Autoimmune myelofibrosis accompanied by Sjögren's syndrome in a 47, XXX/46, XX mosaic woman.

Abstract

This report describes a patient with autoimmune myelofibrosis accompanied by Sjögren's syndrome (SS). A 36-year-old woman was admitted due to petechiae, purpura, gingival bleeding, dyspnea on exertion, and a lack of concentration. She had pancytopenia and was diagnosed with SS. A bone marrow study showed hypercellular marrow with reticulin fibrosis. Lymphocytic infiltrates and aggregates composed of a mixture of T and B cells in the marrow were also observed. A chromosomal analysis of the marrow cells showed 47, XXX and an analysis of peripheral lymphocytes revealed 47, XXX/46, XX mosaic results. The patient's cytopenia resolved following treatment with oral prednisolone.

• Authors: Tohru Takahashi
• Journal: Internal medicine (Tokyo, Japan) (Intern Med) Vol. 53 Issue 7 Pg. 783-7 ( 2014) ISSN: 1349-7235 [Electronic] Japan
• PMID: 24694497 (Publication Type: Case Reports, Journal Article)

Chemical References

• Glucocorticoids
• Prednisolone

Topics

• Adult
• Chromosomes, Human, X (genetics)
• Female
• Follow-Up Studies
• Genetic Testing
• Glucocorticoids (therapeutic use)
• Humans
• Prednisolone (therapeutic use)
• Primary Myelofibrosis (complications, diagnosis, drug therapy)
• Sex Chromosome Aberrations
• Sex Chromosome Disorders of Sex Development (complications, genetics)
• Sjogren's Syndrome (complications, diagnosis)
• Tomography, X-Ray Computed
• Trisomy (genetics)