BJH - 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)
Read moreBJH - volume 7, issue 6, december 2016
M. Camaiani PharmD, C. Chevalier PharmD
A lumbar puncture was performed in a 45-year-old man with Cerebellar and Medullar T-cell lymphoma Not Otherwise Specified treated periodically with intrathecal injections of cytarabine. Laboratory analysis of cerebrospinal fluid resulted in technical interference with potential clinical implications.
(BELG J HEMATOL 2016;7(6):236–9)
Read moreBJH - volume 7, issue 5, october 2016
J. Claessens PhD, PharmD, M. Delforge MD, PhD, K. Poesen MD, PhD
A 79-year old man presented at the emergency ward of our hospital with symptoms characteristic for multiple myeloma. Although serum capillary zone electrophoresis appeared normal, kappa free light chains were found in serum and in urine by immunofixation electrophoresis. The kappa free light chain fraction detected in the serum, albeit appeared as a polyclonal-like smear with an aberrant localisation in the alpha-2- and beta-regions. Subsequent high resolution gel electrophoresis of the serum suggested the presence of a monoclonal fraction in the alpha-2- and beta-regions, but quantification of the band by densitometry was still not possible. Quantification of free light chains in the patient’s serum by immunonephelometry indicated the presence of an excessive amount of kappa free light chains. However, overestimation of the amount of kappa free light chains by the immunonephelometric method was suspected because of discrepancy with the intensity of the fractions on serum and urine immunofixation electrophoresis.
(BELG J HEMATOL 2016;7(5):194–8)
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