Lymphoma and plasma cell neoplasms
T / NK cell disorders
Anaplastic large cell lymphoma, ALK negative

Author: Dragos Luca, M.D. (see Authors page)

Revised: 23 March 2017, last major update September 2011

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PubMed search: anaplastic large cell lymphoma [title] ALK negative

Cite this page: Anaplastic large cell lymphoma, ALK negative. PathologyOutlines.com website. http://pathologyoutlines.com/topic/lymphomanonBanaplasticneg.html. Accessed September 22nd, 2017.
Definition / general
  • ALK negative anaplastic large cell lymphoma (ALCL, ALK-) is included as a provisional entity, and is defined as a CD30+ T cell neoplasm that is not reproducibly distinguishable on morphologic grounds from ALK+ ALCL, but lacks ALK protein (WHO 2008)
Treatment
  • Previously included in the broader category of anaplastic large cell lymphoma
Epidemiology
  • Most frequently 40 - 65 years old
  • Male predominance (M:F ratio 1.5:1)
  • Most epidemiologic studies do not separate ALK+ from ALK- neoplasms
Sites
  • Frequent involvement of both lymph nodes and extranodal sites
  • Usually nodal; extranodal involvement in skin, bone, soft tissue, GI tract
Etiology
  • Uncertain, some suggest it may be the final stage of histologic progression for a number of T cell lymphomas
Clinical features
  • Presentation with advanced stage III - IV disease in most patients
  • Peripheral or abdominal lymphadenopathy, B symptoms, high International Prognostic Index
Case reports
  • ALK negative anaplastic large cell lymphoma with extensive peripheral blood and bone marrow involvements (Leuk Res 2010;34:475)
Treatment
Postulated normal counterpart
  • Activated mature cytotoxic T cell
Microscopic (histologic) description
  • Generally effaced architecture by solid, cohesive sheets of neoplastic cells
  • Intrasinusoidal infiltrate or within T cell areas if architecture is partially preserved (mimics metastatic carcinoma)
  • Large pleomorphic cells, occasional prominent nucleoli, may have multinucleated, wreath-like and "hallmark" cells, higher N / C ratio than ALCL, ALK+
  • May have sclerosis and eosinophilia
  • Similar morphologic variants compared to ALCL, ALK+, except for small cell
Microscopic (histologic) images

Images hosted on other servers:

Contributed by Dr. Doan Minh Khuy, Hanoi Medical University, Hanoi, Vietnam:

23 year old man with a cervical lymphoid nodule, no B symptoms


CK-

EMA+

CD30+

ALK-


CD15-

CD20-

CD5+

Cytology description
  • Deeply basophilic cytoplasm, prominent vacuoles, round or lobate nuclei, prominent nucleoli, clumped chromatin, multinucleation
Positive stains
  • Strong and diffuse uniform CD30 staining in all tumor cells (membrane and Golgi zone pattern, also cytoplasmic)
  • Variable expression / loss of pan-T cell antigens: CD2+ and CD3+ more often than CD5+
  • Almost always CD43+
  • Often CD4+, rarely CD8+
  • TIA1, granzyme B, perforin, clusterin, fascin, rarely EMA
Negative stains
Molecular / cytogenetics description
  • T cell receptor (TCR) gene rearrangement in most cases, irrespective of T cell antigen expression
  • No recurrent cytogenetic abnormality
Differential diagnosis