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Diagnosis and Management of Thrombocytopenia
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Diagnosis and Management of Thrombocytopenia

A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Hematology".

Deadline for manuscript submissions: closed (31 July 2021) | Viewed by 13197

Special Issue Editor

Prof. Dr. Marie Scully

E-Mail Website
Guest Editor
1. Department of haematology, University College Hospital, London, UK
2. Cardiometabolic Programme-NIHR UCLH/UCL BRC, London, UK
Interests: immune thrombocytopenic purpura (ITP); thrombotic thrombocytopenic purpura (TTP); hemolytic uremic syndrome (HUS); disseminated intravascular coagulation (DIC); acute thrombocytopenia

Special Issue Information

Dear Colleagues,

Thrombocytopenia is a common haematological finding that may be isolated or associated with abnormalities of other haematological laboratory parameters. Excluding leukaemia, the differential diagnosis of acute thrombocytopenia includes immune thrombocytopenic purpura (ITP), thrombotic thrombocytopenic purpura (TTP), or thrombotic microangiopathy (TMA) and disseminated intravascular coagulation (DIC). The importance of diagnosing and differentiating these conditions ensures the correct treatment is given, which could have a major impact on patient outcomes. All of these acute conditions may have comparable clinical phenotypes, typically epithelial type bleeding, but may have a thrombotic risk. Treatments may be supportive or specific, for example, for ITP or TMAs for which immunosuppression or plasma exchange would respectively be initiated. These three conditions can affect any age group and are precipitated by an underlying trigger to varying degrees.

ITP is a relatively common condition and is diagnoses of exclusion, requiring steroids, blood products, or supportive pharmacological agents such as tranexamic acid. The use of newer therapies such as thrombopoietin agonists has improved acute and long-term outcomes for patients.

DIC is a syndrome for which there is an underlying trigger, typically sepsis, malignancy, pregnancy, or trauma. The treatment of DIC is symptomatic; treatment of the underlying trigger is critical in order to prevent disease progression.

TMAs are diagnosed by microangiopathic haemolytic anaemia, thrombocytopenia, and organ dysfunction secondary to microvascular thrombi. Confirmation of a diagnosis of TTP is by severe ADAMTS 13 activity deficiency, whereas HUS is a diagnosis of exclusion related to complement dysregulation or toxin from bacterial gut pathogens such as Escherichia coli. These conditions are medical emergencies, and acute and long-term therapies will be discussed.

Prof. Dr. Marie Scully
Guest Editor

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Journal of Clinical Medicine is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2600 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • immune thrombocytopenic purpura (ITP)
  • thrombotic thrombocytopenic purpura (TTP)
  • hemolytic uremic syndrome (HUS)
  • disseminated intravascular coagulation (DIC)
  • acute thrombocytopenia
  • diagnosis
  • treatment

Published Papers (3 papers)

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Research

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13 pages, 2782 KiB  
Article
The Importance of Platelet Glycoside Residues in the Haemostasis of Patients with Immune Thrombocytopaenia
by Andrés Ramírez-López, María Teresa Álvarez Román, Elena Monzón Manzano, Paula Acuña, Elena G. Arias-Salgado, Mónica Martín Salces, María Isabel Rivas Pollmar, Víctor Jiménez Yuste, Raul Justo Sanz, Sara García Barcenilla, Tamara Cebanu, Elena González Zorrilla and Nora V. Butta
J. Clin. Med. 2021, 10(8), 1661; https://doi.org/10.3390/jcm10081661 - 13 Apr 2021
Cited by 8 | Viewed by 2495
Abstract
Loss of sialic acid from the carbohydrate side chains of platelet glycoproteins can affect platelet clearance, a proposed mechanism involved in the etiopathogenesis of immune thrombocytopaenia (ITP). We aimed to assess whether changes in platelet glycosylation in patients with ITP affected platelet counts, [...] Read more.
Loss of sialic acid from the carbohydrate side chains of platelet glycoproteins can affect platelet clearance, a proposed mechanism involved in the etiopathogenesis of immune thrombocytopaenia (ITP). We aimed to assess whether changes in platelet glycosylation in patients with ITP affected platelet counts, function, and apoptosis. This observational, prospective, and transversal study included 82 patients with chronic primary ITP and 115 healthy controls. We measured platelet activation markers and assayed platelet glycosylation and caspase activity, analysing samples using flow cytometry. Platelets from patients with ITP with a platelet count <30 × 103/µL presented less sialic acid. Levels of α1,6-fucose (a glycan residue that can directly regulate antibody-dependent cellular cytotoxicity) and α-mannose (which can be recognised by mannose-binding-lectin and activate the complement pathway) were increased in the platelets from these patients. Platelet surface exposure of other glycoside residues due to sialic acid loss inversely correlated with platelet count and the ability to be activated. Moreover, loss of sialic acid induced the ingestion of platelets by human hepatome HepG2 cells. Changes in glycoside composition of glycoproteins on the platelets’ surface impaired their functional capacity and increased their apoptosis. These changes in platelet glycoside residues appeared to be related to ITP severity. Full article
(This article belongs to the Special Issue Diagnosis and Management of Thrombocytopenia)
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Review

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15 pages, 1109 KiB  
Review
Potential Diagnostic Approaches for Prediction of Therapeutic Responses in Immune Thrombocytopenia
by Anne-Tess C. Jolink, Vivianne S. Nelson, Martin R. Schipperus, Sufia N. Amini, Gestur Vidarsson, C. Ellen van der Schoot, Leendert Porcelijn, Masja de Haas and Rick Kapur
J. Clin. Med. 2021, 10(15), 3403; https://doi.org/10.3390/jcm10153403 - 30 Jul 2021
Cited by 12 | Viewed by 2682
Abstract
Immune thrombocytopenia (ITP) is an autoimmune bleeding disorder in which, via unresolved mechanisms, platelets and megakaryocytes (MKs) are targeted by autoantibodies and/or T cells resulting in increased platelet destruction and impairment of MK function. Over the years, several therapeutic modalities have become available [...] Read more.
Immune thrombocytopenia (ITP) is an autoimmune bleeding disorder in which, via unresolved mechanisms, platelets and megakaryocytes (MKs) are targeted by autoantibodies and/or T cells resulting in increased platelet destruction and impairment of MK function. Over the years, several therapeutic modalities have become available for ITP, however, therapeutic management has proven to be very challenging in several cases. Patients refractory to treatment can develop a clinically worsening disease course, treatment-induced toxicities and are predisposed to development of potentially life-endangering bleedings. It is therefore of critical importance to timely identify potential refractory patients, for which novel diagnostic approaches are urgently needed in order to monitor and predict specific therapeutic responses. In this paper, we propose promising diagnostic investigations into immune functions and characteristics in ITP, which may potentially be exploited to help predict platelet count responses and thereby distinguish therapeutic responders from non-responders. This importantly includes analysis of T cell homeostasis, which generally appears to be disturbed in ITP due to decreased and/or dysfunctional T regulatory cells (Tregs) leading to loss of immune tolerance and initiation/perpetuation of ITP, and this may be normalized by several therapeutic modalities. Additional avenues to explore in possible prediction of therapeutic responses include examination of platelet surface sialic acids, platelet apoptosis, monocyte surface markers, B regulatory cells and platelet microparticles. Initial studies have started evaluating these markers in relation to response to various treatments including glucocorticosteroids (GCs), intravenous immunoglobulins (IVIg) and/or thrombopoietin receptor agonists (TPO-RA), however, further studies are highly warranted. The systematic molecular analysis of a broad panel of immune functions may ultimately help guide and improve personalized therapeutic management in ITP. Full article
(This article belongs to the Special Issue Diagnosis and Management of Thrombocytopenia)
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19 pages, 685 KiB  
Review
Emerging Therapies in Immune Thrombocytopenia
by Sylvain Audia and Bernard Bonnotte
J. Clin. Med. 2021, 10(5), 1004; https://doi.org/10.3390/jcm10051004 - 2 Mar 2021
Cited by 32 | Viewed by 7411
Abstract
Immune thrombocytopenia (ITP) is a rare autoimmune disorder caused by peripheral platelet destruction and inappropriate bone marrow production. The management of ITP is based on the utilization of steroids, intravenous immunoglobulins, rituximab, thrombopoietin receptor agonists (TPO-RAs), immunosuppressants and splenectomy. Recent advances in the [...] Read more.
Immune thrombocytopenia (ITP) is a rare autoimmune disorder caused by peripheral platelet destruction and inappropriate bone marrow production. The management of ITP is based on the utilization of steroids, intravenous immunoglobulins, rituximab, thrombopoietin receptor agonists (TPO-RAs), immunosuppressants and splenectomy. Recent advances in the understanding of its pathogenesis have opened new fields of therapeutic interventions. The phagocytosis of platelets by splenic macrophages could be inhibited by spleen tyrosine kinase (Syk) or Bruton tyrosine kinase (BTK) inhibitors. The clearance of antiplatelet antibodies could be accelerated by blocking the neonatal Fc receptor (FcRn), while new strategies targeting B cells and/or plasma cells could improve the reduction of pathogenic autoantibodies. The inhibition of the classical complement pathway that participates in platelet destruction also represents a new target. Platelet desialylation has emerged as a new mechanism of platelet destruction in ITP, and the inhibition of neuraminidase could dampen this phenomenon. T cells that support the autoimmune B cell response also represent an interesting target. Beyond the inhibition of the autoimmune response, new TPO-RAs that stimulate platelet production have been developed. The upcoming challenges will be the determination of predictive factors of response to treatments at a patient scale to optimize their management. Full article
(This article belongs to the Special Issue Diagnosis and Management of Thrombocytopenia)
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