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Indolent Systemic Mast Cell Disease in Adults: Immunophenotypic
Characterization of Bone Marrow Mast Cells and Its Diagnostic
Implications
Luis Escribano,
Alberto Orfao,
Beatriz Díaz-Agustin,
Jesús Villarrubia,
Carlos Cerveró,
Antonio López,
María A. García Marcos,
Carmen Bellas,
Serafín Fernández-Cañadas,
Manuela Cuevas,
Alberto Sánchez,
José L. Velasco,
José Luis Navarro, and
Jesús F. San Miguel
From the Servicio de Hematología, Anatomía
Patológica, Dermatología, and Inmunología,
Hospital Ramón y Cajal, Universidad de Alcalá de Henares,
Madrid, Spain; and the Servicio Central de Citometría and
Departamento de Medicina, Servicio de Hematología, Universidad
de Salamanca, Salamanca, Spain.
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.
Blood, Vol. 91 No. 8 (April 15), 1998:
pp. 2731-2736
© 1998 by The American Society of Hematology.
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