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 PhD, J. Lemmens MD, L. Rutsaert MD, C. Schuermans MD, T. Eyckmans MD, S. Weekx PhD
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)
Read moreBJH - volume 12, issue 6, october 2021
C. Schuermans MD, D. Mazure MD, K. Van Eygen MD, L. Van Aelst MD, PhD, S. Benghiat Fleur MD, PhD, T. Devos MD, PhD
Polycythemia vera (PV) is classified by the World Health Organization (WHO) under the BCR-ABL-negative myeloproliferative neoplasms (MPNs) and is characterised by clonal proliferation of myeloid cells, which leads primarily to an increased red blood cell mass. Bone marrow morphology remains the cornerstone of diagnosis. Patients can present with thrombosis, microcirculatory symptoms, haemorrhage, splenomegaly, pruritus and other symptoms that reduce their quality of life and they are at risk of transformation to secondary myelofibrosis (MF) or acute myeloid leukaemia (AML). The main goal of therapy in PV is to minimise the thrombotic risk. To achieve this goal PV patients are being treated with low-dose aspirin and phlebotomies to reach a target haematocrit below 45%. In addition, high-risk patients are being treated with cytoreductive agents. Over the last years, new insights in the pathophysiology, diagnosis and prognosis of polycythemia vera were acquired and novel therapeutic options are available. In this paper we give an update on PV and provide diagnostic and therapeutic recommendations, taking into account the Belgian situation.
(BELG J HEMATOL 2021;12(6):258-74)
Read moreBJH - 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
BJH - volume 10, issue 5, september 2019
C. Schuermans MD
At the EHA annual meeting of 2019, interesting new data with regard to myelodysplastic syndromes (MDS) and myeloproliferative neoplasms (MPN) were presented. For patients with high-risk myelofibrosis (MF) who have failed or are intolerant to ruxolitinib (rux) as well as for patients with higher risk MDS, the prognosis is poor and treatment options are few. There is still a large unmet medical need for these patient groups and new therapies are eagerly awaited. The same holds true for patients with advanced systemic mastocytosis (AdvSM). For this patient group very promising data about a new inhibitor of KIT D816V were presented. In contrast, most chronic myeloid leukemia (CML) patients have a much brighter outlook with the currently available tyrosine kinase inhibitors (TKI) treatments and some can even stop their TKI and have a durable treatment-free remission (TFR). A short update of the DASFREE and ENESTfreedom data will be discussed below. Some CML patients however do not reach their treatment goals and are waiting for new treatment options. Also data on pregnancy outcomes in CML patients were discussed.
(BELG J HEMATOL 2019;10(5):188–94)
Read moreBJH - volume 7, issue Abstract Book BHS, january 2016
M. Criel , F. Declau , C. Schuermans MD, K. Ver Elst MD, S. Vermeiren MD, S. Weekx PhD, J. Lemmens MD
BJH - volume 4, issue 4, december 2013
T. Devos MD, PhD, N. Straetmans MD, PhD, C. Schuermans MD, S. Benghiat MD, PhD, V. Robin MD, P. Lewalle MD, PhD, P. Mineur MD, G. Verhoef MD, PhD, L. Knoops MD, PhD
Diagnostic and management guidelines for myelofibrosis patients are presented in this paper. As a consequence of the rapid evolution and progress in this domain over the last years, the need was felt by the BHS MPN subcommittee to update these guidelines for our country. The different prognostic scores in myelofibrosis, the diagnostic tools and treatment options with the focus on new possibilities are discussed.
(BELG J HEMATOL 2013;4(4): 127–137)
Read moreBJH - 2013, issue BHS Abstractbook, january 2013
J. Caers MD, PhD, M-C. Vekemans MD, I. Vande Broek MD, PhD, P.H. Mineur , K. Beel MD, PhD, V. Maertens MD, C. Schuermans MD, F. Leleu , G. Vanstraelen , H. Demuynck MD, W. Schroyens MD, PhD, E. Van den Neste MD, PhD, G. Bries MD, PhD, A. Van De Velde MD, M. Delforge MD, PhD, C. Doyen MD, PhD
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