Hodgkin disease in ataxia‐telangiectasia patients with poor outcomes

Abstract

BackgroundTo determine the presenting clinicopathologic features and treatment outcomes of 11 ataxia‐telangiectasia (A‐T) patients with Hodgkin disease.ProcedureWe 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.ResultsThe 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.ConclusionsA‐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. Med Pediatr Oncol 2003;40:162–166. © 2003 Wiley‐Liss, Inc.

Pulmonary function in adolescents with ataxia telangiectasia

“…Individuals with A-T are also more vulnerable to the development of significant pulmonary complications from chemotherapy used to treat malignancies. 7 With disease progression, decline in lung function may occur as a consequence of impaired respiratory muscle strength and/or coordination. Decline in coordination in children with A-T begins during the early school years and often progresses to widespread and severe impairment of fine and gross motor function.…”

Hodgkin's Disease with Atypical Clinical Presentation, Associated with Ataxia-Telangiectasia

“…Sandoval and Swift analyze 11 cases of HD among 412 A-T patients with additional 14 cases described in literature [3]. The reported case contributes with the unusual clinical presentation of HD in a child with a previously undiagnosed immunodeficiency, good response to standard-dose chemotherapy, and late onset of severe radiation-induced pericarditis.…”

“…This is especially true for the mixed cellularity subtype of the disease diagnosed in our patient. Mixed cellularity was pointed out as the prevailing histological subtype of HD in A-T patients in contrast with the general pediatric population [3,4]. Involvement of the posterior medistinal or paracardiac lymph node groups is found very rarely in HD [5].…”

An unusual manifestation: Papillary thyroid carcinoma in a patient with ataxia-telengiectasia

“…Studies pointed to a high incidence of lymphoid malignancies In patients with A-T, most notably T-cell acute lymphoblastic leukemias. B-cell non-Hodgkin lymphoma and Hodgkin lymphoma were also reported to be frequent 13,14 . In A-T patients, death is usually because of pulmonary infections or malignancies.…”

“…Only one of their patients had thyroid carcinoma. Sandoval et al 14 reported two A-T patients one with follicular thyroid carcinoma, the other with papillary thyroid carcinoma. Even if malignancy risk is greater than normal population in A-T patients, solid tumors are rare than hematologic malignancies.…”