-
Idiopathic refractory sideroblastic anemia: incidence and risk factors for leukemic transformation.
The clinical course of 29 patients with idiopathic refractory sideroblastic anemia studied by us was reviewed. Four patients developed acute leukemia. We were able to find 27 out of 268 cases of idiopathic refractory sideroblastic anemia reported in the literature which terminated in acute leukemia. Nine of these were well described. The overall incidence of acute leukemia is 10%. In an attempt to identify the risk factors for the development of acute leukemia, the clinical and laboratory features of this group of thirteen well-described cases which terminated in acute leukemia were compared to the remaining 25 of our cases which did not undergo leukemic transformation. The patients who died of acute leukemia tended to have a more severe anemia, a lower reticulocyte count, and increased transfusion requirement, and thrombocytopenia. Thrombocytosis appears to be a relatively good prognostic sign.
PMID: 383260 [PubMed - indexed for MEDLINE]
-
Cited by 5 PubMed Central articles
-
Late-onset X-linked sideroblastic anemia. Missense mutations in the erythroid delta-aminolevulinate synthase (ALAS2) gene in two pyridoxine-responsive patients initially diagnosed with acquired refractory anemia and ringed sideroblasts.
Cotter PD, May A, Fitzsimons EJ, Houston T, Woodcock BE, al-Sabah AI, Wong L, Bishop DF.
J Clin Invest. 1995 Oct; 96(4):2090-6.
[J Clin Invest. 1995]
-
Prevalence and distribution of ringed sideroblasts in primary myelodysplastic syndromes.
Juneja SK, Imbert M, Sigaux F, Jouault H, Sultan C.
J Clin Pathol. 1983 May; 36(5):566-9.
[J Clin Pathol. 1983]
-
Haematological features of primary myelodysplastic syndromes (PMDS) at initial presentation: a study of 118 cases.
Juneja SK, Imbert M, Jouault H, Scoazec JY, Sigaux F, Sultan C.
J Clin Pathol. 1983 Oct; 36(10):1129-35.
[J Clin Pathol. 1983]
- » See all...