Article
Hematology
M. Scully, R. Rayment, A. Clark, J. P. Westwood, T. Cranfield, R. Gooding, C. N. Bagot, A. Taylor, V. Sankar, D. Gale, T. Dutt, J. McIntyre, W. Lester, BSH Comm
Summary: The objective of this guideline is to provide healthcare professionals with clear, up-to-date and practical guidance on the management of thrombotic thrombocytopenic purpura (TTP) and related thrombotic microangiopathies (TMAs), including complement-mediated haemolytic uraemic syndrome (CM HUS); these are defined by thrombocytopenia, microangiopathic haemolytic anaemia (MAHA) and small vessel thrombosis. Within England, all TTP cases should be managed within designated regional centres as per NHSE commissioning for highly specialised services.
BRITISH JOURNAL OF HAEMATOLOGY
(2023)
Article
Cell Biology
Mohammad Al-Tamimi, Jianlin Qiao, Elizabeth E. Gardiner
Summary: Primary thrombotic microangiopathies such as TTP and HUS have distinct etiologies, while secondary TMAs have a wide range of causes. Early diagnosis and treatment are crucial for patient well-being, but distinguishing TTP from HUS based on presentation is challenging. Evaluation of platelet function is limited by the central role of thrombocytopenia and platelet activation in different types of TMAs, prompting the urgent need for alternative means of monitoring platelet activity.
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)
Article
Hematology
Jason Tau, Leonor P. Fernando, Meilen C. Munoz, Christina Poh, Viswanathan V. Krishnan, Denis M. Dwyre
Summary: This study retrospectively analyzed the clinical characteristics of patients suspected of having TTP and found that early therapeutic plasma exchange can reduce mortality rates for both TTP and non-TTP TMA. The study also highlighted the diagnostic challenge of TMA.
THERAPEUTIC APHERESIS AND DIALYSIS
(2023)
Article
Hematology
Konstantine Halkidis, Don L. Siegel, X. Long Zheng
Summary: This study demonstrates that a human monoclonal antibody fragment isolated from a patient with acute iTTP can activate ADAMTS-13 and increase proteolytic cleavage, suggesting a potential role in modulating inhibition by other antibodies against ADAMTS-13. These findings provide new insights into the pathogenesis of iTTP.
JOURNAL OF THROMBOSIS AND HAEMOSTASIS
(2021)
Article
Hematology
Marie Scully, Lucy Neave
Summary: A State of the Art lecture on the etiology and outcomes of thrombotic microangiopathies (TMAs) in pregnancy was presented at the 2022 International Society on Thrombosis and Haemostasis Congress. The lecture highlighted the importance of understanding changes in laboratory parameters during normal pregnancy and discussed specific TMAs associated with pregnancy, such as preeclampsia (PET) and hemolysis, elevated liver enzymes, and low platelets (HELLP). The presentation also emphasized the complications in diagnosing TMAs in pregnancy and the management strategies for each subtype.
RESEARCH AND PRACTICE IN THROMBOSIS AND HAEMOSTASIS
(2023)
Article
Medicine, General & Internal
Misbahuddin Khaja, Zaheer A. Qureshi, Sameer Kandhi, Faryal Altaf, Laura Yapor
Summary: Thrombotic thrombocytopenic purpura (TTP) is a rare condition caused by severely reduced ADAMTS13 or the von Willebrand factor-cleaving protease (VWFCP) enzyme, resulting in low platelet and red blood cell counts along with severe renal, cardiac, and neurological dysfunction. Early diagnosis and aggressive management often lead to a favorable outcome.
CUREUS JOURNAL OF MEDICAL SCIENCE
(2022)
Article
Multidisciplinary Sciences
Andry van de Louw, Eric Mariotte, Michael Darmon, Austin Cohrs, Douglas Leslie, Elie Azoulay
Summary: Immediate TPE and early rituximab therapy are associated with improved survival in TTP patients. The use of improved treatments has led to a decrease in mortality over time, and other outcome variables such as hospital resource utilization or long-term outcomes should be considered.
Article
Hematology
Tanja Falter, Heidi Rossmann, Laure de Waele, Charlotte Dekimpe, Charis von Auer, Nadine Mueller-Calleja, Friederike Haeuser, Adriana Degreif, Dana Marandiuc, Xavier Messmer, Martin Sprinzl, Karl J. Lackner, Kerstin Jurk, Karen Vanhoorelbeke, Bernhard Laemmle
Summary: Insufficient proteolytic processing of von Willebrand factor (VWF) multimers (MMs) caused by ADAMTS13 deficiency may contribute to the recurrence of immune-mediated thrombotic thrombocytopenic purpura (iTTP).
Article
Hematology
Jana van den Berg, Johanna A. Kremer Hovinga, Claudia Pfleger, Inga Hegemann, Gregor Stehle, Andreas Holbro, Jan-Dirk Studt
Summary: Immune thrombotic thrombocytopenic purpura (iTTP) is a life-threatening disease that can be treated with the anti-CD38 antibody daratumumab to eradicate persistent ADAMTS13 inhibitory autoantibodies and restore normal ADAMTS13 activity.
Article
Immunology
Cecile L. Karsenty, Susan E. Kirk, Hannah L. Helber, Jose M. Esquilin, Jenny M. Despotovic, Amanda B. Grimes
Summary: This article reviews thrombotic thrombocytopenic purpura (TTP) and discusses two cases of TTP in adolescents, emphasizing the need to consider late-onset congenital TTP (cTTP). The importance of early confirmation of ADAMTS13 enzyme deficiency and the presence or absence of ADAMTS13 inhibitor is demonstrated in order to initiate appropriate life-saving therapies. Ultimately, molecular testing is crucial for distinguishing between congenital and acquired immune-mediated TTP.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Hematology
Cristina Pascual Izquierdo, Maria Eva Mingot-Castellano, Ana E. Kerguelen Fuentes, Jose Garcia-Arroba Peinado, Joan Cid, Maria Moraima Jimenez, David Valcarcel, Ines Gomez-Segui, Javier de la Rubia, Paz Martin, Rosa Goterris, Luis Hernandez, Inmaculada Tallon, Sara Varea, Marta Fernandez, Nadia Garcia-Munoz, Miriam Vara, Miguel Fernandez Zarzoso, Faustino Garcia-Candel, Maria Liz Paciello, Irene Garcia-Garcia, Saioa Zalba, Veronica Campuzano, Jose Maria Gala, Julia Vidan Estevez, Gemma Moreno Jimenez, Jose Luis Lopez Lorenzo, Elena Gonzalez Arias, Carmen Freiria, Maria Sole, Laura Francisca Avila Idrovo, Jose Carlos Hernandez Castellet, Naylen Cruz, Esperanza Lavilla, Albert Perez-Montana, Jon Ander Atucha, Maria Esperanza Moreno Beltran, Juan Ramon Moreno Macias, Ramon Salinas, Julio del Rio-Garma
Summary: In patients with immune thrombotic thrombocytopenic purpura (iTTP), Caplacizumab reduces exacerbations and refractoriness compared to standard therapy and provides a faster clinical response when administered within the first 3 days after plasma exchange (PEX). Additionally, it reduces hospitalization time and the need for PEX.
Article
Pathology
Spencer D. Martin, Eric McGinnis, Tyler W. Smith
Summary: This study analyzed the clinical and laboratory parameters to differentiate TTP from other TMAs, finding that TTP patients had higher frequency of neurological symptoms, more severe thrombocytopenia, and less creatinine elevation compared to other TMAs. High PLASMIC scores had good sensitivity and specificity for TTP diagnoses, but additional ADAMTS13 activity testing was necessary in some cases for accurate diagnosis.
AMERICAN JOURNAL OF CLINICAL PATHOLOGY
(2021)
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)
Review
Biochemistry & Molecular Biology
Martina Mazzariol, Giovanni Camussi, Maria Felice Brizzi
Summary: Extracellular vesicles play a crucial role in cell-to-cell communication and are involved in various diseases by modulating the immune system, coagulation cascade, angiogenetic process, and endothelial dysfunction. They have been investigated as potential biomarkers for renal injury and may have future applications as diagnostic and therapeutic tools in renal diseases.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
