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Sisli Etfal Hastan Tip Bul 2018 21;52(4):279-284. Epub 2018 Mar 21.

Department of Pediatric Hematology, Eskişehir Osmangazi University Faculty of Medicine, Eskişehir, Turkey.

Objectives: In immune thrombocytopenic purpura (ITP) treatment, the main goal is achieving the platelet level most rapidly for hemostasis. Pulse steroid therapy is common due to the rapid increase in the platelet count within the first 48 hours. Intravenous (IV) pulse steroid therapy is usually administered as a single methylprednisolone dose in the morning. Read More

Porcine Health Manag 2020 4;6:18. Epub 2020 Aug 4.

Clinic for Swine, Department of Clinical Veterinary Science, Vetsuisse Faculty, University of Bern, 3012 Bern, Switzerland.

Background: Thrombocytopenia is an immune-mediated disease, which affects suckling piglets. Piglets are pale and inactive, show multiple hemorrhages and often die within days. Pathological examination reveals severe haemorrhages and oedema in several organs. Read More

Case Rep Oncol 2020 May-Aug;13(2):655-658. Epub 2020 Jun 15.

Department of Medical Hematology and Oncology, Hamad Medical Corporation, Doha, Qatar.

Idiopathic thrombocytopenic purpura (ITP) is a disease in which the immune system attacks platelets and causes decrease in its number exposing the patient to risk of bleeding. It is diagnosed by exclusion. Eltrombopag is a thrombopoietin receptor agonist which is used as second-line treatment for patients with ITP. Read More

Front Cell Infect Microbiol 2020 16;10:347. Epub 2020 Jul 16.

Instituto de Medicina Molecular João Lobo Antunes, Faculdade de Medicina, Universidade de Lisboa, Lisbon, Portugal.

HIV-1 infection induces B cell defects, not fully recovered upon antiretroviral therapy (ART). Acute infection and the early start of ART provide unique settings to address the impact of HIV on the B cell compartment. We took advantage of a cohort of 21 seroconverters, grouped according to the presence of severe manifestations likely mediated by antibodies or immune complexes, such as Guillain-Barré syndrome and autoimmune thrombocytopenic purpura, with a follow-up of 8 weeks upon effective ART. Read More

Cureus 2020 Jul 1;12(7):e8955. Epub 2020 Jul 1.

Internal Medicine, Kaiser Permanente Vacaville Medical Center, Vacaville, USA.

Our patient presented with a mild upper respiratory infection, which quickly developed into atypical pneumonia. Although atypical pneumonia is a common clinical presentation, our case is unique due to the rare hematologic complications associated with the underlying etiology of this atypical pneumonia case. Most cases of atypical pneumonia are simple, but ours developed immune thrombocytopenic purpura (ITP), which is a rare complication associated with an acute Mycoplasmapneumoniae infection (evidenced by positive IgM titers). Read More

J Thromb Haemost 2020 Aug 5. Epub 2020 Aug 5.

Department of Haemostaseology and Haemophilia Center, University Hospital Frankfurt, Frankfurt am Main, Germany.

Background: Acquired thrombotic thrombocytopenic purpura (aTTP) is a rare, life-threatening autoimmune thrombotic microangiopathy. Current standard of care is therapeutic plasma exchange, immunosuppression, and caplacizumab, an anti-von Willebrand Factor Nanobody, which is effective in treating aTTP episodes.

Patients/methods: Here we report on 7 episodes of aTTP treated without plasma exchange in 6 female patients in Germany and Austria. Read More

Transfusion 2020 Aug 5. Epub 2020 Aug 5.

Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.

Background: The PLASMIC score was developed to identify patients with thrombotic microangiopathy who are most likely to have immune thrombotic thrombocytopenic purpura (TTP) and benefit from therapeutic plasma exchange (TPE). PLASMIC scores of 0-4, 5, and 6-7 are said to correspond to low, intermediate, and high probability of TTP, respectively.

Study Design And Methods: We conducted a systematic review and meta-analysis on the diagnostic accuracy of the PLASMIC score in adults with suspected TTP. Read More

BMC Nephrol 2020 Aug 3;21(1):323. Epub 2020 Aug 3.

Division of Nephrology and Rheumatology, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan.

Background: Thrombotic microangiopathy (TMA) is a histopathological entity associated with microangiopathic hemolytic anemia, thrombocytopenia, and end-organ ischemic damage. Although TMA is caused by various diseases, there have been few reports regarding children with idiopathic nephrotic syndrome (NS) and TMA. Here we report two 1-year-old infants with steroid-resistant NS (SRNS) who presented with severe hypertension, acute kidney injury (AKI), and TMA. Read More

IJU Case Rep 2019 Sep 2;2(5):250-252. Epub 2019 Aug 2.

Department of Urology Osaka University Graduate School of Medicine Suita Osaka Japan.

Introduction: Several types of cancers are reported to induce secondary immune thrombocytopenia resembling immune thrombocytopenic purpura-like syndrome. However, renal cell carcinoma-induced immune thrombocytopenic purpura is an extremely rare phenomenon.

Case Presentation: A 73-year-old male with right renal tumor and multiple enlarged lymph nodes presented severe thrombocytopenia, without bone or hepatic metastasis. Read More

Curr Opin Hematol 2020 Sep;27(5):320-326

Laboratory for Thrombosis Research, IRF Life Sciences, KU Leuven Campus Kulak Kortrijk, Kortrijk, Belgium.

Purpose Of Review: Fundamental knowledge on the role of a disintegrin and metalloprotease with thrombospondin type one repeats, member 13 (ADAMTS13) has been crucial to better understand the pathophysiology of the rare and life-threatening disease thrombotic thrombocytopenic purpura (TTP).

Recent Findings: ADAMTS13 works through a molecular zipper mechanism to proteolyze its substrate von Willebrand factor (VWF). Recent insights into the structure and function of ADAMTS13 led to the identification of an allosteric activation mechanism. Read More

Clin Neurol Neurosurg 2020 Jul 10;197:106081. Epub 2020 Jul 10.

George Washington University, Department of Neurology, Washington, DC, United States. Electronic address:

Transfus Apher Sci 2020 Jul 22:102885. Epub 2020 Jul 22.

Division of Transfusion Medicine and Hemostasis, Department of Pathology, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd., Dallas, TX, 75390, USA; Division of Hematology and Oncology, Department of Internal Medicine, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd., Dallas, TX, 75390, USA.

The standard dose of rituximab used in B-cell hematological malignancies, 375 mg/m weekly, may be excessive for autoimmune conditions. Successful use of a low, fixed dose of 100-200 mg of rituximab, weekly for 4 weeks, has been reported in the literature in the treatment of autoimmune thrombotic thrombocytopenic purpura (aTTP). We retrospectively analyzed our rituximab data in aTTP over a 13-year-period for 39 patients, with the aim of comparing response and outcomes with a standard lymphoma-dose course versus a low fixed 100 mg-dose course. Read More

Br J Haematol 2020 Aug 1. Epub 2020 Aug 1.

Department of Hematology and Oncology, University of Vermont Medical Center, Burlington, VT, USA.

Adv Ther 2020 Jul 29. Epub 2020 Jul 29.

Department of Anesthesiology, LSU Health Shreveport, Shreveport, LA, USA.

Introduction: Platelet diseases and dysfunction are taught early in medical school to all future physicians. Understanding of the coagulation cascade and hemostatic mechanisms has allowed for targeted pharmacological therapies that have been significantly impactful in clinical practice. Platelets are an early participant in hemostasis physiologically and under pathophysiological states. Read More

Rev Med Interne 2020 Jul 26. Epub 2020 Jul 26.

Centre de Référence des Microangiopathies Thrombotiques, Hôpital Saint-Antoine, AP-HP, Paris, France; Service d'Hématologie, AP-HP.SU, Paris, France; INSERM UMRS 1138, Paris, France. Electronic address:

Introduction: Thrombotic thrombocytopenic purpura (TTP) is a devastating disease characterized by disseminated microvascular thrombosis. Despite pro-thrombotic predisposing conditions, the prevalence of macrovascular venous thrombosis event (VTE) in immune-mediated TTP (iTTP) has rarely been assessed.

Methods: We reviewed data of all iTTP patients of the French reference Center for thrombotic microangiopathies registry prospectively enrolled through a 10-year period, between 2008 and 2018. Read More

Hamostaseologie 2020 Aug 29;40(3):322-336. Epub 2020 Jul 29.

Service d'Hématologie Biologique, Hôpital Lariboisière, and EA3518, Institut de Recherche Saint-Louis, Hôpital Saint Louis, AP-HP.Nord, Université de Paris, Paris, France.

Thrombotic thrombocytopenic purpura (TTP) is a rare, relapsing, and life-threatening disorder with an annual incidence of 10 cases per million people. TTP is a thrombotic microangiopathy characterized by severe thrombocytopenia, microangiopathic hemolytic anemia, and organ ischemia. The disease is caused by a severe deficiency of the enzyme ADAMTS13 (), which can either be acquired, mainly by autoantibodies targeting ADAMTS13, or congenital due to mutations in the gene. Read More

Ann Pharmacother 2020 07 25:1060028020941852. Epub 2020 Jul 25.

Houston Methodist West Hospital, Houston, TX, USA.

Objective: To review the efficacy and safety of caplacizumab and ravulizumab for thrombotic microangiopathy.

Data Sources: A literature search from January 2011 to May 2020 was performed using the key terms caplacizumab (or ALX-0681), ravulizumab (or ALXN1210), atypical hemolytic uremic syndrome (aHUS), acquired thrombotic thrombocytopenic purpura (aTTP), and thrombotic microangiopathy.

Study Selection And Data Extraction: Relevant clinical trials and articles in the English language were identified and reviewed. Read More

Rev Med Interne 2020 Jul 24. Epub 2020 Jul 24.

Service de médecine interne, Centre de références sur les cytopénies auto-immunes de l'adulte, CHU Henri Mondor, APHP, UPEC, 94010 Créteil, France.

The first line treatment of immune thrombocytopenic purpura (ITP) is well established and based on short course of corticosteroids associated with intravenous immunoglobulins (IVIg) for the most severe forms. Predniso(lo)ne is the corticosteroid agent usually given but dexamethasone appears as an alternative. Some guidelines recommend to use dexamethasone as first line when a rapid increase of platelet count is required. Read More

Turk J Haematol 2020 Jul 23. Epub 2020 Jul 23.

Marmara University Faculty of Medicine, Department of Hematology, İstanbul, Turkey.

Cureus 2020 Jul 19;12(7):e9283. Epub 2020 Jul 19.

Internal Medicine, Saint Peter's University Hospital/Rutgers University, New Brunswick, USA.

Thrombotic thrombocytopenic purpura (TTP) is typically characterized by the symptomatic pentad of fever, thrombocytopenia, microangiopathic hemolytic anemia, neurologic abnormalities, and renal failure. Atypical TTP is the diagnosis used to describe the subset of patients with TTP who present with symptoms that deviate from the classic pentad. We report a case an 86-year-old woman who presented to the emergency department complaining of chest pain for one day. Read More

Cureus 2020 Jun 19;12(6):e8705. Epub 2020 Jun 19.

Hematology and Oncology, Hamad General Hospital, Doha, QAT.

Idiopathic thrombocytopenic purpura (ITP) is a disease in which the immune system attacks platelets and causes a decrease in their number, exposing the patient to bleeding risk. It is a diagnosis by exclusion. ITP usually presents as acute disease and is self-limiting in pediatric patients, while it tends to be chronic in adults. Read More

Trop Med Health 2020 20;48:59. Epub 2020 Jul 20.

Base Hospital Mahaoya, Mahaoya, Sri Lanka.

Background: Dengue is a common mosquito-borne infection in tropical countries. Dengue incidence in Sri Lanka is generally showing a rising trend. Both chronic immune thrombocytopenia purpura (ITP) children and chronic ITP triggered by dengue fever in the pediatric age group are rarely reported. Read More

Ann Rheum Dis 2020 Jul 22. Epub 2020 Jul 22.

Department of Internal Medicine, Laboratory of Molecular Medicine and Human Genetics, University of Crete, Heraklion, Greece.

Ann Rheum Dis 2020 Jul 22. Epub 2020 Jul 22.

Institute of Medicine, Chung Shan Medical University, Taichung, Taichung, Taiwan

Blood Transfus 2020 07;18(4):328

Department of Hematology, "San Carlo" Regional Hospital, Potenza, Italy.

Transfusion 2020 Jul 21. Epub 2020 Jul 21.

Department of Pathology and Laboratory Medicine, University of North Carolina, Chapel Hill, North Carolina, USA.

Background: Despite rapid and intensive treatments with therapeutic plasma exchange (TPE) and immunosuppression, immune thrombotic thrombocytopenic purpura (TTP) patients are at risk of disease exacerbation, i.e., early recurrence of TTP within 30 days of achieving treatment response. Read More

Ann Hematol 2020 Sep 20;99(9):2081-2084. Epub 2020 Jul 20.

College of Medicine, University of Arizona, Phonix, AZ, USA.

Aortic valve disease (AVD) has similarities to atherosclerosis in the case of aortic stenosis. The important role of platelet in the pathogenesis of atherosclerosis is known. The goal of this study is to evaluate whether platelet disorders play any role in aortic valve disease. Read More

Thromb Res 2020 Jul 5;195:136-138. Epub 2020 Jul 5.

University of Health Sciences Bakırkoy Dr. Sadi Konuk Training and Research Hospital, Department of Microbiology and Infectious Diseases, Istanbul, Turkey.

Am J Med 2020 Jul 17. Epub 2020 Jul 17.

Department of Medicine, Westchester Medical Center and New York Medical College, Valhalla, NY.

Background: Despite widespread availability of plasmapheresis, the mortality in thrombotic thrombocytopenic purpura remains high. Cardiovascular complications have been reported as an important cause of morbidity in these patients. The burden and prognostic implications of these complications have not been well studied. Read More

Res Pract Thromb Haemost 2020 Jul 25;4(5):918-930. Epub 2020 Jun 25.

Laboratory for Thrombosis Research IRF Life Sciences KU Leuven Campus Kulak Kortrijk Kortrijk Belgium.

Background: In immune-mediated thrombotic thrombocytopenic purpura (iTTP), patients develop an immune response against the multidomain enzyme ADAMTS13. ADAMTS13 consists of a metalloprotease (M) and disintegrin-like (D) domain, 8 thrombospondin type 1 repeats (T1-T8), a cysteine-rich (C), a spacer (S), and 2 CUB domains (CUB1-2). Previous epitope mapping studies have used relatively large overlapping ADAMTS13 fragments. Read More

Res Pract Thromb Haemost 2020 Jul 21;4(5):807-812. Epub 2020 Jun 21.

Division of Hematology Massachusetts General Hospital Harvard Medical School Boston MA USA.

Background: Complement may contribute to platelet destruction in immune thrombocytopenia (ITP), but serum complement levels of ITP patients are not well defined. This study characterized C3, C4, and CH50 levels from 108 ITP patients in comparison with 120 healthy subjects.

Methods: Results of complement testing performed using commercially available turbidimetric immunoassays were retrospectively analyzed. Read More

Clin Exp Immunol 2020 Jul 18. Epub 2020 Jul 18.

Department of Medicine, Division of Hematology and Medical Oncology, Weill Cornell Medical College, New York, NY, USA.

Involvement of the alternative complement pathway (AP) in microvascular endothelial cell (MVEC) injury characteristic of a thrombotic microangiopathy (TMA) is well documented. However, the role of the lectin pathway (LP) of complement has not been explored. We examined mannose-binding lectin associated serine protease (MASP2), the effector enzyme of the LP, in thrombotic thrombocytopenic purpura, atypical hemolytic uremic syndrome and post-allogeneic hematopoietic stem cell transplantation (alloHSCT) TMAs. Read More

Br J Haematol 2020 Jul 17. Epub 2020 Jul 17.

Department of Haematology, University College London NHS Foundation Trust, London, UK.

Haematology patients receiving chemo- or immunotherapy are considered to be at greater risk of COVID-19-related morbidity and mortality. We aimed to identify risk factors for COVID-19 severity and assess outcomes in patients where COVID-19 complicated the treatment of their haematological disorder. A retrospective cohort study was conducted in 55 patients with haematological disorders and COVID-19, including 52 with malignancy, 2 with bone marrow failure and 1 immune mediated thrombotic thrombocytopenic purpura (TTP). Read More

Sci Rep 2020 Jul 16;10(1):11717. Epub 2020 Jul 16.

Preventive Medicine and Public Health Research Center, Psychosocial Health Research Institute, Department of Community and Family Medicine, School of Medicine, Iran University of Medical Sciences, Tehran, Iran.

Atopic dermatitis (AD) is a chronic disease affecting 10-30% of children and 2-10% of adults worldwide. It is manifested by the pruritus eczema lesions on the skin. Immune thrombocytopenic purpura (ITP) is the most common cause of acute onset of thrombocytopenia in childhood. Read More

Int J Hematol 2020 Jul 16. Epub 2020 Jul 16.

Intensive Care Unit, Poissy Saint Germain Hospital, 9-10 rue du champ Gaillard, 78300, Poissy, France.

This case report describes immune thrombocytopenic purpura in a 41-year-old man hospitalized in the intensive-care unit for COVID-19, 13 days after the onset of COVID-19 symptoms with respiratory failure at admission. Acute respiratory distress syndrome was treated with, among other drugs, low-molecular-weight heparin. On day 8, his platelet count began descending rapidly. Read More

Int J Lab Hematol 2020 Jul 16. Epub 2020 Jul 16.

Western Australia Centre for Thrombosis and Haemostasis, Perth Blood Institute, Murdoch University, Perth, WA, Australia.

This guidance document was prepared on behalf of the International Council for Standardization in Haematology (ICSH), by the ADAMTS13 Assay Working Group, which comprises an international group of both clinical and laboratory experts. The document provides recommendations on best practice for the performance of ADAMTS13 assays in clinical laboratories. ADAMTS13 assays support the differential diagnosis of thrombotic microangiopathies and have utility in the management of thrombotic thrombocytopenic purpura (TTP). Read More

Cureus 2020 Jul 9;12(7):e9097. Epub 2020 Jul 9.

Hematology and Oncology, Loyola University Medical Center, Maywood, USA.

Vitamin B12 deficiency is classically associated with megaloblastic anemia. Possible cobalamin deficiency is not investigated once hemolysis is seen. Around 2. Read More

Eur J Case Rep Intern Med 2020 9;7(7):001751. Epub 2020 Jun 9.

Hôpitaux Privés de Metz, Metz, France.

We described three COVID-19-infected patients with profound immune thrombocytopenia causing haemorrhagic mucocutaneous complications. We conclude that an immune mechanism was responsible as common causes were excluded. Since corticoids were considered harmful in the circumstances, the patients were successfully treated with intravenous immunoglobulins without later relapse. Read More

Pediatr Blood Cancer 2020 Jul 13:e28534. Epub 2020 Jul 13.

St Jude Children's Research Hospital, Memphis, Tennessee.

Indian J Hematol Blood Transfus 2020 Jul 10;36(3):575-577. Epub 2020 Jan 10.

Hemato-Oncology Division, University of Massachusetts Medical School, Worcester, MA 01608 USA.

Cell Commun Signal 2020 Jul 9;18(1):107. Epub 2020 Jul 9.

Institute of Biology and Medicine, College of Life and Health Sciences, Wuhan University of Science and Technology, Wuhan, Hubei, 430081, PR China.

Background: Foxp3CD4 regulatory T cells (Treg) constitutes a key event in autoimmune diseases. STAT5b is the critical link between the IL-2/15 and FOXP3, the master regulator of Treg cells.

Methods: The CD3T cell and Foxp3CD4 regulatory T cells were overexpressioned or knockdown MKL-1 and STAT5a and tested for Treg cell development and function. Read More

Cureus 2020 Jun 6;12(6):e8477. Epub 2020 Jun 6.

Oncology, Hematology & Oncology Associates of Northeastern Pennsylvania, Scranton, USA.

Acquired thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy with varied etiology and manifestations. It is uncommon for acute pancreatitis to trigger TTP. A 59-year-old man hospitalized with acute pancreatitis developed fever, acute kidney injury, thrombocytopenia, and microangiopathic hemolytic anemia (MAHA) on his second day in the hospital. Read More

BMJ Case Rep 2020 Jul 7;13(7). Epub 2020 Jul 7.

Service and Central Laboratory of Hematology, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland

We report on a patient with coronavirus disease 2019 (COVID-19) and decompensated cirrhosis who experienced a favourable outcome of severe immune thrombocytopaenic purpura (ITP) after administration of intravenous immunoglobulin and high-dose dexamethasone. The present case suggests that it is reasonable to evoke ITP in case of profound thrombocytopaenia in a patient with COVID-19. Read More

Blood Adv 2020 Jul;4(13):3093-3101

Department of Nephrology and Hypertension, Medical School Hannover, Hannover, Germany.

Introduction of the nanobody caplacizumab was shown to be effective in the treatment of acquired thrombotic thrombocytopenic purpura (aTTP) in the acute setting. The official recommendations include plasma exchange (PEX), immunosuppression, and the use of caplacizumab for a minimum of 30 days after stopping daily PEX. This study was a retrospective, observational analysis of the use of caplacizumab in 60 patients from 29 medical centers in Germany. Read More

Blood Adv 2020 Jul;4(13):3085-3092

Department of Nephrology and Hypertension, Medical School Hannover, Hannover, Germany.

Acquired thrombotic thrombocytopenic purpura (aTTP) is a rare but life-threatening condition. In 2018, the nanobody caplacizumab was approved for the treatment of adults experiencing an acute episode of aTTP, in conjunction with plasma exchange (PEX) and immunosuppression for a minimum of 30 days after stopping daily PEX. We performed a retrospective, observational analysis on the use of caplacizumab in 60 patients from 29 medical centers in Germany during acute disease management. Read More

Int J Clin Pharmacol Ther 2020 07 7. Epub 2020 Jul 7.

Objective: Thrombotic microangiopathy (TMA), often described as thrombotic thrombocytopenic purpura (TTP) or hemolytic uremic syndrome (HUS), is clinically problematic because it is life-threatening. However, up-to-date information on drugs inducing TMA is limited in the real-world setting. The purpose of this study was to clarify drugs associated with TMA using a spontaneous reporting system database. Read More

BMJ Case Rep 2020 Jul 5;13(7). Epub 2020 Jul 5.

Hematology/Oncology, Brookdale University Hospital Medical Center, Brooklyn, New York, USA.

A 21-year-old man presented to the emergency department with generalised weakness, weight loss and decreased appetite for few weeks. He had evidence of severe pancytopenia and haemolysis. His peripheral smear with many schistocytes was suspicious for thrombotic thrombocytopenic purpura (TTP). Read More

Thromb Res 2020 Jun 27;193:154-159. Epub 2020 Jun 27.

Department of Internal Medicine, Seoul National University Hospital, Seoul, Republic of Korea; Center for Precision Medicine, Seoul National University Hospital, Seoul, Republic of Korea; Cancer Research Institute, Seoul National University College of Medicine, Seoul, Republic of Korea; Biomedical Research Institute, Seoul National University Hospital, Seoul, Republic of Korea. Electronic address:

Considering difficulties in on-site ADAMTS13 testing and the performance instability of PLASMIC score according to ethnicity, we developed a prediction tool, MED-TMA (machine learning (ML) method for differential diagnosis (DDx) of thrombotic microangiopathy (TMA)) to support clinical decision. Data from 319 patients visiting 31 hospitals in Korea clinically diagnosed with primary TMA was randomly separated by 2:1 into two groups - the development dataset (D-set, n = 212), the validation dataset (V-set, n = 107). Feature elimination was conducted to select optimal clinical predictors. Read More

Clin Exp Optom 2020 Jul 2. Epub 2020 Jul 2.

School of Optometry, University of Alabama at Birmingha, Birmingham, Alabama, USA.

Haematologica 2020 Jul 2. Epub 2020 Jul 2.

Dept of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy