Case 113 – Summary

Our patient was found to have systemic anaplastic lymphoma kinase (ALK) positive anaplastic large cell lymphoma (ALCL).

ALK-positive ALCL is a rare, mature T-cell non-Hodgkin lymphoma (NHL).

Key learning points:

1. Epidemiology

  • 3% of all adult NHL
  • 10 – 20% of all childhood NHL
  • More common in males 1.5:1 (male:female)
  • Commonly occurs before the age of 30

2. Clinical features

  • Frequently involves extranodal sites such as skin, soft tissue, liver, lung, bone
  • Rarely involves the mediastinum, gut or CNS
  • Majority of patients present with advanced disease (stage III/IV) ~70%
  • Majority of patients present with B symptoms ~75%

3. Pathology

    Lymphoid cells are usually large with abundant cytoplasms and pleiomorphic ‘horseshoe-shaped’ nuclei, this is known as then ‘common pattern’. Variant histology includes the small cell and lymphohistiocytic patterns.
    Pathological cells contain a chromosomal translocation that involves the ALK gene and express ALK protein and CD30.

4. Prognosis

  • Risk stratification should be completed using IPI index
  • ALK positivity is an important prognostic marker as 5 year overall survival is greater in patients with ALK+ ALCL (~80%) than in ALK- ALCL (50%), however this may reflect the comparatively younger patient age group, rather than ALK expression

5.Treatment

First line

  • CHOP – cyclophosphamide, doxorubicin, vincristine, prednisone
  • CHOEP – as above plus etoposide

Consolidation

  • Auto-SCT

Second line

  • GDP – gemcitabine, dexamethasone, cisplatin
  • Brentuximab vedotin (NICE approved in the UK for a minimum of 8 and maximum of 16 cycles)

Consolidation

  • Auto/allo

Clinical trials:

  • Brentuximab vedotin as 1st line therapy?

References:

WHO classification of Tumours of haematopoietic and lymphoid tissues (2017) Swerdlow et. al.

The biology and management of systemic ALCL, Hapgood et. al. 2015 Jul 2;126(1):17-25. doi: 10.1182/blood-2014-10-567461

https://www.ncbi.nlm.nih.gov/pubmed/25869285

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