Mastocytosis with malabsorption, myelofibrosis, and massive ascites.
Mastocytosis is an unusual disorder of unknown etiology characterized by an abnormal proliferation of tissue mast cells. We present a case of a young woman with systemic mastocytosis with malabsorption secondary to small bowel invasion by mast cells, myelofibrosis, and massive exudative ascites secondary to mass cell invasion of the intraabdominal lymph nodes. The patient responded favorably to a combined therapy with histamine H1 and H2 receptor antagonists and corticosteroids. We include a review of the literature.
D000328:Adult; D001201:Ascites / Q000209:etiology / Q000473:pathology*; D005260:Female; D006801:Humans; D007579:Jejunal Diseases / Q000150:complications / Q000473:pathology*; D008198:Lymph Nodes / Q000473:pathology; D008286:Malabsorption Syndromes / Q000209:etiology / Q000473:pathology*; D008407:Mast Cells / Q000473:pathology; D008415:Mastocytosis / Q000150:complications / Q000473:pathology*; D055728:Primary Myelofibrosis / Q000209:etiology / Q000473:pathology*