Rare Diseases Symptoms Automatic Extraction

Expression of the basophil-specific antibodies 2D7 and BB1 in patients with cutaneous Mastocytosis.

[cutaneous mastocytosis]

2D7 and BB1 are thought to be basophil-specific markers. In this study, we tested both antibodies in different skin and mast cell disorders with the aim of determining whether it was possible to differentiate between benign and aggressive presentations of mastocytosis.Using the antibodies 2D7, BB1, and c-Kit, we performed an immunohistochemical study of skin biopsy specimens from patients with cutaneous mastocytosis (15 urticaria pigmentosa and telangiectatic macularis eruptive perstans) and liver or bone marrow biopsy specimens from patients with systemic mastocytosis. A basophil leukemia cell line was used as a reference. Peripheral blood basophils from healthy donors were used as controls.We observed intense expression of 2D7 and BB1 in all skin biopsy specimens from patients with cutaneous mastocytosis. Immunostaining of liver and bone marrow specimens from patients with systemic mastocytosis with 2D7 and BB1 antibodies was negative. Specimens from patients with either type of mastocytosis showed similarly strong expression of c-Kit. The basophil cell line showed a 2D7 and a BB1 profile, with intense expression of c-Kit. Peripheral blood basophils exhibited notable immunostaining for 2D7, BB1, and c-Kit.2D7 and BB1 are expressed in cutaneous mastocytosis, although this expression is lost when mast cell proliferation is systemic, thus reflecting either a different cellular differentiation stage or the presence of basophils in these skin diseases.