Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UNIPROT:Q06643 (non-Hodgkin's lymphoma)
 11,307 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Paraneoplastic pemphigus is an autoimmune vesiculobullous and erosive mucocutaneous disease associated with an underlying malignancy. Reported malignancies include chronic lymphocytic leukemia, non-Hodgkin's lymphoma, Castleman's disease, sarcomas, and rarely solid tumors. Patients with paraneoplastic pemphigus develop characteristic IgG autoantibodies against several antigens including members of the plakin family, bullous pemphigoid antigen 1, and desmosomal proteins. IgA pemphigus is another recently characterized immunobullous disease that presents as a vesiculopustular eruption with neutrophilic infiltration and epidermal acantholysis. Mucous membrane involvement is rare. We report what is to our knowledge a unique case with features of both IgA pemphigus and paraneoplastic pemphigus associated with chronic lymphocytic leukemia.
 ...
PMID:A novel case of IgA paraneoplastic pemphigus associated with chronic lymphocytic leukemia. 1743 44

Peripheral T-cell lymphomas (PTCLs) account for 12% of non-Hodgkin's lymphomas (NHLs). Immunoglobulin (Ig) A pemphigus is an autoimmune blistering disease characterized by tissue-bound and circulating IgA antibodies that target epidermal cell surface components. Malignant lymphomas are often linked with autoimmune disease and the autoimmune blistering disease, paraneoplastic pemphigus, has been associated with NHL. However, cases of PTCLs that are complicated by IgA pemphigus are particularly rare. The current study presents the first known case of PTCL complicated by IgA pemphigus. A 43-year-old male was admitted to the Union Hospital (Wuhan, China) in March 2012 with multiple swollen lymph nodes. Pathology examinations revealed PTCL. Immunohistochemical staining was positive for cluster of differentiation (CD)2, CD3, CD5, CD7 and CD47, and negative for CD20. Ki-67 was ~40% positive. The patient was treated with four cycles of cyclophosphamide, Adriamycin, vincristine and prednisone, and two cycles of gemcitabine, cisplatin and dexamethasone; in addition, the patient received radiation of the retroperitoneal region (total dose, 36 Gy). The patient underwent thalidomide maintenance therapy for 20 days before flaccid blisters appeared on the trunk and limbs. Histopathology and immunofluorescence indicated IgA pemphigus, and intravenous methylprednisolone was administered, followed by treatment with prednisone. Subsequently, no evidence of recurrent lymphoma or pemphigus has been observed.
 ...
PMID:Peripheral T-cell lymphoma complicated by immunoglobulin A pemphigus: A case report and literature review. 2495 19