D. Dierickx MD, PhD

PP30 Retrospective study of de novo and secondary myeloid sarcoma

The role of myeloid-derived suppressor cells in haematology: hype or reality?

Summary

One of the hallmarks of failure of elimination of malignant cells by activated T-cells is the immunosuppressive environment of the tumour. Myeloid-derived suppressor cells contribute to this immunosuppressive environment by inhibition of the adaptive and innate immune system. In this article we describe the current knowledge of the role of myeloid-derived suppressor cells in the progression of haematological malignancies.

(BELG J HEMATOL 2016;7(6):213–6)

Primary central nervous system lymphoma: a case report

Summary

Primary central nervous system lymphoma is a rare lymphoproliferative disorder confined to the central nervous system. Here we report a case of a 73-year-old female with an intracerebral lymphoma, discussing clinical presentation, diagnosis and treatment. Primary central nervous system lymphoma is a subtype of non-Hodgkin lymphoma which, in more than 90% of the cases, are diffuse large B-cell lymphomas. Clinical presentation is broad; the most common presentation in an immunocompetent patient is a focal neurological deficit. The neuroimaging technique of choice for diagnosis is contrast enhanced magnetic resonance. Histopathologic diagnosis is essential, which is mostly performed by a stereotactic guided biopsy. The backbone of treatment for primary central nervous system lymphoma is high-dose methotrexate, which can be combined with other chemotherapy, whole brain radiotherapy, corticosteroids and rituximab.

(BELG J HEMATOL 2016; 7(3):118–22)

BHS guidelines for primary central nervous system lymphoma

Summary

Primary central nervous system lymphoma is a rare form of extranodal B cell lymphoma of the brain, the eyes, the meninges or the spinal cord in the absence of systemic lymphoma. The management of primary central nervous system lymphoma remains controversial, which is related to the rarity of the cases and the small number of controlled studies available. The present consensus report provides the guidelines proposed by the Belgian Hematology Society Lymphoproliferative Working Party for treating immunocompetent adult patients with primary central nervous system lymphoma.

(BELG J HEMATOL 2016;7(2):69–78)

Nanobodies and thrombotic thrombocytopenic purpura

Summary

Thrombotic thrombocytopenic purpura is a non-malignant but life-threatening haematological disorder caused by deficiency of ADAMTS13, a metalloproteinase cleaving ultra-large von Willebrand factor multimer. Urgent initiation of therapeutic plasma exchange, in most cases associated with corticosteroids, has dramatically increased outcome of patients presenting with thrombotic thrombocytopenic purpura. Recently a phase II trial with caplacizumab, a humanised nanobody binding to the A1 domain of von Willebrand factor, has shown promising results by reducing the number of plasma exchanges with an acceptable safety profile.

(BELG J HEMATOL 2016;7(2):79–81)

ADAMTS13 containing the naturally occurring mutation p.Arg1177Gln adopts an open conformation

Waldenstrom’s macroglobulinaemia: Belgian Hematology Society guidelines

summary

Waldenström’s macroglobulinaemia is a B-cell disorder characterised by bone marrow infiltration with lymphoplasmacytic cells, along with demonstration of an IgM monoclonal gammopathy in the blood. This condition belongs to the lymphoplasmacytic lymphomas as defined by the World Health Organization classification (ICD-0 code 9671/3). Approximately one-fourth of patients are asymptomatic. Clinical features of the symptomatic patients are diverse and may relate to overall disease burden (such as peripheral blood cytopaenias, organomegaly and constitutional symptoms) or may be directly attributable to the IgM paraprotein. The latter include hyperviscosity syndrome, amyloidosis, peripheral neuropathy and cold haemagglutinin. Therapeutic options have traditionally involved alkylating agents, nucleoside analogues, and rituximab, either as single therapy or in combination. However, emerging new data on combination therapy as well as novel agents have shown encouraging results. This report provides the Belgian Hematology Society guidelines according to recent clinical studies.

(BELG J HEMATOL 2015;6(4):142–50)