W. Schroyens MD, PhD

P1.02 Hypercalcemia and osteolytic bone lesions in adult B-cell lymphoblastic leukemia: a rare but potentially life-threatening presentation

BHS Guidelines for the treatment of Burkitt’s lymphoma

Summary

Burkitt’s lymphoma is a rare but very aggressive non-Hodgkin’s lymphoma characterised by an isolated translocation t(8;14)(q24;q32). The sporadic form is the sub-entity most frequently encountered in Belgium. Diagnosis and initial work-up must be completed rapidly to start treatment as soon as possible. Positron emission tomography scan is useful for initial staging and to evaluate the chemosensitivity of the tumour during and after treatment. After debulking, it is recommended to add rituximab to chemotherapy. Currently intensive short-cycle and low intensity chemotherapies are two valuable options. Radiotherapy is not indicated except in case of central nervous system involvement. Patients achieving complete remission must be followed carefully during the first year to detect recurrence of the disease. More than 80% of patients sustain their remission one year following initial treatment and are considered cured. For patients in partial remission or with chemosensitive relapse, autologous stem cell transplantation is recommended following re-induction with non-cross-resistant polychemotherapy. Monitoring complete blood counts and cognitive functions is important to detect late toxicity of the applied therapies.

(BELG J HEMATOL 2015;6(2):61–9)

Systemic capillary leak syndrome as a prodrome of extranodal natural killer (NK)/T-cell lymphoma

Summary

We present the case of a 58-year-old male patient with a long-standing, intermittent oedema of the lower extremities and significant spontaneous variations in haematocrit values. Repeated examinations failed to reveal a clear etiology until the patient suffered from a severely painful exacerbation of leg oedema and hypotension. Laboratory analysis showed hypoalbuminemia. The combination of oedema, hypotension, hypoalbuminemia and hemoconcentration was indicative of a systemic capillary leak syndrome. This condition is known to be associated with monoclonal gammopathy, as was the case in our patient. New investigations showed suspicious lesions in the nasopharynx, scrotum and breast. Biopsies of this breast mass as well as bone marrow biopsy showed the presence of an extranodal natural killer/T-cell lymphoma, nasal type. Polychemotherapy was administered according to the SMILE schedule leading to a remission after two cycles. The patient then underwent autologous hematopoietic stem cell transplantation. The patient is currently without signs of systemic capillary leak syndrome. This report illustrates that systemic capillary leak syndrome may occur as a prodrome of haematological malignancies, such as natural killer/T-cell lymphoma and documents that it is responsive to chemotherapy.

(BELG J HEMATOL 2014;5(4):148–53)

Treatment of mantle cell lymphomas: recommendations of the Belgian Hematological Society

Summary

Mantle cell lymphoma was recognised in the nineties and is characterised by the t(11;14)(q13;q32) translocation which results in overexpression of cyclin D1.¹ This disease represents approximately 6% of all non-Hodgkin’s lymphomas. Mantle cell lymphoma generally affects patients over 60 years-old. Most patients have advanced disease (>70 % Ann Arbor stage IV). Several efforts have been made to predict outcome in mantle cell lymphoma. The cell-proliferation marker Ki-67, the Mantle Cell Lymphoma International Prognostic Index, fluorodeoxyglucose positron emission tomography and minimal residual disease are prognostic tools. For young patients, chemoimmunotherapy followed by high-dose chemotherapy plus stem cell transplantation is the treatment of choice. For the main group of older patients, chemo-immunotherapy followed by maintenance with rituximab is the gold standard. In relapses, temsirolimus is actually registered and new drugs, such as ibrutinib, are currently evaluated with promising preliminary results.^2–5

(BELG J HEMATOL 2014;5(3):89–96)

BHS guidelines for the treatment of marginal zone lymphomas

Summary

Marginal zone lymphomas are a heterogeneous subtype of indolent B-non-Hodgkin Lymphoma that includes three distinct diseases: Extranodal mucosa associated lymphoid tissue lymphoma, nodal marginal zone lymphoma and splenic marginal zone lymphoma lymphocytes +/− villous lymphocytes. The different diagnosis, work up and treatment options are discussed in these guidelines.

(BELG J HEMATOL 2014;5(1):12–21)

P2.18 Responding patients show durable responses to bendamustine in double refractory multiple myeloma patients

P4.09 A novel HLA-C null variant allele (C*05:XXN) generated by single nucleotide deletion and premature stop codon in exon 3