ALLOGENEIC HEMATOPOIETIC STEM CELL TRANSPLANTATION IN FANCONI ANEMIA; A SINGLE CENTRE EXPERIENCE

  • Tariq Azam Khattak Armed Forces Bone Marrow Transplant Center/National University of Medical Sciences (NUMS) Rawalpindi Pakistan
  • Muhammad Farhan Armed Forces Bone Marrow Transplant Center/National University of Medical Sciences (NUMS) Rawalpindi Pakistan
  • Tariq Ghafoor Armed Forces Bone Marrow Transplant Center/National University of Medical Sciences (NUMS) Rawalpindi Pakistan
  • Tariq Mahmood Satti Armed Forces Bone Marrow Transplant Center/National University of Medical Sciences (NUMS) Rawalpindi Pakistan
  • Qamar Un Nisa Chaudhry Armed Forces Bone Marrow Transplant Center/National University of Medical Sciences (NUMS) Rawalpindi Pakistan
  • Mehreen Ali Khan Armed Forces Bone Marrow Transplant Center/National University of Medical Sciences (NUMS) Rawalpindi Pakistan
Keywords: Fanconi Anemia, Hematopoietic Stem Cell Transplant, Graft Versus Host Disease

Abstract

Objective: To determine the treatment outcome of Hematopoietic stem cell transplantation in Fanconi Anemia.

Study Design: Case series.

Place and Duration of Study: Armed Forces Bone Marrow Transplant Center, Rawalpindi, from Jan 2001 to Jun 2018.

Methodology: Data of all Fanconi anemia patients who had fully HLA matched bone marrow transplant during this period was analysed for variables affecting the outcome in terms of overall survival. Those fanconi anemia patients who had myelodysplastic changes or acute myeloid leukemia were excluded.

Results: Total 27 patients underwent fully HLA matched allogeneic bone marrow transplant for Fanconi Anemia. Mean age of patients at transplant was 12.12 ± 5.16 years. All patients at transplant were in aplastic phase. Conditioning was done with fludarabine 120mg/m2 , ATG 20 mg/kg and Cyclophosphamide at a dose of 20-40 mg/kg. Mean time to neutrophil engraftment was 12.3 ± 2.92days and for platelets 20 ± 10.3 days. Major posttransplant complications were neutropenic fever in 26 (96%), hypertension in 18 (66.6%), mucositis in 12 (44.4%), azotaemia in 8 (29.6%), gut toxicity in 7 (25.9%) and haemorrhagic cystitis in 5 (18.5%) patients. Four patients (14.8%) had acute graft versus host disease while 7 (26%) patients had chronic GVHD. Overall survival at 6 months, 1, 5 and 8 years was 67%, 63%, 59% and 55% respectively. While overall survival in patients transplanted at younger age (<11 years) was 81.8% compared to 37.5% in older age group (>11years) and was statistically significant (p-value = 0.03).

Conclusion: Our study demonstrated...................

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Author Biographies

Tariq Ghafoor, Armed Forces Bone Marrow Transplant Center/National University of Medical Sciences (NUMS) Rawalpindi Pakistan

Pediatric Oncologist, Associate professor of Pediatrics AM College

Tariq Mahmood Satti, Armed Forces Bone Marrow Transplant Center/National University of Medical Sciences (NUMS) Rawalpindi Pakistan

Professor of Hematology AM college, Clinical hematologist and Bone Marrow Transplant specilaist

Qamar Un Nisa Chaudhry, Armed Forces Bone Marrow Transplant Center/National University of Medical Sciences (NUMS) Rawalpindi Pakistan

Assistant Professor of Hematology AM college, Clinical hematologist and BMT specislist

Published
2021-02-24
How to Cite
Khattak, T., Farhan, M., Ghafoor, T., Satti, T., Chaudhry, Q., & Khan, M. (2021). ALLOGENEIC HEMATOPOIETIC STEM CELL TRANSPLANTATION IN FANCONI ANEMIA; A SINGLE CENTRE EXPERIENCE. PAFMJ, 71(1), 190-95. https://doi.org/10.51253/pafmj.v71i1.3140
Section
Original Articles