Abstract:
:A patient with Jo-1 antibody-associated polymyositis (Jo-1 PM) had a Karnofsky score of 40% and severe muscle, liver and lung damage that was refractory to standard therapy. The female patient received an autologous T-cell-depleted haematopoietic stem cell transplant (HSCT) after myeloablative conditioning. The transplant procedure was complicated by severe adult respiratory distress syndrome (ARDS) and adenovirus-associated haemorrhagic cystitis as well as cytomegalovirus (CMV) reactivation. The patient's creatinine phosphokinase (CPK) and alanine transaminase (ALT) values were normal on day 21. The patient's strength has improved remarkably and her dyspnoea is subjectively improved. At 15 months after the transplant, the patient was well with a Karnofsky score of 80% and had been off any therapy, including steroids, for 14 months.
journal_name
Br J Haematoljournal_title
British journal of haematologyauthors
Baron F,Ribbens C,Kaye O,Fillet G,Malaise M,Beguin Ydoi
10.1046/j.1365-2141.2000.02191.xsubject
Has Abstractpub_date
2000-08-01 00:00:00pages
339-42issue
2eissn
0007-1048issn
1365-2141pii
bjh2191journal_volume
110pub_type
杂志文章abstract::Despite its' central role, the precise mechanisms of the phosphoinositide 3-kinase/Akt (PI3K)/Akt pathway activation in acute myeloid leukaemia (AML) have not been elucidated. Recently, a recurrent novel AKT1 pleckstrin homology domain (PHD) mutation leading to membrane translocation, constitutive AKT activation and l...
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