Articles

Diagnosis of a gamma heavy chain disease in a 71-year-old patient with a persistent leukopenia and thrombocytopenia

BJH - volume 15, issue 2, march 2024

O. Mortelé PhD, K. Ver Elst MD, S. Vermeiren MD, A. Meskal PharmD, S. Schouwers PharmD, J. de Bie MD, PhD, J. Lemmens MD, L. Rutsaert MD, C. Schuermans MD, T. Eyckmans MD, S. Weekx PhD

SUMMARY

A 71-year-old man with persistent leukopenia and thrombocytopenia was referred to the haematology department with a suspicion of a myelodysplastic neoplasm (MDS). Upon presentation, the patient was asymptomatic. Peripheral blood analysis confirmed leukopenia and thrombocytopenia. Furthermore, IgG was elevated, while IgM, total protein and the kappa-lambda free light chain (FLC) ratio were within normal ranges. Protein electrophoresis pattern showed a prominent monoclonal peak in the gamma globulin region. The monoclonal peak was identified as IgG heavy chain without corresponding kappa or lambda light chains by immunofixation analysis. Bone marrow cytology did not provide evidence for MDS; however, an increased plasmocytosis of 8% was detected. Immunophenotyping showed the presence of 6.6% CD19+, CD38++, CD138+, CD45+ and CD56- plasma cells without cytoplasmic light chain expression. The latter was confirmed by histologic review of the bone marrow biopsy using immunohistochemical staining. Immunoglobin gene rearrangement analysis was indicative for the presence of a monoclonal B-cell or plasma cell neoplasm. On positron emission tomography (PET)-scan only a mild splenomegaly was seen. Based on all these results, the diagnosis of a gamma heavy chain disease (gHCD) was made. As the patient was asymptomatic, treatment was not indicated. Blood count and health status were unchanged at a check-up six months later. Further follow-up is performed every six months. This case report presents the diagnostic work-up of a patient with gHCD. Laboratory analysis contributing to the diagnosis of gHCD included protein electrophoresis, immunofixation, bone marrow cytology, immunophenotyping, molecular analysis and pathological examinations of a bone biopsy.

(BELG J HEMATOL 2024;15(2):49–53)

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Rituximab induced serum sickness or immune complex deposition disease: case report and desensitisation protocol

BJH - volume 11, issue 2, march 2020

N.C. Granacher MD, T. Eyckmans MD

SUMMARY

The chimeric monoclonal IgG1 antibody directed to CD20 Rituximab is used to treat various haematological malignancies and auto-immune diseases. Serum sickness is a type III hypersensitivity reaction leading to the formation and tissue deposition of immune antibody-antigen complexes. It has been described as a very rare complication of Rituximab treatment, mainly seen in patients treated for auto-immune diseases. We report the case of a patient with Waldenstrom’s macroglobulinaemia whose Rituximab treatment was complicated by documented immune complex deposition or serum sickness. We successfully applied a Rituximab desensitisation protocol, which allowed us to complete treatment.

(BELG J HEMATOL 2020;11(2):75–8)

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P.24 Rare morphologic phenomena in plasma cell dyscrasia: report of two cases

BJH - volume 11, issue Abstract Book BHS, february 2020

K. Kehoe , S. Weekx PhD, S. Vermeiren MD, K. Ver Elst MD, T. Eyckmans MD, C. Schuermans MD, J. Lemmens MD

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PP45 Blood transfusion in Sickle Cell Disease. Retrospective study in ZNA Hospitals (Antwerp)

BJH - 2018, issue Abstract Book BHS, february 2018

T. Eyckmans MD, S. Mahieu , M.P. Emonds MD, PhD, E. Lazarova , P. Vandenberghe MD, PhD, P. Zachée MD, PhD

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