Systemic therapy for cutaneous T-cell lymphoma: who, when, what, and why?

INTRODUCTION: CTCL are rare neoplasms. Optimal care requires integrated use of diagnostic and treatment modalities spanning multiple specialties. Current instruments for patient risk stratification and disease measurement across all anatomical compartments are suboptimal. A common treatment dichotomy between early (Dermatology) and advanced stage (Hematology-Oncology) has hindered accrual of long term outcome data. Thus, important facts about natural history, such as frequency and determinants of stage progression, and the impact of specific treatment modalities on these endpoints, are not known. Areas covered: One of the most important decisions in the management of CTCL is when to start systemic therapy and what agents to use. This review provides background information to understand why systemic therapy is needed and what goals are currently achievable. Expert commentary: Risk-adapted approaches, based on better knowledge of host and tumor biology, and more accurate disease measurement tools are needed to optimize the use of specific systemic therapies.

Sézary Syndrome: Clinical and Biological Aspects.

Sézary syndrome (SS) is a rare and aggressive type of cutaneous T cell lymphoma (CTCL) characterized by an intensely pruritic, exfoliative rash, known as erythroderma, with cutaneous and systemic dissemination of clonal CD4+ T cells into the blood and lymph nodes. This review aims to present recent advancements in the biological and clinical aspects of SS. We begin by providing an overview of the diagnostic criteria for SS and reviewing some of its epidemiological and clinical aspects. We then discuss updates in the etiology of this elusive disease and the genetic and molecular landscapes that define it. Finally, we provide a short overview of the current therapeutic strategies for SS as well as recent advances in the prognosis of this disease. A brief set of recommendations is provided regarding future directions in research and therapy.

Primary Cutaneous B-Cell Lymphoma: Management and Patterns of Recurrence at the Multimodality Cutaneous Lymphoma Clinic of The Ohio State University.

BACKGROUND: The increasing incidence of primary cutaneous B-cell lymphomas (PCBCLs) presents new challenges for clinicians. Despite advances in the clinical and pathologic characterization of PCBCL, the significance of the current staging approach as a risk profiling tool and the effect of various treatments on outcome remain unclear. MATERIALS AND METHODS: We retrospectively reviewed patients who presented with a diagnosis of PCBCL seen at The Ohio State University between 1998 and 2012. We reviewed the initial presentation and treatment modality. We then assessed whether the treatment modality (conservative skin-directed vs. definitive radiation with or without systemic therapy), stage (T1 or ≥T2), or histologic subtype (primary cutaneous follicle center lymphoma [PCFCL] vs. primary cutaneous marginal zone B-cell lymphoma [PCMZL]) affected the risk of recurrence. RESULTS: We identified 67 patients referred with an initial diagnosis of PCBCL. After imaging, 12 did not meet the criteria for PCBCL and were classified as having systemic B-cell lymphoma with cutaneous involvement. The remaining 55 patients included 25 with PCMZL, 24 with PCFCL, 2 with primary cutaneous large B-cell lymphoma leg type, and 4 with unclassifiable disease. According to the International Society of Cutaneous Lymphoma-European Organization for Research and Treatment of Cancer staging, 30 cases were T1 (55%), 14 T2 (25%), and 11 T3 (20%). Comparing the time to first recurrence (TFR) by indolent PCBCL subtypes, we found no difference in the recurrence risk for either stage (T1, p = .51 vs. T2/T3, p = .30). Comparing TFR by treatment modality, we found no difference in TFR within T1 patients (p = .34) or T2/T3 patients (p = .44). CONCLUSION: Our limited analysis highlights the importance of complete staging at diagnosis and suggests that the treatment modality does not affect the risk of recurrence in T1 indolent PCBCL.

The Role of an Integrated Multidisciplinary Clinic in the Management of Patients with Cutaneous Lymphoma.

The clinical benefit of a multidisciplinary clinic practice model has been well described in a variety of medical specialties and cancer types. It proves particularly valuable when an integrated team is needed to optimally manage patients with rare or complex neoplasms. However, the ideal implementation of an integrated multidisciplinary care program for translational research and education has not been well reported. Herein, we propose how a multimodality cutaneous lymphoma (CL) clinic model can optimally manage CL patients. We offer our perspective on this model as an efficient means for delivering patient care, a continuing education resource for referring physicians, a conduit for translational and clinical research, and an educational tool for medical students, house staff, and fellows.