PRACTICE GUIDELINES

Primary immune thrombocytopenia in adults: Belgian recommendations for diagnosis and treatment anno 2021 made by the Belgian Hematology Society

BJH - volume 12, issue 3, may 2021

A. Janssens MD, PhD, C. Lambert MD, D. Selleslag MD, J. Depaus MD, Y. Beguin MD, PhD

SUMMARY

The Belgian Hematology Society (BHS) updated the 2013 guidelines for diagnosis and treatment of primary immune thrombocytopenia (ITP).1 As knowledge about ITP pathophysiology is increasing, the mode of action of old therapies is better understood and novel drugs are introduced to target more specific pathways. Corticosteroids with or without intravenous immunoglobulins (IgIV) remain the first line treatment. According to the updated international guidelines, a short course of corticosteroids rather than a prolonged treatment has to be recommended. The same guidelines stress that consequent therapies as thrombopoietic agents (TPO-RAs) and rituximab should be available independent of duration of ITP. Although the majority of recommendations is based on very low-quality evidence, it is strongly advised to individualise the ITP management taking patient values and preferences in account. The main treatment goal in all ITP patients must be to maintain a safe platelet count to prevent or stop bleeding with a minimum of toxicity and not to normalise the platelet count.

(BELG J HEMATOL 2020;12(3):112-27)

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Belgian guidelines for diagnosis and treatment of chronic myelomonocytic leukaemia

BJH - 2021, issue 2, march 2021

A. De Becker MD, B. Heyrman MD, D. Mazure MD, M-C. Vekemans MD, dr. M. Beckers MD, PhD, N. Granacher MD, On behalf of the other members of MDS and MPN committee , S. Meers MD, PhD, S. Sid MD

SUMMARY

Chronic myelomonocytic leukaemia (CMML) is a rare haematological disease. Hallmark of the diagnosis is chronic monocytosis. Other clinical features include cytopenia, dysplasia with the associated complaints like fatigue or leucocytosis, splenomegaly with constitutional symptoms. Predicting prognosis and choosing the correct treatment can be challenging for the clinician. These guidelines cover the diagnosis and treatment of CMML and provide information on morphology, cytogenetics and molecular testing, clinical features including autoimmune manifestations, prognosis and risk assessment and a treatment algorithm for both the fit and unfit CMML patient.

(BELG J HEMATOL 2020;12(2):66-76)

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Diagnosis and treatment of AL amyloidosis: Belgian guidelines

BJH - volume 11, issue 8, december 2020

C. Doyen MD, PhD, C. Jacquy MD, G. Bries MD, PhD, J. Caers MD, PhD, M-C. Vekemans MD, M. Cliquennois MD, M. Delforge MD, PhD, M. Vercruyssen MD, N. Meuleman MD, PhD

SUMMARY

Immunoglobulin light chain (AL) amyloidosis is a rare and serious disease due to the deposition of amyloid fibrils. In the past years, improvements have been made in the diagnosis, treatment and response criteria. Based on an extensive review of the recent literature on AL amyloidosis, we propose practical recommenddations that can be used by Belgian haematologists as a reference for daily practice. Management of other types of amyloidosis will not be covered by this review. Levels of evidence and grades of recommendations are based on previously published methods.1 We recommend participation in clinical trials to gain knowledge in this evolving field.

(BELG J HEMATOL 2020;11(8):343-56)

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Practical management of multiple myeloma: Update 2020

BJH - volume 11, issue 8, december 2020

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

SUMMARY

With the introduction of immunomodulatory drugs, proteasome inhibitors and anti-CD38 monoclonal anti-bodies, 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(8):357-75)

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Practical management of multiple myeloma: Update 2020

BJH - volume 11, issue 7, november 2020

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

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

A. Janssens MD, PhD, A. Van De Velde MD, A. Vanderfaeillie MD, J. Loos MD, dr. M. Beckers MD, PhD, M. Hoyoux MD, V. Beckers MD, dr. V. Van Hende MD, prof. dr. W. Peetermans MD, PhD, Y. Van Laethem 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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Diagnosis and treatment of thrombotic thrombocytopenic purpura

BJH - volume 11, issue 6, october 2020

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