J. Van Droogenbroeck MD

Leptomeningeal myelomatosis as a rare presentation of multiple myeloma: A case report

SUMMARY

In this article, we present a case of a 53-year-old female multiple myeloma patient who was diagnosed with leptomeningeal myelomatosis after two years of treatment. This extramedullary presentation is extremely rare and occurs in less than 1% of multiple myeloma patients. Leptomeningeal myelomatosis most commonly manifests with headache, confusion, cerebral nerve palsy and radiculopathy. When leptomeningeal myelomatosis is suspected, the first step in diagnosis is contrast-enhanced magnetic resonance imaging (MRI). However, cerebrospinal fluid (CSF) analysis is imperative for definite diagnosis. Leptomeningeal myelomatosis is commonly associated with high-risk genetic features including deletion of 17p (TP53), absence of CD56, IgA or IgD paraprotein and lambda subtype. Leptomeningeal myelomatosis has a poor prognosis with an expected survival in terms of months after diagnosis. There is no standard treatment. However, there is some evidence for promising results of therapy with marizomib and pomalidomide.

(BELG J HEMATOL 2023;14(2):73–6)

Highlights in relapsed/refractory multiple myeloma

SUMMARY

As our induction therapy in the approach of multiple myeloma (MM) keeps on changing, the character of the relapses is altering too. By moving from doublets over triplets to quadruplets in the induction schemes, and thus intensifying first-line therapies, relapses are very often postponed in time. Nevertheless, they are more resistant to chemotherapy and thus more difficult to treat. In vitro data confirm the increase of the mutational burden of the persistent myeloma cells after chemotherapy and even more after the administration of high dose melphalan, followed by autologous stem cell support (ASCT). This could be an argument to leave ASCT in the first-line treatment of MM in the upcoming future. Moreover, the common use of lenalidomide in maintenance will lead to more refractoriness to this molecule in the relapse setting. The same is of course true when other molecules – e.g. proteasome inhibitors – are used in the maintenance setting. If the addition of daratumumab in first-line would become the standard in induction, this will of course also influence the behaviour of RRMM.

(BELG J HEMATOL 2021;12(1):14-6)

Update on therapy of relapsed and refractory multiple myeloma

SUMMARY

The prognosis for multiple myeloma patients has improved substantially over the past decade with the development of more effective chemotherapeutic agents and regimens that possess a high level of anti-tumour activity. However, nearly all multiple myeloma patients ultimately relapse, even those who experience a complete response to initial therapy. Management of relapsed disease remains a critical aspect of multiple myeloma care and an important area of ongoing research. This manuscript from the Belgian Haematology Society multiple myeloma subgroup provides some recommendations on the management of relapsed disease.

(BELG J HEMATOL 2017;8(2):53–65)

Carfilzomib in the treatment of multiple myeloma

Summary

More than ten years ago the dipeptidyl boronic acid derivative proteasome inhibitor bortezomib changed the therapeutic field of multiple myeloma dramatically. This introduction of a whole new class of chemotherapy lead to a significant extension of disease-free and overall survival for most myeloma patients. But as with most first-in-class molecules, efficacy can probably be improved — primary and secondary bortezomib resistance being common – and treatment is all too often limited by dose-limiting side effects.² Carfilzomib is without doubt a very promising next generation proteasome inhibitor.

(BELG J HEMATOL 2015;6(2):71–3)

Update on the initial therapy of multiple myeloma

Summary

With the introduction of immunomodulatory drugs and proteasome inhibitors, major improvements have been achieved in the treatment and prognosis of multiple myeloma. Different treatment combinations are now in use and innovative therapies are being developed. This rapidly changing therapeutic landscape calls for an update on the Belgian myeloma guidelines, published in 2010.¹ Based on an extensive review of the recent literature, the myeloma study group of the Belgian Hematology Society has revised the consensus recommendations on myeloma care, to be used by haematologists as a reference for daily practice. When applicable, comments with regards to the Belgian reimbursement modalities are included. The full text with appendices can be downloaded from the Belgian Hematology Society website (www.bhs.be) and from the Belgium Journal of Hematology website (www.ariez.com).

(BELG J HEMATOL 2014;5(4):125–36)

Biclonal biphenotypic B-cell lymphocytosis: a CLL in disguise?

Summary

We report a rare case of biclonal biphenotypic B-cell lymphocytosis with chronic lymphocytic leukaemia phenotype. Clonality was initially misjudged by immunophenotyping because of its polyphenotypic pattern. Polymerase chain reaction analysis revealed clonality of the immunoglobulin heavy chain and kappa light chain gene rearrangement, indicating the presence of a monoclonal B-lymphocyte population. Immunophenotyping was repeated after cell sorting and revealed two CD5+CD19+ populations with different light chain restriction. Different genetic abnormalities for both clones, as evidenced by fluorescence in situ hybridisation and hypermutation analysis, support the diagnosis of two independently originated but co-existing B-cell clones. This case illustrates the importance of using multiple techniques in the diagnostic work-up of haematological malignancies.

(BELG J HEMATOL 2014;5(4):143–7)

P2.10 BRAF V600E mutation is not a disease-defining genetic event in classic hairy cell leukemia