Autoimmune myelofibrosis accompanied by Sjögren's syndrome in a 47, XXX/46, XX mosaic woman.
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.
Department of Hematology, Tenshi Hospital, Japan.
Tohru
T
Takahashi
D000328:Adult; D041321:Chromosomes, Human, X / Q000235:genetics; D005260:Female; D005500:Follow-Up Studies; D005820:Genetic Testing; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D011239:Prednisolone / Q000627:therapeutic use; D055728:Primary Myelofibrosis / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D012729:Sex Chromosome Aberrations; D058533:Sex Chromosome Disorders of Sex Development / Q000150:complications* / Q000235:genetics; D012859:Sjogren's Syndrome / Q000150:complications* / Q000175:diagnosis; D014057:Tomography, X-Ray Computed; D014314:Trisomy / Q000235:genetics
D005938:Glucocorticoids; D011239:Prednisolone