Anaplastic Large-Cell Lymphoma, T-/Null-Cell Type

Abstract

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 ...

An unusual patient with shortness of breath—Clinical, radiologic, and pathologic pitfalls

“…Another CD30-immunoreactive lymphoma lacking significant inflammatory background is a T/null cell anaplastic lymphoma [16]. Our case highlights the utility of PAX5 in establishing a B-cell lineage [17] in cases lacking standard T and B markers.…”

Linfoma anaplásico de células grandes: estudio clínico-patológico e inmunohistoquímica de 20 casos clasificados de acuerdo a la revisión de la Organización Mundial de la Salud de neoplasias linfoides de 2016

“…El LACG es un linfoma de alto grado que fue descrito por el grupo de Karl Lennert en 1985 1 , quienes informaron de 45 casos de una neoplasia linfoide con morfología pleomórfi-ca que expresaba Ki-1 (BerH2 / CD30) 1,7 . El Ki-1, que había sido previamente descrito como selectivo para las células de Reed-Sternberg (R-S), también se expresaba en este tipo de linfomas, que presentaban características morfológicas semejantes a histiocitos o a células epiteliales, con pleomorfismo nuclear acentuado, afección a ganglios linfáticos predominante en la zona paracortical con focos de necrosis y diseminación sinusoidal 1,8 2,5 .…”

“…La forma cutánea corresponde a la categoría conocida como «alteración linfoproliferativa CD30+ cutánea», que incluye, por un lado del espectro, la papulosis linfomatoide y, por el otro, el LACG primario cutáneo 12 . La forma sistémica es un linfoma agresivo en que clínicamente los pacientes se presentan en estadios avanzados de la enfermedad (estadios III o IV) y principalmente afecta a los ganglios linfáticos, pero también puede afectar al hueso, la piel, los tejidos blandos y el pulmón 7 . La OMS en la última revisión de la clasificación de neoplasias linfoides de 2016 incluye en el LACG sistémico tres variantes: LACG ALK-1+, LACG ALK-1-y LACG ALK-1-asociado a implantes mamarios 6 .…”

Anaplastic large cell lymphoma: clinicopathological and immunohistochemical study of 20 cases classified according to the 2016 revision of the World Health Organization classification of lymphoid neoplasms

“…ALCL is a high grade lymphoma that was described by Karl Lennert's group in 1985 1 , who reported 45 cases of a lymphoid neoplasm with pleomorphic morphology and that expressed Ki-1 (BerH2/CD30) 1,7 . Ki-1, which was previously described as being selective for Reed-Sternberg (R-S) cells, was also expressed in this type of lymphoma, which had morphological features that were similar to those of histiocytes or epithelial cells, with accentuated nuclear pleomorphism, lymph node involvement predominantly at the paracortical zone, with necrosis foci and sinusoidal dissemination 1,8 .…”

“…The cutaneous form corresponds to the category known as "CD30+ lymphoproliferative cutaneous disorder", which includes, on one side of the spectrum, lymphomatoid papulosis, and, on the other, primary cutaneous ALCL 12 . The systemic form is an aggressive lymphoma where, clinically, patients are present with advanced stages of the disease (stages III or IV), and it involves mainly the lymph nodes, but it can compromise the bone, the skin, soft tissues and lungs as well 7 . The last revision in 2016 of the World Health Organization (WHO) classification of lymphoid neoplasms includes three variants in systemic ALCL: ALK-1+ ALCL, ALK-1-ALCL and breast implant-associated ALK-1-ALCL 6 .…”