J. Maertens MD, PhD

Yellow nail syndrome associated with acute myeloid leukaemia

SUMMARY

Yellow Nail Syndrome (YNS) is a rare entity characterised by the triad of nail discolouration, lung manifestations/sinusitis and lymphoedema. With ongoing debate, the exact aetiological mechanisms of YNS remain unknown. YNS is associated with various conditions such as malignancies, autoimmune and immuno-deficiency diseases. Some authors consider YNS as a paraneoplastic phenomenon due to its association with malignancies. Here we report the first patient presenting with the typical triad of YNS and a consecutive diagnosis of acute myeloid leukaemia with recurrent genetic abnormalities (KMT2A-PTD). Nail symptoms showed partial recovery after initiation of chemotherapy. Currently, the patient is off therapy and remains in first complete remission. More research to identify the exact pathophysiological mechanism and clinical significance of YNS is needed.

(BELG J HEMATOL 2022;13(6):249–52)

Faster to the fungus: rapid diagnostic and prognostic tests for invasive fungal diseases

SUMMARY

Invasive mould infections such as invasive aspergillosis or mucormycosis remain an important infectious complication in haematology patients, especially in those undergoing intensive chemotherapy for acute myeloid leukaemia or undergoing allogeneic stem cell transplantation. An early diagnosis and timely initiation of antifungal therapy improves outcomes. In this thesis, we therefore looked at new possible diagnostic tools to aid in a rapid diagnosis, such as lateral flow assays, PCR tests, or a novel beta-D-glucan assay. Furthermore, we explored how we could get the maximum out of existing tests such as galactomannan, by optimising their use after therapy in an effort to assess the response to therapy.

(BELG J HEMATOL 2020;12(3):138-40)

O.6 Multipotent mesenchymal stromal cells for poor graft function after allogeneic hematopoietic cell transplantation – a multicenter prospective study

Defeating cytomegalovirus, the transplantation troll: can letermovir do the job?

Letermovir (AIC246, MK-8228) is a novel anti-cytomegalovirus (CMV) agent that inhibits CMV replication by targeting the viral terminase complex. In December 2017, letermovir was approved by the Food and Drug Administration (FDA) for the prophylaxis of CMV infection and disease in adult CMV-seropositive recipients of an allogenic haematological stem cell transplantation. Letermovir shows a favourable pharmacokinetic profile in haematological stem cell transplantation recipients after oral administration. The recommended dose for CMV-prophylaxis is once daily 480 mg (oral or intravenous). Letermovir demonstrated superiority in a placebo (plus polymerase chain reaction-monitoring and pre-emptive therapy)-controlled phase III randomised clinical trial. Letermovir is an inhibitor of the cytochrome P450 (CYP)3A family, CYP2B8 and an inducer of the CYP2C9/19. Dose-adjustments (240 mg/day) are necessary when letermovir is combined with cyclosporine. Combinations of letermovir with either voriconazole, midazolam and rosiglitazone require close monitoring of the plasma levels of the latter agents. Letermovir-resistant CMV mutants share mutations that are mostly located between the codon range 230–370 of the UL56 gene. Letermovir is not nephrotoxic nor myelotoxic, but slightly higher rates of atrial fibrillation and tachycardia have been described. In conclusion, letermovir is the first FDA approved anti-CMV agent for prophylaxis in haematological stem cell transplantation patients.

(BELG J HEMATOL 2019;10(4):136–45)

01 A pilot study to assess the feasibility of unrelated umbilical cord blood transplantation with coinfusion of third-party mesenchymal stromal cells after myeloablative or non-myeloablative conditioning in patients with haematological malignancies

Management of severe aplastic anaemia

SUMMARY

Aplastic anaemia is a rare condition characterised by pancytopenia and bone marrow hypocellularity and caused by the immune-mediated destruction of the haematopoietic precursors. The early complications are related to cytopaenias with infections being the major cause of morbi-mortality. The main long-term issue is clonal evolution to myelodysplastic syndrome or acute leukaemia. The diagnosis relies on exclusion of other causes of pancytopenia and characteristic pathologic findings. Severity is stratified according to peripheral blood counts. Nowadays, the survival of treated patients reaches 80–90%. The treatment of the severe form of aplastic anaemia consists on haematopoietic stem cell transplantation in eligible patients and immunosuppressive therapy in non-transplant candidates. Supportive therapy is an option in frail and/or elderly patients. Here, we define and briefly review the pathogenesis of aplastic anaemia. We propose a diagnostic and therapeutic strategy based on existing literature and experts’ recommendations. We finally report three cases illustrating particular clinical associations with pregnancy, hepatitis and paroxysmal nocturnal haemoglobinuria.

(BELG J HEMATOL 2018;9(3):76–85)

P37 Comparison of Multigam^® IV (5% vs. 10%) in hematological patients with secondary immunodeficiencies to evaluate infusion time, tolerability and satisfaction. A monocentric observational Belgian study