Criteria for Severe Aplastic Anaemia

Camitta, BruceM.; Rozmán, C; Marı́n, Pedro; Nomdedeu, Benet; Montserrat, Emilio

doi:10.1016/s0140-6736(88)90388-1

Cited by 37 publications

(30 citation statements)

References 3 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…24 In SDS, an additional complexity is related to cytogenetic abnormalities observed incidentally during routine bone marrow surveillance. Two recurrent cytogenetic anomalies have been observed, i(7)(q10) and del(20q), which can be indolent and sometimes even transient.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2012

View full text Add to dashboard Cite

The online version of this article has a Supplementary Appendix. BackgroundPatients 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. Design and MethodsOne hundred and two patients with Shwachman-Diamond syndrome, with a median followup of 11.6 years, were studied. Major hematologic complications were considered in the case of definitive severe cytopenia (i.e. anemia <7 g/dL or thrombocytopenia <20×10 9 /L), classified as malignant (myelodysplasia/leukemia) according to the 2008 World Health Organization classification or as non-malignant. ResultsSevere cytopenia was observed in 21 patients and classified as malignant severe cytopenia (n=9), non-malignant severe cytopenia (n=9) and malignant severe cytopenia preceded by nonmalignant severe cytopenia (n=3). The 20-year cumulative risk of severe cytopenia was 24.3% (95% confidence interval: 15.3%-38.5%). Young age at first symptoms (<3 months) and low hematologic parameters both at diagnosis of the disease and during the follow-up were associated with severe hematologic complications (P<0.001). Fifteen novel SBDS mutations were identified. Genotype analysis showed no discernible prognostic value. ConclusionsPatients with Shwachman-Diamond syndrome with very early symptoms or cytopenia at diagnosis (even mild anemia or thrombocytopenia) should be considered at a high risk of severe hematologic complications, malignant or non-malignant. Transient severe cytopenia or an indolent cytogenetic clone had no deleterious value.Key words: Shwachman-Diamond syndrome, genotype, aplastic anemia, secondary leukemia, cytopenia, myelodysplasia, monosomy 7.Citation: Donadieu J, Fenneteau O, Beaupain B, Beaufils S, Bellanger F, Mahlaoui N, Lambilliotte A, Aladjidi N, Bertrand Y, Mialou V, Perot C, Michel G, Fouyssac F, Paillard C, Gandemer V, Boutard P, Schmitz J, Morali A, Leblanc T, Bellanné-Chantelot C, and Classification of and risk factors for hematologic complications in a French national cohort of 102 patients with Shwachman-Diamond syndrome

show abstract

Section: Discussionmentioning

confidence: 99%

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

et al. 2012

View full text Add to dashboard Cite

show abstract

“…1 The incidence of SAA varies worldwide from 1.5 to 7.8 per million per year. 2 The pathobiology of SAA is believed to be driven by an abnormal expansion of T lymphocytes that directly affect the hematopoietic stem cells, leading to their destruction or to clonal abnormalities, such as paroxysmal nocturnal hemoglobinuria and myelodysplastic syndrome 2 in most cases, however the etiology remains unknown and the disease is referred to as idiopathic acquired aplastic anemia.…”

Section: Introductionmentioning

confidence: 99%

Survival of patients with documented autologous recovery after SCT for severe aplastic anemia: a study by the WPSAA of the EBMT

Piccin

McCann

Socié

et al. 2009

Bone Marrow Transplant

View full text Add to dashboard Cite

“…included 77 patients who were tested for any correlation between the presence of a minor population in PNH-type cells and the response to IST in our previous study [14]. The severity of AA was classified according to the criteria proposed by Camitta et al [15,16]. All participants provided written, informed consent to all procedures associated with the study, which was approved by the Ethical Committee at our institution (study number 46).…”

Section: Introductionmentioning

confidence: 99%

Roles of DRB1∗1501 and DRB1∗1502 in the pathogenesis of aplastic anemia

Sugimori

Yamazaki

Feng

et al. 2007

Experimental Hematology

View full text Add to dashboard Cite

Category Clinical investigations Word countsTotal text word count: 3412 words Abstract word count: 240 words 2 ABSTRACTObjective. Although a number of reports have documented a significantly increased incidence of HLA-DR15 in aplastic anemia (AA), the exact role of HLA-DR15 in the immune mechanisms of AA remains unclear. We herein clarify the difference between DRB1*1501 and DRB1*1502, the 2 DRB1 alleles which determine the presentation of HLA-DR15, in the pathophysiology of AA. Materials and Methods.We investigated the relationships of the patients' HLA-DRB1 allele with both the presence of a small population of CD55 -CD59 -(PNH-type) blood cells and the response to antithymocyte globulin (ATG) plus cyclosporine (CsA) therapy in 140 Japanese AA patients. Results. Of the 30 different DRB1 alleles, only DRB1*1501 (33.6% vs. 12.8%, P c <0.01) and DRB1*1502 (43.6% vs. 24.4%, P c <0.01) displayed significantly higher frequencies among the AA patients than among a control. AA patients possessing HLA-DR15 tended to be old, and especially, the frequency of DRB1*1502 in patients ≥40 years old (52.4%) was markedly higher than that in those <40 years old (16.2%, P c <0.01). Only DRB1*1501 was significantly associated with the presence of a small population of PNH-type cells and it also showed a good response to ATG plus CsA therapy in a univariate analysis. A multivariate analysis showed only the presence of a small population of PNH-type cells to be a significant factor associated with a good response to the immunosuppressive therapy (P<0.01). Conclusion.Although both DRB1*1501 and DRB1*1502 contribute to the development of AA, the methods of contribution differ between the two alleles.3

show abstract

Criteria for Severe Aplastic Anaemia

Cited by 37 publications

References 3 publications

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

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

Survival of patients with documented autologous recovery after SCT for severe aplastic anemia: a study by the WPSAA of the EBMT

Roles of DRB1∗1501 and DRB1∗1502 in the pathogenesis of aplastic anemia

Contact Info

Product

Resources

About