Indolent Systemic Mast Cell Disease in Adults: Immunophenotypic Characterization of Bone Marrow Mast Cells and Its Diagnostic Implications

Author:

Escribano Luis1,Orfao Alberto1,Dı́az-Agustin Beatriz1,Villarrubia Jesús1,Cerveró Carlos1,López Antonio1,Marcos Marı́a A. Garcı́a1,Bellas Carmen1,Fernández-Cañadas Serafı́n1,Cuevas Manuela1,Sánchez Alberto1,Velasco José L.1,Navarro José Luis1,Miguel Jesús F. San1

Affiliation:

1. From the Servicio de Hematologı́a, Anatomı́a Patológica, Dermatologı́a, and Inmunologı́a, Hospital Ramón y Cajal, Universidad de Alcalá de Henares, Madrid, Spain; and the Servicio Central de Citometrı́a and Departamento de Medicina, Servicio de Hematologı́a, Universidad de Salamanca, Salamanca, Spain.

Abstract

The aim of the present study was to explore the diagnostic value of the immunophenotypic analysis of bone marrow mast cells (BMMC) in indolent systemic mast cell disease (SMCD) patients. For that purpose, a total of 10 SMCD patients and 19 healthy controls were analyzed. Our results show that BMMC from SMCD are different from normal BMMC with regard to both their light scatter and immunophenotypic characteristics. Accordingly, forward light scatter (FSC), side (90°) light scatter (SSC), and baseline autofluorescence levels were higher in BMMC from indolent SMCD patients than they were in control subjects. From the immunophenotypic point of view, the most striking findings were the constant expression of CD2 (P = .0001), CD25 (P = .0001), and CD35 (P = .06) molecules by BMMC from SMCD patients, markers that were absent from all normal controls. In contrast, CD71, absent in BMMC from indolent SMCD, was positive in BMMC from normal subjects. Although, slight differences between BMMC from SMCD patients and normal controls were found in several other markers, they did not reach statistical significance. In conclusion, our results show that simultaneous assessment of FSC/SSC and reactivity for the CD117, CD2, CD25, CD33, and CD35 forms the basis for the immunophenotypic characterization of BMMC from SMCD in adults and should be integrated with clinical and morphologic studies for the diagnosis of the disease.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

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