Ostalo

https://doi.org/10.11613/BM.2018.010802

Gamma heavy-chain disease accompanied with follicular lymphoma: a case report

Paula San-José ; Clinical Laboratory, Parc Taulí Hospital Universitari, Institut d’Investigació i Innovació Parc Taulí I3PT, Universitat Autònoma de Barcelona, Spain
Vicente Aguadero ; Clinical Laboratory, Parc Taulí Hospital Universitari, Institut d’Investigació i Innovació Parc Taulí I3PT, Universitat Autònoma de Barcelona, Spain
Granada Perea ; Clinical Laboratory, Parc Taulí Hospital Universitari, Institut d’Investigació i Innovació Parc Taulí I3PT, Universitat Autònoma de Barcelona, Spain; Hematology Department, Parc Taulí Hospital Universitari, Institut d’Investigació i Innovació Parc Taulí I
Meritxell Estrada ; Clinical Laboratory, Parc Taulí Hospital Universitari, Institut d’Investigació i Innovació Parc Taulí I3PT, Universitat Autònoma de Barcelona, Spain; Hematology Department, Parc Taulí Hospital Universitari, Institut d’Investigació i Innovació Parc Taulí I
Eugenio Berlanga ; Clinical Laboratory, Parc Taulí Hospital Universitari, Institut d’Investigació i Innovació Parc Taulí I3PT, Universitat Autònoma de Barcelona, Spain

Sažetak

Heavy chain diseases (HCD) are B-cell lymphoprolipherative disorders characterized by the production of monoclonal heavy chains without associated
light chains. Some cases of gamma-HCD (γ-HCD) are concurrent with other lymphoid neoplasm. The monoclonal component is not always
detectable by serum electrophoresis, and often an immunofixation procedure is necessary to detect this component. Prognosis is variable, and no
established guidelines for follow-up are available. We describe a case of a challenging diagnosis of γ-HCD due to the absence of clinical signs frequently
reported in the disease (anaemia and palatal oedema among others). Haematological malignancy was the first suspicion but bone marrow
examination was negative. In addition, the presence of an autoimmune bicytopenia and a Klinefelter syndrome complicated the clinical context of the patient. A thoracoabdominal computed tomography reported many small adenopathies whose pathological and immunohystochemical study revealed a follicular lymphoma. Shortly after, serum inmunofixation secondary to an abnormal electrophoretic pattern revealed a gamma paraprotein without light chains. Eventually, γ-HCD in association with follicular lymphoma was the final diagnosis. This is the first case reporting this association.

Ključne riječi

paraproteins; electrophoresis; lymphoma; heavy chain disease

Hrčak ID:

193636

URI

https://hrcak.srce.hr/193636

Datum izdavanja:

15.2.2018.

Posjeta: 1.079 *