BJH - volume 12, issue 8, december 2021
A. Salaroli MD, C. Spilleboudt MD, P. Lewalle MD, PhD, S. Wittnebel MD, PhD
The prognosis of acute promyelocytic leukaemia has passed from nearly desperate to highly curative over the last 40 years due to better understanding of the biology of the disease, the introduction of anthracyclines, all-trans-retinoic acid (ATRA), arsenic trioxide (ATO) and the implementation of better supportive care during the treatment. If this also holds true for older patients will be discussed in this review.
(BELG J HEMATOL 2021;12(8):332–7)
Read moreBJH - volume 12, issue 7, november 2021
V. Labarque MD, PhD
Sickle cell disease (SCD) is one of the most frequently inherited diseases but it no longer only affects children. More and more patients survive well into adulthood. They experience repeated acute complications and inevitably develop chronic organ damage. For years, hydroxyurea and chronic transfusions were the only disease-modifying options in the treatment of SCD patients. Thanks to a better understanding of the pathophysiology, new components have been and are now being tested. Three of these are already used in clinical practice, namely L-glutamine, crizanlizumab and voxelotor. On the other hand, progress has also been made in the field of haematopoietic stem cell transplantation, through the introduction of alternative donors as well as the use of less toxic conditioning regimens. Finally, hopeful results are being achieved in the first studies of gene therapy in patients with SCD but it has yet to be proven that genetically manipulated stem cells maintain the long-term repopulation potential.
(BELG J HEMATOL 2021;12(7):290–5)
Read moreBJH - volume 12, issue 6, october 2021
L. Van Camp MD, T. Lammens PhD, A. Uyttebroeck MD, PhD, B. De Moerloose MD, PhD
Despite huge progress in the past decades, the overall survival (OS) of patients with acute myeloid leukaemia (AML) remains poor. The treatment options run low for those refractory or intolerant to first and second line treatment or in case of relapse. The need for alternative treatment is great and imperative to further improve the OS of these patients. The success of CAR-T19 therapy for the treatment of B cell acute lymphoblastic leukaemia has demonstrated the feasibility of delivering these therapies, and success in further improving survival rates. Among others, the fundamental biological factor limiting the applicability of CAR-T immuno-therapy in the treatment of AML includes the lack of a leukaemia-specific antigen, or an antigen shared by leukaemia blasts and haematopoietic stem and progenitor cells whose sustained depletion could be clinically tolerated. In this review, we describe the most recent developments, clinical results and challenges in CAR-T cell therapy for AML.
(BELG J HEMATOL 2021;12(6):244-50)
Read moreBJH - 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)
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