scholarly journals Treatment of thrombotic thrombocytopenic purpura

2021 ◽  
Vol 93 (6) ◽  
pp. 736-745
Author(s):  
Gennadii M. Galstyan ◽  
Alexei A. Maschan ◽  
Elizaveta E. Klebanova ◽  
Irina I. Kalinina
Keyword(s):  
Cyclosporin A ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Pediatric Patients ◽  
Treatment Options ◽  
Acute Attack ◽  
Thrombocytopenic Purpura

The review discusses approaches to treatment of acquired thrombotic thrombocytopenic purpuгa (aTTP). In patients with aTTP plasma exchanges, glucocorticosteroids allow to stop an acute attack of TTP, and use of rituximab allows to achieve remission. In recent years, caplacizumab has been used. Treatment options such as cyclosporin A, bortezomib, splenectomy, N-acetylcysteine, recombinant ADAMTS13 are also described. Separately discussed issues of management of patients with TTP during pregnancy, and pediatric patients with TTP.

scholarly journals Treatment of thrombotic thrombocytopenic purpura

2021 ◽  
Vol 93 (7) ◽  
pp. 826-829
Author(s):  
Gennadii M. Galstyan ◽  
Alexei A. Maschan ◽  
Elizaveta E. Klebanova ◽  
Irina I. Kalinina
Keyword(s):  
Factor Viii ◽  
Plasma Exchange ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Pediatric Patients ◽  
Treatment Options ◽  
Clotting Factor ◽  
Thrombocytopenic Purpura ◽  
Congenital Thrombotic Thrombocytopenic Purpura

The review discusses approaches to treatment of congenital thrombotic thrombocytopenic purpura (TTP) or Upshaw-Schulman syndrome. In congenital TTP, plasma transfusions are sufficient. Such treatment options as plasma exchange, administration of clotting factor VIII concentrate, recombinant ADAMTS13, are also used. Separately discussed issues of management of patients with TTP during pregnancy, and pediatric patients with TTP.

Cyclosporin A therapy on idiopathic thrombotic thrombocytopenic purpura in the relapse setting: two case reports and a review of the literature

Transfusion ◽  
2012 ◽  
Vol 53 (7) ◽  
pp. 1586-1593 ◽  
Author(s):  
Murvet Yilmaz ◽  
Ahmet Emre Eskazan ◽  
Abdulkadir Unsal ◽  
Hakan Taninmis ◽  
Ekrem Kara ◽  
...  
Keyword(s):  
Cyclosporin A ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Case Reports ◽  
Review Of The Literature ◽  
Thrombocytopenic Purpura

scholarly journals Caplacizumab Model‐Based Dosing Recommendations in Pediatric Patients with Acquired Thrombotic Thrombocytopenic Purpura

2021 ◽  
Author(s):  
Martin Bergstrand ◽  
Emma Hansson ◽  
Bernard Delaey ◽  
Filip Callewaert ◽  
Rui De Passos Sousa ◽  
...  
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Pediatric Patients ◽  
Thrombocytopenic Purpura ◽  
Model Based

scholarly journals Severe Acral Necrosis Complicating Thrombotic Microangiopathy in Two Toddlers

2020 ◽  
Vol 2020 ◽  
pp. 1-3
Author(s):  
H. Nassih ◽  
Z. Lazrak ◽  
S. Younous
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Anticoagulant Therapy ◽  
Thrombotic Microangiopathy ◽  
Pediatric Patients ◽  
Thrombocytopenic Purpura ◽  
Uremic Syndrome ◽  
Acral Necrosis ◽  
Acral Ischemia

Acral ischemia/necrosis is one of the rarest but most dreadful complications of thrombotic microangiopathy in pediatric patients. It is more reported with thrombotic thrombocytopenic purpura than with hemolytic and uremic syndrome. Even with anticoagulant therapy, it is often irreversible, leading to amputation.

Frequent recurrence of pregnancy-triggered congenital thrombotic thrombocytopenic purpura: A rare case report

2020 ◽  
pp. 1-6
Author(s):  
Jialiang Xu ◽  
Sijian Yu ◽  
Fuhua Zhang
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Novel Mutation ◽  
Immunosuppressive Agents ◽  
Acute Attack ◽  
Genetic Mutation ◽  
Plasma Therapy ◽  
Thrombocytopenic Purpura ◽  
Rare Case Report ◽  
Congenital Thrombotic Thrombocytopenic Purpura ◽  
Von Willebrand

Thrombotic thrombocytopenic purpura (TTP) in adults is rare thrombotic microangiopathy (TMA), which is closely related to the lack of specific proteases of von Willebrand factor (vWF) multimers. It is currently believed that both congenital TTP (cTTP) and acquired TTP (aTTP) can induce acute attack through pregnancy. We report a case of a 24-year-old woman who was diagnosed as TTP during early pregnancy. A novel mutation was detected: c.3667G>T (p.E1223*). She responded well to plasma therapy during pregnancy and had a child by cesarean section at 32 weeks. TTP is still recurrent in postpartum patients. The plasma transfusion was effective, but caused a severe transfusion reaction. Cyclosporine was administered with the consent of the patient. This case showed cyclosporine-A (CSA) had a positive effect on ADAMTS13 activity. At 11-months follow-up, the patient’s blood cells and LDH status were stable and no symptom was seen. Our case suggests that the patient had an unreported genetic mutation that causes TTP, immune factors may be involved in the onset of cTTP during pregnancy, and the use of immunosuppressive agents is effective in preventing recurrence.

Relapsed/refractory thrombotic thrombocytopenic purpura treated with N-acetylcysteine: a case report

2018 ◽  
Vol 63 (4) ◽  
pp. 122-124
Author(s):  
Sinan Demircioğlu ◽  
Ömer Ekinci ◽  
Ali Doğan ◽  
Cengiz Demir
Keyword(s):  
Case Report ◽  
Renal Impairment ◽  
Plasma Exchange ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Treatment Options ◽  
Rare Condition ◽  
High Dose ◽  
Thrombocytopenic Purpura ◽  
Loss Of Response ◽  
Refractory Case

Thrombotic thrombocytopenic purpura is a rare condition that presents with microangiopathic haemolytic anaemia, thrombocytopaenia, fever, renal impairment and neurological symptoms. Plasma exchange is a lifesaving treatment for this condition. However, some cases may be non-responsive to plasma exchange, or loss of response may occur. Treatment options for refractory cases include high-dose corticosteroids, rituximab, vincristine, cyclophosphamide, splenectomy, bortezomib and N-acetylcysteine. We present a refractory case of thrombotic thrombocytopenic purpura responding to the last of these therapies.

New treatment options for thrombotic thrombocytopenic purpura

2017 ◽  
Vol 37 (03) ◽  
pp. 211-215 ◽  
Author(s):  
Paul Knöbl
Keyword(s):  
Platelet Aggregation ◽  
Plasma Exchange ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Treatment Options ◽  
Autoimmune Process ◽  
Thrombocytopenic Purpura ◽  
Missing Enzyme ◽  
Disturbed Microcirculation ◽  
Life Threatening ◽  
New Treatment

SummaryThe thrombotic-thrombocytopenic purpura (TTP) is an acute, life-threatening disease, characterised by enhanced platelet aggregation, disturbed microcirculation and organ dysfunction. With the currently available treatment (plasma exchange, infusions, corticosteroids) mortality ist still as high as 10–15 %. Recent, pathophysiology-based developments may improve the outcome. The most promising candidates for future treatment of TTP are: rituximab for termination of the autoimmune process, caplacizumab for prevention of platelet-VWF-interaction, and recombinant ADAMTS13 for replacement of the inhibited or missing enzyme.

scholarly journals Daratumumab for immune thrombotic thrombocytopenic purpura

2021 ◽  
Author(s):  
Jana Van den Berg ◽  
Johanna A Kremer Hovinga ◽  
Claudia Pfleger ◽  
Inga Hegemann ◽  
Gregor Thomas stehle ◽  
...  
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Treatment Options ◽  
Current Treatment ◽  
First Episode ◽  
Thrombocytopenic Purpura ◽  
Life Threatening ◽  
Adamts13 Deficiency ◽  
Treatment Refractory ◽  
Circulating Autoantibodies

Immune thrombotic thrombocytopenic purpura (iTTP) is a life-threatening thrombotic microangiopathy. It is caused by a severe ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 motifs, 13) deficiency due to circulating autoantibodies, and is associated with significant morbidity and mortality. Current treatment options include plasma exchange, immunosuppression, and caplacizumab. When remission is achieved, the risk of relapse is high, especially in patients with persistent ADAMTS13 deficiency. We report the eradication of persistent ADAMTS13 inhibitory autoantibodies and restoration of normal ADAMTS13 activity using the anti-CD38 antibody daratumumab in two patients with iTTP. One patient had a frequently relapsing course, and the other a treatment-refractory first episode. There were no relevant adverse drug reactions.

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