Pilot study of modified LMB-based therapy for children with ataxia telangiectasia and advanced stage high grade mature B-cell malignancies. Pediatr Blood Cancer. 2014 February ; 61(2): 360–362. There is no consensus regarding the optimal strategy for treating children with AT who develop a hematopoietic malignancy. historically, many of these children have been treated with minimal or modified reduced-intensity therapy because of concerns regarding tolerance of therapy. we piloted a curative approach in 5 children with AT who presented with advanced stage (iii, n=2; iv, n=3) B-NHL (diffuse large B-cell lymphoma, n=4; burkitt leukemia, n=1) using a modified LMB-based protocol. two achieved sustained ccr (one, ccr at 6 years; one, pulmonary death after 3 years in ccr). two died from toxicity during induction and 1 failed induction with progressive disease.

Ataxia-telangiectasia and T-Cell Leukemias: No Evidence for Somatic ATM Mutation in Sporadic T-ALL or for Hypermethylation of the ATM-NPAT/E14 Bidirectional Promoter in T-PLL1. Cancer Research 58, 2293-2297, June I. 1998) A-T3 is a recessive pleiotropic syndrome caused by mutations in the ATM gene (1, 2) located at 1Iq22-q23 (3). The risk of malignancies, especially lymphoid neoplasias of T-cell origin, is substantially increased in A-T and was associated previously with spontaneous chromosomal instability observed in A-T. T-PLL, an aggressive malignancy with a median age at diagnosis of 69 years, exhibits immunophenotypic and cytogenetic similarities to a T-cell leukemia seen in A-T . In particular, T-PLL is often associated with translocations and inversions of chromosome 14

Pediatr hematol oncol 1998 Sep-Oct;15(5):425-9. Ataxia telangiectasia associated with B-cell lymphoma: the effect of a half-dose of the drugs administered according to the acute lymphoblastic leukemia standard risk protocol. Because of increased chemosensitivity, the treatment of AT patients with malignancies requires extremely careful planning and caution with respect to the use of chemotherapy. The authors report on a 12-year-old boy with AT who developed B-cell lymphoma. He received a half-dose of the drugs administered according to the acute lymphoblastic leukemia (ALL) protocol issued by our children's cancer study group (9104 Standard Risk Protocol, Tokai Pediatric Oncology Study Group). As a result, he continues to be in complete remission and free of treatment complications 32 months after the diagnosis of B-cell lymphoma.  

J clin immunol 2016 Oct;36(7):667-76 J clin immunol 2016 Oct;36(7):667-76. Lymphoma Secondary to Congenital and Acquired Immunodeficiency Syndromes at a Turkish Pediatric Oncology Center. We summarized the clinical characteristics and treatment results of 17 cases with primary immunodeficiency that developed non-Hodgkin lymphoma (NHL) and Hodgkin lymphoma (HL). 7 patients were diagnosed with AT, two with CVID, two with selective IgA deficiency, one with XLP syndrome, one with Wiskott-Aldrich syndrome, one with EBV related lymphoproliferative syndrome, one with interleukin-2- inducible T-cell kinase (ITK) deficiency, and one with lymphoma developing after ALPS. 7 were diagnosed with HL and 10 with NHL (seven B-cell, three T-cell). The NHL patients were started on the BFM, POG9317, LMB-96, or R-CHOP treatment protocols with reduced chemotherapy dosages. HL cases were started on ABVD and/or COPP protocol, also with modified dosages. six of the ten NHL patients have died. Primary immunodeficiency is a strong predisposing factor for developing lymphoma.

Annals of Oncology 11 (Suppl I): S141-S145, 2000 Annals of Oncology 11 (Suppl I): S141-S145, 2000. Non-Hodgkin's lymphoma in pediatric patients with chromosomal breakage syndromes (AT and NBS): Experience from the BFM trials In three consecutive multicenter therapy, trials for pediatric NHL (NHL-BFM), 1569 patients with newly diagnosed NHL have been registered between 1986 and 1997. Nine patients with AT (n = 5) and NBS (n = 4) were identified. Results: Median age of patients with AT and NBS at diagnosis of NHL was nine years. Diffuse large B-cell lymphoma, n = 1 ; ALCL, n = 1; lymphoblastic T-cell lymphoma, n = 1 Stages were: I and II in 3 patients, III in 5 and IV in 1 patient. All patients received polychemotherapy according to tumor-entity and stage, none received radiation. Dose reductions according to individual tolerance concerned mainly ethotrexate, alkylating agents and epipodophyllotoxines.

Among these 1569 patients, 9 patients were suffering from AT or NBS. Annals of Oncology 11 (Suppl I): S141-S145, 2000. Non-Hodgkin's lymphoma in pediatric patients with chromosomal breakage syndromes (AT and NBS): Experience from the BFM trials From April 1986 to October 1997, 1569 patients up to 18 years of age with newly diagnosed NHL or B-ALL were registered in the NHL-BFM study center. Among these 1569 patients, 9 patients were suffering from AT or NBS. These patients were analyzed regarding clinicopathological features, treatment modalities and outcome.

Therapy and response criteria Annals of Oncology 11 (Suppl I): S141-S145, 2000. Non-Hodgkin's lymphoma in pediatric patients with chromosomal breakage syndromes (AT and NBS): Experience from the BFM trials Therapy and response criteria Patients with lymphoblastic T-cell lymphoma received ALL type therapy consisting of induction, consolidation, re-induction, and maintenance therapy as previously described . Patients with B-cell lymphoma or anaplastic large-cell lymphoma of either immunophenotype received four to six courses of polychemotherapy as described elsewhere. Intensity and duration of therapy was stratified according to stage at diagnosis and to initial tumor mass, determined by serum-concentration of lactate dehydrogenase (LDH). In patients with AT or NBS, the study-center recommended to start therapy with reduced intensity depending on the physical state of the patient, history of previous infections and other ID-related complications . Therapy was intensified during following courses according to tolerance of the first course

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

Modified chop‐chemotherapy plus rituximab for diffuse large B‐cell lymphoma complicating ataxia‐telangiectasia Chemotherapy was considered and, in order to avoid the severe complications reported in patients with AT treated for cancer (Abadir & Hakamin, 1983), a modified dosage of CHOP was chosen. The patient received: cyclophosphamide 300 mg/m2, doxorubicin 15 mg/m2, vincristine 1 mg/m2 on d  1, prednisone 40 mg/m2/d for 5 d. This treatment was repeated every 3 weeks for eight cycles. On d 2 of each cycle, the addition of Rituximab at a dosage of 375 mg/m2 was made.