HEMATOCASE

Immune-mediated early-onset neonatal thrombocytopenia: a double case presentation

Summary

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)

Primary central nervous system lymphoma: a case report

Summary

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)

Occurrence of mild spontaneous graft-versus-host disease after autologous stem cell transplantation for multiple myeloma: a case report and review of the literature

Summary

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)

A case report of an atypical POEMS syndrome

summary

POEMS syndrome is a rare paraneoplastic syndrome that is defined by the presence of peripheral neuropathy, a monoclonal plasma cell disorder, and other paraneoplastic features, of which the most common include organomegaly, endocrinopathy and skin changes.¹ We report a case of POEMS syndrome in a 62-year old female who presented with worsening general condition, weight loss, asthenia and diarrhoea. Clinical examination showed the presence of ascites, peripheral oedema and a thickened skin with the presence of glomeruloid hemangioma. Further investigations showed the presence of three isolated FDG-avid bone laesions on PET-CT, a plasmacytoma with lambda restriction on bone marrow biopsy and elevated VEGF serum levels. The patient was treated with local radiotherapy with a total dose of 39 gray. Two months after radiotherapy, the patient already has a good clinical response with a reduction of ascites, fluid retention and diarrhoea, associated with a significant decline in the VEGF level.

After the case description, a review of the literature is presented.

(BELG J HEMATOL 2015;6(4): 156–61)

Haemolytic crisis induced by rasburicase administration revealing G-6-PD deficiency

Summary

We present a patient with Burkitt’s lymphoma who suffered a severe haemolytic crisis after treatment with rasburicase. This case report underlines the high incidence of glucose-6-phosphate dehydrogenase deficiency in some ethnic groups and the importance of a detailed patient and family history before starting treatment, even in case of emergency. Glucose-6-phosphate dehydrogenase is an essential enzyme since it makes the synthesis of NADPH + H from NADP possible, which determines the reducing power (NADPH) of the cell. Every defect in this physiological process, notably glucose-6-phosphate dehydrogenase deficiency, may thus result simultaneously with the use of rasburicase in acute or chronic haemolysis according to the importance of the deficiency. Management is based on stopping the incriminated drug and on supportive therapy consisting of administering packed red blood cells if the anaemia is poorly tolerated.

(BELG J HEMATOL 2015;6(2): 74–8)

Splenic infarction due to severe hemoconcentration in a case of systemic capillary leak syndrome (Clarkson’s disease)

Summary

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)

Biclonal biphenotypic B-cell lymphocytosis: a CLL in disguise?

Summary

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)