Pure Red Cell Aplasia and Lymphoproliferative Disorders: An Infrequent Association

Table 3

Proposed molecular mechanisms for PRCA, due to lymphoproliferative disorders [2, 15–17, 19, 20].

B cells
IgG antibodies, either complement-binding, directly cytotoxic, targeting erythroblasts in vitro or inhibiting haemoglobin synthesis [15].
Antibodies produced following a viral or bacterial infection that might cross-react with erythroid precursor cells or erythropoietin [16].
Anti-erythropoietin antibodies, immune complexes with erythropoietin, which result in functional inactivation [2, 16].
Obstruction of the erythropoietin receptor by anti-erythroblast antibodies [16].
T cells, NK cells
MHC-I-restricted CD8(+) cytotoxic T-lymphocytes [17].
MHC-unrestricted CD3(+) T-LGLs with TCR expressing KIRs [17].
B cells, T cells, NK cells
MHC-unrestricted CD3(−) NK-LGLs without TCR, which may be positively triggered by circulating antibody against red-cell antigens activating NK-receptors and adhesion molecules [17].
Antibody cross-linking the TCR of the T-LGLs with the Fc receptor on target cells [19, 20].
Antibody cross-linking the Fc receptor on the T-LGLs or NK-LGLs (CD16) with any specific ligand for the antibody on the target cells [19, 20].