Detection of paroxysmal nocturnal hemoglobinuria clones to exclude inherited bone marrow failure syndromes.
Eur J Haematol
; 92(6): 467-70, 2014 Jun.
Article
em En
| MEDLINE
| ID: mdl-24612308
ABSTRACT
BACKGROUND:
Inherited bone marrow failure syndromes (IBMFS) and acquired aplastic anemia (AA) are life-threatening marrow failure disorders. These entities can be difficult to distinguish because they present similarly. Correct diagnosis is imperative for proper therapy. DESIGN ANDMETHODS:
This is a retrospective, single-center study of patients <40 yr of age, evaluated for bone marrow failure, and assayed for the presence of a PNH clone in the pediatric or adult hematology/oncology clinics from 2001 to present. Patients were also evaluated for IBMFS.RESULTS:
We present results from 156 patients with marrow failure, 20 of whom have IBMFS. None of the IBMSF patients had paroxysmal nocturnal hemoglobinuria (PNH) clones.CONCLUSIONS:
Although further studies are needed, our results suggest that the detection of a PNH clone can be a useful diagnostic tool to exclude the diagnosis of IBMFS and focus the work-up and treatment on an acquired form of marrow failure.Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Evolução Clonal
/
Hemoglobinúria Paroxística
Tipo de estudo:
Diagnostic_studies
/
Observational_studies
Limite:
Adolescent
/
Adult
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Child
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Child, preschool
/
Female
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Humans
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Infant
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Male
/
Newborn
Idioma:
En
Ano de publicação:
2014
Tipo de documento:
Article