Sweet's syndrome in a patient with idiopathic myelofibrosis and thymoma- myasthenia gravis-immunodeficiency complex: Efficacy of treatment with etretinate

G. Altomare, G. L. Capella, E. Frigerio

Research output: Contribution to journalArticle

Abstract

Here we present the case of a patient affected with a unique association of Sweet's syndrome, idiopathic myelofibrosis, spindle-cell thymoma, myasthenia gravis and Good's syndrome (a rare form of thymoma-related, combined immunodeficiency presenting with recurrent respiratory infections). Conventional therapies (corticosteroids, colchicine, DDS, dofazimine) were ineffective or were contraindicated. Treatment with etretinate (50 mg/day) proved effective on skin lesions. Moreover, the patient's general condition unexpectedly improved, with long-lasting (11 months) suppression of respiratory infections and a slight but consistent improvement of hematological parameters such as Hct, MCV, Hb level, RBC, WBC and lymphocyte absolute counts.

Original languageEnglish
Pages (from-to)54-58
Number of pages5
JournalHaematologica
Volume81
Issue number1
Publication statusPublished - 1996

Keywords

  • Good's-syndrome
  • Myelofibrosis
  • Retinoids
  • Sweet's syndrome
  • Thymoma

ASJC Scopus subject areas

  • Hematology

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