Anaplastic large cell lymphoma classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [2]

Overview

Anaplastic large cell lymphoma may be classified into 2 subtypes: anaplastic large cell lymphoma, ALK positive and anaplastic large cell lymphoma, ALK negative. Based on clinical presentations, anaplastic large cell lymphoma may be classified into either primary cutaneous anaplastic large cell lymphoma and primary systemic anaplastic large cell lymphoma.

Classification

• Anaplastic large cell lymphoma (peripheral T-cell lymphoma Non-Hodgkin Lymphoma) may be classified into 2 subtypes:

• Anaplastic large cell lymphoma, ALK positive
• Anaplastic large cell lymphoma, ALK negative

• Based on clinical presentations, anaplastic large cell lymphoma may be classified into either

• Primary cutaneous anaplastic large cell lymphoma
• Primary systemic anaplastic large cell lymphoma

• Based on morphology, anaplastic large cell lymphoma may be classified into 3 subtypes:

• Classical Variants
• Atypical Variants
• Rare Variants

Classification based on the clinical presentation

Name	Description
Primary cutaneous anaplastic large cell lymphoma	Confined to the skin Usually have a single lump or tumor in the skin May also spread to lymph nodes in the area Associated with a rare skin condition called lymphomatoid papulosis Less aggressive than primary systemic anaplastic large cell lymphoma Occasionally individuals have a spontaneous remission Have a fairly good prognosis
Primary systemic anaplastic large cell lymphoma	Usually involves the lymph nodes Can also occur in organs or tissues other than the lymph nodes (extranodal sites), including: lungs, liver, bone marrow, bone, gastrointestinal tract, skin and soft tissue Most individuals have advanced stage (stage III or IV) disease when they are diagnosed Usually a fast-growing (aggressive) lymphoma

Morphologic Classification

Name	Description
Classical Variants
Common pattern	ALK positive anaplastic large cell lymphoma
Atypical Variants
Small cell	ALK positive anaplastic large cell lymphoma
Lymphohistiocytic	ALK positive anaplastic large cell lymphoma
Giant cell	ALK positive anaplastic large cell lymphoma
Hodgkin's like	ALK positive anaplastic large cell lymphoma
Rare Variants
Sarcomatoid	ALK positive anaplastic large cell lymphoma