Reduced Intensity Conditioning Regimen Followed by Allogeneic Peripheral Blood Stem Cell Transplantation for Acute Panmyelosis with Myelofibrosis

Hyeok Shim; Young-Jin Lee; Moo Rim Park

doi:10.5045/kjh.2005.40.4.266

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Shim, Lee, and Park: Reduced Intensity Conditioning Regimen Followed by Allogeneic Peripheral Blood Stem Cell Transplantation for Acute Panmyelosis with Myelofibrosis

Case Report

Korean J Hematol 2005;40(4):266-270.

Published online: 21 December 2005

DOI: https://doi.org/10.5045/kjh.2005.40.4.266

Reduced Intensity Conditioning Regimen Followed by Allogeneic Peripheral Blood Stem Cell Transplantation for Acute Panmyelosis with Myelofibrosis

Hyeok Shim, M.D.¹, Young-Jin Lee, M.D.², Moo Rim Park, M.D.¹

¹Department of Internal Medicine, Wonkwang University School of Medicien, Iksan, Korea

²Department of Laboratory Medicine, Wonkwang University School of Medicien, Iksan, Korea

Correspondence to: Moo Rim Park, M.D. Department of Internal Medicine, Wonkwang University School of Medicine 344-2 Sinyong-dong, Iksan 507-711, Korea Tel: +82-63-850-1083, Fax: +82-63-855-2025 E-mail: mrpark21@wonkwang.ac.kr

Received 4 November 2005 Revised 3 December 2005 Accepted 16 December 2005

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

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.

Keywords: Acute panmyelosis with myelofibrosis, Peripheral blood stem cell transplantation, Reduced intensity conditioning

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Fig. 1.

Bone marrow biopsy section at the time of diagnosis reveals marked fibrosis and some immature cells (A, H&E stain, ×400), and shows extensive reticulin fibrosis (B, reticulin stain, ×400).

Fig. 2.

After 1 year of stem cell transplantation, bone marrow biopsy section shows the normocellularity without myelofibrosis (A, H&E stain, ×400), and no evidence of reticulin fibrosis (B, reticulin stain, ×400).

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