PRACTICE GUIDELINES

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)

Read more

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

BJH - volume 11, issue 6, october 2020

A. Delie MD, I. Moors 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)

Read more

Ibrutinib and bleeding management: a Belgian expert consensus

BJH - volume 11, issue 4, june 2020

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

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)

Read more

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)

Read more

BHS guidelines for the treatment of newly diagnosed diffuse large B-cell lymphoma (DLBCL) anno 2020

BJH - volume 11, issue 2, march 2020

A. Janssens MD, PhD, C. Bonnet MD, PhD, C. Jacquy MD, E. Van den Neste MD, PhD, G. Swennen MD, K. Saevels MD, M. Maerevoet MD, S. Bailly MD, S. Snauwaert MD, PhD, V. Vergote MD

SUMMARY

Diffuse large B-cell lymphoma is the most common subtype of non-Hodgkin lymphoma. Prognosis of diffuse large B-cell lymphoma has improved dramatically since the introduction of rituximab and about two thirds of patients can be cured with immunochemotherapy. In the last twenty years, it became clear that diffuse large B-cell lymphoma is a very heterogeneous disease and based on the genetic mutation landscapes numerous efforts have been made to develop novel treatment strategies to improve the prognosis of diffuse large B-cell lymphoma further. This article provides an update of diagnosis, current treatment guidelines and novel treatment strategies for newly diagnosed patients with diffuse large B-cell lymphoma in Belgium. It will also focus on treatment of elderly patients and high-grade B-cell lymphoma.

(BELG J HEMATOL 2020;11(2):56–66)

Read more

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

BJH - volume 11, issue 2, march 2020

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

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)

Read more

Diagnostic testing in myeloid malignancies by next-generation sequencing: recommendations from the Commission Personalised Medicine

BJH - volume 10, issue 6, october 2019

A. Camboni MD, PhD, A. Hébrant PhD, B. Denys MD, B. Maes MD, PhD, E. Boone PhD, E. Heylen PhD, E. Lierman PhD, E. Van Valckenborgh PhD, F. Lambert MD, F. Nollet PhD, MSc, G. Froyen PhD, G. Martens MD, PhD, G. Raicevic PhD, H. Devos MD, H. Louagie MD, PhD, H.A. Poirel MD, PhD, J-P. Defour PhD, K. Jacobs PhD, K. Vandepoele PhD, K. Vermeulen PhD, L. Dewispelaere MD, L. Michaux MD, PhD, M-B. Maes PhD, M. Bakkus PhD, M. Le Mercier Apr, PhD, M. Van Den Bulcke PhD, P. Heimann MD, PhD, P. Hermans MD, PhD, P. Saussoy MD, PhD, P. Vandenberghe MD, PhD, P. Vannuffel PhD, T. Tousseyn MD, PhD, T. Venken PhD

SUMMARY

Molecular diagnostics have an increasing impact on diagnosis, risk stratification and targeted treatment in haemato-oncology. In the framework of a pilot study for the implementation of next-generation sequencing in the Belgian healthcare system, the Commission of Personalised Medicine was founded to give professional and evidence-based advice on the molecular analysis in haemato-oncology. This paper describes its recommendations for NGS analysis in myeloid malignancies. In addition, the minimally required set of genes that must be analysed is defined and algorithms for molecular workflow in myeloid malignancies are proposed.

(BELG J HEMATOL 2019;10(6):241–9)

Read more
X