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1/16. Cyclosporin neurotoxicity with Epstein-Barr virus-associated hemophagocytic syndrome.

    In September 2000, a 22-year-old female was admitted to our hospital due to high grade fever, liver enzymes elevation and pancytopenia. Bone marrow aspiration was performed, and hemophagocytosis was present. Epstein-Barr virus (EBV) dna was positive in her peripheral blood, and we diagnosed the case as EBV-associated hemophagocytic syndrome (EB-VAHS) after excluding other malignancies. The initial therapy including etoposide and dexamethasone was started. As severe leukocytopenia developed, etoposide was stopped and cyclosporin A (CsA) was administered continuously. Four days after administration of CsA, she developed convulsive seizures with loss of consciousness. An MRI demonstrated decreased signal with T1-weighting and high signal with T2-weighting in the subcortical white matter including the posterior lobe. We stopped CsA infusion, and glycerol was administered. Soon the symptom disappeared. When patients developed an episode of convulsive seizure, other diagnostic possibilities were central nervous system (CNS) involvement of hemophagocytosis, EBV encephalitis and acute disseminated encephalomyelitis (ADEM). CsA neurotoxicity must be considered even in the case of EB-VAHS with administration of CsA. As previously reported, Fluid-attenuated Inversion Recovery (FLAIR) imaging improved diagnostic confidence and conspicuity of the T2 hyper intense lesions of CsA neurotoxicity, as well as tacrolimus encephalopathy, typically in the subcortical white matter.Key words; Cyclosporin neurotoxicity; Epstein-Barr virus associated-Hemophagocytic syndrome; Magnetic Resonance Image (MRI).
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2/16. Hemophagocytic syndrome after living-donor liver transplantation for fulminant liver failure: a case report.

    Hemophagocytic syndrome (HPS) is a hypercytokinemia caused by activated T lymphocytes and macrophages in immunologically compromised patients. We report a 37-year-old female who was diagnosed with HPS after undergoing living-donor liver transplantation (LDLT) for fulminant liver failure of unknown etiology. After liver transplantation, recipients with pancytopenia should be tested for serum ferritin. When the serum ferritin is abnormal, the bone marrow should be biopsied to screen for HPS as soon as possible. If the condition is caught early and promptly treated, the outcome of this devastating condition might be improved. In addition, HPS should be ruled out in LDLT candidates with acute liver failure before their operations.
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3/16. Non-fatal haemophagocytic syndrome in an elderly patient.

    A frail 78-year-old man presented with lethargy, fever, splenomegaly and pancytopenia. Bone marrow aspirate showed marked haemophagocytosis. A diagnosis of haemophagocytic syndrome secondary to diffuse splenic large B-cell lymphoma was eventually made. Treatment with laparascopic splenectomy was successful. Secondary haemophagocytic syndrome is a rare complication of many common conditions, and is fatal if untreated. A brief literature review is included.
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4/16. Haemophagocytic syndrome associated with hepatitis-B virus infection responding to etoposide.

    Haemophagocytic syndrome (HPS) secondary to viral infections usually has a variable course and can be life-threatening. We report a 53-year-old male patient who presented with fever, hepatosplenomegaly and pancytopenia. He had deranged liver function, abnormal clotting and markedly elevated serum ferritin. Bone marrow biopsy showed prominent haemophagocytosis. The patient was investigated thoroughly and found to have evidence of chronic hepatitis b-virus (HBV) infection by serological tests and liver biopsy. Other conditions associated with HPS such as lymphoma, malignancy and other viral or bacterial infections were not present. The patient did not respond to steroids, intravenous immunoglobulins or cyclosporin but responded to etoposide and became apyrexial. He also became HBV negative on lamivudine. The patient died of infection later on but there was no evidence of recurrence of HPS. To the best of our knowledge this is the first case report of HPS associated with isolated HBV infection.
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5/16. Aggressive NK-cell leukaemia associated with reactive haemophagocytic syndrome.

    We report a case of aggressive NK-cell leukaemia associated with reactive haemophagocytic syndrome in a 29-year-old Korean woman who had several small purpuric patches on both thighs. She also had high fever. Laboratory tests revealed pancytopenia and deranged liver function, and atypical lymphocytes containing toxic granules were detected from peripheral blood and bone marrow. The bone marrow examination showed diffuse histiocytic proliferation with several haemophagocytic macrophages, suggesting an associated reactive haemophagocytic syndrome. skin biopsy from her thigh lesion demonstrated atypical CD56 lymphoid cellular infiltrates with angiocentric pattern, and in situ hybridization test for Epstein-Barr virus was positive. Although we treated her with chemotherapy, she died 1 month later.
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6/16. toxoplasmosis-associated hemophagocytic syndrome in renal transplantation.

    toxoplasmosis is an infrequent, often difficult to diagnose and potentially lethal disease in kidney transplant recipients. Among reported cases, a few were associated with hemophagocytic syndrome (HPS), a rare condition characterized by widespread proliferation of macrophages phagocytizing blood elements, accompanied by fever and pancytopenia. We report here the case of a patient who received a toxoplasma gondii positive kidney allograft and developed invasive toxoplasmosis 10 days after surgery, with high fever, skin rash, arthralgias, and renal failure, followed by pneumonia, anemia, thrombocytopenia, liver dysfunction, and encephalitis. Mislead by the absence of toxoplasma on blood smears, alveolar fluid, renal graft biopsy, and negative brain computed tomography, confusion with serum sickness, and simultaneous herpetic infection, we failed to make the right diagnosis and the patient died with septic shock 11 days later. An HPS was revealed by a late bone marrow analysis. This may well be the fourth case ever reported of toxoplasmosis-associated HPS in renal transplant recipients.
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7/16. Success with infliximab in treating refractory hemophagocytic lymphohistiocytosis.

    Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder characterized by fever, pancytopenia, hepatosplenomegaly, liver dysfunction, and hemophagocytosis. A 29-year-old woman, diagnosed with systemic lupus erythematosus in 1996, developed HLH in early June 2002. HLH remained refractory during 1.5 months of treatment including corticosteroid, cyclosporine, plasma exchange, vincristine, and etoposide. Infliximab (5 mg/kg/day) was then administered twice. After the second administration, the patient attained remission. Because HLH itself is not a neoplasm but an uncontrolled immune reaction, blocking cytokines involved in the reaction should have therapeutic potentials. For HLH patients not responding to conventional therapy, anticytokine treatment with infliximab may represent one of promising options.
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8/16. Disseminated hyperkeratotic and granulomatous nodules in a child with fatal Epstein-Barr-virus-associated hemophagocytic lymphohistiocytosis.

    Hemophagocytic lymphohistiocytosis is a rare and potentially fatal syndrome associated with a variety of genetic, malignant, autoimmune, or infectious conditions. The importance of cutaneous presentations of this syndrome has only recently been brought forward. We report the first case of Epstein-Barr-virus-associated hemophagocytic lymphohistiocytosis presenting with papulonodular and granulomatous skin lesions. A girl of African origin developed several umbilicated papules on her extremities and face at the age of 18 months. She was born in germany, had never visited africa, and was otherwise healthy. Over the next 5 months the lesions progressed in size and number and became hyperkeratotic. Histopathologic analysis of early lesions revealed a superficial lympho- and plasmacellular dermatitis with some features of panniculitis. Later biopsy specimens from nodular lesions showed the formation of tuberculoid granulomas in the deep dermis. At the age of 23 months she became severely ill, rapidly developing high fever, hepatosplenomegaly, icterus, pancytopenia, and ascites. On the basis of bone marrow and lymph node biopsies, the diagnosis of hemophagocytic lymphohistiocytosis was established. However, this phenomenon could not be detected in any of the skin specimens. An active Epstein-Barr virus infection was diagnosed by polymerase chain reaction in blood, lymphoid tissue, and skin. Despite chemotherapy with etoposide and cortisone, the girl expired 14 days after clinical onset of her systemic disease.
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9/16. Subcutaneous panniculitis-like T-cell lymphoma: successful initial treatment with prednisolone and cyclosporin A.

    A case of subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is reported. A 27-year-old man presented with fever and abdominal swelling. His laboratory examination revealed pancytopenia and liver dysfunction. The diagnosis of SPTCL was made by biopsy based on thickened subcutaneous tissue. In addition, bone marrow specimen showed a hemophagocytosis syndrome (HPS). methylprednisolone pulse therapy was initiated followed by prednisolone (60 mg/day) and cyclosporin A (150 mg/day). He responded to the treatment and remained asymptomatic for at least for 6 months. Our results suggest that a trial of cyclosporin A is warranted in patients with SPTCL complicated by HPS.
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10/16. Secondary hemophagocytic syndrome in a patient with methicillin-sensitive staphylococcus aureus bacteremia due to severe decubitus ulcer.

    A 51-year-old man with poliomyelitis was admitted to emergency because of a severe decubitus ulcer on his right hip that was associated with infection. His general condition deteriorated and he was malnourished and dehydrated. Despite adequate hyperalimentation and antibiotic administration, laboratory data indicated pancytopenia 4 days later. He was diagnosed as having secondary hemophagocytosis (HPS) associated with methicillin-sensitive staphylococcus aureus sepsis due to decubitus inflammation based on bone marrow aspiration and a blood culture. Although granulocyte colony stimulating factor, packed red blood cell transfusions, platelet transfusions, and antibiotics gradually improved the pancytopenia, the patient died of massive gastrointestinal tract bleeding.
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