Acute Vulvar Ulceration as the Main Presenting Manifestation of Hemophagocytic Syndrome
A 14-year-old female exhibited an acute vulvar ulcer during the course of hemophagocytic syndrome (HPS). The patient presented persistent high fever and a deep painful vulvar ulcer lasting for more than 2 weeks. Neither infection with sexually transmitted agents nor autoimmune disorder were found to...
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Veröffentlicht in: | Pediatric hematology and oncology 2003, Vol.20 (5), p.421-425 |
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Format: | Artikel |
Sprache: | eng |
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Zusammenfassung: | A 14-year-old female exhibited an acute vulvar ulcer during the course of hemophagocytic syndrome (HPS). The patient presented persistent high fever and a deep painful vulvar ulcer lasting for more than 2 weeks. Neither infection with sexually transmitted agents nor autoimmune disorder were found to be positive. The presence of hemophagocytosis in the bone marrow and elevation of urinary g -2-microglobulin ( g -2M) prompted the diagnosis of HPS. Acute vulvar ulcer is rare, but it should be recognized as a mucous manifestation of HPS. During the clinical course, urinary g -2M was the most sensitive marker for the evaluation of the disease activity of HPS. |
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ISSN: | 0888-0018 1521-0669 |
DOI: | 10.1080/08880010390203990 |