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Diagnosis and Management of Acquired Aplastic Anemia: Consensus Statement of Indian Academy of Pediatrics

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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.

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Author information

Authors and Affiliations

  1. Action Balaji Hospital, New Delhi, India

    Arun Danewa

  2. Pediatric Hematology Oncology and Bone Marrow Transplantation unit, Institute for Child Health, Sir Ganga Ram Hospital, New Delhi, 110 060, India

    Manas Kalra, Anupam Sachdeva, Pallavi Sachdeva & Divij Sachdeva

  3. PGIMER, Chandigarh, India

    Deepak Bansal

  4. Narayana Health City, Bangalore, Karnataka, India

    Sunil Bhat

  5. Rainbow Children’s Hospital, Hyderabad, Andhra Pradesh, India

    Sirisha Rani

  6. Medanta Hospitals, Gurugram, Haryana, India

    Satya P. Yadav & Dhwanee Desai

  7. Manipal Hospital, Jaipur, Rajasthan, India

    Satyendra Katewa

  8. KGMU, Lucknow, Uttar Pradesh, India

    Archana Kumar

  9. Rela Institute and Medical Centre, Chennai, Tamil Nadu, India

    Deendayalan Muniratnam

  10. BJ Wadia Hospital for Children, Mumbai, Maharashtra, India

    Bharat R. Agarwal

  11. AIIMS, New Delhi, India

    Tulika Seth

  12. Indraprastha Apollo Hospitals, New Delhi, India

    Amita Mahajan & Gaurav Kharya

  13. Fortis Memorial Research Institute, Gurugram, Haryana, India

    Vikas Dua

  14. SRCC Children’s Hospital, Mumbai, Maharashtra, India

    Ruchira Misra

  15. Kauvery Hospital, Tiruchirappalli, Tamil Nadu, India

    Vinod Gunasekaran

  16. Ministry of Health and Family Welfare, New Delhi, India

    Vinita Srivastava

Authors
  1. Arun Danewa
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  2. Manas Kalra
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  3. Anupam Sachdeva
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  4. Pallavi Sachdeva
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  5. Deepak Bansal
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  6. Sunil Bhat
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  7. Divij Sachdeva
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  8. Sirisha Rani
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  9. Satya P. Yadav
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  18. Ruchira Misra
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  19. Dhwanee Desai
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Contributions

AD, MK, AS, PS, DB: designed the manuscript; AS, SB, DS, SR, SPY, SK, AK, DM, BRA, TS, AM, VD, GK, RM, DD, VG, VS: analyzed and critically reviewed the manuscript. All authors approved the final version of manuscript.

Corresponding author

Correspondence to Anupam Sachdeva.

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Danewa, A., Kalra, M., Sachdeva, A. et al. Diagnosis and Management of Acquired Aplastic Anemia: Consensus Statement of Indian Academy of Pediatrics. Indian Pediatr 59, 467–475 (2022). https://doi.org/10.1007/s13312-022-2538-x

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  • DOI: https://doi.org/10.1007/s13312-022-2538-x

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