title: [Acquired hemoglobin H disease associated with a myelodysplastic syndrome].
abstract: Some patients found to have clonal panmyelopathies develop an acquired defect of haemoglobin synthesis clinically similar to haemoglobin H disease. A 58 year-old male diagnosed of simple refractory anaemia developed microcytosis and hypochromia. At the same time, his myelodysplastic syndrome became a refractory anaemia with excess of blasts. 33% of the red blood cells had "golf ball" inclusions after incubation with brilliant cresyl blue. Cellulose acetate electrophoresis revealed an haemoglobin H band. The globin chain synthesis alpha/beta ratio was 0.69. The molecular analysis demonstrated the integrity of both alpha genes in each chromosome. There were no familiar antecedent of haemoglobinopathy.
authors.affiliation: Servicio de Hematología, Hospital Universitario San Carlos, Madrid.
authors.forename: J
authors.initials: J
authors.lastname: Pérez Calvo
authors.forename: F A
authors.initials: FA
authors.lastname: González Fernández
authors.forename: T
authors.initials: T
authors.lastname: Santillana
authors.forename: C
authors.initials: C
authors.lastname: Alarcón
authors.forename: M
authors.initials: M
authors.lastname: Fariñas
authors.forename: J
authors.initials: J
authors.lastname: Sánchez
authors.forename: R
authors.initials: R
authors.lastname: Martínez Martínez
authors.forename: A
authors.initials: A
authors.lastname: Villegas
mesh_terms: D000754:Anemia, Refractory, with Excess of Blasts / Q000097:blood / Q000150:complications* / Q000473:pathology; D001773:Blood Cells / Q000473:pathology; D001853:Bone Marrow / Q000473:pathology; D004801:Eosine Yellowish-(YS); D005914:Globins / Q000096:biosynthesis / Q000235:genetics; D006801:Humans; D002479:Inclusion Bodies / Q000648:ultrastructure; D008297:Male; D008751:Methylene Blue; D008875:Middle Aged; D010078:Oxazines; D013194:Staining and Labeling; D017085:alpha-Thalassemia / Q000097:blood / Q000209:etiology* / Q000473:pathology
chemical_list: D010078:Oxazines; C006797:Brilliant Cresyl Blue; D005914:Globins; D008751:Methylene Blue; D004801:Eosine Yellowish-(YS)