BJH - volume 12, issue 6, october 2021
A. Kentos MD, PhD, N. Mavroudakis MD, PhD, M. Delforge MD, PhD
Monoclonal gammopathy of undetermined significance (MGUS) is quite frequent in the general population. The association between MGUS and peripheral neuropathy (PN) was described in various studies demonstrating a higher than expected prevalence of PN in patients with MGUS. The demonstration of causality remains a diagnostic challenge as a coincidental association may also occur. Specific diagnostic criteria are available for only a few disorders: DADS, POEMS, amyloidosis, cryoglobulinemia. Data to guide management are quite limited. We present a short review of the literature and emphasise the need of a close collaboration between haematologists and neurologists for an optimal management.
(BELG J HEMATOL 2021;12(6):251-7)
Read moreBJH - volume 13, issue 8, december 2022
V. Havelange MD, PhD, S.N. Constantinescu MD, PhD
Calreticulin mutations are driver mutations detected in around 20–25% of essential thrombocythemia and in 25–30% of primary myelofibrosis patients. The most recurrent mutations are type-1 (a deletion of 52 bp in the exon 9) and type-2 (an insertion of 5 bp in the exon 9). This review describes the distinct clinical features, prognosis and outcome of calreticulin mutated patients from JAK2V617F or MPL (thrombopoietin receptor) mutated patients. The subtypes of calreticulin mutations were also associated with distinct clinical characteristics. Several treatment guidelines were adapted for calreticulin-mutated patients. The mechanism by why the three driver mutations, which all activate JAK/STAT signalling pathway can trigger diseases with quite different features is still not known. The recent progress in the understanding of calreticulin mutation biology will allow the development of new target therapies with the hope to cure the disease in the next years.
(BELG J HEMATOL 2022;13(8):293–301)
Read moreBJH - volume 13, issue 8, december 2022
A.M. Laheij, DDS, PhD, J.E. Raber-Durlacher, DDS, PhD, M.D. Hazenberg MD, PhD, M.C. Schoordijk , M.C. Huysmans, DDS, PhD, J.G. de Visscher, DDS, MD, PhD
Allogeneic stem cell transplantation recipients may develop chronic graft-versus-host disease (cGVHD). The oral and perioral tissues are commonly involved, clinically manifesting as mucosal lesions, salivary gland dysfunction and/or sclerotic changes. Oral cGVHD is associated with mucosal sensitivity and pain, (severe) oral dryness, altered taste, decreased mouth opening, all of which may contribute to a significant decrease of the patient’s quality of life. Hyposalivation may put patients at risk for mucosal infections and rampant dental caries. In addition, patients with (a history) of oral cGVHD are at increased risk for oral squamous cell carcinoma. The diagnosis of cGVHD is based on the patient’s medical history, clinical signs, and symptoms. In rare cases, a biopsy may provide clinically relevant information as the histopathology findings are mostly not very specific. Treatment of cGVHD is based on the patient’s symptoms and consists preferably of local immunosuppressants. In case of severe oral complaints and when other non-oral body are also involved systemic immunosuppressive therapy should be considered. Xerostomia can be alleviated with mechanical stimulation, topical dry mouth relief products or sialagogues. Dental professionals can provide supportive care aimed at reducing symptoms and prevention of further deterioration of oral health.
(BELG J HEMATOL 2022;13(8):302–9)
Read moreBJH - volume 13, issue 8, december 2022
S. Haggenburg MD, Q. Hofsink MD, A.E.C. Broers MD, PhD, J.A. van Doesum MD, C. van Elssen MD, PhD, R.S. van Binnendijk PhD, G. den Hartog PhD, J. Heijmans MD, PhD, P.G.N.J. Mutsaers MD, PhD, T. van Meerten MD, PhD, C.J.M. Halkes MD, PhD, M.H.M. Heemskerk MD, PhD, A. Goorhuis MD, PhD, C.E. Rutten MD, PhD, M.D. Hazenberg MD, PhD, I.S. Nijhof MD, PhD
Patients with haematologic diseases are at high risk for severe coronavirus disease 2019 (COVID-19) and COVID-19-related death. In early 2021, haematology patients were therefore prioritized for SARS-CoV-2 vaccination by the Dutch government. It was however not known whether they would be able to generate a protective immune response to SARS-CoV-2 vaccines, given the immunodeficiencies that often accompany hematologic conditions and the therapy thereof. National and international cohort studies demonstrated an adequate antibody response after a standard 2-dose mRNA vaccination schedule in a larger number of patients than expected. After the third dose, the majority of immunocompromised haematology patients obtained SARS-CoV-2 antibody concentrations similar to the antibody concentrations obtained by healthy individuals after the standard 2-dose mRNA-1273 schedule. The primary COVID-19 vaccination schedule for haematology patients should therefore consist of three instead of two mRNA vaccinations. B cell depleted patients and patients who received allogeneic hematopoietic progenitor cell transplantation (HCT) should be revaccinated. The number and the exact timing of revaccinations remains to be determined however. In conclusion, SARS-CoV-2 vaccination should not be postponed in patients on or shortly after therapy for hematologic conditions.
(BELG J HEMATOL 2022;13(8):310–5)
Read moreBJH - volume 12, issue 4, june 2021
F. Massaro MD, C. Vandevoorde , J. Ku MD, D. Papazoglou MD, A. Van Uytvanck MD, N. Meuleman MD, PhD, D. Bron MD, PhD
The majority of CLL patients are elderly, with a median age of those requiring a first line treatment, close to 76 years old. Nowadays, multiple treatment options are available for this disease, ranging from chemo immunotherapy regimens to biological therapies. The treatment decision in an older CLL patient is a four-step procedure, starting firstly with the assessment of treatment criteria. The second step is to evaluate the life-expectancy of the patient, its autonomy, vulnerabilities and the socio-economic status. The subsequent step is to define treatment options according to prognostic factors. Last, but not least, the patient should be involved in the final decision to know to what extend he is willing to receive a treatment with a potential curative or palliative intent. The assessment of an elderly CLL patient is a complex procedure, not only comprehending the evaluation of biological and hematological parameters but also clinical, social and psychological features, which equally contribute to the selection of the most valuable strategy.
(BELG J HEMATOL 2021;12(4):147-54)
Read moreBJH - volume 12, issue 3, may 2021
C. Van Laer PharmD, M. Jacquemin MD, PhD, K. Peerlinck MD, PhD, K. Freson PhD
The international study ThromboGenomics has developed and tested a targeted high-throughput sequencing (HTS) multi-gene panel test for diagnostics of patients with rare bleeding, thrombotic or platelet disorders (BTPD). After the initial validation of this research platform, 2396 index patients were sequenced and a mean diagnostic rate of 49.2% was reached for all thrombotic, coagulation, platelet count and function disorder patients while this rate dropped to 3.2% for patients with unexplained bleeding disorders that were characterised by normal haemostasis test results. Since early 2019, a similar HTS test for BTPD has been implemented in Belgium in a clinical diagnostic setting. This test screens 96 diagnostic-grade genes and is updated yearly with novel genes. Upon inclusion, clinicians can opt for one of the three panels: 1) (anti)coagulation panel test for abnormal bleeding or thrombosis, 2) platelet disorder panel test for inherited thrombocytopenia or known platelet dysfunctions and 3) the unexplained bleeding panel test but with evidence for Ehlers-Danlos Syndrome or Rendu-Osler-Weber disease. Inclusion and exclusion criteria for patients eligible for such HTS will be discussed. The submission of detailed information about clinical phenotype, family history and laboratory test results is critical for the interpretation of the genetic results. The aim is to provide results to clinicians and patients with a detailed report that discusses variant interpretation.
(BELG J HEMATOL 2020;12(3):99-105)
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BJH - volume 12, issue 3, may 2021
A. Janssens MD, PhD, L. Stas MSc, M. Van den Enden MD, E. Van den Neste MD, PhD
Chronic lymphocytic leukaemia (CLL) is a slow-progressing cancer that results in uncontrolled proliferation and accumulation of B-lymphocytes in the blood and bone marrow and is the most common form of leukaemia in the western world. CLL patients harbouring a deletion of chromosome 17 (del17p) or the TP53 mutation, who progress after treatment with immunological, chemotherapeutic as well as targeted agents such as ibrutinib have poor prognosis signifying a population with an unmet medical need. Clinical studies showed that venetoclax, a selective; orally bioavailable Bcl-2 inhibitor, induces CLL cell apoptosis, and offers an alternative therapeutic option for CLL, either as a monotherapy or in combination with rituximab. BRAVe was a multicentre, observational retrospective study, conducted in Belgium. The main objectives of this study were to evaluate the safety and effectiveness of venetoclax monotherapy in Belgian patients with CLL, as well as the utilisation of resources in a real life setting. The results show a manageable/favourable safety profile for venetoclax with limited burden for patients and sites, and a good overall response rate in pre-treated CLL patients in the relapsed/refractory setting.
(BELG J HEMATOL 2020;12(3):106-11)
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