BJH - volume 11, issue 7, november 2020
D. Papazoglou MD, A. Salaroli MD, P. Heimann MD, PhD, P. Lewalle MD, PhD, D. Bron MD, PhD
A 47-year-old patient was diagnosed with FIP1L1-PDGFRA-positive myeloid/lymphoid neoplasm with eosinophilia (F/P+ MLN-eo) and was successfully treated with Imatinib, achieving a sustained molecular treatment free remission (TFR) persisting three years after discontinuation.
(BELG J HEMATOL 2020;11(7):320-4)
Read moreBJH - volume 11, issue 7, november 2020
J. Deuson MD, C. Motet MD, L. Delval MD, A-L. Trepant MD, J. Brauner MD
A 65-year-old woman presented with thrombocytopenia. A bone marrow biopsy was done and showed a cellular bone marrow infiltration by 94% of blast-like cells making the diagnosis of acute leukaemia. However, flow cytometry excluded hematological malignancy. After an extensive panel of immunohistochemical stains and genetic analysis, the diagnosis of metastatic alveolar rhabdomyosarcoma was made.
(BELG J HEMATOL 2020;11(7):317-9)
Read moreBJH - volume 11, issue 6, october 2020
E. Damen MD, M. van den Akker MD, S. Diallo MD, S. Uyttebroeck MD, B. Hauser MD, PhD, J. van der Werff ten Bosch MD, PhD
DNAJC21 mutations have recently been discovered as the rare cause of a Swachmann Diamond-like syndrome. So far, fifteen children have been reported in literature. We here describe two new patients from two different families, both harbouring previously undescribed mutations. Our patients had different mutations, but looked phenotypically alike and both presented with oral aversion. One patient presented with amegakaryocytosis only, which is not described as such in literature. The other patient presented with low platelets and only a mild decrease in neutrophils and haemoglobin, had only amegakaryocytosis in the bone marrow and developed pancytopenia soon afterwards. Finally, both patients had low elastase in stool, although they had no clinical signs of pancreatic insufficiency, hinting that this easy test should not be forgotten in the work up of patients with congenital amegakaryocytosis or bone marrow failure syndrome.
(BELG J HEMATOL 2020;11(6):268-71)
Read moreBJH - volume 11, issue 3, may 2020
J. Loos MD, D. Dierickx MD, PhD
Oxaliplatin-based chemotherapy is commonly used to treat colorectal cancer. After prolonged administration it can rarely lead to hypersensitivity reactions such as immune mediated haemolytic anaemia and thrombocytopenia. We present the case of a 50-year old patient admitted with acute onset of fever, dark urine and back pain during the ninth infusion of oxaliplatin. Two weeks before the current event he experienced similar though less severe symptoms. Laboratory signs were compatible with severe Coombs positive haemolytic anaemia, thrombocytopenia and acute kidney injury. Signs of haemolysis abated quickly, however, the patient developed anuria and required dialysis for thirty days. Immune mediated haemolytic anaemia is a rare but potentially life-threatening complication of prolonged oxaliplatin therapy, especially when kidney failure develops. A careful history and high index of suspicion may identify warning signs leading to increased vigilance and possible prevention of such events.
(BELG J HEMATOL 2020;11(3):128–32)
Read moreBJH - volume 11, issue 2, march 2020
N.C. Granacher MD, T. Eyckmans MD
The chimeric monoclonal IgG1 antibody directed to CD20 Rituximab is used to treat various haematological malignancies and auto-immune diseases. Serum sickness is a type III hypersensitivity reaction leading to the formation and tissue deposition of immune antibody-antigen complexes. It has been described as a very rare complication of Rituximab treatment, mainly seen in patients treated for auto-immune diseases. We report the case of a patient with Waldenstrom’s macroglobulinaemia whose Rituximab treatment was complicated by documented immune complex deposition or serum sickness. We successfully applied a Rituximab desensitisation protocol, which allowed us to complete treatment.
(BELG J HEMATOL 2020;11(2):75–8)
Read moreBJH - volume 10, issue 7, november 2019
M. Verstraeten MD, C. Verbeke MD, B. De Moerloose MD, PhD
We describe a 7-month-old girl with severe neutropenia born to a mother treated for ulcerative colitis with infliximab until the 24th week of pregnancy. Despite the recommendation of using Tumour Necrosis Factor inhibitors (TNFi) only in the first and second trimester of pregnancy, significant levels of TNFi in offspring are possible. Hence, drug-induced neutropenia should be considered in the differential diagnosis of infants with severe neutropenia if these were exposed to TNFi in utero. Moreover, additional information is given on the risk of infection and dysfunctional immune development in these new-borns.
(BELG J HEMATOL 2019;10(7):285–9)
Read moreBJH - volume 10, issue 6, october 2019
A. Louwagie PharmD, M. Tajdar PharmD, B. Cauwelier MD, PhD, H. Devos MD, J. Robbrecht MD, S. Van Erum MD, J. Emmerechts MD, PhD
We report a case of a falsely increased mean corpuscular volume (MCV) due to severe hyperglycaemia in a patient with diabetic ketoacidosis. This phenomenon results from in vitro swelling of hyperosmolar red blood cell size when diluted in an iso-osmolar buffer of a haematology analyser, and does not reflect a true macrocytosis in vivo. The magnitude of this effect is dependent on the glucose concentration of the sample and time of incubation prior to analysis. Haematology analysers from three different manufacturers were found equally sensitive to this phenomenon. Therefore, it is suggested to use reluctance when reporting and interpreting MCV results in the case of severe hyperglycaemia to avoid unnecessary additional investigation.
(BELG J HEMATOL 2019;10(6):250–4)
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