BJH - volume 6, issue 1, march 2015
K. De Man MD, F. Van Ryckeghem MD, J. Dierick MD, B. Leus MD, V. Van Hende MD, M. Schurgers MD, H. Hannon MD
Systemic capillary leak syndrome is a potentially fatal disorder characterised by transient but severe hypotension, resulting in vascular collapse and shock, in combination with extreme hemoconcentration and anasarca oedema accompanied by a monoclonal gammopathy of unknown significance. We describe a case of Clarkson’s disease, complicated with severe hemoconcentration leading to splenic infarction and pulmonary oedema treated with ultrafiltration. The pathogenesis of systemic capillary leak syndrome remains unknown. We determined the serum concentration of soluble mediators erythropoietin and vascular endothelial growth factor, in order to attribute their role in the underlying pathophysiology of the disease.
(BELG J HEMATOL 2015;6(1):33–6)
Read moreBJH - volume 5, issue 4, december 2014
B. Depreter PhD, PharmD, E. Dumoulin PharmD, J. Billiet MD, B. Cauwelier MD, PhD, B. Maes MD, PhD, C. Matthys MD, J. Van Droogenbroeck MD, F. Nollet PhD, MSc, J. Emmerechts MD, PhD
We report a rare case of biclonal biphenotypic B-cell lymphocytosis with chronic lymphocytic leukaemia phenotype. Clonality was initially misjudged by immunophenotyping because of its polyphenotypic pattern. Polymerase chain reaction analysis revealed clonality of the immunoglobulin heavy chain and kappa light chain gene rearrangement, indicating the presence of a monoclonal B-lymphocyte population. Immunophenotyping was repeated after cell sorting and revealed two CD5+CD19+ populations with different light chain restriction. Different genetic abnormalities for both clones, as evidenced by fluorescence in situ hybridisation and hypermutation analysis, support the diagnosis of two independently originated but co-existing B-cell clones. This case illustrates the importance of using multiple techniques in the diagnostic work-up of haematological malignancies.
(BELG J HEMATOL 2014;5(4):143–7)
Read moreBJH - volume 5, issue 4, december 2014
B. Hodossy MD, I. Vrelust MD, S. Anguille MD, PhD, V. Van Marck MD, PhD, M. Maes PhD, PharmD, K. Vermeulen PhD, A. Van De Velde MD, PhD, A. Gadisseur MD, PhD, W. Schroyens MD, PhD, Z. Berneman MD, PhD
We present the case of a 58-year-old male patient with a long-standing, intermittent oedema of the lower extremities and significant spontaneous variations in haematocrit values. Repeated examinations failed to reveal a clear etiology until the patient suffered from a severely painful exacerbation of leg oedema and hypotension. Laboratory analysis showed hypoalbuminemia. The combination of oedema, hypotension, hypoalbuminemia and hemoconcentration was indicative of a systemic capillary leak syndrome. This condition is known to be associated with monoclonal gammopathy, as was the case in our patient. New investigations showed suspicious lesions in the nasopharynx, scrotum and breast. Biopsies of this breast mass as well as bone marrow biopsy showed the presence of an extranodal natural killer/T-cell lymphoma, nasal type. Polychemotherapy was administered according to the SMILE schedule leading to a remission after two cycles. The patient then underwent autologous hematopoietic stem cell transplantation. The patient is currently without signs of systemic capillary leak syndrome. This report illustrates that systemic capillary leak syndrome may occur as a prodrome of haematological malignancies, such as natural killer/T-cell lymphoma and documents that it is responsive to chemotherapy.
(BELG J HEMATOL 2014;5(4):148–53)
Read moreBJH - volume 5, issue 3, september 2014
P. Mineur MD, F. Hubert , E. Van den Neste MD, PhD, K. Peeters PhD
We observed severe hyponatremia in a patient who was treated with bortezomib for multiple myeloma. The patient was diagnosed with the syndrome of inappropriate secretion of antidiuretic hormone due to bortezomib.
(BELG J HEMATOL 2014;5(3):104–5)
Read moreBJH - volume 5, issue 3, september 2014
E. Macken MD, L. Lewi MD, PhD, D. Dierickx MD, PhD
A 26-year-old female was referred to our hospital in the 20th week of a second pregnancy because of severe intra-uterine growth restriction, hypertension, thrombocytopenia and proteinuria. Working diagnosis was thrombotic thrombocytopenic purpura, but it was difficult to differentiate it from haemolysis, elevated liver enzymes, and low platelet count. Therapeutic plasma exchange was urgently initiated and pregnancy was terminated with prostaglandin induction. The activity level of ADAMTS13 (a disintegrin and metalloproteinase with trombospondin type-1 motifs 13, the von Willebrand Factor cleaving protease), was immeasurably low. Furthermore, the patient was positive for ADAMTS13 inhibitor, which confirmed a diagnosis of acquired thrombotic thrombocytopenic purpura.
(BELG J HEMATOL 2014;5(3):106–9)
Read moreBJH - volume 5, issue 2, june 2014
A. Piette MD, B. De Moerloose MD, PhD, P. Schelstraete MD, PhD, V. Bordon MD, PhD, T. De Baere PhD, G. Claeys MD, PhD
We report a Myceliophthora thermophila infection in a boy who underwent haematopoietic (cord blood) stem cell transplantation as treatment for relapsed acute myeloid leukaemia. Despite neutrophil engraftment and maximal supportive treatment, the child died of multi-organ failure. Infections with environmental moulds are rare, but potentially disastrous in immunocompromised patients. Rapid diagnosis and early treatment is of upmost importance.
(BELG J HEMATOL 2014;5(2):60–3)
Read moreBJH - volume 5, issue 1, march 2014
L. Duquenne MD, V. Schlesser PhD, Z. Jedidi MD, L. Plawny MD
Haemophilia C, also known as Rosenthal syndrome, is a rare disorder affecting essentially persons of Ashkenazi Jewish ascendancy. We report the case of a 79 year old patient without previous bleeding history, except for slight bruising, who presented with a severe retinal bleeding in the absence of trauma. Biology showed elevated activated partial thromboplastin time (77,7 seconds vs. normal range 30–36 seconds). Factor analysis showed isolated decreased Factor XI of 1%. No Factor XI inhibitor could be found. The patient was subsequently treated with Factor XI infusions which allowed a reduction of the bleeding and normal eyesight within four days. Spontaneous bleeding in old age is a rare inaugural sign of Factor XI deficiency, such episodes mostly occur after haemostatic challenge such as surgery or trauma leading to blood analysis and coagulation tests. Intraocular haemorrhage is an uncommon presentation of mild bleeding disorders.1–5
(BELG J HEMATOL;5(1):22–24)
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