BJH - volume 6, issue 1, march 2015
M. Maerevoet MD, J. Vouriot MD, N. Meuleman MD, PhD, D. Bron MD, PhD
Diffuse large B-cell lymphoma is a frequent pathology in older individuals, and though curable by R-CHOP 21, treatment toxicity increases in frail patients. Therefore, therapeutic choices have to take into account Comprehensive Geriatric Assessment in addition to Performance Status, but reliable and standardised clinical decision-making tools are sorely lacking. However, Mabthera-containing treatments adapted for frail patients and co-morbidities can be used with a satisfactory survival rate at two years. Nevertheless, the main cause of death remains disease progression.
(BELG J HEMATOL 2014;6(1):4–9)
Read moreBJH - volume 5, issue 4, december 2014
H. Helsmoortel PhD, T. Lammens PhD, N. Van Roy PhD, J. Philippé MD, PhD, P. De Paepe MD, PhD, Y Benoit MD, PhD, F. Speleman PhD, P. Van Vlierberghe PhD, B. De Moerloose MD, PhD
Juvenile myelomonocytic leukaemia is a very rare, aggressive stem cell disorder predominantly affecting infants and young children. Current survival rates are disappointing and the only available curative therapy is haematopoietic stem cell transplantation. Over the last years, intensive research efforts elucidated a plethora of molecular aberrations involved in the pathogenesis of juvenile myelomonocytic leukaemia. Current investigations are mainly directed towards the complete unravelling of the molecular biology behind the disease in order to find more specific drugs. This review will focus on the diagnosis, genomic characterisation and the use of experimental therapies in juvenile myelomonocytic leukaemia.
(BELG J HEMATOL 2014; 5(4): 119–24)
Read moreBJH - volume 5, issue 3, september 2014
J. Van Der Straeten MSc, B. De Moerloose MD, PhD, M-F. Dresse MD, PhD, S. Dupont MD, A. Ferster MD, PhD, P. Philippet MD, A. Uyttebroeck MD, PhD, J. van der Werff ten Bosch MD, PhD, C. Demanet MD, PhD, Y Benoit MD, PhD, M. Bakkus PhD
In Belgium approximately 70 children are diagnosed with acute lymphoblastic leukaemia annually. For these children, the monitoring of minimal residual disease has an important prognostic value. The level of minimal residual disease during the first three months of therapy is used to recognise subgroups that differ substantially in outcome. Two techniques are used for minimal residual disease monitoring: the Genescan method and the allele specific oligonucleotide polymerase chain reaction. The Genescan method is a less sensitive method (10−3) but is fast and less expensive. The allele specific oligonucleotide polymerase chain reaction requires more time and budget but has a sensitivity of 10−4–10−5. Both techniques have proven their value in minimal residual disease monitoring in childhood acute lymphoblastic leukaemia.
(BELG J HEMATOL 2014;5(3):81–8)
Read moreBJH - volume 5, issue 2, june 2014
J. Van Meirhaeghe MD
Balloon kyphoplasty is a minimally invasive procedure for the treatment of painful vertebral compression fractures due to primary or secondary osteoporosis, osteolytic lesions due to myeloma or bone metastasis, or trauma. Balloon kyphoplasty aims to reduce and stabilise the fracture, thereby providing immediate and sustained pain relief, improved physical function and better quality of life. Balloon kyphoplasty differs from vertebroplasty in that it is also designed to restore diminished vertebral height and correct kyphotic deformity.
(BELG J HEMATOL 2014;5(2):36–43)
Read moreBJH - volume 5, issue 1, march 2014
N. Put MD, PhD, L. Michaux MD, PhD, P. Vandenberghe MD, PhD
The presence, number and/or type of chromosomal aberrations represent an independent predictor of prognosis in several haematological disorders. Therefore, (cyto)genetic analysis is now routinely performed in many haematological malignancies. Different techniques are available to detect chromosomal abnormalities. Conventional cytogenetic analysis can be performed, and also interphase fluorescent in situ hybridisation is widely used. In addition, multiplex ligation-dependent probe amplification and more recently analysis by means of different array-platforms have been used in research and routine setting. Newly developed techniques, such as next-generation sequencing are only available for research purposes thus far. All these techniques are complementary, and each technique has its own (dis)advantages.
(BELG J HEMATOL 2014;5(1):3–11)
Read moreBJH - volume 4, issue 4, december 2013
K. Beel MD, PhD
The development of new immunomodulatory therapies and their implementation in the treatment of multiple myeloma in the past years, offer new perspectives for the treatment of other plasma cell dyscrasias. Light chain amyloidosis is historically associated with a very poor prognosis, despite the small size of the monoclonal plasma cell population, due to progressive amyloid deposition in vital organs. Hence, advances in treatment are eagerly awaited. Luckily, myeloma patients are paving the way for light chain amyloidosis treatment, clearly demonstrating that immunomodulatory drugs and proteasome inhibitors are capable of controlling plasma cell proliferation. Two recently published trials have shown a remarkable survival benefit with CyBorD, a bortezomib containing regimen in light chain amyloidosis, possibly setting a new standard for the treatment of this disease. In this article, we review current insights in the pathogenesis, diagnostic challenges, prognostic markers and available treatments for light chain amyloidosis.
(BELG J HEMATOL 2013;4(4): 120–126)
Read moreBJH - volume 4, issue 3, september 2013
G. Deslypere MD, T. Devos MD, PhD, M. Delforge MD, PhD, G. Verhoef MD, PhD
Systemic mastocytosis is an orphan myeloproliferative disease characterised by an excessive mast cell accumulation. Benign forms present with urticaria pigmentosa while aggressive subtypes or leukaemic variants lead to organ dysfunction. In patients with unexplained hypotensive syncope’s or anaphylaxis, flushing and angio-oedema with a basal tryptase >20 ng/mL, one should think of systemic mastocytosis. Pathophysiology is based on mutations in KIT, encoding the c-kit receptor (CD117) on the surface of mast cells. Diagnosis is based on bone marrow biopsies with clusters of atypical mast cells and co-expression of CD2 or CD25 and/or a KIT mutation. Treatment consists of avoiding triggers of mast cell release and antihistaminic drugs. Patients with aggressive subtypes are candidates for cytoreductive therapies. CD30 is thought to be a novel predictor of prognosis.
(BELG J HEMATOL 2013;4(3):85–89)
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