PRACTICE GUIDELINES

Practical management of multiple myeloma: Update 2020

BJH - volume 11, issue 7, november 2020

M.C. Vekemans MD, C. Doyen MD, K.L. Wu MD, PhD, A. Kentos MD, PhD, P. Mineur MD, L. Michaux MD, PhD, J. Caers MD, PhD, N. Meuleman MD, PhD, M. Delforge MD, PhD, On behalf of the BHS Myeloma Subgroup

SUMMARY

With the introduction of immunomodulatory drugs, proteasome inhibitors and anti-CD38 monoclonal antibodies, major improvements have been achieved in the treatment and outcome of multiple myeloma (MM). Different treatment combinations are now in use and other therapies are being developed. This rapidly changing therapeutic landscape urges for an update on practical guidelines. Based on an extensive review of the recent literature, we propose recommendations on myeloma management, to be used by haematologists as a reference for daily practice.

(BELG J HEMATOL 2020;11(7):286-304)

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Vaccination policy in adult patients with hematological malignancies or asplenia without stem cell transplantation: Recommendations from the advisory board on vaccination of the Belgian Hematological Society

BJH - volume 11, issue 7, november 2020

J. Loos MD, M. Beckers MD, PhD, V. Beckers MD, M. Hoyoux MD, prof. dr. W. Peetermans MD, PhD, A. Van De Velde MD, PhD, V. Van Hende MD, A. Vanderfaeillie MD, Y. Van Laethem MD, PhD, A. Janssens MD, PhD

SUMMARY

Patients with hematological malignancies suffer from widely varying degrees of immunodeficiency, which leads to an increased susceptibility to a wide range of infections. Some of these, such as influenza and invasive pneumococcal disease, are vaccine preventable. During the Covid19 pandemic these past months patients with hematological malignancies have already shown to be at greater risk of dying, with mortality rates of up to 30% in hospitalized patients.1,2 This has once again highlighted the importance of robust and widely spread vaccination strategies, also we eagerly await an available vaccine for Covid19. In this review, the advisory board on vaccination of the Belgian Hematological Society (BHS), consisting of experts from various disease committees as well as two infectious disease experts attempts to provide clear recommendations regarding vaccinations in patients with hematological malignancies and asplenia. Although transplant recipients share many of the immunodeficiencies of those not transplanted, clear guidelines and vaccination schedules have already been published.3

(BELG J HEMATOL 2020;11(7):305-316)

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Therapy-related myeloid neoplasms: epidemiology, pathogenesis and therapeutic options

BJH - volume 11, issue 6, october 2020

I. Moors MD, dr. A. Delie MD

SUMMARY

Therapy-related myeloid neoplasms are increasingly seen in our daily practice, as a consequence of increased long-term cancer survivorship and an aging population. Typically, there is an overrepresentation of high-risk cytogenetics and TP53 mutations. In recent years, there have been new insights in the pathogenesis of these neoplasms, especially with regard to the role of CHIP (clonal haematopoiesis of indeterminate potential) in patients receiving cytotoxic therapy for a malignant or non-malignant disorder. Unfortunately, prognosis seems worse in comparison to de novo AML, despite intensive induction and consolidation with allogeneic stem cell transplantation, with a high frequency of treatment-related toxicity and relapse. However, there is hope for the future with the emergence of novel therapies that could be of special interest in the context of these poor-risk leukaemias.

(BELG J HEMATOL 2020;11(6):261-7)

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Diagnosis and treatment of thrombotic thrombocytopenic purpura

BJH - volume 11, issue 6, october 2020

E. Roose PhD, S. Deconinck , C. Dekimpe , A. Curie , SF. De Meyer PhD, K. VanHoorelbeke PhD, D. Dierickx MD, PhD

SUMMARY

Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic microangiopathic disorder (TMA) due to a severe deficiency of ADAMTS13 (A Disintegrin And Metalloprotease with Thrombo-Spondin type 1 repeats, member 13). The deficiency in ADAMTS13 can either be caused by mutations in ADAMTS13 (congenital TTP or Upshaw-Schulman syndrome, cTTP) or by anti-ADAMTS13 autoantibodies (immune-mediated TTP, iTTP). Diagnosis of TTP is challenging but crucial for the survival of the patient. TTP should be suspected when microangiopathic haemolytic anaemia and severe thrombocytopenia are observed. A severely decreased ADAMTS13 activity (activity <10%) should confirm the diagnosis of TTP. Standard treatment of TTP is plasma therapy (plasma exchange for iTTP, while plasma infusion for cTTP), but novel therapeutics like rituximab, caplacizumab and recombinant ADAMTS13 show promising results regarding the recovery and sustained remission of TTP patients. However, although major advances have been made in the management of TTP, TTP is a chronic disease and patients still relapse, careful and stringent patient follow-up is needed to improve the patients’ quality of life.

(BELG J HEMATOL 2020;11(6):253-60)

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Ibrutinib and bleeding management: a Belgian expert consensus

BJH - volume 11, issue 4, june 2020

A. Janssens MD, PhD, D. Bron MD, PhD, V. Van Hende MD, V. Galle MD, K. Jochmans MD, PhD, S. Meers MD, PhD, M. André MD, PhD, M-C. Ngirabacu MD, PhD, K.L. Wu MD, PhD, B. De Prijck MD, P. Verhamme MD, PhD, C. Hermans MD, PhD

SUMMARY

In recent years ibrutinib emerged as a paradigm shifting agent in the treatment of chronic lymphocytic leukaemia (CLL), mantle cell lymphoma (MCL) and Waldenström’s macroglobulinemia (WM). In clinical trials and in real-world studies ibrutinib proved to be an effective agent with an overall favourable tolerability profile. However, compared with standard chemo-immunotherapy (CIT), ibrutinib was associated with a higher incidence of clinically significant bleeding. This has been hypothesized to be linked to the platelet-specific effects of inhibiting Bruton’s tyrosine kinase (BTK). Most bleeding events under ibrutinib are low-grade with a decreasing incidence over time. However, bleeding can have a significant impact on patients and interfere with persistence and compliance of ibrutinib treatment. Currently, no clear consensus exists on the use of ibrutinib in patients with an increased bleeding risk, on the management of ibrutinib-induced bleeding and on the use of ibrutinib around surgery or invasive procedures. In this paper, a panel of Belgian haematology and haemostasis specialists formulated practical advice on bleeding prevention and management in ibrutinib-treated patients.

(BELG J HEMATOL 2020;11(4):174–84)

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BHS Guidelines for the Management of Small Lymphocytic Lymphoma and Chronic Lymphocytic Leukaemia, anno 2020

BJH - volume 11, issue 3, may 2020

A. Janssens MD, PhD

SUMMARY

The Belgian Haematological Society (BHS) Lymphoproliferative Disease Committee updated the existing recommendations on diagnosis, prognostic scores, treatment indications, best strategies for front-line and subsequent-line treatment of small lymphocytic lymphoma (SLL)/ chronic lymphocytic leukaemia (CLL), according robust new data.

(BELG J HEMATOL 2020;11(3):108–19)

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Guidelines of the Belgian Hematological Society for newly diagnosed and relapsed follicular lymphoma anno 2019

BJH - volume 11, issue 2, march 2020

M. Clauwaert MD, V. Galle MD, M. Maerevoet MD, A. Janssens MD, PhD, K. Saevels MD, S. Snauwaert MD, PhD, C. Springael MD, PhD, V. Van Hende MD, G. Verhoef MD, PhD, F. Offner MD, PhD

SUMMARY

Follicular lymphoma is the most common low-grade non-Hodgkin lymphoma. Survival rates have been rising over time mainly due to advancing therapeutic strategies. As the last Belgian guidelines date from 2012, we present an update of the scientific evidence regarding diagnosis, staging, treatment and follow-up, and confront these to the Belgian reimbursement rules anno 2019. Follicular lymphoma grade 3B is classified as high-grade lymphoma and treated accordingly, and will not be discussed in this paper. Early stage disease can be treated with involved-field radiotherapy, which has curative potential. Advanced stage disease is virtually incurable, but many treatment options are available with good results. In first line, treatment is mostly based on chemotherapy combined with rituximab; the latter can be continued as maintenance therapy. In relapsed setting, introduction of the newer and more potent anti-CD20-antibody obinutuzumab, also in combination with chemotherapy, can lead to improved survival in high-risk patients. For older patients with comorbidities, rituximab monotherapy is the preferred option. In further lines, PI3K-inhibition with idelalisib and radioimmunotherapy are available. Finally, autologous or allogeneic stem cell transplantation remain an option in a small group of selected patients.

(BELG J HEMATOL 2020;11(2):67–74)

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