HEMATOCASE

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)

Systemic capillary leak syndrome as a prodrome of extranodal natural killer (NK)/T-cell lymphoma

Summary

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)

Thrombotic thrombocytopenic purpura in pregnancy: a case report

Summary

A 26-year-old female was referred to our hospital in the 20^th 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)

Syndrome of inappropriate antidiuretic hormone secretion associated with bortezomib

Summary

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)