Detection of CD55- and/or CD59-Deficient Red Cell Populations in Patients With Plasma Cell Dyscrasias

Abstract

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal disorder characterized by a decrease or absence of glycosylphosphatidylinositol (GPI)-anchored molecules such as CD55 and CD59 from the surface of affected cells, resulting in intravascular hemolysis, cytopenia, and venous thrombosis. A PNH-like phenotype has been detected in various hematological disorders, mainly in aplastic anemia and myelodysplastic syndromes, but also in lymphoproliferative syndromes (LPSs). To the best of our knowledge, CD55- or CD59-deficient red cells have not been detected in plasma cell dyscrasias (PCDs). The aim of this study was the detection of CD55- and/or CD59-deficient red cell populations in patients with PCD. Seventy-seven patients were evaluated; 62 with multiple myeloma (MM), 7 with Waldenstrom macroglobulinemia (WM), 6 with monoclonal gammopathy of undetermined significance (MGUS), and 2 with heavy chain disease (HCD). The sephacryl gel microtyping system was applied; Ham and sucrose lysis tests were also performed on all samples with CD55- or CD59-negative populations. Red cells deficient in both molecules were detected in 10 (12.9%) of 77 patients with PCD: 2 (28.6%) of 7 with WM, 1 (16.6%) of 6 with MGUS, 6 (9.6%) of 62 with MM, and 1 of 2 patients with HCD. Isolated CD55 deficiency was found in 28.5% of all PCD patients, whereas isolated CD59 deficiency was not observed in any patients. These findings illustrate the existence of the PNH phenotype in the red cells of patients with PCD; further investigation is needed into the mechanisms and significance of this phenotype.

A Paroxysmal Nocturnal Haemoglobinuria Progress with Waldenström Macroglobulinemia Along with T Cell Monoclonal Expansion

“…However the potential progress to B-cell malignant disease was rarely reported. The John Meletis [6] study about the information on the PNH phenotype in plasma cell dyscrasias (PCDs) found the two of seven patients with confirmed Waldenström macroglobulinemia (WM) can simultaneously present CD55…”

“…Reviewing literature, malignant transformation is mainly acute myeloid leukemia [4,5]. Terminating B cell lymphoma even more seldom seen in literature [6]. Here, we report a PNH case terminating with Waldenström macroglobulinemia (WM) and T cell monoclonal expansion and review the literature.…”

A patient with paroxysmal nocturnal hemoglobinuria, T cell large granular lymphocyte clonal expansion, and monoclonal gammopathy of undetermined significance

“…Ten patients were found to have PNH and plasma cell dyscrasias, including 1 patient with PNH and MGUS and 4 patients with PNH and IgG multiple myeloma [11]. Monoclonal gammopathies can also occur in association with T cell clonal expansions [24][25][26][27].…”

“…PNH has also been described with concurrent plasma cell dyscrasias [11]. In this report, we describe a patient with PNH associated with T cell LGL oligoclonal expansion and IgG l monoclonal gammopathy of undetermined significance (MGUS).…”

O papel das proteínas reguladoras do complemento CD55/CD59 em células de sangue periférico de pacientes com lúpus eritematoso sistêmico

“…CD55 and CD59 deficiency has been studied in other diseases and correlated with its severity. [29][30][31][32][33][34][35][36] Yamaguchi et al 37 showed that 28.6% of the patients with aplastic anemia (AA) and 27.8% of the patients with myelodysplasia syndrome (MDS) presented a poor population of CD59 in erythrocytes. Wang et al 38 observed a significant CD55 and CD59 reduction in 52% of neutrophils in patients with AA not treated.…”