Biomaterial Database

Mastocytosis with malabsorption, myelofibrosis, and massive ascites. 1987

I R Reisberg, and S Oyakawa

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.

UI	MeSH Term	Description	Entries
D007579	Jejunal Diseases	Pathological development in the JEJUNUM region of the SMALL INTESTINE.	Disease, Jejunal,Diseases, Jejunal,Jejunal Disease
D008198	Lymph Nodes	They are oval or bean shaped bodies (1 - 30 mm in diameter) located along the lymphatic system.	Lymph Node,Node, Lymph,Nodes, Lymph
D008286	Malabsorption Syndromes	General term for a group of MALNUTRITION syndromes caused by failure of normal INTESTINAL ABSORPTION of nutrients.	Malabsorption Syndrome,Syndrome, Malabsorption,Syndromes, Malabsorption
D008407	Mast Cells	Granulated cells that are found in almost all tissues, most abundantly in the skin and the gastrointestinal tract. Like the BASOPHILS, mast cells contain large amounts of HISTAMINE and HEPARIN. Unlike basophils, mast cells normally remain in the tissues and do not circulate in the blood. Mast cells, derived from the bone marrow stem cells, are regulated by the STEM CELL FACTOR.	Basophils, Tissue,Basophil, Tissue,Cell, Mast,Cells, Mast,Mast Cell,Tissue Basophil,Tissue Basophils
D008415	Mastocytosis	A rare neoplastic disorder characterized by a clonal proliferation of MAST CELLS, associated with KIT-D816 mutations, and accompanied by aberrant mast cell activation. The abnormal increase of MAST CELLS may occur in only the skin (MASTOCYTOSIS, CUTANEOUS), in extracutaneous tissues involving multiple organs (MASTOCYTOSIS, SYSTEMIC), or in solid tumors (MASTOCYTOMA).
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328	Adult	A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available.	Adults
D001201	Ascites	Accumulation or retention of free fluid within the peritoneal cavity.
D055728	Primary Myelofibrosis	A de novo myeloproliferation arising from an abnormal stem cell. It is characterized by the replacement of bone marrow by fibrous tissue, a process that is mediated by CYTOKINES arising from the abnormal clone.	Agnogenic Myeloid Metaplasia,Bone Marrow Fibrosis,Chronic Idiopathic Myelofibrosis,Fibrosis, Bone Marrow,Idiopathic Myelofibrosis,Myelofibrosis,Myelofibrosis With Myeloid Metaplasia,Myeloid Metaplasia,Myelosclerosis,Myelosis, Nonleukemic,Agnogenic Myeloid Metaplasias,Bone Marrow Fibroses,Fibroses, Bone Marrow,Metaplasia, Agnogenic Myeloid,Metaplasia, Myeloid,Metaplasias, Agnogenic Myeloid,Metaplasias, Myeloid,Myelofibroses,Myelofibroses, Primary,Myelofibrosis, Primary,Myeloid Metaplasia, Agnogenic,Myeloid Metaplasias,Myeloid Metaplasias, Agnogenic,Myeloscleroses,Myeloses, Nonleukemic,Nonleukemic Myeloses,Nonleukemic Myelosis,Primary Myelofibroses