Platelet counts and haemorrhagic diathesis in patients with myelodysplastic syndromes

Neukirchen, Judith; Blum, Sabine; Kuendgen, Andrea; Strupp, Corinna; Aivado, Manuel; Haas, Rainer; Aul, Carlo; Gattermann, Norbert; Germing, Ulrich

doi:10.1111/j.1600-0609.2009.01299.x

Cited by 64 publications

(67 citation statements)

References 14 publications

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“…16,17,50 In addition, the immunophenotypic platelet score showed an important prognostic impact on the outcome of MDS patients, independently of the WHO and IPSS subtypes, the presence of megakaryocytic dysplasia and the PB platelet count, cases with high immunophenotypic scores showing a shorter overall survival. Interestingly, although an association between thrombocytopenia and poor patient outcome has been described in large series of MDS patients, 33,34 in our study approximately 26% (7 of 26 cases) of those patients showing normal platelet counts had a high immunophenotypic score, reinforcing the potential added value of immunophenotyping to the number of platelets in PB.…”

Section: Discussionsupporting

confidence: 67%

“…At the same time it is associated with a poor prognosis of the disease. 30,31 Similarly, multiple reports continue to show that thrombocytopenia is also associated with both a worse outcome and decreased survival in MDS, 8,[32][33][34] even among low-risk MDS cases. 35 In our series, megakaryocytic dysplasia was not seen to have a significant impact on the outcome of MDS patients.…”

Section: Discussionmentioning

confidence: 98%

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Altered immunophenotypic features of peripheral blood platelets in myelodysplastic syndromes

Sandes¹,

Yamamoto²,

Matarraz³

et al. 2012

Haematologica

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BackgroundMultiparameter flow cytometric analysis of bone marrow and peripheral blood cells has proven to be of help in the diagnostic workup of myelodysplastic syndromes. However, the usefulness of flow cytometry for the detection of megakaryocytic and platelet dysplasia has not yet been investigated. The aim of this pilot study was to evaluate by flow cytometry the diagnostic and prognostic value of platelet dysplasia in myelodysplastic syndromes. Design and MethodsWe investigated the pattern of expression of distinct surface glycoproteins on peripheral blood platelets from a series of 44 myelodysplastic syndrome patients, 20 healthy subjects and 19 patients with platelet alterations associated to disease conditions other than myelodysplastic syndromes. Quantitative expression of CD31, CD34, CD36, CD41a, CD41b, CD42a, CD42b and CD61 glycoproteins together with the PAC-1, CD62-P, fibrinogen and CD63 platelet activation-associated markers and platelet light scatter properties were systematically evaluated. ResultsOverall, flow cytometry identified multiple immunophenotypic abnormalities on platelets of myelodysplastic syndrome patients, including altered light scatter characteristics, over-and under expression of specific platelet glycoproteins and asynchronous expression of CD34; decreased expression of CD36 (n=5), CD42a (n=1) and CD61 (n=2), together with reactivity for CD34 (n=1) were only observed among myelodysplastic syndrome cases, while other alterations were also found in other platelet disorders. Based on the overall platelet alterations detected for each patient, an immunophenotypic score was built which identified a subgroup of myelodysplastic syndrome patients with a high rate of moderate to severe alterations (score>1.5; n=16) who more frequently showed thrombocytopenia, megakaryocytic dysplasia and high-risk disease, together with a shorter overall survival. ConclusionsOur results show the presence of altered phenotypes by flow cytometry on platelets from around half of the myelodysplastic syndrome patients studied. If confirmed in larger series of patients, these findings may help refine the diagnostic and prognostic assessment of this group of disorders.

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Section: Discussionsupporting

confidence: 67%

Section: Discussionmentioning

confidence: 98%

Altered immunophenotypic features of peripheral blood platelets in myelodysplastic syndromes

Sandes¹,

Yamamoto²,

Matarraz³

et al. 2012

Haematologica

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“…The proportions of patients receiving an ESA were, however, similar in Cross-sectional study on MDS management haematologica | 2010; 95 (6) 897 There are only few published data on the frequency of platelet transfusions in MDS. 16 In our series, patients dependent on platelet transfusions had received a median of eight platelet transfusions during the preceding 6 months; among the patients who had not received myelosuppressive treatments, the median was one platelet transfusion every 6 weeks. Of note, severe bleeding occurred in 38.5% of patients with a platelet count below 50¥10 9 /L in our series.…”

Section: Discussionmentioning

confidence: 79%

Daily practice management of myelodysplastic syndromes in France: data from 907 patients in a one-week cross-sectional study by the Groupe Francophone des Myelodysplasies

Kelaïdi

Stamatoullas²,

Beyne‐Rauzy³

et al. 2009

Haematologica

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BackgroundThere is little published information on the everyday clinical management of myelodysplastic syndromes in real world practice. Design and MethodsWe conducted a cross-sectional study of all patients with myelodysplastic syndromes attending 74 French centers in a 1-week period for inpatient admission, day-hospital care or outpatient visits. ResultsNine hundred and seven patients were included; 67.3% had lower-risk myelodysplastic syndromes (International Prognostic Scoring System: low or intermediate-1). Karyotype had been analyzed in 82.5% of the cases and was more often of intermediate or poor risk in patients under 65 years old compared with those who were older. Red blood cell transfusions accounted for as many as 31.4% of the admissions. Endogenous erythropoietin level was less than 500 IU/L in 88% of the patients tested. Erythroid stimulating agents had been or were being used in 36.8% of the lower risk patients, iron chelation in 31% of lower risk patients requiring red blood cell transfusions and lenalidomide in 41% of lower risk patients with del 5q. High-dose chemotherapy, hypomethylating agents, low dose cytarabine and allogeneic stem cell transplantation had been or were being used in 14.8%, 31.1%, 8.8% and 5.1%, respectively, of higher-risk patients. ConclusionsKaryotype is now assessed in most patients with myelodysplastic syndromes, and patients under 65 years old may have more aggressive disease. Apart from erythroid-stimulating agents and, in higher-risk myelodysplastic syndromes, hypomethylating agents, specific treatments are used in a minority of patients with myelodysplastic syndromes and red blood cell transfusions still represent the major reason for hospital admission.Key words: MDS, cross-sectional study, outpatient visit, erythropoietin, erythropoiesis stimulating agents. Citation: Kelaidi C, Stamatoullas A, Beyne-Rauzy O, Raffoux E, Quesnel B, Guerci A, Dreyfus F, Brechignac S, Berthou C, Prebet T, Hicheri Y, Hacini M, Delaunay J, Gourin M-P , Camo J-M, Zerazhi H, Taksin A-L, Legros L, Choufi B, and Fenaux P, on behalf of the Groupe Francophone des Myélodysplasies (GFM). Daily practice management of myelodysplastic syndromes in France:data from 907 patients in a one-week cross-sectional study by the Groupe Francophone des Myélodysplasies. Haematologica. 2010;95:892-899. doi:10.3324/haematol.2009

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“…As a result, there are no imperative reasons for bleeding complications and infections, which are the most frequent causes of death in the general MDS population. 27 Another clinical difference between MDS without del(5q) and MDS with del(5q) contributing to the good overall survival are age and gender: MDS with del(5q) more frequently affects females and the median age at diagnosis is between 5 and 10 years lower than that in the other MDS patients. Again, transfusion dependency, along with low platelet count and complex karyotype were the most important risk factors in our study.…”

Section: Discussionmentioning

confidence: 99%

Survival, prognostic factors and rates of leukemic transformation in 381 untreated patients with MDS and del(5q): A multicenter study

et al. 2012

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Myelodysplastic syndromes (MDS) with del(5q) are considered to have a benign course of the disease. In order to address the issue of the propensity of those patients to progress to acute myeloid leukemia (AML), data on 381 untreated patients with MDS and del(5q) characterized by low or intermediate I International Prognostic Scoring System (IPSS) risk score were collected from nine centers and registries. Median survival of the entire group was 74 months. Transfusion-dependent patients had a median survival of 44 months vs 97 months for transfusion-independent patients (Po0.0001). Transfusion need at diagnosis was the most important patient characteristic for survival. Of the 381 patients, 48 (12.6%) progressed to AML. The cumulative progression rate calculated using the Kaplan --Meier method was 4.9% at 2 years and 17.6% at 5 years. Factors associated with the risk of AML transformation were high-risk World Health Organization adapted Prognostic Scoring System (WPSS) score, marrow blast count 45% and red-cell transfusion dependency at diagnosis. In conclusion, patients with MDS and del(5q) are facing a considerable risk of AML transformation. More detailed cytogenetic and molecular studies may help to identify the patients at risk of progression.

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Platelet counts and haemorrhagic diathesis in patients with myelodysplastic syndromes

Abstract: Thrombocytopenia is a strong predictor of short survival, with or without haemorrhagic complications.

Cited by 64 publications

References 14 publications

Altered immunophenotypic features of peripheral blood platelets in myelodysplastic syndromes

Altered immunophenotypic features of peripheral blood platelets in myelodysplastic syndromes

Daily practice management of myelodysplastic syndromes in France: data from 907 patients in a one-week cross-sectional study by the Groupe Francophone des Myelodysplasies

Survival, prognostic factors and rates of leukemic transformation in 381 untreated patients with MDS and del(5q): A multicenter study

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