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- Title
Diagnosis and Management of Acquired Aplastic Anemia: Consensus Statement of Indian Academy of Pediatrics.
- Authors
Danewa, Arun; Kalra, Manas; Sachdeva, Anupam; Sachdeva, Pallavi; Bansal, Deepak; Bhat, Sunil; Sachdeva, Divij; Rani, Sirisha; Yadav, Satya P.; Katewa, Satyendra; Kumar, Archana; Muniratnam, Deendayalan; Agarwal, Bharat R.; Seth, Tulika; Mahajan, Amita; Dua, Vikas; Kharya, Gaurav; Misra, Ruchira; Desai, Dhwanee; Gunasekaran, Vinod
- Abstract
Justification: In India, there is a lack of uniformity of treatment strategies for aplastic anemia (AA), and many children are managed only with supportive care due to non-availability of hematopoietic stem cell transplantation (HSCT). Process: Eminent national faculty members were invited to participate in the process of forming a consensus statement in Hyderabad in July, 2016. Draft guidelines were circulated to all members, and comments received in a online meeting in October, 2020 were incorporated into the final draft. These were approved by all experts. Objective: To facilitate appropriate management of children with acquired aplastic anemia. Recommendations: Key recommendations are: i) A bone marrow biopsy is must to make a diagnosis of AA; ii) Rule out inherited bone marrow failure syndromes (IBMFS), connective tissue disorders, viral infections, paroxysmal nocturnal hemoglobinuria (PNH), drug or heavy metal induced marrow suppression in all cases of AA; iii) Conservative approach to transfusions should be followed, with a target to keep hemoglobin >6 g/dL in children with no co-morbidities; iv) HLA-matched sibling donor HSCT is the preferred choice of treatment for newly diagnosed very severe/severe AA; v) In absence of HLA-matched family donor, a matched unrelated donor (MUD) transplant or immunosuppressive therapy (IST) should be considered as alternate choice based on physician expertise; vi) Fludarabine, cyclophosphamide and anti-thymocyte globulin (ATG) based conditioning with cyclosporine and methotrexate as graft versus host disease (GvHD) prophylaxis is the preferred regimen; vii) Horse ATG and cyclosporine are the recommended drugs for IST. One should wait for 3–6 months for the response assessment and consideration of next line therapy.
- Subjects
HYDERABAD (India); INDIA; APLASTIC anemia; HEMATOPOIETIC stem cell transplantation; GRAFT versus host disease; DIAGNOSIS; PAROXYSMAL hemoglobinuria; IMMUNOSUPPRESSIVE agents
- Publication
Indian Pediatrics, 2022, Vol 59, Issue 6, p467
- ISSN
0019-6061
- Publication type
Article
- DOI
10.1007/s13312-022-2538-x