Biomaterial Database

Familial mastocytosis: a clinical, immunophenotypic, light and electron microscopic study. 1991

A Anstey, and D G Lowe, and J D Kirby, and M A Horton

Department of Dermatology, St Bartholomew's Hospital, London, U.K.

A women and her daughter both developed diffuse cutaneous mastocytosis in early infancy. The immunophenotypes of the cutaneous mast cells in the two cases were shown to be identical: leucocyte common antigen (CD45) and macrophage/monocyte marker EBM11 were both strongly positive, HLA-DR was positive and CD25 was weakly positive. The peripheral lymphocytes of the patients were analysed by flow cytometry and this revealed that there was no increase in the activation markers HLA-DR and CD25, contrary to recent claims that lymphocytes have a contributory role in driving mast-cell proliferation. Mast-cell granules in both cases were found on electron microscopy to have an abnormal ultrastructure. The close similarity between the two cases at clinical, immunophenotypic and ultrastructural levels strongly suggests that genetic factors are important in the development of this type of mastocytosis.

UI	MeSH Term	Description	Entries
D007223	Infant	A child between 1 and 23 months of age.	Infants
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
D008854	Microscopy, Electron	Microscopy using an electron beam, instead of light, to visualize the sample, thereby allowing much greater magnification. The interactions of ELECTRONS with specimens are used to provide information about the fine structure of that specimen. In TRANSMISSION ELECTRON MICROSCOPY the reactions of the electrons that are transmitted through the specimen are imaged. In SCANNING ELECTRON MICROSCOPY an electron beam falls at a non-normal angle on the specimen and the image is derived from the reactions occurring above the plane of the specimen.	Electron Microscopy
D005260	Female		Females
D006649	Histocompatibility Antigens	A group of antigens that includes both the major and minor histocompatibility antigens. The former are genetically determined by the major histocompatibility complex. They determine tissue type for transplantation and cause allograft rejections. The latter are systems of allelic alloantigens that can cause weak transplant rejection.	Transplantation Antigens,Antigens, Transplantation,Histocompatibility Antigen,LD Antigens,SD Antigens,Antigen, Histocompatibility,Antigens, Histocompatibility,Antigens, LD,Antigens, SD
D006684	HLA-DR Antigens	A subclass of HLA-D antigens that consist of alpha and beta chains. The inheritance of HLA-DR antigens differs from that of the HLA-DQ ANTIGENS and HLA-DP ANTIGENS.	HLA-DR,Antigens, HLA-DR,HLA DR Antigens
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
D012867	Skin	The outer covering of the body that protects it from the environment. It is composed of the DERMIS and the EPIDERMIS.
D014582	Urticaria Pigmentosa	The most common form of cutaneous mastocytosis (MASTOCYTOSIS, CUTANEOUS) that occurs primarily in children. It is characterized by the multiple small reddish-brown pigmented pruritic macules and papules.	Maculopapular Cutaneous Mastocytosis,Cutaneous Mastocytoses, Maculopapular,Cutaneous Mastocytosis, Maculopapular,Maculopapular Cutaneous Mastocytoses