In:
European Journal of Haematology, Wiley, Vol. 91, No. 6 ( 2013-12), p. 557-560
Abstract:
Ataxia telangiectasia ( AT ) is a rare autosomal recessive multisystem disorder characterised by cerebellar degeneration, immunodeficiency and cancer predisposition. Around 10% of AT patients develop lymphoid malignancies, but the development of myeloid leukaemia with AT ( AT ‐ AML ) is extremely rare, and there have been no previous publications regarding suitable therapies. Here, we first describe a successful therapeutic experience in a patient with AT ‐ AML ( FAB ‐M1) who attained remission after induction therapy and maintained stable disease for a year. To minimise therapy‐induced toxicity, low‐dose induction was applied first, though this was obviously insufficient and the patient subsequently responded well to dose‐intensified short‐term chemotherapy. In this report, we suggest a curative therapeutic approach for AT ‐ AML , though the issue of how best to manage patients with cancer complicated by immunodeficiency remains undecided.
Type of Medium:
Online Resource
ISSN:
0902-4441
,
1600-0609
DOI:
10.1111/ejh.2013.91.issue-6
Language:
English
Publisher:
Wiley
Publication Date:
2013
detail.hit.zdb_id:
2027114-1
Permalink