BJH - volume 9, issue 5, september 2018
I. Depoortere MD, V. Maertens MD, M. Criel , M. Vanden Driessche MD, I. Geerts MD
We present a case of a 73-year old patient with polycythaemia vera in whom cyclic thrombocytopenia was diagnosed. Strong fluctuations in platelet count, ranging from 31 to 1334 × 103/µL, were noticed after onset of hydroxyurea therapy. We did a literature search to find possible underlying causes of cyclic thrombocytopenia that could guide us towards a fast and appropriate diagnosis and an optimal treatment. In literature, provoked and unprovoked oscillations in platelet numbers have been described. Unprovoked oscillations can most likely be attributed to an unstable haematopoietic stem cell pool, as can be seen in polycythaemia vera. Provoked oscillations could be associated with myelosuppressive agents such as hydroxyurea. In both situations, a decrease in platelet count can be followed by a compensatory thrombopoietin-induced stimulation of megakaryocytes. Frequent hydroxyurea dose adjustments may be carried out in an attempt to control this cyclic pattern but, by contrast, may provoke a bouncing ball effect on platelet count. Certain patients will therefore benefit from maintaining therapy at a constant dose; while certain others require withholding or switching therapy. Cyclic thrombocytopenia is a rare finding and is frequently misdiagnosed as immune thrombocytopenia. If hydroxyurea-treated patients with a chronic myeloproliferative disorder present with thrombocytopenia, cyclic thrombocytopenia should be considered. Intensive follow-up with regular control of platelet count and personalised therapy is mandatory.
(BELG J HEMATOL 2018;9(5):188–91)
Read moreBJH - volume 9, issue 2, march 2018
F. Baro MD, J. Bastin MD, S. Schifflers MD, N. Francotte MD, P. Philippet MD, C.F. Chantrain MD, PhD
Immune thrombocytopenic purpura is an isolated thrombocytopenia consisting of premature platelet destruction mediated by self-reacting antibodies and an impaired platelet production. In children, most of the cases resolve spontaneously within six months. Several studies have shown a high incidence of vitamin D deficiency in auto-immune disorders, including immune thrombocytopenic purpura. We report the clinical history of an eight-year-old boy who presented with refractory immune thrombocytopenic purpura and major vitamin D deficiency. Supplementation in vitamin D was followed by a rapid normalisation of thrombocytosis. After six months of evolution, the child developed a relapse of immune thrombocytopenic purpura concomitant with a decrease of his serum level of vitamin D. Treatment with vitamin D associated with dapsone resulted in a prolonged remission. After thirteen months, dapsone treatment was stopped and the platelet count remained normal. This article reviews the approach of refractory immune thrombocytopenic purpura in children and discusses the potential interest of vitamin D in this disease.
(BELG J HEMATOL 2018;9(2):64–7.)
Read moreBJH - volume 8, issue 7, december 2017
V. Beckers MD, R. Schots MD, PhD, K. Fostier MD
Bleeding diathesis in light chain amyloidosis may be due to an acquired coagulation factor deficiency, most commonly factor X deficiency. This report describes the case of an elderly myeloma patient with associated light chain amyloidosis, nephrotic syndrome and factor X deficiency. Treatment with nine cycles of subcutaneous bortezomib + melphalan + prednisone resulted in a complete haematological remission with resolution of the nephrotic syndrome and normalisation of coagulation tests. This case report highlights the importance of performing coagulation screening tests in light chain amyloidosis. In addition, it illustrates that bortezomib-based regimens can induce rapid and complete haematological response with long-term correction of factor X levels.
(BELG J HEMATOL 2017;8(7):272–5)
Read moreBJH - volume 8, issue 6, october 2017
G. Van den Bosch PhD, M. Ramael MD, PhD, P. Storms MD, M. Develter MD, J. Willemse PhD, B. Maes MD, PhD, G. Bries MD, PhD
In this hematocase, a patient is presented with a spontaneous rupture of the spleen due to massive splenomegaly caused by a previously undiagnosed diffuse large B-cell lymphoma. Diagnosis and differentiation of this non-Hodgkin lymphoma is discussed with attention for the prognostic implications of the results. The association between spontaneous rupture of the spleen and haematological malignancies is further explored by means of earlier described cases. A take home message is given regarding this rare initial presentation of a lymphoma since immediate surgical intervention is imperative in these cases.
(BELG J HEMATOL 2017;8(6):239–43)
Read moreBJH - volume 8, issue 5, september 2017
B. Van Aelst PhD, J. Coene MD, H.B. Feys PhD, M.P. Emonds MD, PhD, K. Van Poucke MD, J. Moerman MD, E. Verhoye MPharm , Prof V. Compernolle PhD
We report three cases of massive ex vivo coagulation in a leukocyte depleted red cell concentrate during transfusion. Molecular blood typing indicated that patient blood was present in the filter housing of the infusion set and/or in the blood bag itself. Together with the history of events, this suggested that the observed clotting was caused by backflow of patient blood. Therefore, we recommend maintaining the blood bag above heart level during the entire transfusion procedure, until the infusion set is removed.
(BELG J HEMATOL 2017;8(5):195–7)
Read moreBJH - volume 8, issue 3, june 2017
C. Meert MD, S. Vanderschueren MD, PhD, K. Poesen MD, PhD, R. Sciot MD, PhD, S. Pans MD, M. Delforge MD, PhD
Amyloid myopathy is a rare manifestation of amyloid light chain amyloidosis. We present a case of a 41-year old male with multiple myeloma with muscle hypertrophy, muscle weakness and enlargement of the submandibular glands as the only presenting clinical symptoms, illustrating the sheer difficulty of diagnosing amyloid light chain amyloidosis in patients with mainly soft tissue involvement. Even if there is a clinical suspicion, it is often hard to verify as Congo red stain and immunohistochemistry on muscle biopsy are not always reliable. After bortezomib-based induction treatment followed by autologous stem cell transplantation with high dose melphalan conditioning, he achieved complete haematological remission as well as a significant clinical response. We would like to highlight the importance of early diagnosis and treatment, as progression to more extensive visceral involvement can lead to rapid occurrence of organ failure and death.
(BELG J HEMATOL 2017;8(3):113–7)
Read moreBJH - volume 8, issue 2, march 2017
A-S. De Koninck PharmD, C. Dhooge MD, PhD, B. Denys MD, K. Vandepoele PhD, N. Van Roy PhD, M. Hofmans MD, PhD, J. Philippé MD, PhD
We describe a case of a four-year-old boy diagnosed with an Early T-cell Precursor Lymphoblastic Leukaemia. This type of leukaemia is recognised as a high-risk subgroup characterised by very early arrest in T-cell differentiation. Early T-cell Precursor Lymphoblastic Leukaemia cases have characteristic gene expression profiles, increased genomic instability and a distinct immature immunophenotype (CD1a–, CD8–, CD5+dim and positivity for at least one marker of stem cell or myeloid lineage). This type of leukaemia is associated with poor prognosis and a poor response to intensive chemotherapy, though this finding is still debated. Our patient displayed an inferior response to induction therapy. The main purpose of this report is to make Belgian physicians aware of this entity and its controversial prognostic significance.
(BELG J HEMATOL 2017;8(2):75–9)
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