Review
Urology & Nephrology
Lilian Monteiro Pereira Palma, Meera Sridharan, Sanjeev Sethi
Summary: Thrombotic microangiopathy (TMA) is a condition characterized by thrombocytopenia and microangiopathic hemolytic anemia (MAHA) with involvement of complement. TMA can be classified into primary and secondary forms, each with distinct etiology and treatment approaches.
KIDNEY INTERNATIONAL REPORTS
(2021)
Article
Genetics & Heredity
I Mancini, P. Agosti, M. Boscarino, B. Ferrari, A. Artoni, R. Palla, M. Spreafico, G. Crovetti, E. Volpato, S. Rossini, C. Novelli, S. Gattillo, L. Barcella, M. Salmoiraghi, A. Falanga, F. Peyvandi
Summary: This study aimed to evaluate the role of centralized, rapid testing of ADAMTS13 in patients with acute thrombotic microangiopathy (TMAs) and estimate the incidence of TTP in a large Italian Region. The study found that severe ADAMTS13 deficiency distinguished two groups of patients with overlapping clinical features but different treatment and disease course.
ORPHANET JOURNAL OF RARE DISEASES
(2022)
Article
Urology & Nephrology
Esra Karabag Yilmaz, Memnune Nur Cebi, Iremnaz Karahan, Seha Saygili, Ruveyda Gulmez, Ebru Burcu Demirgan, Cansu Durak, Fatih Aygun, Fatih Ozaltin, Salim Caliskan, Nur Canpolat
Summary: This report describes two cases of thrombotic microangiopathy (TMA) associated with COVID-19, one of which was successfully treated with eculizumab. PCR tests confirmed SARS-CoV-2 infection in both cases, and laboratory results showed typical signs of TMA. No known cause for TMA was found in either case.
Review
Hematology
Michelle Schoettler, Satheesh Chonat, Kirsten Williams, Leslie Lehmann
Summary: The review discusses the impact of transplant-associated thrombotic microangiopathy (TA-TMA) on haematopoietic cell therapy recipients and the role of complement activation and endothelial dysfunction in the disease. It highlights the use of eculizumab in high-risk patients but emphasizes the need for novel approaches due to high mortality rates. Ongoing clinical trials are investigating novel complement agents and prophylactic strategies for TA-TMA, aiming to improve patient care and outcomes in the future.
CURRENT OPINION IN HEMATOLOGY
(2021)
Review
Hematology
Gemma L. Thonnpson, David Kavanagh
Summary: Thrombotic microangiopathy (TMA) is a disease characterized by low platelet count, microangiopathic hemolytic anemia, and organ damage. TMAs can present with a variety of clinical manifestations due to different underlying pathophysiology. Kidney involvement is common due to its susceptibility to endothelial damage and microvascular occlusion. Rapid assessment, diagnosis, and treatment are necessary for TMAs due to their high morbidity and mortality rates.
INTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY
(2022)
Review
Biology
Sapna Devi, Neelesh Sharma, Touqeer Ahmed, Zul Huma, Savleen Kour, Bijayalaxmi Sahoo, Amit Kumar Singh, Nino Macesic, Sung Jin Lee, Mukesh Kumar Gupta
Summary: The paper emphasizes the importance of fast and precise diagnosis of animal diseases and targeted treatments. It discusses the limitations of current diagnostic methods and highlights the potential of aptamers in developing biosensors for sensitive detection and therapy in veterinary medicine.
SAUDI JOURNAL OF BIOLOGICAL SCIENCES
(2021)
Review
Oncology
Leonidas Trakolis, Athanasios K. Petridis
Summary: Recent advances in diagnostics and adjuvant therapy of meningiomas have raised questions about the oncological relevance of complete tumor removal. Some authors consider the Simpson grading system to be outdated and opt for a more conservative approach. This narrative review discusses the significance of the extent of resection in aggressive meningiomas in terms of oncological outcomes and postoperative functional outcomes.
Article
Pediatrics
Saeed AlZabali, Sawsan AlBatati, Khawla Rahim, Hassan Faqeehi, Abubaker Osman, Abdulaziz Bamhraz, Mohammed A. Saleh, Jameela A. Kari, Majed Aloufi, Loai Eid, Haydar Nasser, Abubakr Imam, Entesar AlHammadi, Omar Alkandari, Mohammed Al Riyami, Sidharth Sethi, Christoph Licht, Khalid A. Alhasan, Abdulkarim AlAnazi
Summary: This study aimed to evaluate the rate and risk factors of recurrence in patients with aHUS who discontinued eculizumab treatment. During the follow-up period after discontinuation, 28.5% of patients experienced HUS relapse, with positive genetic mutations being the main risk factor for relapse. In terms of renal outcomes after discontinuation, most patients achieved remission with normal renal function, but a few patients developed chronic kidney disease or required kidney transplantation.
Article
Hematology
Eric Mariotte, Lara Zafrani, Jehane Fadlallah, Lionel Galicier, Etienne Ghrenassia, Lionel Kerhuel, Laure Calvet, Audrey De Jong, Virginie Lemiale, Sandrine Valade, Berangere S. Joly, Alain Stepanian, Elie Azoulay, Michael Darmon
Summary: This study evaluated the accuracy of diagnostic scores for thrombotic thrombocytopenic purpura (TTP) in an intensive care unit (ICU) setting, finding that platelet count could be similarly used to differentiate TTP from non-TTP patients as the Coppo score.
THROMBOSIS AND HAEMOSTASIS
(2021)
Review
Hematology
Konstantine Halkidis, X. Long Zheng
Summary: ADAMTS13 is a crucial plasma enzyme for normal hemostasis, and deficiency caused by acquired autoantibodies can lead to a potentially fatal blood disorder. Recent research has shown that ADAMTS13 exists in multiple conformations and can be regulated by antibodies in different ways.
JOURNAL OF THROMBOSIS AND HAEMOSTASIS
(2022)
Review
Pharmacology & Pharmacy
Binbin Chen, Mengli Liu, Chengzhi Huang
Summary: The outbreak and spread of COVID-19 has posed a great threat to global public health, highlighting the importance of deeper understanding of this viral illness for the development of vaccines and treatment options.
JOURNAL OF PHARMACEUTICAL ANALYSIS
(2021)
Review
Cardiac & Cardiovascular Systems
Manzhi Wang, Tao Sun, Liang Dong, Shengshi Huang, Ju Liu
Summary: Symmetrical peripheral gangrene (SPG) is a rare condition characterized by ischemic damage and tissue death in the extremities. Recent reports have shown SPG in severe COVID-19 patients, presenting with cyanosis and necrosis. Microthrombi, immunothrombosis, endothelial dysfunction, and hypercoagulation contribute to SPG development. Thrombotic microangiopathy, shock, disseminated intravascular coagulation, and anticoagulant depletion further exacerbate the condition. This review discusses the clinical features, mechanisms, and potential treatments for COVID-19-associated SPG.
FRONTIERS IN CARDIOVASCULAR MEDICINE
(2023)
Review
Immunology
Michelle L. L. Schoettler, Harshil Bhatt, Sumithira Vasu
Summary: Transplant-associated thrombotic microangiopathy (TA-TMA) is a complication of hematopoietic cellular therapy (HCT) with significant morbidity and mortality. Although the disease is associated with endothelial damage and complement activation, specific diagnostic biomarkers have not been identified. This study reviewed the diagnostic, early risk, and prognostic biomarkers of TA-TMA, finding that sC5b-9 was the most robust biomarker. However, further research is needed to validate and establish cut-off points for biomarkers in separate cohorts.
FRONTIERS IN IMMUNOLOGY
(2023)
Editorial Material
Critical Care Medicine
Anthony W. Martinelli, William Dunn, Mark E. McClure, Ieuan Walker, Andrew Stewart, Sumit Karia, Stephen D. Preston, Sathia Thiru, Nicholas Torpey, Sanjay Ojha, Emily Symington, James A. Nathan
Summary: This article describes a rare case of acute presentation of sarcoidosis, resulting in severe renal and hematological dysfunction. Despite the absence of significant respiratory symptoms, radiological and histological findings support sarcoidosis as the underlying cause of the multiorgan presentation. This case highlights the importance of considering sarcoidosis in unusual multiorgan cases and involving multidisciplinary teams early for optimal treatment.
Review
Hematology
Aneeqa Zafar, Ming Yeong Lim, Mouhamed Yazan Abou-Ismail
Summary: Drug-induced TMA (DI-TMA) is a thrombotic microangiopathy caused by certain drugs, and its management usually involves drug discontinuation and supportive measures. This study found that eculizumab, a complement-inhibiting drug, appears to be effective in achieving hematologic and renal recovery in severe or refractory DI-TMA cases. The findings suggest that eculizumab may be considered as a potential treatment option, but larger studies are needed.
THROMBOSIS RESEARCH
(2023)