Document Detail

Acquired hemoglobin H disease associated with a myelodysplastic syndrome
MedLine Citation:
PMID:  7524169     Owner:  NLM     Status:  MEDLINE    
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.
J Pérez Calvo; F A González Fernández; T Santillana; C Alarcón; M Fariñas; J Sánchez; R Martínez Martínez; A Villegas
Publication Detail:
Type:  Case Reports; English Abstract; Journal Article    
Journal Detail:
Title:  Sangre     Volume:  39     ISSN:  0036-4355     ISO Abbreviation:  Sangre (Barc)     Publication Date:  1994 Jun 
Date Detail:
Created Date:  1994-11-10     Completed Date:  1994-11-10     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0404373     Medline TA:  Sangre (Barc)     Country:  SPAIN    
Other Details:
Languages:  spa     Pagination:  211-3     Citation Subset:  IM    
Servicio de Hematología, Hospital Universitario San Carlos, Madrid.
Vernacular Title:
Enfermedad de la hemoglobina H adquirida asociada a síndrome mielodisplásico.
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MeSH Terms
Anemia, Refractory, with Excess of Blasts / blood,  complications*,  pathology
Blood Cells / pathology
Bone Marrow / pathology
Eosine Yellowish-(YS)
Globins / biosynthesis,  genetics
Inclusion Bodies / ultrastructure
Methylene Blue
Middle Aged
Staining and Labeling
alpha-Thalassemia / blood,  etiology*,  pathology
Reg. No./Substance:
0/Oxazines; 17372-87-1/Eosine Yellowish-(YS); 4712-70-3/Brilliant Cresyl Blue; 61-73-4/Methylene Blue; 9004-22-2/Globins

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