B-Cell Acute Lymphoblastic Leukemia in a Child With Ataxia Telangiectasia

Sharma, Lalit Mohan; Kashyap, Rajesh; Gupta, Sunil Kumar; Bhargava, Manorama

doi:10.1080/08880010802106614

Cited by 9 publications

(2 citation statements)

References 5 publications

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“…Children afflicted with A-T can develop acute lymphocytic leukemia of T cell origin, and leukemia in older A-T patients is usually an aggressive T cell cancer similar to a chronic lymphoblastic leukemia (4,6). However, B cell disease is also prevalent in A-T (7,8). ATM mutations in heterozygote carriers are also considered cancer predisposing, and these mutations have been strongly linked to breast and other cancers (9)(10)(11).…”

mentioning

confidence: 99%

ATM and the Molecular Pathogenesis of Ataxia Telangiectasia

McKinnon

2012

Annu. Rev. Pathol. Mech. Dis.

206

193

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Ataxia telangiectasia (A-T) results from inactivation of the ATM protein kinase. DNA-damage signaling is a prime function of this kinase, although other roles have been ascribed to ATM. Identifying the primary ATM function(s) for tissue homeostasis is key to understanding how these functions contribute to the prevention of A-T-related pathology. In this regard, because A-T is primarily a neurodegenerative disease, it is essential to understand how ATM loss results in degenerative effects on the nervous system. In addition to delineating the biochemistry and cell biology of ATM, important insights into the molecular basis for neurodegeneration in A-T come from a spectrum of phenotypically related neurodegenerative diseases that directly result from DNA-repair deficiency. Together with A-T, these syndromes indicate that neurodegeneration can be caused by the failure to appropriately respond to DNA damage. This review focuses on defective DNA-damage signaling as the underlying cause of A-T.

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confidence: 99%

ATM and the Molecular Pathogenesis of Ataxia Telangiectasia

McKinnon

2012

Annu. Rev. Pathol. Mech. Dis.

206

193

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“…All patients suff ered from T-ALL. Up to now, only 1 pa tient of AT and mature B-cell leukaemia has been published [15] . Compared to patients without underlying immunodefi ciency or chromosomal breakage disorder such as AT or Nijmegen Breakage syndrome (NBS), outcome of AT patients with concomitant leukaemia is almost invariably fatal [6,9] .…”

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confidence: 99%

Complicated but Successful Treatment of a Patient with Ataxia Telangiectasia and Pre-B-Acute Lymphoblastic Leukemia

Brummel

Bernbeck²,

Schneider³

2010

Klin Padiatr

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Lymphoma Secondary to Congenital and Acquired Immunodeficiency Syndromes at a Turkish Pediatric Oncology Center

et al. 2016

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The prevalence of lymphoma in primary immunodeficiency cases and autoimmune diseases, as well as on a background of immunodeficiency following organ transplants, is increasing. The lymphoma treatment success rate is known to be a low prognosis. Our study aimed to emphasize the low survival rates in immunodeficient vs. immunocompetent lymphoma patients and also to investigate the effect of rituximab in patients with ataxia telangiectasia and other immunodeficiencies. We summarized the clinical characteristics and treatment results of 17 cases with primary immunodeficiency that developed non-Hodgkin lymphoma (NHL) and Hodgkin lymphoma (HL) retrospectively. Seven patients were diagnosed with ataxia-telangiectasia, two with common variable immunodeficiency, two with selective IgA deficiency, one with X-related lymphoproliferative syndrome, one with Wiskott-Aldrich syndrome, one with Epstein-Barr virus-related lymphoproliferative syndrome, one with interleukin-2-inducible T-cell kinase (ITK) deficiency, and one with lymphoma developing after autoimmune lymphoproliferative syndrome (ALPS). One patient underwent a renal transplant. Of the nine males and eight females (aged 3-12 years, median = 7) that developed lymphoma, seven were diagnosed with HL and ten with NHL (seven B-cell, three T-cell). The NHL patients were started on the Berlin-Frankfurt-Münster, POG9317, LMB-96, or R-CHOP treatment protocols with reduced chemotherapy dosages. HL cases were started on the doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD) and/or cyclophosphamide, vincristine, procarbazine, and prednisone (COPP) protocol, also with modified dosages. Importantly, all seven cases of HL are alive and in remission, while six of the ten NHL patients have died. Primary immunodeficiency is a strong predisposing factor for developing lymphoma. Low treatment success rates relative to other lymphomas and difficulties encountered during treatment indicate that new treatment agents are needed. While some success has been achieved by combining rituximab with lymphoma treatment protocols in B-NHL cases with primary immunodeficiency, the need for new treatment approaches for these patients remains critical.

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B-Cell Acute Lymphoblastic Leukemia in a Child With Ataxia Telangiectasia

Cited by 9 publications

References 5 publications

ATM and the Molecular Pathogenesis of Ataxia Telangiectasia

ATM and the Molecular Pathogenesis of Ataxia Telangiectasia

Complicated but Successful Treatment of a Patient with Ataxia Telangiectasia and Pre-B-Acute Lymphoblastic Leukemia

Lymphoma Secondary to Congenital and Acquired Immunodeficiency Syndromes at a Turkish Pediatric Oncology Center

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