25,095 results match your criteria thrombotic microangiopathy

Loss of diacylglycerol kinase ε causes thrombotic microangiopathy by impairing endothelial VEGFA signaling.

JCI Insight 2021 May 10;6(9). Epub 2021 May 10.

Department of Internal Medicine, University of Iowa, Iowa City, Iowa, USA.

Loss of function of the lipid kinase diacylglycerol kinase ε (DGKε), encoded by the gene DGKE, causes a form of atypical hemolytic uremic syndrome that is not related to abnormalities of the alternative pathway of the complement, by mechanisms that are not understood. By generating a potentially novel endothelial specific Dgke-knockout mouse, we demonstrate that loss of Dgke in the endothelium results in impaired signaling downstream of VEGFR2 due to cellular shortage of phosphatidylinositol 4,5-biphosphate. Mechanistically, we found that, in the absence of DGKε in the endothelium, Akt fails to be activated upon VEGFR2 stimulation, resulting in defective induction of the enzyme cyclooxygenase 2 and production of prostaglandin E2 (PGE2). Read More

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Atypical posterior reversible encephalopathy syndrome in a case of thrombotic thrombocytopenic purpura with Sjogren's syndrome.

Radiol Case Rep 2021 Jun 19;16(6):1552-1556. Epub 2021 Apr 19.

Department of Radiology, Yale New Haven Health System, Bridgeport Hospital, Bridgeport, CT.

Thrombotic thrombocytopenic purpura (TTP) is a rare hematologic disorder characterized by thrombotic microangiopathy. Neurologic symptoms are frequently seen in its presentation and the most common finding on neuroimaging of TTP is posterior reversible encephalopathy syndrome (PRES). Acute strokes, hemorrhages and atypical PRES are uncommonly seen. Read More

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Reversible renal-limited thrombotic microangiopathy due to gemcitabine-dexamethasone-cisplatin therapy: a case report.

BMC Nephrol 2021 May 12;22(1):175. Epub 2021 May 12.

Department of Hematology, Kobe City Medical Center General Hospital, 2-1-1 Minami-machi, Minatojima, Chuo-ku, Kobe, Hyogo, 650-0047, Japan.

Background: Gemcitabine and cisplatin are chemotherapeutic agents used for treating multiple cancers, and these agents are sometimes used in combination. Drug-induced thrombotic microangiopathy (TMA) is a rare but potentially fatal complication. It typically presents as a systemic disease with the classical triad of hemolytic anemia, thrombocytopenia, and organ damage. Read More

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Fatal cerebral hemorrhage in a patient with thrombotic thrombocytopenic purpura with a normal platelet count during treatment with caplacizumab.

Platelets 2021 May 12:1-2. Epub 2021 May 12.

Department of Internal Medicine, Meander Medical Centre, Amersfoort, The Netherlands.

Acquired thrombotic thrombocytopenic purpura (aTTP) is a thrombotic microangiopathy with a severe mortality and morbidity. Caplacizumab has recently been approved in the Netherlands as a new therapeutic option in patients with life-threatening organ failure due to aTTP. We describe the case of a 50 year old patient with aTTP who was referred to our hospital for treatment with caplacizumab. Read More

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Clinical characteristics and outcomes of adult patients with acquired thrombotic thrombocytopenic purpura: a single center retrospective study.

Ann Palliat Med 2021 May 11. Epub 2021 May 11.

Department of Intensive Care Medicine, The First Affiliated Hospital of Soochow University, Suzhou, China.

Background: Thrombotic thrombocytopenic purpura (TTP) is a rare disease and a potentially lifethreatening thrombotic microangiopathy. Although the diagnostic and therapeutic techniques have improved, it is still difficult for clinicians to identify early due to different initial clinical manifestations and the incidence and survival rate are reported inconsistently. This study investigated the clinical characteristics, treatment strategies, and treatment outcomes of adult patients with acquired TTP. Read More

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Complement-Mediated Thrombotic Microangiopathy Associated with Lupus Nephritis Treated with Eculizumab: A Case Report.

Case Rep Nephrol Dial 2021 Jan-Apr;11(1):95-102. Epub 2021 Mar 31.

Division of Nephrology, Hypertension and Kidney Transplantation, Department of Medicine, University of California, Irvine, California, USA.

Thrombotic microangiopathies (TMAs) involve multiple organ systems due to the presence of microangiopathic hemolysis. One such condition, atypical hemolytic uremic syndrome (aHUS), is a complement-mediated process that is part of a spectrum of disorders that have underlying complement dysfunction of the alternative pathway due to overactivity or decreased self-nonself discrimination by innate immunity. Complement-amplifying conditions such as pregnancy may unmask a diagnosis of aHUS. Read More

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Humoral Acute Rejection in a Kidney Transplant Recipient with Idiopathic Thrombocytopenic Purpura.

Case Rep Transplant 2021 22;2021:9933354. Epub 2021 Apr 22.

Departamento de Nefrología y Trasplantes, UMAE, Hospital de Especialidades, CMNO, IMSS, Guadalajara, Jalisco, Mexico.

A 47-year-old male was diagnosed with chronic kidney disease (CKD) in 2011; idiopathic thrombocytopenic purpura (ITP) was also diagnosed in 2011 refractory to medical treatment and finally treated with splenectomy (2017) without relapses since that date, 5 blood transfusions, and 4 platelet apheresis in 2017. Renal transplant from a living related donor (brother), ABO compatible, crossmatch were negative, sharing 1 haplotype. Donor-specific anti-HLA antibody was negative. Read More

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Atypical Evolution of Secondary Hemolytic Uremic Syndrome Defined as Paraneoplastic Syndrome under Eculizumab and Palbociclib Therapies.

Case Rep Oncol 2021 Jan-Apr;14(1):676-680. Epub 2021 Apr 12.

Nephrology, Hemodialysis, Apheresis and Kidney Transplantation Department, Grenoble Alpes University Hospital, Grenoble, France.

Thrombotic microangiopathy (TMA) is most of the time caused by thrombotic thrombocytopenic purpura or hemolytic uremic syndrome. A 60-year-old female was diagnosed in 2014 with mammary breast adenocarcinoma treated by several-line therapy: mastectomy, docetaxel, cyclophosphamide, radiotherapy, doxorubicine, and capecitabine. By mid-November, the patient was admitted to the hospital with regenerative, mechanical, and hemolytic anemia, schistocytes at 3%, and thrombopenia (99 G/L), associated with high blood transfusion requirement. Read More

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Complement levels at admission as a reflection of Coronavirus Disease 19 (COVID-19) severity state.

J Med Virol 2021 May 11. Epub 2021 May 11.

Division of Nephrology and Hypertension, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.

Background: Complement system hyperactivation has been proposed as a potential driver of adverse outcomes in severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infected patients, given prior research of complement deposits found in tissue and blood samples, as well as evidence of clinical improvement with anticomplement therapy. Its role in augmenting thrombotic microangiopathy mediated organ damage has also been implicated in coronavirus disease 2019 (COVID-19). This study aimed to examine associations between complement parameters and progression to severe COVID-19 illness, as well as correlations with other systems. Read More

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The hemostatic mechanism of "Treated the Spleen" therapy on immune thrombocytopenia based on the characteristics of vasoactive factors.

Ann Palliat Med 2021 Apr;10(4):4612-4622

Department of Hematology, Dongfang Hospital Affiliated to Beijing University of Chinese Medicine, Beijing, China.

Background: To explore the effect mechanism of "treat the spleen" therapy on immune thrombocytopenia (ITP) based on the characteristics of vasoactive factors.

Methods: The ITP mice model was established by passive immunomodeling. 120 successfully modeled BALB/c mice were randomly divided into 6 groups: normal group, model group, prednisone group, Guipi Decoction group, Jianpi Yiqi group, and Jianpi Shexue group. Read More

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Lenvatinib-related renal microangiopathy: a case series.

Virchows Arch 2021 May 6. Epub 2021 May 6.

Department of Pathology, Johns Hopkins School of Medicine, Baltimore, MD, USA.

Tyrosine kinase inhibitors play an important role in the armamentarium against cancer. Lenvatinib is a multiple kinase inhibitor approved by the Food and Drugs Administration (FDA) for the treatment of advanced and radioresistant thyroid carcinomas and, in combination with everolimus, for renal cell carcinoma and unresectable hepatocellular carcinoma. The anti-tumoral activity is largely dependent on inhibition of neo-angiogenesis, and established side effects of anti-angiogenetic therapeutics include renal thrombotic microangiopathy (TMA). Read More

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Transplant-associated thrombotic microangiopathy and immune haematological complications following intestine-containing organ transplantation: experience from over 100 consecutive cases.

Br J Haematol 2021 May 5. Epub 2021 May 5.

Department of Gastroenterology, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK.

Descriptions of passenger lymphocyte syndrome (PLS), immune cytopenias and transplant-associated thrombotic microangiopathy (TA-TMA) after intestine-containing transplants remain scarce. We describe our centre's experience of these complications from 2007 to 2019. Ninety-six patients received 103 transplants. Read More

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Thrombotic microangiopathy in aHUS and beyond: clinical clues from complement genetics.

Nat Rev Nephrol 2021 May 5. Epub 2021 May 5.

Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Service d'Immunologie, Paris, France.

Studies of complement genetics have changed the landscape of thrombotic microangiopathies (TMAs), particularly atypical haemolytic uraemic syndrome (aHUS). Knowledge of complement genetics paved the way for the design of the first specific treatment for aHUS, eculizumab, and is increasingly being used to aid decisions regarding discontinuation of anti-complement treatment in this setting. Complement genetic studies have also been used to investigate the pathogenic mechanisms that underlie other forms of HUS and provided evidence that contributed to the reclassification of pregnancy- and postpartum-associated HUS within the spectrum of complement-mediated aHUS. Read More

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Diagnosis and management of coronavirus disease-associated immune thrombocytopenia: a case series.

Rev Soc Bras Med Trop 2021 28;54:e0029 2021. Epub 2021 Apr 28.

University of Dicle, School of Medicine, Department of Hematology, Diyarbakır, Turkey.

Data on health problems and fatal complications associated with coronavirus disease (COVID-19) have consistently been reported. Although immune thrombocytopenia has been associated with multiple viral infections, only few studies have shown its association with COVID-19. Here, we have reported a case series of two cases pertaining to patients diagnosed with COVID-19-associated immune thrombocytopenia, elaborating on the clinical course, management, and response to treatment. Read More

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Clinico-epidemiology of Hypnale zara (hump-nosed pit viper) envenoming in Sri Lanka.

Trans R Soc Trop Med Hyg 2021 May 4. Epub 2021 May 4.

Department of Medicine, Faculty of Medicine, University of Peradeniya, Sri Lanka.

Background: Hump-nosed vipers of the genus Hypnale are the most common cause of venomous snakebites in Sri Lanka. The genus includes three species: Hypnale hypnale, Hypnale zara and Hypnale nepa. Of them, H. Read More

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SARS-CoV-2 vaccine and thrombosis: Expert opinions.

Thromb Haemost 2021 May 4. Epub 2021 May 4.

Department of Internal Medicine and Clinical Immunology, Sorbonne Universités, AP-HP, Groupe Hospitalier Pitié-Salpêtrière,, Paris, France.

Historically, the vaccination strategies developed in the second half of the 20th century have permitted to eradicate infectious diseases. From the onset of COVID-19 pandemic to March 2021, more than 141 million cases and 3 million deaths were documented worldwide with disruption of the economic and social activity, devastating material, physical and psychological consequences. Reports of unusual and severe thrombotic events, including cerebral and splanchnic venous thrombosis and other autoimmune adverse reactions such as immune thrombocytopenia or thrombotic microangiopathies (TMA) in connection with some of the SARS-CoV-2 vaccine have caused a great deal of concern within the population and the medical community. Read More

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Thrombotic Microangiopathy (aHUS/iTTP) Reported So Far in Covid -19 patients: The virus alone or an omnium gatherum of mechanisms and etiologies?

Tajamul H Mir

Crit Rev Oncol Hematol 2021 Apr 30:103347. Epub 2021 Apr 30.

Department of Nephrology and Lupus/Vasculitis Centre, Khyber Medical Institute, Room No: 126, Khyam Chowk (Munawarabad) Khanyar, Srinagar, 190001, Jammu and Kashmir, India. Electronic address:

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C5 Inhibition in Secondary Thrombotic Microangiopathies: A Yet Unresolved Question.

Kidney Int Rep 2021 Apr 22;6(4):878-880. Epub 2021 Feb 22.

Intensive Care Nephrology and Transplantation Department, SINRA, Hôpital Tenon, Assistance Publique-Hôpitaux de Paris, Paris, France.

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A Novel Case of Cytomegalovirus Pneumonia in an Acquired Thrombotic Thrombocytopenic Purpura Patient Treated With Rituximab.

Cureus 2021 Mar 30;13(3):e14182. Epub 2021 Mar 30.

Internal Medicine and Nephrology, McLaren Health Care, Flint/MSU, Flint, USA.

Thrombotic thrombocytopenic purpura (TTP) is thrombotic microangiopathy that is universally fatal if not promptly recognized and treated. Standard treatment includes plasma exchange (PLEX) therapy and immunosuppression. We present a case of an 80 years old African American male with a past medical history significant for essential hypertension, chronic obstructive pulmonary disease, and a recent TTP diagnosis for which he was treated with PLEX, glucocorticoids, and rituximab. Read More

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Erythrocytosis, embolism in a woman with antiphospholipid antibodies: A diagnostic challenge.

Clin Case Rep 2021 Apr 24;9(4):2138-2143. Epub 2021 Feb 24.

Hematology and Oncology BronxCare Health System Bronx NY USA.

Systemic thromboembolism, along with acute multiorgan failure, should raise clinical suspicion for CAPS. Timely management, even in suspected cases, can have a significant impact on mortality. Read More

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Extracellular vesicles in renal inflammatory and infectious diseases.

Free Radic Biol Med 2021 Apr 29;171:42-54. Epub 2021 Apr 29.

Department of Pediatrics, Clinical Sciences Lund, Lund University, 22185, Lund, Sweden.

Extracellular vesicles can mediate cell-to-cell communication, or relieve the parent cell of harmful substances, in order to maintain cellular integrity. The content of extracellular vesicles includes miRNAs, mRNAs, growth factors, complement factors, cytokines, chemokines and receptors. These may contribute to inflammatory and infectious diseases by the exposure or transfer of potent effectors that induce vascular inflammation by leukocyte recruitment and thrombosis. Read More

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Shiga Toxin-Associated Hemolytic Uremic Syndrome: Specificities of Adult Patients and Implications for Critical Care Management.

Toxins (Basel) 2021 04 26;13(5). Epub 2021 Apr 26.

Centre de Référence des Microangiopathies Thrombotiques (CNR-MAT), Assistance Publique-Hôpitaux de Paris, Hôpital Saint-Antoine, 75012 Paris, France.

Shiga toxin-producing -associated hemolytic uremic syndrome (STEC-HUS) is a form of thrombotic microangiopathy secondary to an infection by an enterohemorrhagic . Historically considered a pediatric disease, its presentation has been described as typical, with bloody diarrhea at the forefront. However, in adults, the clinical presentation is more diverse and makes the early diagnosis hazardous. Read More

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Successful Treatment of NDM Sepsis and Intestinal Decolonization with Ceftazidime/Avibactam Plus Aztreonam Combination in a Patient with TTP Complicated by SARSCoV-2 Nosocomial Infection.

Medicina (Kaunas) 2021 Apr 28;57(5). Epub 2021 Apr 28.

Department of Anesthesia and Intensive Care Unit, Università degli Studi di Foggia, Azienda Ospedaliero Universitaria Ospedali Riuniti di Foggia, 1, 71122 Viale Pinto, FG, Italy.

Carbapenem-resistant (CRE) are a serious public health threat. Infections due to these organisms are associated with significant morbidity and mortality. Among them, metallo-β-lactamases (MBLs)-producing are of global concern today. Read More

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Extracellular Vesicles Tune the Immune System in Renal Disease: A Focus on Systemic Lupus Erythematosus, Antiphospholipid Syndrome, Thrombotic Microangiopathy and ANCA-Vasculitis.

Int J Mol Sci 2021 Apr 18;22(8). Epub 2021 Apr 18.

Department of Medical Sciences, University of Turin, Corso Dogliotti 14, 10126 Turin, Italy.

Extracellular vesicles (EV) are microparticles released in biological fluids by different cell types, both in physiological and pathological conditions. Owing to their ability to carry and transfer biomolecules, EV are mediators of cell-to-cell communication and are involved in the pathogenesis of several diseases. The ability of EV to modulate the immune system, the coagulation cascade, the angiogenetic process, and to drive endothelial dysfunction plays a crucial role in the pathophysiology of both autoimmune and renal diseases. Read More

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Functional and Genetic Landscape of Complement Dysregulation Along the Spectrum of Thrombotic Microangiopathy and its Potential Implications on Clinical Outcomes.

Kidney Int Rep 2021 Apr 3;6(4):1099-1109. Epub 2021 Feb 3.

Department of Nephrology and Clinical Immunology.

Introduction: The syndromes of thrombotic microangiopathy (TMA) are diverse and represent severe endothelial damage caused by various mechanisms. The complement system plays a major role in a subset of patients with TMA, and its recognition is of clinical importance because it guides choice and duration of treatment.

Methods: We studied a well-defined cohort of patients with TMA and hypothesized that assessment of serum-induced   C5b9 formation on the endothelium and screening for rare variants in complement genes can better categorize TMA. Read More

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Role of C5 inhibition in Idiopathic Inflammatory Myopathies and Scleroderma Renal Crisis-Induced Thrombotic Microangiopathies.

Kidney Int Rep 2021 Apr 29;6(4):1015-1021. Epub 2021 Jan 29.

Département de Néphrologie et transplantation d'Organes-Unité de Réanimation, Centre de référence des maladies rénales rares, Centre Hospitalier Universitaire, Institut National de la Santé et de la Recherche Médicale U1048 (Institut des Maladies Métaboliques et Cardiovasculaires), Toulouse, France.

Introduction: Connective tissue diseases, including systemic sclerosis and idiopathic inflammatory myopathies (IIMs), are a very rare cause of thrombotic microangiopathies (TMAs). Whether dysregulation of the complement pathways underlies these secondary forms of TMA and may be targeted by complement blocking agents remains elusive.

Methods: Kidney pathology and outcomes of 18 critically ill patients with TMA related to inflammatory myopathy flare-up (IIM, =7) or scleroderma renal crisis (SRC, =11; biopsy =9) are assessed. Read More

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Thrombotic Microangiopathy: A Rare Breast Cancer-associated Complication Treated Successfully With Doxorubicin and Cyclophosphamide.

In Vivo 2021 May-Jun;35(3):1885-1888

Department of Medical Oncology, Moffitt Cancer Center, Tampa, FL, U.S.A.

Background: Thrombotic microangiopathy (TMA) is a clinical syndrome consisting of hemolytic anemia, thrombocytopenia, and presence of schistocytes on peripheral blood smear secondary to disorders of systemic microvascular thrombosis. Malignancy-associated TMA is a rare entity and shares clinical features with that of HUS and TTP usually seen in patients with metastatic cancer, tumor cell infiltration of the bone marrow and/or response to cancer-directed therapy.

Case Report: We present a rare case of TMA secondary to breast cancer without evidence of bone marrow infiltration responsive to doxorubicin and cyclophosphamide treatment, after failed plasmapheresis with prednisone and later, eculizumab. Read More

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February 2021