Reduced Intensity Conditioning Regimen Followed by Allogeneic Peripheral Blood Stem Cell Transplantation for Acute Panmyelosis with Myelofibrosis.
10.5045/kjh.2005.40.4.266
- Author:
Hyeok SHIM
1
;
Young Jin LEE
;
Moo Rim PARK
Author Information
1. Department of Internal Medicine, Wonkwang University School of Medicien, Iksan, Korea. mrpark21@wonkwang.ac.kr
- Publication Type:Case Report
- Keywords:
Acute panmyelosis with myelofibrosis;
Peripheral blood stem cell transplantation;
Reduced intensity conditioning
- MeSH:
Adult;
Biopsy;
Bone Marrow;
Busulfan;
Cell Line;
Chimerism;
Cytarabine;
Dizziness;
Dyspnea;
Fibrosis;
Humans;
Idarubicin;
Male;
Microsatellite Repeats;
Pancytopenia;
Peripheral Blood Stem Cell Transplantation*;
Primary Myelofibrosis*;
Remission Induction;
Reticulin;
Siblings
- From:Korean Journal of Hematology
2005;40(4):266-270
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
A 44-year-old male presented with a month history of exertional dyspnea and dizziness. A peripheral blood smear revealed a pancytopenia with 3% of blasts. We were not able to obtain a bone marrow aspirate, but a biopsy specimen showed hypercellularity, proliferation of trilineage cell lines (panmyelosis) with extensive myelofibrosis, and clusters of immature cells at the paratrabecular area. After remission induction therapy with idarubicin 12mg/m2 (D1-3) and cytosine arabinoside 100mg/m2 (D1-7), the bone marrow blast count was decreased, but the marrow fibrosis and pancytopenia persisted. Peripheral blood stem cell transplantation from his HLA-matched brother was performed after administering fludarabine 30mg/m2 for 5 days and busulfan 3.2mg/kg for 2 days. Early engraftment occurred and the bone marrow reticulin fibrosis disappeared. Full-donor chimerism was demonstrated at day 22 by performing short tandem repeats analysis and this was maintained for 1 year. The patient has survived 20 months after transplantation without any complication.