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J Thromb Haemost 2021 Aug;19(8):2082-2088

Department of Medicine, McMaster Centre for Transfusion Research, McMaster University, Hamilton, ON, Canada.

Background: Immune thrombocytopenia (ITP) is an autoimmune disease characterized by low platelet counts and increased risk of bleeding. In preparation for an upcoming guideline, the ITP Emergency Management Guideline Panel, including clinical experts in hematology, emergency medicine, research methodology, and patient representatives, identified the need for a standardized definition of a critical ITP bleed. The goal of the definition was to distinguish critical bleeds from bleeds that may not require urgent treatment, typically in the context of severe thrombocytopenia. Read More

Hamostaseologie 2021 Jul 29. Epub 2021 Jul 29.

Department of Internal Medicine III, Comprehensive Cancer Center, University Medical Center of the Johannes Gutenberg University, Mainz, Germany.

Immune thrombotic thrombocytopenic purpura (iTTP) is a rare autoimmune disorder characterized by severely reduced activity of the von Willebrand factor (VWF)-cleaving protease ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) due to autoantibodies. This leads to the development of pathogenic multimers of VWF, causing a thrombotic microangiopathy with decreased number of platelets, hemolysis, and life-threatening tissue ischemia of mostly brain, heart, and kidneys. Standard treatment of iTTP involves daily plasma exchange to remove ultra large multimers of VWF, inhibitors, substituting ADAMTS13, and the accompaniment of an immunosuppressive treatment with steroids. Read More

Blood 2021 Jul 23. Epub 2021 Jul 23.

University Medical Center Hamburg Eppendorf, Hamburg, Germany.

J Pediatr Hematol Oncol 2021 Jul 23. Epub 2021 Jul 23.

Departments of Pediatric Hematology and Oncology Pathology Otorhinolaryngology-Head and Neck Surgery, Süleyman Demirel University Medical Faculty, Isparta Molecular Medicine Laboratory, Department of Pediatrics, Ege University Medical Faculty, İzmir, Turkey.

Ann Hematol 2021 Jul 26. Epub 2021 Jul 26.

Klinik Für Innere Medizin III, Abteilung Für Nephrologie, Universitätsklinikum Jena, Jena, Germany.

Open Forum Infect Dis 2021 Jul 24;8(7):ofab343. Epub 2021 Jun 24.

Department of Internal Medicine, Lincoln Medical Center, Bronx, New York, USA.

Immune thrombocytopenia (ITP) is an autoimmune condition associated with multiple risk factors including viral infections (hepatitis B virus/hepatitis C virus/cytomegalovirus, HIV, and recently severe acute respiratory syndrome coronavirus 2) and vaccines. Though immune mechanisms have been proposed to explain the pathogenesis of acute ITP, autoimmunity with the coronavirus disease 2019 (COVID-19) vaccine is still unclear and needs further research. We report a case of acute ITP after administration of the Pfizer-BioNTech mRNA COVID-19 vaccine in a patient with previously stable ITP. Read More

Case Rep Hematol 2021 6;2021:5516863. Epub 2021 Jul 6.

Umm Al-Qura University, Makkah, Saudi Arabia.

Introduction: Congenital thrombotic thrombocytopenic purpura (cTTP) is a rare autosomal recessive disease characterized by ADAMTS13 deficiency or a severe decrease in its activity that is caused by homozygous or combined heterozygous mutations in its encoding gene. Here, we describe a de novo genetic mutation of the ADAMTS13 gene and a rare complication of cTTP in a neonate. . Read More

Transl Lung Cancer Res 2021 Jun;10(6):2614-2624

Department of Oncology, The First Affiliated Hospital of Shandong First Medical University & Shandong Provincial Qianfoshan Hospital, Jinan, China.

Background: Immune checkpoint inhibitors (ICIs) and bevacizumab-based therapy are a promising treatment approach to significantly improving overall survival (OS) of non-small cell lung cancer (NSCLC) patients. However, the incidence of adverse events induced by a combination treatment with programmed cell death-1 or programmed death ligand 1 [PD-(L)1] inhibitor and bevacizumab remains unknown. The current evidence from prospective studies is limited. Read More

Int J Hematol 2021 Jul 22. Epub 2021 Jul 22.

Department of Blood Transfusion Medicine, Nara Medical University, 840 Shijo-Cho, Kashihara, 634-8522, Japan.

Plasma exchange (PEX) using fresh frozen plasma has considerably reduced the mortality rate in patients with immune-mediated thrombotic thrombocytopenic purpura (iTTP). However, some patients still do not survive even with treatment, but little information is available regarding which treatment these patients received. This study was conducted to obtain this information in 240 patients who met the current iTTP diagnostic criteria and completed at least 30 days of follow-up except for deceased cases. Read More

Int J Lab Hematol 2021 Jul;43 Suppl 1:103-108

Department of Pathology, ARUP Laboratories, University of Utah, Salt Lake City, UT, USA.

TTP is a life-threatening disorder diagnosed using a combination of clinical information and laboratory results. ADAMTS13 activity and antibody testing represent a major advance in the field, but results can sometimes be difficult to interpret due to technical aspects of the tests and characteristics of the causative antibodies in acquired TTP. Genetic testing for ADAMTS13 mutations is also now available to assist with the diagnosis of inherited TTP. Read More

Biomol NMR Assign 2021 Jul 20. Epub 2021 Jul 20.

Department of Molecular Engineering, Graduate School of Engineering, Kyoto University, Kyoto-Daigaku Katsura, Nishikyo-Ku, Kyoto, 615-8510, Japan.

von Willebrand factor (vWF) is an adhesive plasma protein that is important for platelet adhesion in normal hemostasis in response to vascular injury. Although large vWF multimers are released from storage granules of platelets and (sub-)endothelial cells in response to hemostatic stimuli, for normal physiological function, vWF multimers are required to be cleaved into smaller multimeric forms. The plasma metalloproteinase ADAMTS13 specifically cleaves the peptide bond located in the middle of the A2 domain of vWF (vWF-A2), but the cleavage site is buried inside the structure of vWF and is difficult to access in the absence of elevated flow shear stress. Read More

Am J Case Rep 2021 Jul 21;22:e931478. Epub 2021 Jul 21.

Department of Family Medicine and Public Health Sciences, Wayne State University, Detroit, MI, USA.

BACKGROUND Immune thrombocytopenic purpura (ITP) is an immune response that destroys platelets and increases the risk of bleeding, which can range from bruising to intracranial hemorrhage. ITP is a known complication of coronavirus disease 2019 (COVID-19). In the first studies of the BNT162b2 messenger RNA (mRNA) COVID-19 vaccine, there were no reports of ITP and the incidence of serious adverse events (AEs) was low overall. Read More

BMJ Case Rep 2021 Jul 20;14(7). Epub 2021 Jul 20.

Haemato-Oncology, Barts Health NHS Trust, London, UK.

A 61-year-old woman who had tested positive for COVID-19 in the community 5 days prior to admission presented with new onset severe headache and mild shortness of breath. She had an acute reduction in her platelet counts from 153×10⁹/L to 5×10⁹/L. She was diagnosed with immune thrombocytopenia purpura and after treatment with intravenous immunoglobulin, her platelet count increased to 15×10⁹/L. Read More

Mediterr J Hematol Infect Dis 2021 1;13(1):e2021047. Epub 2021 Mar 1.

Division of Hematology, AOU "Policlinico G. Rodolico-San Marco", Via Santa Sofia 78, 95124 Catania, Italy.

Expert Rev Hematol 2021 Jul 19. Epub 2021 Jul 19.

Servicio de Hematología y Hemoterapia, Hospital Universitari i Politècnic La Fe, Valencia (Spain).

Introduction: Thrombotic thrombocytopenic purpura (TTP) is a life-threatening thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, thrombocytopenia, and organ injury. TTP pathophysiology is based on a severe ADAMTS13 deficiency, and is a medical emergency with fatal outcome if appropriate treatment is not initiated promptly.

Areas Covered: Authors will review the best options currently available to minimize mortality, prevent relapses, and obtain the best clinical response in patients with immune TTP (iTTP). Read More

Exp Clin Transplant 2021 Jul 16. Epub 2021 Jul 16.

From the Department of Pharmacotherapy, Liver Transplantation Research Center, Tehran University of Medical Sciences, Tehran, Iran.

The present COVID-19 pandemic is a cause for concern among solid-organ transplant recipients, who are generally at high risk for infection and for whom infection with COVID-19 carries additional risks for complications and mortality that are higher than the COVID-19-associated risks for the general population. We report the case of a liver transplant recipient who presented with COVID-19 and multiple complications. A 39-year-old woman with a liver transplant was diagnosed with COVID-19 within the first week after transplant surgery. Read More

Front Cell Infect Microbiol 2021 29;11:614963. Epub 2021 Jun 29.

Department of Food Science and Biotechnology, College of Agriculture and Life Sciences, Kangwon National University, Chuncheon, South Korea.

Shiga toxin-producing (STEC) is a global foodborne bacterial pathogen that is often accountable for colon disorder or distress. STEC commonly induces severe diarrhea in hosts but can cause critical illnesses due to the Shiga toxin virulence factors. To date, there have been a significant number of STEC serotypes have been evolved. Read More

J Hematol 2021 Jun 16;10(3):139-142. Epub 2021 Jun 16.

Morsani School of Medicine, University of South Florida, Tampa, FL, USA.

Leflunomide has not been previously associated with thrombotic thrombocytopenic purpura (TTP), a rare life-threatening clinical syndrome characterized by thrombotic microangiopathy (TMA) due to inability to cleave ADAMTS13. Here, we present the first case of leflunomide-induced TTP. Our patient developed encephalopathy, thrombocytopenia, anemia and hyperbilirubinemia 2 months after starting leflunomide. Read More

Blood Coagul Fibrinolysis 2021 Jul 15. Epub 2021 Jul 15.

Baxalta Innovations GmbH, A Takeda Company, Vienna, Austria.

Insufficiency of ADAMTS13 (a disintegrin and metalloprotease with thrombospondin motif repeats-13) is the cause of thrombotic thrombocytopenic purpura (TTP) and contributes in microangiopathy in sickle cell disease (SCD). Recombinant ADAMTS13 effectively cleaves prothrombotic ultra-large von Willebrand factor (VWF) multimers. It is being tested as replacement therapy for TTP, and at supra-physiologic concentrations, for moderating vaso-occlusive crisis in SCD. Read More

Med Clin (Barc) 2021 Jul 12. Epub 2021 Jul 12.

Servicio de Transfusión. Servicio de Hematología. Complexo Hospitalario Universitario de Ourense, Galicia, España. Electronic address:

Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy (TMA) characterized by the development of microangiopathic haemolytic anaemia, thrombocytopenia, and ischaemic organ dysfunction associated with ADAMTS13 levels lower than 10% in most cases. Recently there have been numerous advances in the field of PTT, new, rapid and accessible techniques capable of quantifying ADAMTS13 activity and inhibitors. The massive sequencing systems facilitate the identification of polymorphisms in the ADAMTS13 gene. Read More

Int J Hematol 2021 Jul 15. Epub 2021 Jul 15.

Department of Internal Medicine, Dow University of Health Sciences, Karachi, Pakistan.

Thrombotic thrombocytopenic purpura (TTP) is a known menace in hematology and is quite rare in practice with known triggers. Lately, in the COVID-19 pandemic, hematology has seen a new pathology amongst which TTP associated with COVID-19 messenger RNA (mRNA) vaccine is unique. We report a case of a 69-year-old male with multiple comorbidities who presented to the hospital with severe fatigue and shortness of breath. Read More

Clin Case Rep 2021 Jul 6;9(7):e04364. Epub 2021 Jul 6.

Department of Internal Medicine, Hematology Al- Mouwasat Hospital Damascus University Damascus Syria.

TTP, Preeclampsia have similar manifestations in pregnancy. Establishing the right diagnosis is essential as the treatment is different. Endocarditis-induced TTP should be suspected when neurological symptoms, thrombocytopenia are present. Read More

J Neurol 2021 Jul 13. Epub 2021 Jul 13.

Department of Neurology, Tampere University Hospital, Tampere, Finland.

Background: Alemtuzumab is an effective disease-modifying therapy (DMT) for highly active multiple sclerosis (MS). However, safety concerns limit its use in clinical practice.

Objectives: To evaluate the safety of alemtuzumab in a nationwide cohort of Finnish MS patients. Read More

Sci Rep 2021 Jul 12;11(1):14263. Epub 2021 Jul 12.

Pediatrics, Nihon University Itabashi Hospital, 30-1 Ohyaguchi-kamicho, Itabashi-ku, Tokyo, Japan.

Treatment of children with refractory immune thrombocytopenic purpura (ITP) is challenging and poorly established. We retrospectively reviewed the clinical data of 87 patients under the age of 16 years who were diagnosed with ITP from April 1998 to March 2017 in our institution. Refractory ITP was defined as a platelet count of < 50 × 10/L at 14 days after receiving intravenous immunoglobulin (IVIG) and prednisolone. Read More

Microvasc Res 2021 Jul 9;138:104226. Epub 2021 Jul 9.

Kidney Research Center, Tabriz University of Medical Sciences, Tabriz, Iran. Electronic address:

Pregnancy is a high-risk time for the development of different kinds of thrombotic microangiopathy (TMA). Three major syndromes including TTP (thrombotic thrombocytopenic purpura), PE/HELLP (preeclampsia/hemolysis, elevated liver function tests, low platelets), and aHUS (atypical hemolytic- uremic syndrome) should be sought in pregnancy-TMA. These severe disorders share multiple clinical features and overlaps and even the coexistence of more than one pathologic mechanism. Read More

Acta Med Indones 2021 Apr;53(2):208-212

Department of Medicine, Universiti Kebangsaan Malaysia Medical Centre, JalanYaacob Latif, Bandar TunRazak, 56000 Cheras, Wilayah Persekutuan Kuala Lumpur, Malaysia.

Thrombotic thrombocytopenic purpura is a rare but life threatening medical condition. Early recognition and treatment of thrombotic thrombocytopenic purpura is important especially in patients who do not present with the classic pentad to reduce the high mortality. Herein, we describe a case of a patient who does not fulfil the classic pentad features thrombotic thrombocytopenic purpura that was induced by dengue fever. Read More

Rinsho Ketsueki 2021 ;62(5):480-485

Department of Blood Transfusion Medicine, Nara Medical University.

The key clinical symptoms of thrombotic thrombocytopenic purpura (TTP) are severe thrombocytopenia, microangiopathic hemolytic anemia, and organ ischemia/infarction due to microthrombi. Hemolytic anemia in TTP is characterized by mechanical damage to red blood cells. TTP is caused by a severe deficiency of ADAMTS13 activity, which is caused by mutations in the ADAMTS13 gene (congenital TTP) or by autoantibodies affecting the function or clearance of ADAMTS13 (immune TTP). Read More

J Fr Ophtalmol 2021 Jul 7. Epub 2021 Jul 7.

Department of Pediatrics, Virgen del Rocío University Hospital, Av. Manuel Siurot, 41013 Seville, Spain.