J. Depaus MD

Practical recommendations for the management of late relapsed and refractory multiple myeloma in 2024

SUMMARY

Despite significant advances in therapeutic modalities, the treatment of relapsed and refractory multiple myeloma (RRMM) is still challenging. In this publication, we aim to provide an update on therapeutic modalities for RRMM in Belgium. First, novel combinations of well-established therapeutic agents will be discussed. Second, T-cell redirection therapies will be addressed. These include bispecific antibodies, both anti-BCMA x CD3 and anti-GPRC5D x CD3, as well as chimeric antigen receptor (CAR) T cell therapy. Third, we discuss novel modalities such as antibody-drug conjugates, selinexor, venetoclax, melflufen and CELMoDs. Finally, a general flowchart regarding overall treatment sequencing will be proposed, providing an integrated treatment recommendation from frontline to relapse.

(BELG J HEMATOL 2024;15(6):225–32)

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

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)

POEMS Syndrome

SUMMARY

Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes (POEMS) syndrome is a rare disorder due to an underlying plasma cell clone (PC). The syndrome can affect several organs. The diagnosis is based on the presence of mandatory criteria (polyneuropathy, monoclonal plasma cell disorder) and at least one major and one minor criteria. The therapeutic regimen is determined according to the extent of the patient’s sclerotic lesions and the presence of bone marrow involvement.

(BELG J HEMATOL 2020;11(8):381-6)

Management of systemic mastocytosis: A heterogeneous disease requiring a correct diagnosis and adapted treatment

Editorial for the contribution of T. Goos et al., entitled: A single-center retrospective study of patients with systemic mastocytosis at University Hospital Leuven
BELG J HEMATOL 2019;10(7):265-76)

(BELG J HEMATOL 2019;10(7):264)

Diffuse large B-cell lymphoma refractory to R-CHOP

SUMMARY

Rituximab with cyclophosphamide, adriamycin, vincristine and prednisone (R-CHOP) is the standard treatment for diffuse large B-cell lymphoma and is able to cure 50–60% of the patients. However, patients resistant to or in early relapse after R-CHOP have a very poor prognosis with a median overall survival of only six months, and very few patients have a long survival. Double-hit lymphoma (rearrangement MYC and BCL2) has a major risk of refractoriness, and more intense chemotherapy than R-CHOP is recommended. Early PET-CT could identify resistance to conventional chemotherapy. Intensification with autologous or allogeneic stem cell transplantation is recommended in case of a response to salvage regimen. New agents are expected and chimeric antigen receptor T-cell therapy is a very promising approach.

(BELG J HEMATOL 2018;9(7):249–53)

Management of extended breast implant-associated anaplastic large cell lymphoma

SUMMARY

We report the case of a 69-year-old woman who presented an aggressive breast implant-associated anaplastic large cell lymphoma with supra- and infradiaphragmatic disease. The diagnosis was made 17 years after her first prosthesis, following a right breast carcinoma, and three years after the replacement of this first prosthesis. Breast implant-associated anaplastic large cell lymphoma is a rare form of non-Hodgkin lymphoma caused by a breast implant. Unique features of this case include the fast clinical extension of a lymphoma that is indolent in the vast majority of the cases. Indeed, less than two months after the first symptoms on the breast, cutaneous metastasis appeared on the right arm. The key diagnosis exams are histology and immunohistochemistry including CD30 and cytotoxic markers and a PET-scan to evaluate the extension of the disease. The treatment should include removal of the prosthesis and any associated mass. Local residual or unresectable disease may benefit from radiation therapy to the chest wall. For regional lymph node involvement or confirmed extended disease, adjuvant chemotherapy more in line with systemic anaplastic large cell lymphoma anaplastic lymphoma kinase-negative treatments is recommended. Finally, brentuximab vedotin, an anti-CD30 monoclonal antibody, showed encouraging results in refractory disease but still needs more prospective trials.

(BELG J HEMATOL 2018;9(7):279–84)

P25 Extended Breast Implant-Associated Anaplastic Large Cell Lymphoma: case report