Best practices and recommendations for drug regimens and plasma exchange for immune thrombotic thrombocytopenic purpura

Gómez-Seguí, Inés; Izquierdo, Cristina Pascual; Comos, Javier de la Rubia

doi:10.1080/17474086.2021.1956898

Cited by 9 publications

(10 citation statements)

References 126 publications

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“…In thrombotic thrombocytopenic purpura (TTP) the presence of fragmented red blood cells/schistocytes is pathognomonic. In addition, thrombocytopenia, increased reticulocyte count, increased indirect bilirubin levels, increased lactate dehydrogenase levels and absent haptoglobin are usually present 41 . Coagulation tests are normal in TTP.…”

Section: Vasculopathymentioning

confidence: 99%

Inflammatory skin diseases and wounds

Dissemond¹,

Romanelli

2022

Br J Dermatol

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Inflammatory wounds of the skin can be caused by many different diseases. Of particular importance here are the very heterogeneous groups of vasculitides and vasculopathies. These are usually interdisciplinary relevant diseases that require extensive diagnostics in specialized centres. Clinically, these inflammatory wounds present as very painful ulcers that develop from necrosis and are surrounded by erythematous livid borders. The healing process is often difficult and protracted. Moreover, it considerably impairs the quality of life of the affected patients. In addition to clinical evaluation, histopathological examination of biopsies taken as early as possible is particularly important in diagnosis. Numerous differential diagnoses must be ruled out. Therapeutically, in addition to the often necessary systemic therapies, which include immunosuppressants (immunomodulating drugs and/or rheologics), appropriate topical wound treatment, usually in combination with compression therapy, should always be considered. Whenever possible, the causative factors should be diagnosed early and avoided or treated.

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Section: Vasculopathymentioning

confidence: 99%

Inflammatory skin diseases and wounds

Dissemond¹,

Romanelli

2022

Br J Dermatol

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“…Thrombotic thrombocytopenic purpura (TTP), a severe and often deadly disease, has been extensively studied over the past several decades. The widely accepted mechanism of TTP involves the deficiency of congenital and/or antibodies to immune-mediated a disintegrin and metalloprotease with a thrombospondin type I motif, member 13 (ADAMTS13) [ 1 , 2 ]. The lack of ADAMTS13 leads to an inability to cleave ultralong von Willebrand factor (UL-vWF) multimers, which aggregate with platelets to create microthrombi [ 1 , 2 , 3 ].…”

Section: Introductionmentioning

confidence: 99%

“…The widely accepted mechanism of TTP involves the deficiency of congenital and/or antibodies to immune-mediated a disintegrin and metalloprotease with a thrombospondin type I motif, member 13 (ADAMTS13) [ 1 , 2 ]. The lack of ADAMTS13 leads to an inability to cleave ultralong von Willebrand factor (UL-vWF) multimers, which aggregate with platelets to create microthrombi [ 1 , 2 , 3 ]. This leads to thrombotic microangiopathy (TMA) and characteristic symptoms of TTP.…”

Section: Introductionmentioning

confidence: 99%

Pathological Mechanisms and Novel Testing Methods in Thrombotic Thrombocytopenic Purpura

Dolin,

Maitta

2024

Biomedicines

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Thrombotic thrombocytopenic purpura (TTP) is an uncommon, but potentially disabling or even deadly, thrombotic microangiopathy with a well-studied mechanism of ADAMTS13 deficiency or dysfunction. While established treatments are largely effective, the standard ADAMTS13 testing required to definitively diagnose TTP may cause delays in diagnosis and treatment, highlighting the need for rapid and effective diagnostic methods. Additionally, the heterogeneous presentation and varied inciting events of TTP suggest more variation in its mechanism than previously thought, implying three potential pathways rather than the accepted two. The recent discovery of ADAMTS13 conformation as a potential contributor to TTP in addition to the proposal of using the absolute immature platelet count (A-IPC) as a biomarker, present novel areas for monitoring and treatment. A-IPC in particular may serve as a more rapid and accurate diagnostic test to distinguish TTP from non-TTP TMAs and to monitor treatment response and relapse. These considerations highlight the need to further study TTP in order to improve best practices and patient care.

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“…Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a diagnostic emergency that demands prompt therapy initiation to prevent severe known complications associated with the disease. 2 Deficiency of the metalloprotease ADAMTS13 with generation of autoantibodies to this enzyme characterize iTTP, resulting in the formation of diffuse microthrombi that can affect all organ systems. Thus, therapy to treat this disease requires not only active removal of these autoantibodies from circulation [through therapeutic plasma exchange (TPE)] but use of immunosuppression to reduce the amount of autoantibody produced.…”

mentioning

confidence: 99%

“…Immune‐mediated thrombotic thrombocytopenic purpura (iTTP) is a diagnostic emergency that demands prompt therapy initiation to prevent severe known complications associated with the disease 2 . Deficiency of the metalloprotease ADAMTS13 with generation of autoantibodies to this enzyme characterize iTTP, resulting in the formation of diffuse microthrombi that can affect all organ systems.…”

mentioning

confidence: 99%

Anti‐CD20 therapeutic options in immune‐mediated thrombotic thrombocytopenic purpura

Maitta

2022

Br J Haematol

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Summary Immunosuppression with rituximab in immune‐mediated thrombotic thrombocytopenic purpura helps decrease production of autoantibody mediating ADAMTS13 clearance from circulation. Failure to respond to rituximab in a satisfactory way or made difficult by adverse events to the medication does not represent a reason to stop considering anti‐CD20 therapies to control antibody production. Therefore, both of atumumab and obinutuzumab with specificity to CD20, represent potentially valuable therapeutic tools in patients who are not candidates for rituximab. Commentary on: Doyle et al. The use of obinutuzumab and ofatumumab in the treatment of immune thrombotic thrombocytopenic purpura. Br J Haematol. 2022;198:391‐396.1

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Best practices and recommendations for drug regimens and plasma exchange for immune thrombotic thrombocytopenic purpura

Cited by 9 publications

References 126 publications

Inflammatory skin diseases and wounds

Inflammatory skin diseases and wounds

Pathological Mechanisms and Novel Testing Methods in Thrombotic Thrombocytopenic Purpura

Anti‐CD20 therapeutic options in immune‐mediated thrombotic thrombocytopenic purpura

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