BJH - 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)
Read moreBJH - volume 3, issue 3, september 2012
D. Selleslag MD
Haploidentical stem cell transplantation is an attractive form of alternative donor transplantation because of the immediate donor availability, ease of stem cell procurement, and the possibility to collect donor cells for further cellular therapy. T-cell depleted haploidentical transplantation (the Perugia approach) has been limited by a high nonrelapse mortality related to infectious complications as a result of delayed immune reconstitution posttransplantation. Research in this field is focusing on improving immune reconstitution by immunotherapy with different types of T-cells that do not cause graft-versus-host disease. A more recent modality (Hopkins approach), resulting in a decreased risk of graft-versus-host disease, is the use of T-cell replete haploidentical stem cells in combination with posttransplantation high-dose cyclophosphamide to eliminate expanding alloreactive T-cells. Research with this approach is focusing on the prevention of disease relapse posttransplantation. It seems that the most important barriers against successful haploidentical transplantation can now be overcome. This review evaluates the opposing modalities (T-cell replete versus T-cell depleted approach) and future directions of haploidentical stem cell transplantation in adults.
(BELG J HEMATOL 2012;3:74–81)
Read moreBJH - volume 3, issue 3, september 2012
S. Simoens PhD, MSc
Anticoagulants reduce blood clotting and are effective in preventing and treating venous thromboembolism, stroke and myocardial infarction. New oral agents such as dabigatran and rivaroxaban have recently been approved for these indications. Dabigatran and rivaroxaban benefit from oral administration, but have a higher potential for drug interactions than low molecular weight heparins. As compared with warfarin, dabigatran and rivaroxaban have a rapid onset of action and a predictable anticoagulant effect, obviating the need for routine coagulation monitoring. Although there are few economic evaluations of anticoagulants, the existing evidence suggests that the cost-effectiveness of anticoagulants depends on the alternative with which the anticoagulant is compared and on the specific disease.
(BELG J HEMATOL 2012;3:82–87)
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