Monoclonal gammopathy is a uncommon finding in pediatric age. Only viral infections, bone marrow or solid organ transplantation, and immunosuppressive therapy may induce B-lymphocyte clonal proliferation leading to monoclonal components (MC) in serum. Here we present a case of clinically relevant monoclonal gammopathy in pediatric age. After receiving an unusual request for a serum protein electrophoresis (SPE) test in 12 years-old boy, information on the patient was collected. The boy was affected by Crohn’s disease and treated with prednisone, azathioprine, and antibodies against tumor necrosis factor-V (infliximab and, later, adalimumab). As both disease and treatment made the patient at risk for developing lymphoid/myeloid malignancies (LMM), the search for MC in serum was indeed recommended. On SPE three MC were detected and typed by agarose gel immunofixation (IFE) as IgA , IgM , and IgG. Moreover, a -type Bence Jones protein was detected by urine IFE. Bone marrow examination was then carried out to exclude LMM, resulting in a negative pattern. It is known that Crohn’s disease in adults, particularly when treated by means of immunosuppresive therapies, can be associated with monoclonal gammopathies and an increased risk of developing leukemia or lymphoma and, rarely, myeloma. However, at our best knowledge, no report of such association has been previously reported in pediatrics. This case illustrates a specific situation in which the request and execution of a SPE in the young is not only appropriate, but even recommended to prevent the risk of silent LMM development.

Un caso clinicamente rilevante di gammopatia monoclonale in età pediatrica / I. Infusino, D. Dilillo, E. Galli, G.V. Zuccotti, A. Dolci, M. Panteghini. - In: BIOCHIMICA CLINICA. - ISSN 0393-0564. - 36:5(2012), pp. 378-383.

Un caso clinicamente rilevante di gammopatia monoclonale in età pediatrica

G.V. Zuccotti;A. Dolci;M. Panteghini
2012

Abstract

Monoclonal gammopathy is a uncommon finding in pediatric age. Only viral infections, bone marrow or solid organ transplantation, and immunosuppressive therapy may induce B-lymphocyte clonal proliferation leading to monoclonal components (MC) in serum. Here we present a case of clinically relevant monoclonal gammopathy in pediatric age. After receiving an unusual request for a serum protein electrophoresis (SPE) test in 12 years-old boy, information on the patient was collected. The boy was affected by Crohn’s disease and treated with prednisone, azathioprine, and antibodies against tumor necrosis factor-V (infliximab and, later, adalimumab). As both disease and treatment made the patient at risk for developing lymphoid/myeloid malignancies (LMM), the search for MC in serum was indeed recommended. On SPE three MC were detected and typed by agarose gel immunofixation (IFE) as IgA , IgM , and IgG. Moreover, a -type Bence Jones protein was detected by urine IFE. Bone marrow examination was then carried out to exclude LMM, resulting in a negative pattern. It is known that Crohn’s disease in adults, particularly when treated by means of immunosuppresive therapies, can be associated with monoclonal gammopathies and an increased risk of developing leukemia or lymphoma and, rarely, myeloma. However, at our best knowledge, no report of such association has been previously reported in pediatrics. This case illustrates a specific situation in which the request and execution of a SPE in the young is not only appropriate, but even recommended to prevent the risk of silent LMM development.
Settore MED/38 - Pediatria Generale e Specialistica
Settore BIO/12 - Biochimica Clinica e Biologia Molecolare Clinica
2012
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/208162
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