Chemo- And Radiosensitivity Testing in a Patient With Ataxia Telangiectasia and Hodgkin Disease

Tamminga, Rienk; Dolsma, W.V.; Leeuw, Jan A.; Kampinga, Harm H.

doi:10.1080/088800102753541314

Cited by 24 publications

(17 citation statements)

References 27 publications

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“…The problem of the appropriate chemotherapy regimen is still debated and controversial because of the small series of A-T patients with HD [3,14]. Our case report supports the opinion that standard chemotherapy regimens are well tolerated [3,11]. On the other hand, the patient experienced severe radiation-induced complications: esophagitis and pericarditis at a very low total dose of irradiation.…”

Section: Discussionsupporting

confidence: 70%

“…Elevated AFP occurs in about 95% of the A-T patients. Thus, AFP appears to be a reliable diagnostic tool when other causes for its elevation are excluded, keeping in mind that another laboratory evidence like increased chromosomal breakage after radiation exposure of cell cultures or sequencing of the ATM gene is expensive and not routinely available [10,11].…”

Section: Discussionmentioning

confidence: 99%

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

Spasova

Ivanov

Grudeva-Popova

et al. 2009

Scholarly Research Exchange

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The authors report a 4-year-old male with Hodgkin's disease with atypical clinical presentation, laboratory data and imaging studies as well as an increased radiosensitivity. These were the first symptoms which raised the suspicion of ataxia-telangiectasia. The lymphoma presented as a solid tumor mass in the right upper posterior mediastinum without peripheral lymphadenopathy. The alpha-fetoprotein was significantly increased and the diagnosis of a germ-cell tumor or neuroblastoma was suspected. After partial resection of the tumor the histology indicated Hodgkin's disease-mixed cellularity. The boy received chemotherapy according to DAL HD-95 protocol without any unusual toxicity. Low-dose radiation therapy of the upper and middle mediastinum was followed by early radiation-induced esophagitis and appearance of a large pericardial effusion one year later. Ataxia and bulbar and skin telangiectasias at the radiation site appeared in the next years. He suffered recurrent pulmonary infections and succumbed to severe pneumonia with respiratory failure and pulmonary hypertension five years after the diagnosis of malignant lymphoma without evidence of recurrence of Hodgkin's disease. Case ReportThe child was a 4-year-old male of Roma origin, born after second normal pregnancy without family history of immune deficiency. He had a past history of cerebral palsy, diagnosed at the age of 20 months because of a delay in motor and intellectual development. His present complaints were productive cough and fever for one month. After admission to the regional hospital physical and Xray signs of right pulmonary infiltrate were found, and he received antibiotic treatment with Clindamycin, Amikacin, Imipenem, and Cefepime without any improvement. He was referred for further diagnostic evaluation to the Department of Pediatrics, Medical University-Plovdiv. Physical examination on admission showed mask-like, sad, and relaxed face with progeroid features, growth retardation, reduced subcutaneous fat, generalized muscle hypotonia and atrophy, bronchial breathing in the right parascapular region, tracheal stridor, and enlarged liver 3 cm below the costal margin and lack of peripheral lymphadenopathy. Mental retardation was mild (IQ: 60%) with predominantly affected expressive speech and fine motor skills. Computer tomography (CT) scan showed tumor mass in the right upper posterior mediastinum (Figure 1). It deviated the trachea and was well demarked from the pulmonary parenchyma. No destruction of the adjacent bones was evident.Laboratory data showed leukocytosis, thrombocytosis; elevated erythrocyte sedimentation rate, serum fibrinogen and C-reactive protein 221 mg/L. The diagnostic workup for the mediastinal tumor revealed significant increase of alpha-fetoprotein (AFP) 238.98 ng/mL (reference range 0-5 ng/mL), moderate elevation of vanylmandelic acid 80.9 µmol/l, anergic tuberculin test, and negative blood and sputum cultures and serology for ecchinococcus.Germ-cell tumor or neuroblastoma was suspected and the boy was referred to ...

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Section: Discussionsupporting

confidence: 70%

Section: Discussionmentioning

confidence: 99%

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

Spasova

Ivanov

Grudeva-Popova

et al. 2009

Scholarly Research Exchange

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“…Eight patients received chemotherapy only (four at 50-75% of standard dose and in four the doses were not documented), two received radiation therapy, two did not have therapy documented in the case report, one received combined modality therapy, and one patient was not treated. In five patients, the Hodgkin disease remitted after reduced-dose chemotherapy (n ¼ 3; 75% of standard dose [10]), after reduced combined modality therapy (n ¼ 1; [14]), and after radiation therapy [9]. Three of these five patients ultimately died of pulmonary complications, one of recurrent Hodgkin disease, and one died of an untreated preleukemic syndrome.…”

Section: Discussionmentioning

confidence: 91%

“…Fourteen cases (13 girls and one boy) of A-T and Hodgkin disease have been described (Table II) [6][7][8][9][10][11][12][13][14]. These patients had a median age at diagnosis of 8 years (range, 3-25 years) and nine had advanced disease.…”

Section: Discussionmentioning

confidence: 98%

“…In our continuing study of A-T patients and their families, we noted that all 11 A-T patients with Hodgkin disease died. This review of the diagnostic features and treatment outcomes of these patients and of other published cases [6][7][8][9][10][11][12][13][14] was undertaken to determine the reasons for this unusually high rate of mortality.…”

Section: Introductionmentioning

confidence: 99%

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Hodgkin disease in ataxia‐telangiectasia patients with poor outcomes

Sandoval

Swift

2003

Med. Pediatr. Oncol.

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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.

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Identifying ataxia‐telangiectasia in cancer patients: Novel insights from an interesting case and review of literature

Cao

Tan

et al. 2020

Clinical Case Reports

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Chemo- And Radiosensitivity Testing in a Patient With Ataxia Telangiectasia and Hodgkin Disease

Cited by 24 publications

References 27 publications

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

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

Hodgkin disease in ataxia‐telangiectasia patients with poor outcomes

Identifying ataxia‐telangiectasia in cancer patients: Novel insights from an interesting case and review of literature

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