title: An unusual case of myelodysplastic syndrome with prolonged clonal stability, indolent clinical course over a decade, and spontaneous regression of AML in the terminal phase.
abstract: An unusual case of secondary acute myeloid leukemia (AML) with indolent clinical course is described. The patient, a 67-yr-old female, had first been diagnosed to suffer from low-risk myelodysplastic syndrome, subtype refractory anemia with ringed sideroblasts, in 1992. In 2001, transformation to secondary AML with an increase in bone marrow blasts (>20%) and thrombocytopenia, was found. The patient did not require cytoreductive drugs. Rather, during the following months, spontaneous improvement of peripheral blood cells with normalization of platelets and decrease in the red cell transfusion frequency, were noted. In October 2002, she even became transfusion independent. However, the bone marrow still showed AML with >20% blasts. These blast cells exhibited a monoclonal pattern in the human androgen receptor (HUMARA) assay. However, no chromosomal defects occurred during a total observation period of 14 yr. We hypothesize that clonal stability may have contributed to the indolent course of the disease in this patient. The exact mechanisms underlying clinical and genetic stability remain unknown, however.
authors.affiliation: Division of Hematology and Hemostaseology, Department of Internal Medicine I, Medical University of Vienna, Währinger Gürtel 18-20, A-1090 Vienna, Austria.
authors.forename: Karoline
authors.initials: K
authors.lastname: Sonneck
authors.forename: Christine
authors.initials: C
authors.lastname: Mannhalter
authors.forename: Maria-Theresa
authors.initials: MT
authors.lastname: Krauth
authors.forename: Wolfgang R
authors.initials: WR
authors.lastname: Sperr
authors.forename: Ilse
authors.initials: I
authors.lastname: Schwarzinger
authors.forename: Christa
authors.initials: C
authors.lastname: Fonatsch
authors.forename: Oskar
authors.initials: O
authors.lastname: Haas
authors.forename: Klaus
authors.initials: K
authors.lastname: Geissler
authors.forename: Peter
authors.initials: P
authors.lastname: Valent
mesh_terms: D001853:Bone Marrow / Q000473:pathology / Q000503:physiopathology; D005260:Female; D006801:Humans; D015470:Leukemia, Myeloid, Acute / Q000209:etiology / Q000473:pathology / Q000503:physiopathology*; D008875:Middle Aged; D009190:Myelodysplastic Syndromes / Q000150:complications*; D011944:Receptors, Androgen / Q000097:blood; D012074:Remission Induction; D013921:Thrombocytopenia / Q000209:etiology / Q000473:pathology / Q000503:physiopathology
chemical_list: D011944:Receptors, Androgen