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Platelet and immune characteristics of immune thrombocytopaenia patients non‐responsive to therapy reveal severe immune dysregulation
Author(s) -
Monzón Manzano Elena,
Álvarez Román María Teresa,
Justo Sanz Raúl,
Fernández Bello Ihosvany,
Hernández Diana,
Martín Salces Mónica,
Valor Larissa,
Rivas Pollmar Isabel,
Butta Nora V.,
Jiménez Yuste Víctor
Publication year - 2020
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/bjh.16459
Subject(s) - immune system , platelet , sialic acid , immunology , medicine , thrombopoietin , endocrinology , chemistry , haematopoiesis , biology , biochemistry , stem cell , genetics
Summary Multifactorial mechanisms leading to diminished platelet counts in immune thrombocytopaenia (ITP) might condition the ability of patients with ITP to respond to treatments. Examining their platelet and immune features, we aimed to detect singular characteristics of patients with ITP who do not respond to any treatment. We studied patients with chronic primary ITP who had been without treatment, or untreated (UT‐ITP), for at least six months; included were responders to agonists of thrombopoietin receptors (TPO‐RA), patients who showed no response to first‐ and second‐line treatments (NR‐ITP), and healthy controls. Platelets from NR‐ITP patients exposed a reduced amount of sialic acid residues. Increased loss of platelet surface sialic acid residues was associated with increased platelet apoptosis. NR‐ITP patients had an increased fraction of naive lymphocyte (L) B cells and a reduced LTreg (Lymphocyte T‐regulator) subset. They also presented an anomalous monocyte and NK (Natural Killer) cells distribution. TPO‐RA‐treated patients seemed to recover an immune homeostasis similar to healthy controls. In conclusion, our results indicate a severe deregulation of the immune system of NR‐ITP. The inverse correlation between loss of sialic acid and LTreg count suggests a potential relationship between glycan composition on the platelet surface and immune response, positing terminal sugar moieties of the glycan chains as aetiopathogenic agents in ITP.