Open AccessSYSTEMIC MASTOCYTOSIS WITHOUT CUTANEOUS INVOLVEMENT
Author(s) -
Katsuda Shogo,
Okada Yoshikatsu,
Oda Yoshio,
Tanimoto Kazuo,
Takabatake Satoru
Publication year - 1987
Publication title -
acta patholigica japonica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.73
H-Index - 74
ISSN - 0001-6632
DOI - 10.1111/j.1440-1827.1987.tb03144.x
Subject(s) - pathology , autopsy , medicine , bone marrow , hepatosplenomegaly , spleen , lymph , systemic mastocytosis , urticaria pigmentosa , infiltration (hvac) , malaise , immunology , disease , physics , thermodynamics
An autopsy case of systemic mastocytosis without cutaneous involvement in a 76‐year‐old woman was described. The patient presented with general malaise, chest and epigastric discomfort, flushing of the face and progressive hepatosplenomegaly, and she terminated in hemorrhagic complications of DIC within 2 months. There was neither rash nor urticaria pigmentosa recognizable in the entire course. The diagnosis was made by the histologic identification of abnormal aggregates of mast cells in a bone marrow aspirate. These mast cell granules were chloroacetate esterase‐positive, peroxidase‐negative, and electronmicroscopically they were composed of fine granular materials containing variable numbers of lamellar structures. At autopsy, diffuse infiltration of the mast cells was found in the liver, spleen, bone marrow, lymph nodes, lungs, kidneys, stomach, and adrenal glands.