Platelet features allow to differentiate immune thrombocytopenia from inherited thrombocytopenia

Bonnard, G.; Babuty, Antoine; Collot, R.; Costes, Domitille; Drillaud, Nicolas; Eveillard, Marion; Néel, A.; Espitia, Alexandra; Masseau, A.; Wahbi, A.; Hamidou, M.; Béné, Marie C.; Fouassier, Marc

doi:10.1007/s00277-021-04651-4

Cited by 7 publications

(10 citation statements)

References 20 publications

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“…This is in agreement with previous studies that have attempted to define IPF thresholds to distinguish central from peripheral thrombocytopenias 43,44 . It has been shown that, in MTP, the IPF is increased (three‐ to five‐times higher than in ITP) 45,46 . Nevertheless, there are no validated normal values for IPF levels to date, although several studies have measured IPF in cohorts of healthy adults, yielding ranges of 0.2% to 7.7% 43–46 .…”

Section: Discussionsupporting

confidence: 90%

“…43,44 It has been shown that, in MTP, the IPF is increased (three-to five-times higher than in ITP). 45,46 Nevertheless, there are no validated normal values for IPF levels to date, although several studies have measured IPF in cohorts of healthy adults, yielding ranges of 0.2% to 7.7%. [43][44][45][46] Finally, a recent study found that an IPF level of >11 × 10 9 /l is a strong argument in favour of constitutional thrombocytopenia, 46 which is confirmed here.…”

Section: Discussionmentioning

confidence: 99%

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Biological, clinical features and modelling of heterozygous variants of glycoprotein Ib platelet subunit alpha (GP1BA) and glycoprotein Ib platelet subunit beta (GP1BB) genes responsible for constitutional thrombocytopenia

et al. 2022

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Constitutional thrombocytopenias are rare disorders, often difficult to discriminate from acquired thrombocytopenias. More than 80 genes have been described as being at the origin of these diseases. Among them, several variants of the glycoprotein Ib platelet subunit alpha (GP1BA) and glycoprotein Ib platelet subunit beta (GP1BB) genes, coding for the GpIb-IX-V glycoprotein complex, have been reported in the literature. The study reported here aimed at describing newly identified monoallelic anomalies affecting the GP1BA and GP1BB genes on a clinical, biological and molecular level. In a cohort of nine patients with macrothrombocytopenia, eight heterozygous variants of the GP1BA or GP1BB genes were identified. Five of them had never been described in the heterozygous state. Computer modelling disclosed structure/ function relationships of these five variants.

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Section: Discussionsupporting

confidence: 90%

Section: Discussionmentioning

confidence: 99%

Biological, clinical features and modelling of heterozygous variants of glycoprotein Ib platelet subunit alpha (GP1BA) and glycoprotein Ib platelet subunit beta (GP1BB) genes responsible for constitutional thrombocytopenia

et al. 2022

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“…Another mechanism that contributes to the platelet clearance is platelet activation ( 4 ). In two pilot studies, it was reported that platelet activation markers, such as P-selectin, CD62 and CD11b expression, were significantly higher in ITP patients ( 5 , 6 ). In the ITP mouse model, Fc-independent platelet activation was observed, which led to platelet clearance in the liver ( 7 ).…”

Section: Introductionmentioning

confidence: 99%

Associations of Gut Microbiota and Fatty Metabolism With Immune Thrombocytopenia

Zheng

et al. 2022

Front. Med.

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ObjectiveTo determine whether gut microbiota, fatty metabolism and cytokines were associated with immune thrombocytopenia (ITP).MethodsIn total, 29 preliminarily diagnosed ITP patients and 33 healthy volunteers were enrolled. Fecal bacterial were analyzed based on 16S rRNA sequencing. Plasma cytokines and motabolites were analyzed using flow cytometry and liquid chromatography-mass spectrometry (LC-MS), respectively.ResultsBacteroides, Phascolarctobacterium, and Lactobacillus were enriched at the genus level in ITP patients, while Ruminococcaceae UCG-002, Eubacterium coprostanoligeues, Megamonas, and Lachnospiraceae NC2004 were depleted. At the phylum level, the relative abundance of Proteobacteria and Chloroflexi increased in ITP patients, while Firmicutes, Actinobacteria, and the Firmicutes/Bacteroidetes ratio decreased. Plasma levels of 5-hydroxyeicosatetraenoic acid (5-HETE), 6-trans-12-epi-leukotriene B4 (6t,12e-LTB4), and resolvin D2 (RvD2) were upregulated, and stachydrine, dowicide A, dodecanoylcarnitine were downregulated in ITP patients. Furthermore, RvD2 is positively correlated with order Bacteroidetes VC2.1 Bac22, 5-HETE is positively correlated with genus Azospirillum, and 6t,12e-LTB4 is positively correlated with genus Cupriavidus. In addition, stachydrine is positively correlated with family Planococcaceae, dowicide A is positively correlated with class MVP-15, and dodecanoylcarnitine is positively correlated with order WCHB1-41. Plasma levels of interleukin-6 (IL-6) and tumor necrosis factor-α (TNF-α) were upregulated in ITP patients.ConclusionOur study revealed a relationship between microbiota and fatty metabolism in ITP. Gut microbiota may participate in the pathogenesis of ITP through affecting cytokine secretion, interfering with fatty metabolism.

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“…RCA-I and ECL preferentially bind to glycans terminating in Gal or GalNAc, the monosaccharides expected to be exposed following cleavage of Sia. In several flow cytometry studies, ITP platelets were shown to have increased binding to RCA-I and/or ECL compared with healthy platelets, suggesting desialylation of platelet glycans [27–31].…”

Section: Sialic Acid In Immune Thrombocytopenia Pathophysiologymentioning

confidence: 99%

New insights into the glycobiology of immune thrombocytopenia

Tiemeyer

Kuter

Cairo

et al. 2023

Current Opinion in Hematology

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Purpose of review The platelet surface harbors a lush forest of glycans (carbohydrate polymers) attached to membrane proteins and lipids. Accumulating evidence suggests that these glycans may be relevant to the pathophysiology of immune thrombocytopenia (ITP). Here, we critically evaluate data that point to a possible role for loss of sialic acid in driving platelet clearance in ITP, comment on the potential use of neuraminidase inhibitors for treatment of ITP, and highlight open questions in this area. Recent findings Multiple lines of evidence suggest a role for loss of platelet sialic acid in the pathophysiology of thrombocytopenia. Recent work has tested the hypothesis that neuraminidase-mediated cleavage of platelet sialic acid may trigger clearance of platelets in ITP. Some clinical evidence supports efficacy of the viral neuraminidase inhibitor oseltamivir in ITP, which is surprising given its lack of activity against human neuraminidases. Summary Further study of platelet glycobiology in ITP is necessary to fill key knowledge gaps. A deeper understanding of the roles of platelet glycans in ITP pathophysiology will help to guide development of novel therapies.

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Platelet features allow to differentiate immune thrombocytopenia from inherited thrombocytopenia

Cited by 7 publications

References 20 publications

Biological, clinical features and modelling of heterozygous variants of glycoprotein Ib platelet subunit alpha (GP1BA) and glycoprotein Ib platelet subunit beta (GP1BB) genes responsible for constitutional thrombocytopenia

Biological, clinical features and modelling of heterozygous variants of glycoprotein Ib platelet subunit alpha (GP1BA) and glycoprotein Ib platelet subunit beta (GP1BB) genes responsible for constitutional thrombocytopenia

Associations of Gut Microbiota and Fatty Metabolism With Immune Thrombocytopenia

New insights into the glycobiology of immune thrombocytopenia

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