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Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis
Published on: December 15, 2011
[Immunocytoma IgA. Case report]
Bebiana Conde1, Ana Fernandes, Manuel Cunha
1Centro Hospitalar de Trás-os-Montes e Alto Douro Av. da Noruega 5000 Vila Real. bebianaconde@gmail.com
Abstract:
Immunocytoma is a non-Hodgkin's indolent evolution B cell lymphoma. It accounts for approximately 1-3% of non-Hodgkin's limphomas and usually onsets in adults aged over 50 years old. It manifests as lymphadenopathy, splenomegaly, hepatomegaly and lymphcytosis in 15 -30% of cases and is rarely seen with pulmonary involvement. Monocloncal peaks of serum immunoglobulin often occur. These are IgM and rarely IgA. We present as an example a male patient aged 52 years old, with recurrent respiratory infections. Clinical work -up identified an immunocytoma IgA stage IV. Diagnosing an indolent lymphoma, we prophylactic polyspecific human immunoglobulin to treat the respiratory infection. Evidence of lymphoma progression leads us to prescribe combined cyclo- phosphamide (C), vincristine (V), prednisone (P) e rituximab (R) (CVP-R), which has obtained a partial response over two years.
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