التفاصيل البيبلوغرافية
| العنوان: |
Narsoplimab for severe transplant-associated thrombotic microangiopathy |
| المؤلفون: |
Ambreen Pandrowala, Parth Ganatra, V. P. Krishnan, Ajay Narayan Sharma, Saroj Chavan, Minnie Bodhanwala, Bharat Agarwal, Prashant Hiwarkar |
| المصدر: |
Thrombosis Journal, Vol 21, Iss 1, Pp 1-6 (2023) |
| بيانات النشر: |
BMC, 2023. |
| سنة النشر: |
2023 |
| المجموعة: |
LCC:Diseases of the blood and blood-forming organs |
| مصطلحات موضوعية: |
Transplant-associated thrombotic microangiopathy, Narsoplimab, Lectin pathway inhibitor, Diseases of the blood and blood-forming organs, RC633-647.5 |
| الوصف: |
Abstract Background Transplantation-associated thrombotic microangiopathy (TA-TMA) is an endothelial injury syndrome linked to the overactivation of complement pathways. It manifests with microangiopathic hemolytic anemia, consumptive thrombocytopenia, and microvascular thrombosis leading to ischemic tissue injury. Mannose residues on fungi and viruses activate the mannose-binding lectin complement pathway, and hence activation of the lectin pathway could be one of the reasons for triggering TA-TMA. Narsoplimab, a human monoclonal antibody targeting MASP-2 is a potent inhibitor of the lectin pathway. We describe the transplant course of a pediatric patient who developed TA-TMA following Candida-triggered macrophage activation syndrome and was treated with Narsoplimab. The data collection was performed prospectively. Case presentation The six-year-old girl underwent a human leucocyte antigen (HLA) haploidentical hematopoietic stem cell transplant using post-transplant Cyclophosphamide for severe aplastic anemia. In the second week of the transplant, the patient developed macrophage activation syndrome necessitating treatment with steroids and intravenous immunoglobulin. Subsequently, USG abdomen and blood fungal PCR revealed the diagnosis of hepatosplenic candidiasis. Candida-triggered macrophage activation syndrome responded to antifungals, steroids, intravenous immunoglobulin, and alemtuzumab. However, the subsequent clinical course was complicated by thrombotic microangiopathy. The patient developed hypertension in the 2nd week, followed by high lactate dehydrogenase (1010 U/L), schistocytes (5 per hpf), low haptoglobin ( |
| نوع الوثيقة: |
article |
| وصف الملف: |
electronic resource |
| اللغة: |
English |
| تدمد: |
1477-9560 |
| Relation: |
https://doaj.org/toc/1477-9560 |
| DOI: |
10.1186/s12959-023-00464-9 |
| URL الوصول: |
https://doaj.org/article/b98160e98b744cd4ac5a842072ef86e5 |
| رقم الانضمام: |
edsdoj.b98160e98b744cd4ac5a842072ef86e5 |
| قاعدة البيانات: |
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