Anaplastic large-cell lymphoma, T-/null-cell type (ALCL), is a rare disease that has only been well characterized for two decades. Despite this, the biology of ALCL is better understood than that of many other more common variants of lymphoma. This review focuses on the pathophysiology, clinical presentation, and therapy of ALCL, including stem cell transplantation. In particular, the text emphasizes how novel prognostic features and the evolving understanding of the biology of this disease will influence treatment selection and drug development. The Oncologist 2006;11:831-840
Epidemiology and Clinical PresentationAnaplastic large-cell lymphoma, T-/null-cell type (ALCL), is a rare disease, accounting for <5% of all cases of nonHodgkin's lymphoma (NHL) [1]. ALCL was first described as a clinical entity in 1985 based upon its unique characteristic of cohesive proliferation of large pleomorphic cells expressing CD 30 (Ki-1) [2]. ALCL has a peak incidence in childhood and accounts for approximately 40% of NHL cases diagnosed in pediatric populations [3]. There appears to be a male predominance, particularly in anaplastic lymphoma kinase (ALK) + cases, in which the male/female ratio is approximately 3:1. In one series, the male predominance of ALK + cases was especially striking in patients under the age of 30, with a male/female ratio of 6.5:1 [4].There are no clear risk factors for developing ALCL. Some reports have suggested that Epstein-Barr virus (EBV) is important in the pathogenesis of ALCL. However, a recent series of 64 ALCL cases revealed no EBVencoded RNA (EBER) or immunohistochemistry evidence for EBV-latent membrane protein type 1 [5]. The authors concluded that previous reports of EBV in Western patients with ALCL were probably a result of the inclusion of tumors no longer considered to be ALCL, such as CD30 + anaplastic tumors of B-cell origin. EBV, however, may be important in the pathogenesis of ALCL occurring in Asia and in immune-compromised hosts [6, 7].ALCL occurs as two distinct clinical entities, a cutaneous and a systemic variant [8]. Cutaneous ALCL falls under
Learning ObjectivesAfter completing this course, the reader will be able to:1. Identify areas of diagnostic confusion in anaplastic large-cell lymphoma (ALCL).2. Describe risk stratification in ALCL.
Discuss therapeutic options in ALCL.Access and take the CME test online and receive 1 AMA PRA Category 1 Credit ™ at CME.TheOncologist.com CME CME + lymphoproliferative disorders of the skin that represents a spectrum of disease ranging from the benign lymphomatoid papulosis (LP) to the malignant primary cutaneous ALCL (PCALCL) [9]. Both PCALCL and LP generally occur in older adults and are rare in children [10]. LP resembles lymphoma and is essentially indistinguishable from PCALCL histologically [11]. Thus the distinction between the two disorders is largely clinical.Patients with LP tend to have more diffuse lesions, whereas PCALCL is more likely to be solitary [12]. LP lesions are generally <3 cm, whereas PCALCL lesions are ...