Classification of and risk factors for hematologic complications in a French national cohort of 102 patients with Shwachman-Diamond syndrome

Patients with the Shwachman-Diamond syndrome often develop hematologic complications. No risk factors for these complications have so far been identified. The aim of this study was to classify the hematologic complications occurring in patients with Shwachman-Diamond syndrome and to investigate the...

Full description

Saved in:
Bibliographic Details
Published in:Haematologica (Roma) 2012-09, Vol.97 (9), p.1312-1319
Main Authors: DONADIEU, Jean, FENNETEAU, Odile, PEROT, Christine, MICHEL, Gérard, FOUYSSAC, Fanny, PAILLARD, Catherine, GANDEMER, Virginie, BOUTARD, Patrick, SCHMITZ, Jacques, MORALI, Alain, LEBLANC, Thierry, BELLANNE-CHANTELOT, Christine, BEAUPAIN, Blandine, BEAUFILS, Sandrine, BELLANGER, Florence, MAHLAOUI, Nizar, LAMBILLIOTTE, Anne, ALADJIDI, Nathalie, BERTRAND, Yves, MIALOU, Valérie
Format: Article
Language:English
Subjects:
Biological and medical sciences
Bone Marrow Diseases - complications
Bone Marrow Diseases - mortality
Chromosome aberrations
Development Biology
Exocrine Pancreatic Insufficiency - complications
Exocrine Pancreatic Insufficiency - mortality
Female
Follow-Up Studies
France
Gastroenterology. Liver. Pancreas. Abdomen
Genetics
Hematologic and hematopoietic diseases
Hematologic Diseases - classification
Hematologic Diseases - etiology
Hematologic Diseases - mortality
Hematology
Human health and pathology
Humans
Infant
Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis
Life Sciences
Lipomatosis - complications
Lipomatosis - mortality
Liver. Biliary tract. Portal circulation. Exocrine pancreas
Male
Medical genetics
Medical sciences
Original and Brief Reports
Other diseases. Hematologic involvement in other diseases
Other diseases. Semiology
Prognosis
Prospective Studies
Registries
Risk Factors
Survival Rate
Citations: Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Patients with the Shwachman-Diamond syndrome often develop hematologic complications. No risk factors for these complications have so far been identified. The aim of this study was to classify the hematologic complications occurring in patients with Shwachman-Diamond syndrome and to investigate the risk factors for these complications. One hundred and two patients with Shwachman-Diamond syndrome, with a median follow-up of 11.6 years, were studied. Major hematologic complications were considered in the case of definitive severe cytopenia (i.e. anemia
ISSN:0390-6078
1592-8721
DOI:10.3324/haematol.2011.057489