pci-32765 and Pemphigus

Paraneoplastic pemphigus (PNP) is a severe autoimmune blistering disease associated with an underlying malignancy, and its prognosis is poor. We herein report the first patient with B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma (B-CLL/SLL)-associated PNP successfully treated with the Bruton's tyrosine kinase inhibitor ibrutinib and rituximab. Although his PNP lesions did not improve with ibrutinib monotherapy, the combination of ibrutinib and rituximab was effective against B-CLL/SLL-associated PNP. This case suggests that ibrutinib plus rituximab may be a potent therapeutic option for B-CLL/SLL-associated PNP that is hard to control with ibrutinib alone.

Topics: Adenine; Antineoplastic Agents, Immunological; Humans; Leukemia, Lymphocytic, Chronic, B-Cell; Male; Middle Aged; Paraneoplastic Syndromes; Pemphigus; Piperidines; Prognosis; Protein Kinase Inhibitors; Pyrazoles; Pyrimidines; Rituximab; Treatment Outcome

We present the case of a 51-year-old man who developed paraneoplastic pemphigus (PNP) in the context of chronic lymphocytic leukemia (CLL). His CLL was successfully controlled with ibrutinib. Concurrently, there was significant improvement of his PNP, suggesting that ibrutinib may be a very useful addition to the treatment options in this potentially life-threatening autoimmune disorder.

Topics: Adenine; Agammaglobulinaemia Tyrosine Kinase; Antineoplastic Agents; Humans; Leukemia, Lymphocytic, Chronic, B-Cell; Male; Middle Aged; Paraneoplastic Syndromes; Pemphigus; Piperidines; Protein-Tyrosine Kinases; Pyrazoles; Pyrimidines