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J Clin Apher 2022 Aug 18. Epub 2022 Aug 18.

Department of Pathology, Johns Hopkins University, Baltimore, Maryland, USA.

Per the American Society for Apheresis, therapeutic plasma exchange (TPE) is a Category III indication in the management of immune thrombocytopenia (ITP). This nationally representative study evaluates TPE utilization in hospitalized adults with a primary admission diagnosis of ITP. Hospitalizations with ITP as the primary admitting diagnosis were analyzed from the 2010 to 2014 National Inpatient Sample, the largest all-payer inpatient database in the United States. Read More

Front Pediatr 2022 1;10:931669. Epub 2022 Aug 1.

Department of Pediatrics, Gunma University Graduate School of Medicine, Maebashi, Japan.

Background: Thrombotic microangiopathy (TMA) is a syndrome associated with hemolytic anemia, thrombocytopenia, and various organ disorders. Thrombotic thrombocytopenic purpura (TTP) is a disease that develops when a disintegrin-like and metalloproteinase with thrombospondin type l motif 13 (ADAMTS13) activity decreases to < 10% of that in normal plasma, causing platelet thrombosis in microvessels throughout the body. Currently, ADAMTS13-deficient TMA is diagnosed as TTP. Read More

Clin Lab 2022 Jul;68(7)

Background: Immune thrombocytopenia (ITP) is an autoimmune hemorrhagic disease with distinct clinical manifestations such as extensive skin petechiae, mucosal bleeding, and even visceral hemorrhage. In this study, CD3+T lymphocytes from ITP patients were screened for differentially expressed genes. The expression of miR-21 and miR-155 in T lymphocytes of ITP patients were investigated. Read More

Nat Commun 2022 Aug 15;13(1):4800. Epub 2022 Aug 15.

Usher Institute, The University of Edinburgh, Edinburgh, UK.

We investigated thrombocytopenic, thromboembolic and hemorrhagic events following a second dose of ChAdOx1 and BNT162b2 using a self-controlled case series analysis. We used a national prospective cohort with 2.0 million(m) adults vaccinated with two doses of ChAdOx or 1. Read More

Blood Transfus 2022 Aug 9. Epub 2022 Aug 9.

Department of Clinical Medicine and Surgery, "Federico II" University, Naples, Italy.

Background: The PLASMIC score is a rapid and inexpensive clinical assessment tool for predicting severe ADAMTS13 deficiency (<10% activity) in patients with suspected thrombotic thrombocytopenic purpura (TTP). The score includes 7 parameters: absence of active cancer, patient not having received stem cell transplant or organ transplant, platelet count <30×10/L, haemolysis, mean corpuscular volume <90 fl, International Normalized Ratio <1.5, and serum creatinine <2 mg/dL. Read More

Case Rep Dermatol Med 2022 4;2022:1469410. Epub 2022 Aug 4.

Campbell University at Sampson Regional Medical Center, Clinton 28328, NC, USA.

Our case highlights leukocytoclastic vasculitis as a potential side effect of the elasomeran COVID-19 vaccine. As the elasomeran vaccine becomes more widely available to the public, cutaneous reactions should be noted and looked for as potential side effects of the vaccine. Our patient had a history of immune thrombocytopenic purpura, making this a potential predisposing condition to the development of vasculitis following elasomeran administration. Read More

Rheumatol Int 2022 Aug 12. Epub 2022 Aug 12.

Department of Rheumatology, Tohoku University Hospital, Sendai, Miyagi, Japan.

Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by the production of various autoantibodies and deposition of immune complexes on tissues. Acquired thrombotic thrombocytopenic purpura (TTP) is a life-threatening hematological disorder that rarely develops in SLE, mainly caused by inhibitory or clearing autoantibody against ADAMTS13. Although B cells play critical roles in the pathogenesis of two diseases, the role of B-cell depletion therapy using rituximab (RTX), a chimeric monoclonal antibody targeting CD20, in the management of TTP associated with SLE remains unclear. Read More

Ugeskr Laeger 2022 Jul;184(29)

Hæmatologisk Afdeling, Odense Universitetshospital.

Despite significant improvements in the prognosis of thrombotic thrombocytopenic purpura (TTP), long-term neuropsychological deficits are frequent but probably under-recognised. Regular assessment of cognitive impairment using screening tools is therefore recommended. In this case report we describe two patients with neuropsychological late effects severely affecting their work capacity and quality of life. Read More

J Crit Care Med (Targu Mures) 2022 Apr 12;8(2):131-135. Epub 2022 May 12.

George Emil Palade University of Medicine, Pharmacy, Science, and Technology of Targu Mures, Targu Mures, Romania.

Postoperative coronary vasospasm is a well-known cause of angina that may lead to myocardial infarction if not treated promptly. We report a case of a 70-year-old female with severe mitral regurgitation submitted to mitral valve replacement, and a history of diabetes mellitus type II, stroke, idiopathic thrombocytopenic purpura on steroid therapy, and previous percutaneous coronary intervention (PCI) for severe obstruction of the circumflex coronary artery, 4 months prior to surgery. Immediately after intensive care unit admission, the patient developed pulseless electrical activity which required extracorporeal membrane oxygenation for hemodynamic support. Read More

Oman Med J 2022 Jul 31;37(4):e407. Epub 2022 Jul 31.

Department of Hematology, Dubai Hospital, Dubai, UAE.

Acquired thrombotic thrombocytopenic purpura (aTTP) is a rare hematological emergency characterized by microangiopathic hemolytic anemia, thrombocytopenia, fever, and multiorgan failure due to autoimmune-mediated deficiency in ADAMTS-13 activity. Currently, plasma exchange, with or without steroids, is the frontline option for the management of aTTP. The treatment should be started promptly once the disorder is clinically suspected. Read More

Exp Ther Med 2022 Sep 19;24(3):580. Epub 2022 Jul 19.

Department of Hematology, The 940th Hospital of Joint Logistics Support Force of The Chinese People's Liberation Army, Lanzhou, Gansu 730050, P.R. China.

Coronavirus disease 2019 (COVID-19) has become a global pandemic, but treatment options remain limited. Up to now, vaccination has been the main strategy to prevent transmission and reduce disease severity. However, with follow-up observations after massive vaccination, immune thrombocytopenic purpura (ITP) induced by COVID-19 vaccines has attracted the attention of investigators. Read More

Genome Med 2022 Aug 9;14(1):85. Epub 2022 Aug 9.

Department of Dermatology, University of Michigan Medical School, Ann Arbor, MI, USA.

Background: Rare diseases collectively affect up to 10% of the population, but often lack effective treatment, and typically little is known about their pathophysiology. Major challenges include suboptimal phenotype mapping and limited statistical power. Population biobanks, such as the UK Biobank, recruit many individuals who can be affected by rare diseases; however, investigation into their utility for rare disease research remains limited. Read More

Transplant Cell Ther 2022 Aug 5. Epub 2022 Aug 5.

National clinical research center for hematologic diseases, Jiangsu Institute of Hematology, The First Affiliated Hospital of Soochow University, Suzhou, China; Institute of Blood and Marrow Transplantation, Collaborative Innovation Center of Hematology, Soochow University, Suzhou, China; Key Laboratory of Thrombosis and Hemostasis of Ministry of Health, Suzhou, China; State Key Laboratory of Radiation Medicine and Protection, Soochow University, Suzhou, China. Electronic address:

Background: Transplant-associated thrombotic microangiopathy (TA-TMA) is a life-threatening complication for patients undergoing hematopoietic stem cell transplantation (HSCT). N-acetylcysteine (NAC) has recently been considered as a potential treatment for patients with thrombotic thrombocytopenic purpura.

Objectives: To assess the value of NAC for the prevention of TA-TMA, we conducted a prospective study at the First Affiliated Hospital of Soochow University. Read More

Comput Math Methods Med 2022 28;2022:5002681. Epub 2022 Jul 28.

Department of Gynecology, The People's Hospital of Hechi, Hechi, 547000 Guangxi, China.

The risk factors of upper respiratory tract infection (URI) within 6 months after diagnosis in patients with idiopathic thrombocytopenic purpura (ITP) were analyzed, and the nomogram model was established and verified, with 242 and 50 ITP patients as the training and validation set, respectively. The patients were followed up for six months after the diagnosis of ITP. The clinical data of patients were collected, and the risk factors of URI in ITP patients within six months after diagnosis were analyzed using univariable, followed by multivariable logistic regression. Read More

Ann Hematol 2022 Aug 6. Epub 2022 Aug 6.

Division of Hematology, Department of Medicine, Johns Hopkins University School of Medicine, 720 Ross Research Building Rutland Avenue, Room 1025, Baltimore, MD, 21205, USA.

Pregnancy is a well-established trigger for a first episode or relapse of immune thrombotic thrombocytopenic purpura (iTTP). Other outcomes of subsequent pregnancy after a diagnosis of iTTP are less well described. We conducted this retrospective cohort study to evaluate maternal and fetal outcomes of pregnancy in women with prior iTTP from the Johns Hopkins Thrombotic Microangiopathy Cohort. Read More

Blood Adv 2022 Aug 5. Epub 2022 Aug 5.

Hospital Universitario Juan Ramón Jiménez, Huelva, Spain.

Immune thrombotic thrombocytopenic purpura (iTTP) is a thrombotic microangiopathy caused by anti-ADAMTS13 antibodies. Caplacizumab is approved for adults with an acute episode of iTTP in conjunction with PEX and immunosuppression. The objective of this study is to analyze and compare the safety and efficacy of caplacizumab versus the standard of care and assess the effect of the concomitant use of rituximab. Read More

Orphanet J Rare Dis 2022 08 4;17(1):308. Epub 2022 Aug 4.

Division of Hematology-Oncology, CHU Sainte-Justine, Université de Montréal, 3175, chemin de la Côte-Ste-Catherine, Montreal, QC, H3T 1C5, Canada.

Background: Thrombotic microangiopathies (TMA) are serious medical conditions requiring a prompt diagnosis to adapt treatment. The determination of ADAMTS-13 activity enables discriminating thrombotic thrombocytopenic purpura (TTP) from other forms of TMA. The purpose of this study was to provide an estimate of the incidence of TTP and TMA in the Canadian Quebec province using data collected from a laboratory centralizing ADAMTS-13 testing for the whole province. Read More

Clin Appl Thromb Hemost 2022 Jan-Dec;28:10760296221106779

Department of Laboratory Medicine, the Second People's Hospital of Lianyungang City, Jiangsu Province, China.

To investigate the application value of bone marrow megakaryocyte count, the proportion of megakaryocytes at each stage, and the platelet parameter in the clinical diagnosis of primary immune thrombocytopenia (ITP). The megakaryocyte and platelet parameter level in 62 ITP and 40 control group patients were compared and analyzed. Linear correlation analysis, Pearson correlation analysis, and ROC curves were performed for the correlation between megakaryocytes and platelet parameters. Read More

Ther Adv Hematol 2022 26;13:20406207221112217. Epub 2022 Jul 26.

Department of Haematology, UCLH, London, UK.

Modern therapy for acute TTP has resulted in a dramatic improvement in outcomes, with the combination of plasma exchange, immunosuppression, and caplacizumab being associated with >90% survival rates following an acute episode. TTP is no longer associated with just the acute episode, but requires long-term follow-up. There remains significant morbidity associated with acute TTP, and many patients suffer marked neuropsychological sequelae, including impairment in cognitive functioning, affective disorders, and reduction in health-related quality of life measures. Read More

Elife 2022 Aug 3;11. Epub 2022 Aug 3.

Center for Immune-Related Diseases at Shanghai Institute of Immunology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China.

IgG4 is the least potent human IgG subclass for the FcγR-mediated antibody effector function. Paradoxically, IgG4 is also the dominant IgG subclass of pathogenic autoantibodies in IgG4-mediated diseases. Here, we show that the IgG subclass and Fc-FcγR interaction have a distinct impact on the pathogenic function of autoantibodies in different IgG4-mediated diseases in mouse models. Read More

Blood Res 2022 Aug 3. Epub 2022 Aug 3.

Clinical and Chemical Pathology Department, Kasr Al Ainy, Cairo University, Cairo, Egypt.

Background: The pathophysiology underlying primary adult immune thrombocytopenic purpura (ITP) has not yet been identified. However, many mechanisms affect the immune system, causing defective tolerance to self-platelets and megakaryocytes. Cluster of differentiation 40 (CD40) contributes to both humoral and cell-mediated immune responses. Read More

Blood Res 2022 Aug 3. Epub 2022 Aug 3.

Medical Genetic, Firat University Faculty of Medicine, Elazig, Turkey.

Background: Nitric oxide (NO) can induce apoptosis in megakaryocytes. Stimulatory function of NO on platelet production may be important in the pathophysiology of idiopathic thrombocytopenic purpura (ITP). NO is produced by three isoforms of NO synthase (NOS). Read More

Caspian J Intern Med 2022 ;13(2):368-374

Surgical Oncology Research Center, Mashhad University of Medical Sciences, Mashhad, Iran.

Background: Chronic idiopathic thrombocytopenic purpura (ITP), is an autoimmune disease associated with a reduction in circulating blood platelets under 150±10g/L which persists longer than 6 months without any specific cause. With the current study, we aimed to evaluate the efficacy and safety of laparoscopic splenectomy in ITP patients with a very low platelet count and normal coagulation status.

Methods: From April 2007 to January 2012, laparoscopic splenectomy was performed on 60 patients with chronic ITP who could not achieve a sustained recovery after steroid therapy. Read More

BMC Infect Dis 2022 Jul 28;22(1):657. Epub 2022 Jul 28.

Iran University of Medical Science, Tehran, Iran.

Introduction: Immunosuppressive chemotherapy increase the risk of vaccine-preventable infectious diseases in children; nevertheless, chemotherapy may result in delay or miss updated immunization schedules. The predictable antibody waning after incomplete primary immunization series may be intensified at the end of chemotherapy. This study aimed to investigate post-chemotherapy vaccine immunity waning at the end of immunosuppressive therapy in children with malignancy and hematologic disorders. Read More

BMJ Case Rep 2022 Jul 28;15(7). Epub 2022 Jul 28.

Departments of Medicine and Laboratory Medicine & Pathobiology, University of Toronto, Toronto, Ontario, Canada

Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease that may be triggered by inflammation, including infection or vaccination. Since the start of the COVID-19 pandemic, several case reports were published on de novo or relapsed immune TTP (iTTP) in COVID-19-infected patients. Case reports of iTTP episodes following vaccination against COVID-19 are also emerging. Read More

Indian J Pathol Microbiol 2022 Jul-Sep;65(3):702-704

Department of General Medicine, D. M. Wayanad Institute of Medical Sciences, Wayanad, Kerala, India.

Introduction: While disseminated intravascular coagulation (DIC) is a serious complication of COVID-19, a close differential in critically ill patients with thrombocytopenia is Thrombotic thrombocytopenic purpura (TTP).

Case Report: We describe the case of a middle-aged lady admitted with COVID-19 pneumonia who developed progressive thrombocytopenia, altered sensorium and renal failure. The absence of coagulation abnormalities alerted to the possibility of TTP, strengthened by presence of schistocytes in peripheral smear. Read More

J Thromb Haemost 2022 Jul 27. Epub 2022 Jul 27.

Department of Pathology and Laboratory Medicine, The University of Kansas Medical Center, Kansas City, Kansas, USA.

Background: Severe deficiency of plasma ADAMTS13 activity may result in potentially fatal thrombotic thrombocytopenic purpura and relative deficiency of plasma ADAMTS13 activity may be associated with adverse outcomes of certain malignancies. Here, we report the role of ADAMTS13 or lack of von Willebrand factor (VWF) in reducing irradiation and melanoma-induced thrombotic microangiopathy (TMA) and mortality in zebrafish.

Methods: Zebrafish melanoma cell line (ZMEL) was injected subcutaneously into wild-type (wt), adamts13 (a13 ), von Willebrand factor (vwf ), and a13 vwf zebrafish following total body irradiation; the tumor growth, its gene expression pattern, the resulting thrombocytopenia, and the mortality were determined. Read More

Vaccines (Basel) 2022 Jun 24;10(7). Epub 2022 Jun 24.

Medical Intensive Care Unit, Faculty of Medicine of Sousse, University of Sousse, Sousse 4000, Tunisia.

The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) outbreak in December 2019, causing millions of deaths all over the world, and the lack of specific treatment for severe forms of coronavirus disease 2019 (COVID-19) have led to the development of vaccines in record time, increasing the risk of vaccine safety issues. Recently, several cases of thrombotic thrombocytopenic purpura (TTP) have been reported following COVID-19 vaccination. TTP is a rare disease characterized by thrombocytopenia, microangiopathic hemolytic anemia and ischemic end-organ lesions. Read More

Diagnostics (Basel) 2022 Jun 27;12(7). Epub 2022 Jun 27.

Department of Pathology, County Clinical Hospital of Targu Mures, 540072 Targu Mures, Romania.

Thrombocytopenic purpura (TTP) is a rare, potentially fatal pathology characterized by microangiopathic thrombotic syndrome and caused by an acute protease deficiency of von Willebrand factor, ADAMTS13. Moreover, ADAMTS13 deficiency promotes microthrombosis led by the persistence of ultra-large VWF multimers in the blood circulation. According to the few studies involving pregnant participants, the heterogeneity of manifestations has made this pathology difficult to diagnose, with an unexpected occurrence and increased risk of maternal and fetal morbidity and mortality. Read More

Cureus 2022 Jun 20;14(6):e26112. Epub 2022 Jun 20.

Internal Medicine, Howard University, Washingon DC, USA.

A rare complication of infectious mononucleosis is immune thrombocytopenic purpura (ITP). The majority of people affected by Epstein-Barr Virus (EBV) are below the age of 30, while ITP is usually seen with peaks of incidence in the elderly and children. The unique case of an otherwise healthy 22-year-old female will be discussed, with an initial presentation of ecchymosis, rash, and epistaxis, and was subsequently found to have severe thrombocytopenia. Read More