Limited treatment safe, successful for pediatric patients with lymphocyte-predominant Hodgkin lymphoma

A majority of highly selected pediatric patients with lymphocyte-predominant Hodgkin lymphoma may be successfully treated with surgery alone, according to study results from the Children’s Oncology Group.

Patients treated with a less intensive chemotherapy regimen experienced excellent EFS outcomes, with the majority avoiding radiation therapy, results showed.

Lymphocyte-predominant Hodgkin lymphoma (LPHL) is rare, and standard of care has yet to be established.

“There have been two recurring themes in the literature about LPHL in the last few decades,” Burton E. Appel, MD, MBA, pediatric hematologist–oncologist at the Institute for Pediatric Cancer and Blood Disorders at Hackensack University Medical Center’s Joseph M. Sanzari Children’s Hospital in New Jersey, told HemOnc Today. “The first is that some patients with very limited disease that is totally resected can be cured by surgery alone, with case reports and small series to support this. The second is that LPHL may behave more like a type of non-Hodgkin lymphoma than classical Hodgkin lymphoma, and may be cured with less therapy.”

No prospective study had been conducted to determine which treatment produces the best outcomes for children with LPHL.

Appel and colleagues from the Children’s Oncology Group observed 178 patients (mean age, 12.3 years; range, 4.2-20.7; 84% boys) with stage IA LPHL in a single node.

Patients who underwent complete resection of the single node (n = 52) received no further therapy. After recurrence, they underwent three cycles of doxorubicin, vincristine, prednisone and cyclophosphamide (AV-PC).

Patients with unresected stage IA or stage IIA LPHL (n = 126) received three cycles of AV-PC as treatment. Those who achieved less than a complete response underwent 21-Gy involved-field radiation therapy.

The 5-year EFS rate was 77.1% (95% CI, 62.4-86.6).

Thirteen patients treated with surgical resection alone relapsed a median of 11.5 months (95% CI, 1.1-79.1) from study enrollment.

Median follow-up was 56.3 months (range, 3.9-107.4) for the remaining patients who had not relapsed.

A total of 135 patients received AV-PC, included nine patients who relapsed after surgical resection. Ninety-two percent (n = 124) achieved a confirmed complete response and did not proceed to radiation therapy; the remaining 11 patients were assigned radiation after achieving less than a complete response.

Fourteen events have occurred in this cohort, including 12 relapses. Two patients developed non-Hodgkin lymphoma. The median time to a first event was 28.1 months (range, 5.1-45.5), and the cohort had a median 5-year EFS of 88.8% (95% CI, 81.8-93.2).

No deaths had occurred at the time of reporting; OS rate was 100%.

“There are a number of interesting directions for this research to take, including whether the surgery-only approach can be safely extended to patients with more than a single node,” Appel said. “It will also be important to observe whether there will be more relapses or increased surgical morbidity from more extensive surgeries, especially in the neck. Additionally, most adult patients with localized LPHL are treated with radiation alone; it will be important to determine whether we can study the efficacy of this approach in older patients.” – by Cameron Kelsall

For more information:

Burton E. Appel, MD, MBA, can be reached at bappel@hackensackUMC.org.

Disclosure: Appel reports a consultant role with United Therapeutics. One other study researcher reports travel expenses from IBA. The other researchers report no relevant financial disclosures.