CT and MR findings in familial erythrophagocytic lymphohistiocytosis.

Huddle, Daniel C.; Rosenblum, Jordan D.; Diamond, C K

doi:10.2214/ajr.162.6.8192058

Cited by 8 publications

(10 citation statements)

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“…The major two radiologic findings are: 1) diffuse leptomeningeal enhancement with or without subdural fluid collection, and 2) focal or diffuse T2 hyperintensity with or without parenchymal volume loss of the white matter that usually begins in the peritrigonal regions 2,6-10. These findings are well-correlated with the neuropathologic findings, which have been known to be: 1) diffuse infiltration of the leptomeninges by lymphocytes and histiocytes, 2) dense perivascular lymphohistiocytic infiltration in all brain regions, and 3) demyelination of the white matter with reactive gliosis, which is predominantly observed in the cerebellum 2,7,8,12,14…”

Section: Discussionsupporting

confidence: 57%

MR Findings of Fulminent Leukoencephalopathy in EBV-Associated Hemophagocytic Syndrome

et al. 2006

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Various manifestations of brain involvement for patients with virus-associated hemophagocytic syndrome have been reported. Here, we report on the sequential magnetic resonance (MR) findings of acute demyelination of the entire brain with subsequent brain atrophy in a follow-up study of a 25-month-old boy who was admitted with fever and then diagnosed with infectious mononucleosis and EBV-associated hemophagocytic syndrome. We also review other conditions that should be included in the differential diagnosis of this disease.

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Section: Discussionsupporting

confidence: 57%

MR Findings of Fulminent Leukoencephalopathy in EBV-Associated Hemophagocytic Syndrome

et al. 2006

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“…MR findings show a good correlation with areas of parenchymal and meningeal lymphohistiocytic infiltration 5 , demonstrating diffuse white matter abnormalities in the early stages and necrotic areas with parenchymal volume loss as terminal findings 4 . In addition, MR allows the follow-up after chemotherapy and bone marrow transplantation 2, 3 .…”

Section: Summary -Erithrophagocytic Lymphohistiocytosis Is a Rare Dismentioning

confidence: 88%

“…L'esito fatale è precoce, con una media di sopravvivenza dal momento della diagnosi di circa 6 mesi a causa di fenomeni emorragici, insufficienza epatica, problemi neurologici od infezioni intercorrenti; il tasso di sopravvivenza a 4 mesi è del 10% 5 .…”

Section: Discussioneunclassified

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Linfoistiocitosi eritrofagocitica

Caranci

D’Amico²,

Briganti³

et al. 2003

Rivista di Neuroradiologia

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Erithrophagocytic lymphohistiocytosis is a rare disease characterized by exaggerated histiocytic proliferation and activation 12 and multisystem involvement including visceral organs, lymph nodes, bone marrow and central nervous system 4 .Magnetic Resonance (MR) examination was performed in 10 patients with previously diagnosed Erithrophagocytic lymphohistiocytosis. This study was aimed at assessing MR accuracy in the identification and characterization of central nervous system lesions.MR findings show a good correlation with areas of parenchymal and meningeal lymphohistiocytic infiltration 5 , demonstrating diffuse white matter abnormalities in the early stages and necrotic areas with parenchymal volume loss as terminal findings 4 . In addition, MR allows the follow-up after chemotherapy and bone marrow transplantation 2,3 .Although rare, EL should be differentiated from other pediatric patchy white matter abnormalities 5 .Rare sono in letteratura le descrizioni di casi studiati con metodiche per immagini, soprattutto a causa del breve periodo di sopravvivenza dopo l'esordio sintomatologico 6 .Vengono descritti 10 casi di osservazione personale, allo scopo di valutare il ruolo della Risonanza Magnetica (RM) nella definizione dell'entità dell'interessamento cerebrale e nel follow-up dei pazienti dopo trattamento chemioterapico o trapianto di midollo osseo.

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“…Diffuse, nonspecific brain atrophy and low attenuated parenchymal lesions have been revealed by brain CT (2,5), while MRI has revealed low signal intensity on T1-weighted images and high signal intensity on T2-weighted images of the gray and white matter of the cerebrum and cerebellum, including both basal ganglia and thalami (3,5,6). Pathologically, white matter lesions correspond to demyelination and gliosis, and begin in the peritrigonal area, progressing, in severe cases, to the entire cerebral and cerebellar white matter (2 5).…”

Section: Discussionmentioning

confidence: 99%

Serial Brain MRI Findings in CNS Involvement of Familial Erythrophagocytic Lymphohistiocytosis: A Case Report

Cho

Yoo

Suh

et al. 2002

J Korean Radiol Soc

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Familial erythrophagocytic lymphohistiocytosis (FEL) is a rare and fatal hereditary disorder of unknown cause with multisystem involvement. Involvement of the central nervous system (CNS) is reported in approximately 30% of cases. Leptomeningitis, gliosis, demyelination, and lymphocyte and histiocyte infiltration of the cerebrum and cerebellum are the characteristic neuropathologic findings. We describe the serial brain MRI findings and neurologic symptoms in a case of FEL, and review the literature. Case ReportA 16-month-old male was transferred to our hospital due to dyspnea, tachypnea, fever, and aggravated abdominal distention, present for one week. He had previously been admitted to another hospital, with similar symptoms, five months earlier. His first and third older female siblings died at two months and two years of age, respectively of fever of unknown origin and hepatosplenomegaly. The second male sibling, eight years old, is currently alive.Physical examination revealed hepatosplenomegaly, shifting dullness, anemic conjunctiva, and jaundice, and hematological study showed pancytopenia, hypofibrinogenemia, and hyperferritinemia : Hb, 6.1 g/dl; WBC, Familial erythrophagocytic lymphohistiocytosis is a fatal early childhood disorder characterized by multiorgan lymphohistiocytic infiltration and active hemophagocytosis. Involvement of the central nervous system (CNS) is not uncommon and is characterized by rapidly progressive tissue damage affecting both the gray and white matter. We encountered a case of familial erythrophagocytic lymphohistiocytosis with CNS involvement. Initial T2-weighted MRI of the brain demonstrated high signal intensity in the right thalamus, though after chemotherapy, which led to the relief of neurologic symptoms, this disappeared. After four months, however, the patient s neurologic symptoms recurred, and follow-up T2-weighted MR images showed high signal intensity in the thalami, basal ganglia, and cerebral and cerebellar white matter.Brain MRI is a useful imaging modality for the evaluation of CNS involvement and monitoring the response to treatment.

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CT and MR findings in familial erythrophagocytic lymphohistiocytosis.

Cited by 8 publications

References 0 publications

MR Findings of Fulminent Leukoencephalopathy in EBV-Associated Hemophagocytic Syndrome

MR Findings of Fulminent Leukoencephalopathy in EBV-Associated Hemophagocytic Syndrome

Linfoistiocitosi eritrofagocitica

Serial Brain MRI Findings in CNS Involvement of Familial Erythrophagocytic Lymphohistiocytosis: A Case Report

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