The Role of CD40/CD40 Ligand Interactions in Bone Marrow Granulopoiesis
Table 1
Pathogenetic and pathophysiologic features of X-linked Hyper IgM Syndrome and Chronic Idiopathic Neutropenia.
X-linked hyper-IgM syndrome
Chronic idiopathic neutropenia
Clinical features
Infections, arthritis,mucosal ulcers, and neutropenia [70–72]
Neutropenia, and osteopenia/osteoporosis, usually uncomplicated course [75]
Immunologic Features/cytokines
Elevated IgM, reduced IgG, IgA, and IgE levels [70–72]
Elevated TNFα, IFNγ, TGFβ, IL-1β, IL-6, and FasL [75–77]
Pathophysiologic features
Mutations in the CD40L gene, abnormal T-cell mediated signals, failure of B-cell differentiation, and defective regulation of immunoglobulin isotype switching [70–72]
Impaired BM granulopoiesis and T-cell and cytokine-mediated suppression of hematopoiesis [75–78]