Abstract
Paraneoplastic pemphigus (PNP) is a severe autoimmune syndrome commonly triggered by neoplasms. The prognosis of CLL-associated PNP is dismal due to its refractory course and secondary infection and no standard treatment was recommended. We retrospectively reported six CLL with PNP cases from 842 cases of CLL including diagnosis, treatment and prognosis. The median time between the initial of CLL to PNP was 36 months while the median overall survival from the diagnosis of PNP was 26 months. And three cases died of lung infection while 5 developed pulmonary symptoms. And 5 cases received fludarabine-based chemotherapy before developing PNP, which suggesting fludarabine was one of potential causes of PNP. For the treatment, five patients were rescued by combined regimens including rituximab, methylprednisolone, immunoglobulin, fresh frozen plasma and the last received ibrutinib combined with short-term prednisone. Fludarabine-based regimen may be one of the potential causes of PNP. The combined regimen might shed a new light, while ibrutinib is a promising drug for CLL with PNP, but needs much more evidence. PNP should be carefully treated to guide early diagnosis and intervention for a better prognosis.
Highlights
Paraneoplastic pemphigus (PNP) is a severe autoimmune syndrome commonly triggered by neoplasms
All the 6 patients developed PNP after the treatment of chronic lymphocytic leukemia (CLL) and half of them were diagnosed PNP during the partial remission of CLL while the rest of them manifested as disease progression in CLL
CLL accompanied with PNP as a rare and fetal disease, is resistant to treatment resulting in a highly mortality rate over 75%
Summary
Paraneoplastic pemphigus (PNP) is a severe autoimmune syndrome commonly triggered by neoplasms. Abbreviations PNP Paraneoplastic pemphigus NHL Non-Hodgkin’s lymphoma CLL Chronic lymphocytic leukemia LPD Lymphoproliferative diseases FISH Fluorescence in situ hybridization IGHV Immunoglobulin heavy-chain variable region LDH Lactate dehydrogenase β2-MG β2-Microglobulin IVIG Intravenous immunoglobulin PR Partial remission CHOP Cyclophosphamide, epirubicin, vindesine and prednisolone FCM Fludarabine, cyclophosphamide and mitoxantrone HDMP High-dose methylprednisolone COP Cyclophosphamide, vindesine sulfate, hydroprednisone. Paraneoplastic pemphigus (PNP), as a severe autoimmune disease, is categorized by autoantibodies like IgG that injures desmoplakins, desmogleins, desmocollins and presenting a poor prognosis with a high mortality. According to the research reported to the US Food and Drug Administration, a total of 12 in 100,000 cases of non-Hodgkin’s lymphoma (NHL) and chronic lymphocytic leukemia (CLL) were found to be complicated by PNP4. CLL is a most prevalent adult lymphoid malignance in western hemisphere accounting for 30% of all leukemia, but relatively rare in eastern c ountries[6]. Compared with the hematologic autoimmune complications, non-hematologic autoimmune complications such as PNP, glomerulonephritis, C1 esterase deficiency and pernicious anemia are relatively r are[2,8]
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