BJH - 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)
Read moreBJH - volume 7, issue 5, october 2016
K. Vanhouteghem PharmD, H. Vanhouteghem PharmD, K. De Schynkel MD, K. Van Haver MD, J. Dierick MD, A. Luyckx MD, PhD
Neonatal thrombocytopenia comprises a platelet count of less than 150×109/L, with severe neonatal thrombocytopenia in case of a platelet count less than 50×109/L. Severe thrombocytopenia is an uncommon finding in newborns requiring immediate diagnostic work-up with the need for urgent intervention to avoid severe bleeding complications. Neonatal thrombocytopenia can be classified by time of onset in early-onset (<72h after birth) versus late-onset (>72h after birth) thrombocytopenia. Multiple causes may be involved. In term, otherwise healthy neonates, one important cause of early-onset thrombocytopenia is antibody-mediated platelet destruction, and this can be allo- or auto-immune-mediated. In this case presentation, we discuss two neonates with severe early-onset immune-mediated thrombocytopenia presenting simultaneously at our general hospital.
(BELG J HEMATOL 2016;7(5):188–93)
Read moreBJH - volume 7, issue 3, june 2016
L. Barrios MD, M. Beckers MD, PhD, G. Verhoef MD, PhD, D. Dierickx MD, PhD
Primary central nervous system lymphoma is a rare lymphoproliferative disorder confined to the central nervous system. Here we report a case of a 73-year-old female with an intracerebral lymphoma, discussing clinical presentation, diagnosis and treatment. Primary central nervous system lymphoma is a subtype of non-Hodgkin lymphoma which, in more than 90% of the cases, are diffuse large B-cell lymphomas. Clinical presentation is broad; the most common presentation in an immunocompetent patient is a focal neurological deficit. The neuroimaging technique of choice for diagnosis is contrast enhanced magnetic resonance. Histopathologic diagnosis is essential, which is mostly performed by a stereotactic guided biopsy. The backbone of treatment for primary central nervous system lymphoma is high-dose methotrexate, which can be combined with other chemotherapy, whole brain radiotherapy, corticosteroids and rituximab.
(BELG J HEMATOL 2016; 7(3):118–22)
Read moreBJH - volume 7, issue 2, april 2016
I. Moors MD, L. Noens MD, PhD, T. Kerre MD, PhD
We present the case of a 68 year old woman with multiple myeloma, who developed mild persistent upper and lower gastrointestinal symptoms, together with a rash and abnormal liver function tests, in the early phase post-autologous stem cell transplantation. Pathology findings were compatible with graft-versus-host disease. Local treatment with corticosteroids resulted in a durable remission of the autologous graft-versus-host disease. A literature review reveals an incidence of mild gastrointestinal graft-versus-host disease in 13% of patients after autologous stem cell transplantation. Severe fatal autologous graft-versus-host disease has rarely been reported, and solely in multiple myeloma patients. In this article we discuss incidence, pathophysiology, outcome and treatment of these entities.
(BELG J HEMATOL 2016;7(2):83–6)
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