CASI CLINICI – Case Reports
Biochimica Clinica 2012; 36(5) 378-383
Un caso clinicamente rilevante di gammopatia monoclonale in età pediatrica
1Laboratorio Analisi Chimico-Cliniche, Azienda Ospedaliera “Luigi Sacco", e Cattedra di Biochimica Clinica e Biologia Molecolare Clinica, Dipartimento di Scienze Biomediche e Cliniche “Luigi Sacco”, Università degli Studi, Milano
2Unità Operativa di Pediatria, Azienda Ospedaliera “Luigi Sacco", e Cattedra di Clinica Pediatrica, Dipartimento di Scienze Biomediche e Cliniche “Luigi Sacco”, Università degli Studi, Milano
A clinically relevant monoclonal gammopathy in pediatric age
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 IgAk, IgMk, and IgGg. Moreover, a k-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.