Korean J Hematol 1997; 32(3):
Published online September 30, 1997
© The Korean Society of Hematology
원발성 철적아구성 빈혈 환자의 임상양상
신동건, 최지용, 장성국, 이상채, 배정동, 손상균, 이규보, 황기석
대구효성가톨릭대학교 의과대학 내과학교실,
경북대학교 의과대학 내과학교실
Acquired Idiopathic Sideroblastic Anemia - A clinical study of 15 patients -
Background: Acquired idiopathic sideroblastic anenlia(AISA) is a heterogeneous condition. Most instances, involving only the erythroid line, are benign disease with a longer survival and a low propensity for evolution into acute leukemia. A subset of patients have severe clinical course and evidence of other cell line involvement at
presentation, may develop the emergence of blast cells and evolution into acute leukemia. In an attempt to identify the natural history and the risk factors for the development of acute leukemia, the clinical, hematological and outcome data were studied in the patients with AISA.
Methods: We reviewed retrospectively the medical records of 15 patients of AISA treated at the Catholic University of Taegu-Hyosung and Kyungpook National University Hospital from March 1989 to December 1995.
Results: The median age at diagnosis was 41 years and the male to female ratio was 8 : 7. On bone marrow examination, erythroid abnormalities were prominent in all
cases, 5 patients also showed involvement of the granulocytic and/or megakaryocytic cell lines(AISA with myelodysplastic features, AISA-M). The median follow-up duration was 32 months. Transfusion dependence occurred in 11 of 16 cases. Progression towards refractory anemia with excess of blasts or acute leukemia(M2) was observed in two
patients with AISA-M after follow-up period of 16 months and 24 months, respectively. Infections and hemorrhages were causes of death in 3 patients with AISA-M but not in
patients with dyserythropoiesis only (AISA-erythroid, AISA-E).
Conclusions : Most patients with AISA have a relatively benign course with prolonged survival after the onset of anemia. Patients with features of dysgranulopoiesis and/or
dysmegakaryopoiesis in addition to dyserythropoiesis at presentation were increased risk of transformation to refractory anemia with excess of blasts or acute leukemia and shorter surtival. But further study of larger numbers of patients and longer follow-up may be warranted.
Keywords Acquired idiopathic sideroblastic anemia; Acute leukemia; Dyserythropoiesis;
PDF
Standard view
Export citation
Share Article
Korean J Hematol 1997; 32(3): 360-366
Published online September 30, 1997
Copyright © The Korean Society of Hematology.
원발성 철적아구성 빈혈 환자의 임상양상
신동건, 최지용, 장성국, 이상채, 배정동, 손상균, 이규보, 황기석
대구효성가톨릭대학교 의과대학 내과학교실,
경북대학교 의과대학 내과학교실
Acquired Idiopathic Sideroblastic Anemia - A clinical study of 15 patients -
Dong Gun Shin, Ji Yong Choi, Sun Kug Chang, Sang Chae Lee, Jyung Dong Bae, Sang Kyun Sohn, Kyu Bo Lee, Kee Suk Whang
Department of Internal Medicine, College of Medicine, Catholicd University of Taegu, Hyosung
College of Medicine Kyougpook National University Hospital, Taegu, Korea
Abstract
Background: Acquired idiopathic sideroblastic anenlia(AISA) is a heterogeneous condition. Most instances, involving only the erythroid line, are benign disease with a longer survival and a low propensity for evolution into acute leukemia. A subset of patients have severe clinical course and evidence of other cell line involvement at
presentation, may develop the emergence of blast cells and evolution into acute leukemia. In an attempt to identify the natural history and the risk factors for the development of acute leukemia, the clinical, hematological and outcome data were studied in the patients with AISA.
Methods: We reviewed retrospectively the medical records of 15 patients of AISA treated at the Catholic University of Taegu-Hyosung and Kyungpook National University Hospital from March 1989 to December 1995.
Results: The median age at diagnosis was 41 years and the male to female ratio was 8 : 7. On bone marrow examination, erythroid abnormalities were prominent in all
cases, 5 patients also showed involvement of the granulocytic and/or megakaryocytic cell lines(AISA with myelodysplastic features, AISA-M). The median follow-up duration was 32 months. Transfusion dependence occurred in 11 of 16 cases. Progression towards refractory anemia with excess of blasts or acute leukemia(M2) was observed in two
patients with AISA-M after follow-up period of 16 months and 24 months, respectively. Infections and hemorrhages were causes of death in 3 patients with AISA-M but not in
patients with dyserythropoiesis only (AISA-erythroid, AISA-E).
Conclusions : Most patients with AISA have a relatively benign course with prolonged survival after the onset of anemia. Patients with features of dysgranulopoiesis and/or
dysmegakaryopoiesis in addition to dyserythropoiesis at presentation were increased risk of transformation to refractory anemia with excess of blasts or acute leukemia and shorter surtival. But further study of larger numbers of patients and longer follow-up may be warranted.
Keywords: Acquired idiopathic sideroblastic anemia, Acute leukemia, Dyserythropoiesis,
Previous Article
