Síndrome de Plummer-Vinson: uma rara associação na talassemia / Plumer-Vinson syndrome: a rare association with thalassemia
Rev. bras. hematol. hemoter
; 29(4): 412-415, out.-dez. 2007. ilus, tab
Article
in Portuguese
| LILACS
| ID: lil-476784
Responsible library:
BR408.1
Localization: Br408.1
RESUMO
A síndrome de Plummer-Vinson é caracterizada por disfagia cervical, deficiência de ferro e presença de membrana esofágica. Neste estudo, relatam-se dois casos dessa síndrome em irmãos adolescentes. Eles não obtiveram aumento dos níveis hematimétricos após reposição com ferro oral, o que, associado à eletroforese de hemoglobinas, sustentou o diagnóstico de talassemia concomitante. Devido ao quadro dos filhos, os pais foram também submetidos à eletroforese de hemoglobinas cujo diagnóstico do pai foi talassemia alfa/beta menor e da mãe, talassemia alfa menor. Os irmãos tiveram disfagia refratária e necessitaram de dilatação endoscópica. Ambos necessitaram de terapia com ferro venoso com melhora dos níveis hematimétricos.
ABSTRACT
Plummer-Vinson syndrome is characterized by cervical dysphagia, iron deficiency and the presence of esophageal membranes. We report two cases of this syndrome present in adolescent brothers with associated thalassemia. After oral iron therapy, their hematimetric levels showed no increase, which associated with the results of hemoglobin electrophoresis, sustained the diagnosis of thalassemia. Due to the condition of the children, the parents were submitted to hemoglobin electrophoresis examinations; the father was diagnosed as having minor alpha/beta thalassemia and the mother as minor beta thalassemia. Both patients suffered from refractory dysphagia and required endoscopic dilatation. They both underwent venous iron therapy, which improved the hematimetric levels.
Full text:
Available
Collection:
International databases
Database:
LILACS
Main subject:
Plummer-Vinson Syndrome
Type of study:
Risk factors
Language:
Portuguese
Journal:
Rev. bras. hematol. hemoter
Journal subject:
Hematology
Year:
2007
Document type:
Article
Affiliation country:
Brazil
Institution/Affiliation country:
UFTM/BR