Multicentric Castleman disease and systemic lupus erythematosus phenotype in a boy with Klinefelter syndrome: Long-term disease stabilization with interferon therapy

R. Simko, K. Nagy, B. Lombay, A. Kiss, K. Minik, V. H. Lukacs, I. Vamosi

Research output: Contribution to journalArticle

21 Citations (Scopus)

Abstract

An 11-year-old boy with Klinefelter syndrome had Castleman disease (CD) of plasma cell type develop. Nonregulated antibody production mimicked systemic lupus erythematosus (SLE). Hepatitis C virus (HCV) infection caused significant disease worsening. The patient was treated with a daily dosage of 2 million units/m2 of IFN-α. Dramatic clinical improvement and decreasing autoimmune phenomenon were observed. HCV RNA were cleared. Hypergammaglobulinemia did not change. The boy has been living for 8 years with his disease. Plasma cell type CD can mimic collagenosis. Disease worsening is caused by HCV, though it can be reversed with IFN-α. Klinefelter syndrome may be a genetic susceptibility factor for CD in some cases.

Original languageEnglish
Pages (from-to)180-183
Number of pages4
JournalJournal of Pediatric Hematology/Oncology
Volume22
Issue number2
DOIs
Publication statusPublished - 2000

Fingerprint

Giant Lymph Node Hyperplasia
Klinefelter Syndrome
Hepacivirus
Systemic Lupus Erythematosus
Interferons
Plasma Cells
Phenotype
Hypergammaglobulinemia
Virus Diseases
Genetic Predisposition to Disease
Antibody Formation
Therapeutics
RNA
Multi-centric Castleman's Disease

Keywords

  • Castleman disease
  • Interferon therapy
  • SLE-Klinefelter syndrome

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Oncology
  • Hematology

Cite this

Multicentric Castleman disease and systemic lupus erythematosus phenotype in a boy with Klinefelter syndrome : Long-term disease stabilization with interferon therapy. / Simko, R.; Nagy, K.; Lombay, B.; Kiss, A.; Minik, K.; Lukacs, V. H.; Vamosi, I.

In: Journal of Pediatric Hematology/Oncology, Vol. 22, No. 2, 2000, p. 180-183.

Research output: Contribution to journalArticle

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