X-linked agammaglobulinemia (XLA) is a primary immunodeficiency disorder caused by germline mutations of B-cell tyrosine kinase (BTK) gene. It is characterized by decreased serum immunoglobulins levels and circulating mature B cells. This defect in humoral immunity leads to increased susceptibility to infection. Pyoderma gangrenosum (PG) is an uncommon, ulcerating, neutrophilic dermatosis. Here...

Research over the role of Bruton's agammaglobulinemia tyrosine kinase (BTK) in B-lymphocyte development, differentiation, signaling and survival has led to better understanding of the pathogenesis of B-cell malignancies. Down-regulation of BTK activity is an attractive novel strategy for treating patients with B-cell malignancies. Ibrutinib (PCI-32765), a potent inhibitor of BTK induces impress...

By DOROTHEA ZUCKER-FRANKLIN, EDWARD C. FRANKLIN AND NORMAN S. COOPER T HE ROLE of the plasma cell in the synthesis of y-globulin has been readily accepted because of the excellent correlation found between the number of plasma cells in tissues, the ability to form antibodies, and the level of y-globulin in the serum. Thus, there is a lack of plasma cells in agammaglobulinemia, an increase in pl...