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Prepublished online as a Blood First Edition Paper on April 3, 2003; DOI 10.1182/blood-2002-12-3706.
Submitted December 5, 2002
Accepted March 25, 2003
Polyclonal hematopoiesis maintained in patients with bone marrow failure harboring a minor population of paroxysmal nocturnal hemoglobinuria-type cells
Ken Ishiyama, Tatsuya Chuhjo, Hongbo Wang, Akihiro Yachie, Mitsuhiro Omine, and Shinji Nakao*
Cellular Transplantation Biology, Division of Cancer Medicine, Kanazawa University Graduate School of Medical Science, Kanazawa, Japan
Preventive Environment Unit, Kanazawa University Hospital, Kanazawa, Japan
School of Health Sciences, Faculty of Medicine, Kanazawa University, Kanazawa, Japan
Division of Hematology, Fujigaoka Hospital, Showa University School of Medicine, Yokohama, Japan
* Corresponding author; email: snakao{at}med3.m.kanazawa-u.ac.jp.
Although a minor population of paroxysmal nocturnal hemoglobinuria (PNH)-type blood cells is often detected in patients with aplastic anemia (AA) and refractory anemia (RA), the significance of such cells in the pathophysiology of bone marrow failure remains obscure. We therefore examined clonality in peripheral blood granulocytes from 118 female patients with AA or myelodysplastic syndrome utilizing the X-chromosome inactivation pattern. Clonality, defined as a clonal population accounting for 35% or more of total granulocytes, was confirmed in 22 of 68 (32.4%) AA patients, in 13 of 44 (29.5%) RA patients, in all 4 RA with excess blasts (RAEB), and in 4 with PNH. When the frequency of patients with granulocyte clonality was compared with respect to the presence of increased PNH-type cells, the frequency was significantly lower in AA patients with (PNH+; 21.2%) than without (PNH-; 42.9%) increased numbers of PNH-type cells (p=0.049). Clonality was absent in granulocytes from the 15 PNH+ RA patients but present in 13 of 29 (44.8%) PNH- RA patients (p=0.0013). The absence of clonality in AA and RA patients before treatment was strongly associated with positive response to immunosuppressive therapy (without clonality, 74.4%; with clonality, 33.3%; p=0.0031) in all patients as well as in PNH+ patients (without clonality, 96.2%; with clonality 66.6%, p=0.026). These results suggest that AA and RA with a minor population of PNH-type cells are benign types of bone marrow failure with immune pathophysiology that have little relationship with clonal disorders such as RAEB or acute myeloid leukemia.
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