Allogeneic Hematopoietic Stem Cell Transplantation in Advanced Stage Mycosis Fungoides and Sézary Syndrome

A Concise Review

William T. Johnson; Reetu Mukherji; Saritha Kartan; Neda Nikbakht; Pierluigi Porcu; Onder Alpdogan

Disclosures

Chin Clin Oncol. 2019;8(1) 

In This Article

Abstract and Introduction

Abstract

Mycosis fungoides and Sézary syndrome encompass over 70% of all cases of cutaneous T-cell lymphoma (CTCL). While early stage disease has excellent long-term survival rates, advanced stage disease (IIB–IV) carries a poor prognosis with a median 5-year overall survival rate of approximately 50%. Early stage and advanced stage disease have different treatment algorithms with systemic therapy being indicated upfront in the later. The role of allogeneic hematopoietic stem cell transplant (HSCT) has gained considerable interest in recent years as a treatment option for CTCL given the increasingly promising long-term outcomes in an otherwise incurable disease. Herein, we provide a brief update on the current advances in HSCT followed by a concise review of the role of HSCT for CTCL. We conclude with our recommendations to approaching HSCT as a curative treatment option for CTCL.

Introduction

Cutaneous T-cell lymphomas (CTCL) are a heterogeneous group of non-Hodgkin lymphoma (NHL) that originate from skin-homing or skin-resident T-cells. Mycosis fungoides (MF) and Sézary syndrome (SS) comprise the majority of cases accounting for 70–75% of all newly diagnosed CTCL with the median age at diagnosis of 55–60 years.[1,2] Staging of MF and SS through the TNMB (tumor, node, metastasis, blood) staging system continues to be the most important prognostic tool,[3,4] and approximately 70% of cases present with early stage (IA–IIA) disease.[4,5] Stage IA and IB disease are associated with 5-year overall survival (OS) rates of 96–99% and 75–86%, respectively.[4,5] Treatment options for early stage disease include expectant management or skin directed therapies such as topical steroids, topical nitrogen mustards, light therapy and radiation.[6] Advanced stage CTCL carries with it a much less favorable prognosis and often warrants systemic therapy.[6] In a recently published series of 1,275 patients with advanced disease (stage IIB or greater), the median OS was just 63 months, with 2 and 5-year survival rates of 77% and 52%, respectively. Median survival for stage IIB disease was 68 months, 48 months for stage IVA, and 33 months for stage IVB.[7] Treatment options include, but are not limited to bexarotene, vorinostat, romidepsin and most recently, brentuximab vedotin (for CD30+ MF) and mogamulizumab-kpkc. Unfortunately, for these agents and others the overall response rates (ORRs) are averaging only 30–40% with limited durations of response. Excellent reviews on systemic therapies are widely available,[6,8,9] but beyond the scope of this review (it is discussed in detail in this issue's review of systemic chemotherapy of CTCL by Dr. Alpdogan). Several other systemic therapies are recommended by the National Comprehensive Cancer Network (NCCN), the European Organization for Research and Treatment of Cancer (EORTC), and other cutaneous lymphoma consortiums. Similarly, the NCCN and the EORTC as well as the American Society of Blood and Marrow Transplantation (ASBMT) list allogeneic hematopoietic stem cell transplant (HSCT) as an option for advanced stage CTCL, which as of this writing remains the only potential curative treatment option.[9–11]

In this review we first discuss the advancements HSCT transplantation has made in recent years. We then focus on the role of HSCT in advanced CTCL. To date there remains no randomized published control trials of HSCT versus systemic treatment; thus, this review is limited to published data consisting of small case series and retrospective analyses. We will conclude this review with our recommendations.

Comments

3090D553-9492-4563-8681-AD288FA52ACE

processing....