Paroxysmal nocturnal hemoglobinuria: New discoveries about an old disease

Riley, Roger S.

doi:10.1002/jcla.1860080602

Cited by 3 publications

(1 citation statement)

References 90 publications

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“…CRs are either associated with host defence (cellular responses) ‐ CD11b/CR3, CD11c/CR4, CD35/CR1 and CD88/C5aR (Walport, 2001a) ‐ or they are linked to inactivation of potentially self‐destructive autologous activated complement proteins. These regulatory receptors include the glycosylphosphatidylinositol‐associated proteins (GPI‐AP) CD55/DAF and CD59/MIRL (Navenot et al , 1993, 1996; Riley, 1994). During the last two decades, the expression and distribution of CRs has been investigated on many different types of cells of the immune system.…”

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confidence: 99%

Overexpression of complement receptors and related antigens on the surface of bone marrow mast cells in patients with systemic mastocytosis

et al. 2003

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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 < 0AE05). In addition, the levels of CD11c, CD35 and CD88 expressed per MC (sites per cell) were significantly higher (P < 0AE05) 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 complementmediated cell activation. Whether this pathological expression of complement receptors is of pathophysiological significance remains to be determined.

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confidence: 99%