myeloid malignancies arise from the acquisition of somatic mutations among various genes implicated in essential functioning of hematopoietic stem cells and progenitor cells. In this second part of our two-part review, we discuss the use of mutation profiling in the diagnosis, prognosis, and treatment of patients with myeloproliferative neoplasms and other myeloid diseases. We also discuss the entity known as clonal hematopoiesis of indeterminate potential, awareness of which is a result of the increasing availability and improved quality of mutation profiling.

Implications of Mutation Profiling in Myeloid Malignancies-PART 1: Myelodysplastic Syndromes and Acute Myeloid Leukemia.

The advent of high-throughput gene sequencing has revolutionized our understanding of the genetic mutations that drive myeloid malignancies. While these mutations are of interest pathobiologically, they are increasingly being recognized as clinically meaningful in providing diagnostic, prognostic, and therapeutic information to guide patient care. In this first part of our two-part review, we introduce mutation profiling as a relevant clinical tool for hematologists treating patients with myeloid malignancies. Next, we discuss the diagnostic and prognostic role of mutation profiling in myelodysplastic syndrome and acute myeloid leukemia. Finally, we detail the therapeutic implications of specific mutations in myelodysplastic syndrome and acute myeloid leukemia. In Part 2, we will discuss similar clinical approaches using mutation profiling in myeloproliferative neoplasms and other myeloid malignancies.

Masks for prevention of respiratory viruses on the BMT unit: results of a quality initiative.

Respiratory viral infections (RVI) cause significant morbidity and mortality in hospitalized oncology patients. These viruses are easily spread from asymptomatic and/or symptomatic healthcare workers and visitors to immunocompromised patients, and literature review of facemasks for prevention of infection revealed mixed results. The Bone Marrow Transplant (BMT) Quality Assurance (QA) Committee at Mount Sinai began a surgical mask initiative on the BMT unit. The purpose of our initiative was to assess the impact of surgical mask implementation for healthcare workers and visitors on nosocomial RVI in all patients hospitalized on the BMT unit. We hypothesized that implementing surgical masks would reduce the number of hospital-acquired RVI. We performed a retrospective study involving all patients with malignancy hospitalized on the BMT unit for 4 years. During the latter 2 years, all healthcare workers and visitors were required to wear a surgical mask in every patient room on the BMT unit. Primary endpoint was incidence of RVI after implementation of surgical masks. The 2-year incidence of RVI in the pre-mask period was 14 out of a total of 15 001 patient days on the unit vs 2 out of 15 608 patient days after mask implementation. The difference in incidence of RVI within the two time intervals was noted to be statistically significant (P<.05, 2-proportion z-test). Our quality initiative demonstrated that surgical masks are an infection control modality that may provide benefit to oncology/BMT units by decreasing the risk for hospital-acquired RVI.