Hodgkin disease in ataxia-telangiectasia patients with poor outcomes.

Background: To determine the presenting clinicopathologic features and treatment outcomes of 11 ataxia-telangiectasia (A-T) patients with Hodgkin disease.

Procedure: We reviewed the charts of 412 A-T patients to ascertain cases of Hodgkin disease. The data analyzed included date of diagnosis, duration of symptoms, chest radiographic findings, stage and histology, therapy, and outcome.

Results: The six male and five female patients had a median age at diagnosis of 12.2 years. Eight patients presented with fever, cough, and/or cervical lymphadenopathy with a median duration of symptoms of 3 months. Five patients had abnormal chest radiographic findings a median of 3 months prior to diagnosis and were treated with antibiotics for presumed pneumonia. Mediastinal and hilar adenopathy in addition to bilateral infiltrates were present. Histopathology reports were available for nine patients. Three had nodular sclerosing and two each had lymphocyte depleted, mixed cellularity, and not otherwise specified histology. Eight patients had stage IV disease, one had stage III, and in two the staging was not documented. Six patients received reduced-dose chemotherapy, two received radiation therapy, two did not receive therapy, and in one the treatment was not documented. In no patient did the Hodgkin disease remit and all died with a median survival of 3 months. Eight died of pneumonia and three of multiple system organ failure.

Conclusions: A-T patients with Hodgkin disease have markedly reduced survival compared to Hodgkin disease in the general population. Their poor outcomes may be due to advanced Hodgkin disease, failure to recognize coincident chronic lung disease, and the use of non-standard treatment regimens.