Overexpression of complement receptors and related antigens on the surface of bone marrow mast cells in patients with systemic mastocytosis
Rosa Núñez-López
Mast Cell Unit, Hospital Ramón y Cajal, Madrid, Spain,
Search for more papers by this authorLuis Escribano
Mast Cell Unit, Hospital Ramón y Cajal, Madrid, Spain,
Search for more papers by this authorGerit-Holger Schernthaner
Department of Internal Medicine I, Division of Haematology and Hemostaseology, University of Vienna, Vienna, Austria, and
Search for more papers by this authorAránzazu Prados
Mast Cell Unit, Hospital Ramón y Cajal, Madrid, Spain,
Search for more papers by this authorRamón Rodríguez-González
Mast Cell Unit, Hospital Ramón y Cajal, Madrid, Spain,
Search for more papers by this authorBeatriz Díaz-Agustín
Mast Cell Unit, Hospital Ramón y Cajal, Madrid, Spain,
Search for more papers by this authorAntonio López
Servicio General de Citometría, Centro de Investigación del Cáncer, Universidad de Salamanca, Salamanca, Spain
Search for more papers by this authorAlexander Hauswirth
Department of Internal Medicine I, Division of Haematology and Hemostaseology, University of Vienna, Vienna, Austria, and
Search for more papers by this authorPeter Valent
Department of Internal Medicine I, Division of Haematology and Hemostaseology, University of Vienna, Vienna, Austria, and
Search for more papers by this authorJulia Almeida
Servicio General de Citometría, Centro de Investigación del Cáncer, Universidad de Salamanca, Salamanca, Spain
Search for more papers by this authorPilar Bravo
Mast Cell Unit, Hospital Ramón y Cajal, Madrid, Spain,
Search for more papers by this authorAlberto Orfao
Servicio General de Citometría, Centro de Investigación del Cáncer, Universidad de Salamanca, Salamanca, Spain
Search for more papers by this authorRosa Núñez-López
Mast Cell Unit, Hospital Ramón y Cajal, Madrid, Spain,
Search for more papers by this authorLuis Escribano
Mast Cell Unit, Hospital Ramón y Cajal, Madrid, Spain,
Search for more papers by this authorGerit-Holger Schernthaner
Department of Internal Medicine I, Division of Haematology and Hemostaseology, University of Vienna, Vienna, Austria, and
Search for more papers by this authorAránzazu Prados
Mast Cell Unit, Hospital Ramón y Cajal, Madrid, Spain,
Search for more papers by this authorRamón Rodríguez-González
Mast Cell Unit, Hospital Ramón y Cajal, Madrid, Spain,
Search for more papers by this authorBeatriz Díaz-Agustín
Mast Cell Unit, Hospital Ramón y Cajal, Madrid, Spain,
Search for more papers by this authorAntonio López
Servicio General de Citometría, Centro de Investigación del Cáncer, Universidad de Salamanca, Salamanca, Spain
Search for more papers by this authorAlexander Hauswirth
Department of Internal Medicine I, Division of Haematology and Hemostaseology, University of Vienna, Vienna, Austria, and
Search for more papers by this authorPeter Valent
Department of Internal Medicine I, Division of Haematology and Hemostaseology, University of Vienna, Vienna, Austria, and
Search for more papers by this authorJulia Almeida
Servicio General de Citometría, Centro de Investigación del Cáncer, Universidad de Salamanca, Salamanca, Spain
Search for more papers by this authorPilar Bravo
Mast Cell Unit, Hospital Ramón y Cajal, Madrid, Spain,
Search for more papers by this authorAlberto Orfao
Servicio General de Citometría, Centro de Investigación del Cáncer, Universidad de Salamanca, Salamanca, Spain
Search for more papers by this authorAbstract
Summary. Depending on their stage of maturation and other factors, mast cell (MC) subsets differ from each other in terms of the expression of complement-associated antigens. This study analysed the expression of various complement-related cell surface antigens (CD11b/CR3, CD11c/CR4, CD35/CR1, CD55/DAF, CD59/MIRL, CD88/C5aR) on bone marrow mast cells (BMMC) in patients suffering from systemic mastocytosis (SM), other haematological diseases and non-haematological disorders (control groups). Expression of complement-associated cell surface antigens was analysed by flow cytometry. There were clear immunophenotypic differences between BMMC obtained from patients with SM and those from the control subjects: the percentage of patients expressing surface CD11c, CD35 and CD88 was significantly higher in patients with SM (76%, 100%, 54%) than in the control subjects (58%, 11%, 18%) (P < 0·05). In addition, the levels of CD11c, CD35 and CD88 expressed per MC (sites per cell) were significantly higher (P < 0·05) in SM than in the control group. Expression of the complement regulatory molecules CD55 and CD59 was detected in BMMC in all patients analysed. However, the levels of CD59 per BMMC were higher in patients with SM as compared with the control subjects, which could help to explain the formation of BMMC aggregates in the former group of individuals. Together, our results showed that BMMC in systemic mastocytosis overexpressed the cell surface membrane receptors involved in binding of complement components and complement-mediated cell activation. Whether this pathological expression of complement receptors is of pathophysiological significance remains to be determined.
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