Abstract
ABSTRACTAnaplastic lymphoma kinase negative anaplastic large cell lymphoma (ALK- ALCL) is a definite entity in the WHO 2016 Classification that represents 2–3% of non-Hodgkin lymphoma (NHL) and 12% of T-cell NHL cases. ALK- ALCL lacks ALK protein expression, but expresses CD30 and has morphologic features similar to ALK positive anaplastic large cell lymphoma (ALK+ ALCL). Some studies indicate that ALK- ALCL and ALK+ ALCL possess different molecular and genetic characteristics. Besides, ALK- ALCL is worse than ALK+ ALCL in terms of treatment outcome, prognosis, and long-term survival. This review is aimed at summarizing information about ALK- ALCL, especially with respect to the treatment and prognosis.
Highlights
Anaplastic large cell lymphoma (ALCL) is a rare and heterogeneous malignant tumor, with high expression of CD30 (Ki-1) and large cell proliferation
This study suggested that CD30+ PTCL patients treated with bentuximab vedotin followed by CHOP or combined with CHP had good safety and anti-tumor activity
The molecular biology, clinical manifestation, treatment and prognosis of ALK- ALCL are summarized, which is a definite entity in the WHO 2016 Classification
Summary
Anaplastic large cell lymphoma (ALCL) is a rare and heterogeneous malignant tumor, with high expression of CD30 (Ki-1) and large cell proliferation. It was named ALCL after Stein et al [1]. According to the expression state of ALK protein, ALCL is classified into ALK+ ALCL and ALKALCL. The WHO 2008 classification recognized 3 ALCL entities: ALK+ ALCL, ALK- ALCL (a provisional entity), and primary cutaneous ALCL (pcALCL) [3]. ALK- ALCL was recognized as a definite entity in the WHO 2016 Classification; the breast implant-associated ALCL (BIA ALCL) was newly proposed [4]
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