How is anaplastic large cell lymphoma (ALCL) staged?

Updated: May 08, 2020
  • Author: Delong Liu, MD, PhD; Chief Editor: Emmanuel C Besa, MD  more...
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The Cotswold modification of the Ann Arbor staging system is the standard anatomic staging system for non-Hodgkin lymphoma and Hodgkin disease; it is used to evaluate the extent of disease in patients with anaplastic large cell lymphoma (ALCL). Accurate staging allows appropriate therapeutic selection and contributes to predicting the prognosis.

Staging for anaplastic large cell lymphoma (ALCL) is as follows [2, 26] :

  • Stage I – Involvement of a single lymph node region or lymphoid structure

  • Stage II – Involvement of 2 or more lymph node regions on the same side of the diaphragm

  • Stage III – Involvement of lymph node regions or structures on both sides of the diaphragm

  • Stage IV – Involvement of extranodal sites beyond that designated as E (see below)

Further staging designations include the following:

  • Suffix A – No symptoms (any disease stage)

  • Suffix B – Fever (temperature >38°C); drenching sweats; unexplained weight loss (eg, 10% of body weight within preceding 6 mo) (any disease stage)

  • Suffix E – Involvement of a single extranodal site that is contiguous or proximal to the known nodal site (stages I-III)

Cotswold modifications are as follows:

  • Suffix X – Denotes bulky disease (a widening of the mediastinum by more than one third or the presence of a nodal mass with a maximal dimension of >10 cm)

  • Subscripts – Used to indicate the number of anatomic regions

  • Stage III subdivisions – May be subdivided to include III(1), with or without splenic, hilar, celiac, or portal nodes, and III(2), with para-aortic, iliac, or mesenteric nodes

  • Further identification – Staging identified as clinical stage (CS) or pathologic stage (PS)

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