1/7. Isolated central nervous system hemophagocytic lymphohistiocytosis: case report.OBJECTIVE AND IMPORTANCE: The first case of histologically proven hemophagocytic lymphohistiocytosis (HLH) isolated to the central nervous system (CNS) is reported. HLH affecting the CNS mimics several neurological disorders and may be misdiagnosed. The diagnostic and therapeutic problems of this disease are discussed. CLINICAL PRESENTATION: We report a case of a 5-year-old girl with a 2-month history of right hemiparesis. The initial magnetic resonance imaging scan mimicked the appearance of malignant glioma or cerebral infarction. By use of neuroimaging alone, it was extremely difficult to reach an appropriate diagnosis. INTERVENTION: Pathological examination of a surgical specimen of the lesion revealed histological characteristics typical of HLH. Because of the absence of both physical and blood chemical findings of systemic HLH, the patient was diagnosed as having HLH isolated in the CNS without systemic HLH. radiotherapy with corticosteroid administration led to complete resolution of the CNS lesions, but the duration of remission was only 3 months. The patient died secondary to refractory progression of the CNS lesion. CONCLUSION: radiotherapy with corticosteroid administration led to a complete resolution of the lesions, although for only a transitory remission. Although HLH is extremely rare, the existence of this disease isolated in the CNS should be documented, and further case accumulation and therapeutic investigations are needed to clarify the pathophysiological characteristics of this disease.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
2/7. 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).- - - - - - - - - - ranking = 0.2keywords = central nervous system, nervous system (Clic here for more details about this article) |
3/7. 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.- - - - - - - - - - ranking = 0.0067610782792836keywords = brain (Clic here for more details about this article) |
4/7. Acute necrotizing encephalopathy associated with hemophagocytic syndrome.A 7-year-old female suddenly exhibited high fever and convulsions, and entered a semi-coma. She also had thrombocytopenia, elevated aminotransferase, prolonged prothrombin time and activated partial thromboplastin time, and hemophagocytes in the bone marrow. The brain magnetic resonance imaging revealed multiple low-intensity areas on the T1-weighted images, and high-intensity areas on the T2-weighted images bilaterally in the thalamus, the dorsal part of the pons, and the cerebellar white matter. The patient was diagnosed as having both acute necrotizing encephalopathy and hemophagocytic syndrome. Serum and cerebrospinal fluid interleukin-6 and tumor necrosis factor-alpha were elevated to the same high levels (serum:cerebrospinal fluid interleukin-6, 103:101 pg/mL; tumor necrosis factor-alpha 753:753 pg/mL). The clinical symptoms and the magnetic resonance imaging findings improved immediately after the administration of dexamethasone. These results suggest that the hypercytokinemia and the hyperpermeability of both the blood-brain barrier and the capillary walls of the central nervous system might be essential in the pathogenesis of acute necrotizing encephalopathy, and that early steroid therapy might be effective in these conditions.- - - - - - - - - - ranking = 0.21352215655857keywords = central nervous system, nervous system, brain (Clic here for more details about this article) |
5/7. diffusion-weighted cranial MR imaging findings in a patient with hemophagocytic syndrome.Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder of the immune system that is associated with frequent involvement of the central nervous system (CNS). The MR imaging and CT findings of the CNS infiltration have been reported in the past; however, the diffusion-weighted imaging (DWI) findings have not been previously described. We present MR imaging findings in a case of secondary HLH with CNS involvement, with an emphasis on the DWI findings.- - - - - - - - - - ranking = 0.2keywords = central nervous system, nervous system (Clic here for more details about this article) |
6/7. Successful father-to-son stem cell transplantation in a child with hemophagocytic lymphohistiocytosis using a reduced-intensity conditioning regimen.OBJECTIVE: Hemophagocytic lymphohistiocytosis (HLH) is an uncommon disorder, usually lethal without allogeneic stem cell transplantation (SCT). MATERIALS AND methods: We report a 9-month-old boy, the first child of consanguineous parents, diagnosed with HLH and neurological involvement demonstrated by magnetic resonance imaging (MRI), who received an allogeneic SCT from his HLA genetically matched father. Transplant was performed after a reduced-intensity conditioning (RIC) regimen consisting of cyclophosphamide, fludarabine, and melphalan. Graft vs. host disease (GVHD) prophylaxis included cyclosporine a and methotrexate. RESULTS: An absolute neutrophil count of 0.5 x 10(9)/L was documented on day 20 and a platelet count >20 x 10(9)/L was shown by day 33. Full donor chimerism was showed on day 175. A follow-up brain MRI was reported normal. Twenty months after SCT, the child shows no evidence of HLH or GVHD activity, and has a normal psychomotor development. CONCLUSION: Given the reduced toxicity of SCT with RIC, it could represent an attractive transplant method for children with HLH, in whom myeloablation plays no role in disease eradication, and in whom mixed chimerism may be enough to cure the disease.- - - - - - - - - - ranking = 0.0067610782792836keywords = brain (Clic here for more details about this article) |
7/7. Three patients with hemophagocytic syndrome who developed acute organic brain syndrome.INTRODUCTION: We describe three patients with hemophagocytic syndrome (HPS) who developed acute organic brain syndrome. All three presented with high-grade fever and twilight state, and were admitted to our hospital. After admission, delirium developed in all three. As delirium improved, various other psychiatric symptoms, including hallucinations, agitation, hypoactivity, affective lability and insomnia, were noted. DISCUSSION: When treated with steroid hormones, immunoglobulin and neuroleptics, all patients demonstrated improvement in their psychiatric symptoms, as well as in their general condition and laboratory findings. Ultimately, they all recovered and were discharged. CONCLUSION: It needs to be noted that organic brain syndrome might be observed at the onset of HPS. Consequently, early diagnosis and treatment for psychiatric symptoms, as well as for HPS, are crucial.- - - - - - - - - - ranking = 0.040566469675702keywords = brain (Clic here for more details about this article) |