T-cell Acute Lymphoblastic Leukemia in a Child With Ataxia-telangiectasia

Abstract

We present a patient with acute lymphoblastic leukemia and ataxia-telangiectasia (A-T). The 4-year-old girl is the first child of young nonconsanguineous parents of Serbian origin. Gait problems appearing in the second year of life were treated by physiotherapy. At the age of 4 she was diagnosed with T-cell acute lymphoblastic leukemia and treated according to Berlin-Frankfurt-Munster strategy. Owing to typhlitis developing after 15 days of cytotoxic treatment, frequent radiologic examinations were performed causing profound aplasia. Typhlitis did not respond to conservative treatment but necessitated extensive bowel resection. At that time the A-T was suspected by our team and confirmed by increased chromosomal radiosensitivity and markedly reduced level of A-T mutated protein. Chemotherapy was continued without alkylating agents and further radiologic imaging ran an uncomplicated course. At present, the patient is in first remission and 2.5 years since the beginning of the treatment. We stress the importance of careful initial neurologic evaluation of children with malignancy.

The natural history of ataxia-telangiectasia (A-T): A systematic review

“…Of the 18246 cases, family history of A-T was reported in 1274 cases ( Table 2 ) and 142 cases (53 studies) had 199 illnesses or symptoms other than A-T in a relative ( Fig 3A ). 1279 cases (109 studies [ 15 , 53 , 65 , 71 , 72 , 78 , 87 , 88 , 95 , 99 – 101 , 109 , 110 , 115 , 118 – 221 ]) were the children of consanguineous relationships, and 186 cases (86 studies [ 23 , 33 , 41 , 50 , 54 , 62 , 63 , 65 , 71 , 74 , 84 , 88 , 95 , 111 , 116 , 142 , 146 , 151 – 153 , 188 , 208 – 210 , 212 , 214 , 216 , 218 , 219 , 221 – 277 ]) were reported as being born of non-consanguineous relationships.…”

Successful treatment of acute myeloid leukaemia in a patient with ataxia telangiectasia

“…Its molecular attributions mean that therapy for tumour‐bearing AT is complicated and usually unsuccessful. Reports of acute lymphoblastic leukaemia have provided controversial recommendations for chemotherapy doses; some recommend a standard dose, while others used a reduced dose, especially of alkylating agents . In this case, we therefore started with low‐dose induction, though this was obviously insufficient, and the patient responded well to subsequent dose‐intensified short‐term chemotherapy.…”

“…Furthermore, radiosensitivity and therapy‐induced toxicity could not be ignored . In this case, progressive dyspnoea was repeatedly assessed by chest X‐ray, as these recurrent episodes could cause alveolar destruction and lead to the development of obstructive pulmonary disease.…”

Detection of Acute Lymphoblastic Leukemia Involvement in Pleural Fluid in an Adult Patient with Ataxia Telangiectasia by Flow Cytometry Method

“…Because of increased chemosensitivity, the treatment of AT patients with malignancies requires extremely careful planning and caution with respect to the use of chemotherapy [9][10][11]. ALL, cancer of the white blood cells, is a heterogenous disease that mainly occurs due to the malignant cloning of lymphocytes.…”