Articles

Highlights in myeloproliferative neoplasms

BJH - volume 15, issue 5, september 2024

N. Granacher MD

SUMMARY

The annual EHA meeting again featured several interesting oral abstracts and poster presentations in the field of myeloproliferative neoplasms (MPN). Below the highlights in Philadelphia positive (Ph+) chronic myeloid leukaemia (CML), Philadelphia negative MPN, polycythemia vera (PV) and myelofibrosis are summarised.

(BELG J HEMATOL 2024;15(5):197–200)

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Novel JAK inhibitors for the treatment of myelofibrosis

BJH - volume 15, issue 4, june 2024

N. Granacher MD

SUMMARY

Myelofibrosis (MF) is a rare Philadelphia chromosome negative myeloproliferative bone marrow disorder characterised by a high symptom burden due to splenomegaly, inflammatory status and cytopenias. Until recently, the only approved and reimbursed pharmacotherapy for non-haematopoietic stem cell transplant candidates in Belgium was ruxolitinib. However, as of September 1st, 2023, the novel JAK (janus kinase) 1/2 inhibitor (JAKi) fedratinib has obtained its reimbursement in second line. The other FDA approved JAKi, momelotinib and pacritinib, play an important role in patient-tailored JAKi treatment but are not yet reimbursed in Belgium.

(BELG J HEMATOL 2024;15(4):165–71)

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Highlights in chronic myeloid leukaemia

BJH - volume 14, issue 5, september 2023

N. Granacher MD

SUMMARY

The focus on CML this year was on how to improve patient’s eligibility and success of treatment free remission (TFR). The longest TFR follow-up comes from the STIM trial, Imatinib +/- Interferon (IFN), in which has been shown that at 7-year follow-up only 38% of patients maintain TFR.1 Previously, additional prognostic features such as IFN pre-treatment and increased NK cell count at baseline have been suggested to be associated with increased success of TFR however these are not always validated in larger cohorts. Several investigators are therefore looking at how to better define TFR eligible patients and at how to increase the TFR success rate.

(BELG J HEMATOL 2023;14(5):207–10)

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Systemic bevacizumab to treat refractory bleeding in hereditary haemorrhagic telangiectasia: A single centre case series

BJH - volume 14, issue 3, may 2023

J. Nelissen MD, K.L. Wu MD, PhD, N. Granacher MD, D. Breems MD, PhD

SUMMARY

Hereditary haemorrhagic telangiectasia (HHT) is a rare genetic disorder that causes mucocutaneous telangiectasia and visceral arteriovenous malformations (AVMs). Recurrent iron deficiency and anaemia are significant complications often treated by haematologists. Bevacizumab, an anti-VEGF monoclonal antibody, has been implemented to target elevated levels of VEGF as seen in HHT patients. We present a single centre case series of three patients with recurrent bleeding issues due to HHT who have been treated with bevacizumab. All three patients were benefited in terms of mean haemoglobin, need for iron infusions and number of haemostatic interventions. Based on our own case series and existing literature, systemic bevacizumab appears to be effective in the treatment of bleeding-related conditions. However, the optimal dose and treatment strategy has yet to be determined. Randomised controlled studies are needed to further support the indication of bevacizumab in HHT.

(BELG J HEMATOL 2023;14(3):135–8)

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Highlights in myeloproliferative neoplasms

BJH - volume 14, issue 1, february 2023

N. Granacher MD

SUMMARY

With the identification of novel therapeutic targets in different MPN and the movement of second-/third-line treatment upfront, the MPN therapeutic landscape is continuously changing. For Ph+ CML, additional genetic mutations are probably going to change our currently known risk stratification and determine choice of front-line treatment. In Ph- MPN, focus is on molecular response and disease modification and scientific evidence that this might actually have beneficial effect on patient outcome is gradually being gathered.

(BELG J HEMATOL 2023;14(1):10–5)

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Highlights in chronic myeloid leukaemia

BJH - volume 13, issue 5, september 2022

N. Granacher MD

SUMMARY

The treatment landscape of chronic myeloid leukaemia in chronic phase (CP-CML) is continuously changing. During the annual congress of the European Hematology Association (EHA 2022), new strategies to improve the management of CP-CML were presented. These novel strategies include new drugs, alternative treatment strategies to reduce toxicity without affecting efficacy, and new techniques to help identify the best patient subpopulation for treatment-free remission. The highlights of these new strategies are addressed in this article.

(BELG J HEMATOL 2022;13(5):183–7)

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Leishmaniasis: A potential lymphoma Mimicker

BJH - volume 13, issue 2, march 2022

B. Heyrman MD, S. Heyman MD, N. Granacher MD, G. Van den Eynden MD, PhD

SUMMARY

In Belgium, a 53-year old Caucasian man presented at the haematology consultations with weight loss and night sweats in the last six weeks. Apart from a cholecystectomy and appendectomy, he had no medical history. He had no pets; his last journey abroad was to Spain eight months ago. On physical examination, splenomegaly was noted. Blood testing revealed a microcytic anaemia (Hb 9.3g/dl), leukopenia (2.63x10E9/L) with normal formula, normal liver testing and crp of 47.9g/dl. Serologic testing for HIV, CMV, HepB, HepC, and Toxoplasma was negative. Trephine biopsy was normal. PET/CT scan demonstrated splenomegaly with high FDG-avidity. Splenectomy was performed. Small granules (amastygotes) were seen by the pathologist suggestive for Leishmaniasis. Serologic testing and PCR confirmed the diagnosis. He was subsequently treated with liposomal amphotericin B. Our patient is now in optimal condition. Earlier serologic testing for this rare tropical disease in a non-endemic region could have prevented splenectomy.

(BELG J HEMATOL 2022;13(2):92–4)

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