BJH - 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)
Read moreBJH - volume 4, issue 2, june 2013
L. Rozen PharmD, D. Noubouossie MD, A. Demulder MD, PhD
D–dimer (DD) assay is a widely used laboratory test in thrombosis-related conditions because it is rapid and easy to perform. This test is highly sensitive to thrombus formation and degradation. However DD levels are increased in many clinical conditions so that its positive predictive value is poor. Improvements of its usefulness have been mainly realised by combining the test with clinical scores and by adapting positive threshold to particular settings of patients. In this article, different methods of DD testing are presented with the aim to review their benefits and pitfalls in various clinical applications.
(BELG J HEMATOL 2013;4(2):47–50)
Read moreBJH - volume 4, issue 1, march 2013
M.A. Azerad MD, F. Debaugnies PharmD, A. Demulder MD, PhD, D. Bron MD, PhD, A. Efira MD
Microvesicles (MV) are since quite recently recognized as forming a unique network between cells. These very little fragments (<1 µm size) are actively released from their parent cells and are able to transfer both cellular and nuclear material. Although active debate remains on how to best detect MV, rendering some results questionable, high MV levels have been reported in aggressive tumours and have been correlated with a poor clinical outcome. Some tumour cell derived MV exhibit strong tissue factor dependent procoagulant activity. Their detection could actually predict the thrombotic risk in selected cancer patients. A growing body of evidence suggests cell microvesicles to be a major link between cancer and thrombosis. Current knowledge on MV in cancer will be reviewed here.
(BELG J HEMATOL 2013;1:3–8)
Read moreBJH - volume 3, issue 4, december 2012
D. Dierickx MD, PhD, X. Vanoeteren MD, G. Verhoef MD, PhD
Prevention of organ rejection following solid organ transplantation requires long term immunosuppressive therapy, leading to an increased risk of both infections and malignancies. Although skin cancers are the most common malignancies, posttransplant lymphoproliferative disorder (PTLD) comprises one of the most serious complications following transplantation with high morbidity and mortality rates. Here we will review current treatment options for PTLD following solid organ transplantation (SOT).
(BELG J HEMATOL 2012;3: 121–127)
Read moreBJH - volume 3, issue 4, december 2012
J. Cornillie MD, T. Tousseyn MD, PhD, G. Verhoef MD, PhD
T-cell/histiocyte rich large B-cell lymphoma (THRLBCL) is a rare variant of diffuse large B-cell lymphoma (DLBCL) with an aggressive behaviour. Clinically, THRLBCL affects a young, predominantly male population. Pathologically, it is characterised by fewer than 10% of large neoplastic B-cells in a background of abundant T-cells with or without the presence of histiocytes. Differentiating THRLBCL from other lymphoproliferative disorders can be difficult but is achieved by morphologic and immunohistochemical characterisation of the tumour cells in the appropriate stromal microenvironment. Despite these clinical and pathologic differences, treating THRLBCL is not different from treating stage-matched DLBCL and can result in a comparable outcome. Comparative studies, however, on outcome of THRLBCL and DLBCL are methodologically weak and include small numbers of patients. Recently, gene expression profiling showed a predominant role for a distinct host immune response in THRLBCL, leading to tumor tolerance. Targeting specific molecules responsible for this tumour tolerance could lead to novel therapeutic options.
(BELG J HEMATOL 2012;3: 128–133)
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