Initial treatment of systemic anaplastic large cell lymphoma

This is an overview of our approach to the management of newly diagnosed systemic anaplastic large cell lymphoma (ALCL). It should be used in conjunction with other UpToDate content on ALCL. While the general treatment principles for ALCL apply to most disease presentations and patient populations, this algorithm does not apply to patients with primary cutaneous ALCL and patients with breast implant-associated ALCL.

PET/CT: positron emission tomography/computed tomography; CR: complete response; PR: partial response; SD: stable disease; PD: progressive disease; IPI: international prognostic index; ALK: anaplastic lymphoma kinase; HCT: hematopoietic cell transplantation; ECOG: eastern cooperative oncology group.
* In patients over age 60, CHOEP (cyclophosphamide, doxorubicin, vincristine, etoposide, prednisone, administered every 21 days) has increased toxicity and no clear benefit over CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone, administered every 21 days) in retrospective analyses. The addition of etoposide for especially fit patients in this age category is considered on an individual basis.
¶ The Lugano criteria for response assessment in lymphoma are used to determine complete response (CR), partial response (PR), stable disease (SD), and progressive disease (PD).
Δ For those with limited stage (I/II) disease who achieve a PR after six cycles of induction therapy, we offer radiation therapy in an attempt to achieve complete response. All others proceed with second-line chemotherapy.
◊ To calculate the International Prognostic Index (IPI), one point is given for each of the following variables: age >60 years; serum lactate dehydrogenase concentration greater than normal; ECOG performance status of 2 or greater; clinical stage III or IV disease; and >1 involved extranodal site.

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Initial treatment of systemic anaplastic large cell lymphoma