Résumé
BACKGROUND: Acquired idiopathic sideroblastic anemia (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.
Sujets)
Femelle , Humains , Mâle , Anémie , Anémie réfractaire avec excès de blastes , Anémie sidéroblastique , Myélogramme , Cause de décès , Lignée cellulaire , Diagnostic , Études de suivi , Hémorragie , Leucémies , Dossiers médicaux , Histoire naturelle , Études rétrospectives , Facteurs de risqueRésumé
PURPOSE: Chronic lymphocytic leukemia (CLL) is the most common form of leukemia in western countries but it has been reported rarely in Korea. The purpose of this study was to evaluate the clinical characteristics, treatment responses and prognosis of the patients with CLL. MATERIALS AND METHODS: We retrospectively reviewed the medical records of 17 patients with CLL diagnosed at the Catholic University of Taegu-Hyosung & Kyungpook National University Hospital from Jan. 1986 to Mar. 1996. RESULTS: There were 9 males and 8 females and the mean age was 55 years. The majority of patients had systemic symptoms (77%) and advanced clinical stage (Binet stage C, 70%) at the time of diagnosis. The bone marrow histology was reviewed in 13 cases and patients were subdivided into those with a diffuse (9cases) and nondiffuse (4cases) pattern of bone marrow histology. In patients with advanced clinical stage, diffuse pattern was predominated and showed poor survival. 14 patients were treated with chlorambucil/steroid or combination chemotherapy. Among the 13 evaluable patients, 1 (8%) achieved a complete response and 7 (54%) achieved partial responses, and median survival duration was 18months. 11 patients died after 2~18 months of follow-up and infection was the main cause of death. CONCLUSION: Poor prognostic factors, such as high blood lymphocyte counts, diffuse bone marrow involvement pattern & advanced clinical stage, are common in our patients at presentation & showed poor survival.
Sujets)
Femelle , Humains , Mâle , Moelle osseuse , Cause de décès , Diagnostic , Association de médicaments , Études de suivi , Corée , Leucémies , Leucémie chronique lymphocytaire à cellules B , Numération des lymphocytes , Dossiers médicaux , Pronostic , Études rétrospectivesRésumé
No abstract available.
Sujets)
Néoplasie endocrinienne multiple de type 2a , Néoplasie endocrinienne multipleRésumé
No abstract available.
Sujets)
Néoplasie endocrinienne multiple de type 2a , Néoplasie endocrinienne multipleRésumé
The authors carried out the funduscopic examination in 53 cases of leukemia (acute myeloblastic leukemia 17, acute lymphoblastic leukemia 2.0, acute monocytic leukemia 9, chronic myelogenous leukemia 5, acute progranulocytic leukemia 2) and 51 cases of various anemias (aplastic anemia 37, iron deficiency anemia, secondary anemia due to bleeding 4, miscellaneous 4) which were confirmed by hematological studies at Kyung-Pook National University Hospital from October 1969 to October 1975. The following results were obtained. 1) Of 53 cases of leukemia, 62% revealed retinopathy with retinal homorrhage. In 65% of 17 cases of acute myeloblastic leukemia, 55% of 2.0 cases of acute lymphoblastic leukemia, 77 % of 9 cases of acute monocytic leukemia, and 4.0% of 5 cases of chronic myelogenous leukemia, retinopathies were noted. 2) Of 51 cases of anemia, 45% revealed retinopathy with retinal hemorrhage. In 49% of 37 cases of aplastic anemia, and 33% of 6 cases of iron deficiency anemia, retinopathies were noted, but none of secondary anemia due to blood loss had retinal hemorrhage. 3) Acute leukemia had more retinal hemorrhage frequency than chronic leukemia. 4) The extent of the retinal hemorrhages seemed to parallel the severity of the anemia in aplastic anemia. 5) Generally the thrombocytopenia was noted in the aplastic anemia but the extent of the retinal hemorrhages not seemed to parallel the severity of the thrombocytopenia. 6) The extent of the retinal hemorrhages seemed to parallel the severity of the anemia in iron deficiency anemia. 7) Specific retinal pictures could not be noted in the various blood dyscrasias. 8) With the retinal pictures, no differentiation on type of the leukemia and the anemia could be noted.