A Rare Case of Neonatal Systemic Xanthogranulomatosis With Severe Hepatic Disease and Metachronous Skin Involvement

Papadakis, Vassilios; Volonaki, Eleni; Katsibardi, Κaterina; Stefanaki, Kalliopi; Valari, Manthoula; Anagnostakou, Marina; Polychronopoulou, Sophia

doi:10.1097/mph.0b013e3182203086

Cited by 21 publications

(23 citation statements)

References 12 publications

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“…This is due to the possibility of spontaneous remission, uncertain efficacy and high risk of toxic side‐effects in the usually very young patients . However, there is growing evidence to support the use of chemotherapeutics in the treatment of symptomatic multisystemic JXG with vital organ involvement …”

Section: Discussionsupporting

confidence: 79%

“…jaundice and ascites in liver dysfunction, seizures and neurological deficits in CNS affection) and range from an asymptomatic to a fatal course . Particularly, progressive CNS and liver disease were associated with significant morbidity and mortality …”

Section: Discussionmentioning

confidence: 99%

“…Based on the report of three other patients with systemic JXG with involvement of the skin and liver, and also one case involving the CNS with failure to respond to vinblastine and prednisone, therapy was changed to cytarabine and 2CDA, In line with the previous reports, this treatment was successful, supporting cytarabine and 2CDA as a therapeutic alternative in severe cases of systemic JXG . However, due to the limited number of cases reported, no treatment guidelines have been established yet and individualized management is required in this rare and multifaceted disease …”

Section: Discussionmentioning

confidence: 99%

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Successful treatment of systemic juvenile xanthogranulomatosis with cytarabine and 2‐chlorodeoxyadenosine: case report and review of the literature

et al. 2016

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The non-Langerhans cell histiocytosis (LCH) juvenile xanthogranulomatosis (JXG) is usually a benign disease limited to the skin. Only a few cases of systemic disease with at least two affected organs and lethal outcomes have been reported to date. Treatment is controversial and no standard protocol is available. We report the rare case of a 22-month-old boy presenting multiple erythematous brownish papules of the head, trunk and legs, which had developed starting from his 6th month of life. Additional symptoms were delayed psychomotor development, hydrocephalus and hepatosplenomegaly. Further diagnostics revealed a systemic JXG with involvement of the skin, central nervous system, liver and spleen. The patient did not respond to initial therapy with prednisone and vinblastine according to protocol III for LCH. However, further therapy with cytarabine and 2-chlorodeoxyadenosine followed by a consolidation phase with 2-chlorodeoxyadenosine alone was successful and the patient is in his 4th year of remission. We provide a comprehensive review of the reported cases of systemic JXG to date.

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Section: Discussionsupporting

confidence: 79%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Successful treatment of systemic juvenile xanthogranulomatosis with cytarabine and 2‐chlorodeoxyadenosine: case report and review of the literature

et al. 2016

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“…This immunophenotype may facilitate the differential diagnosis from Langerhans cell histiocytosis and Rosai–Dorfman disease (described below). In systemic JXG, survival rates are 58–75%, with significant morbidity and mortality related to liver involvement …”

Section: Infiltrative Lesions Of the Sinusoidsmentioning

confidence: 99%

Pathology of the liver sinusoids

et al. 2014

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The hepatic sinusoids comprise a complex of vascular conduits to transport blood from the porta hepatis to the inferior vena cava through the liver. Under normal conditions, portal venous and hepatic artery pressures are equalized within the sinusoids, oxygen and nutrients from the systemic circulation are delivered to the parenchymal cells and differentially distributed throughout the liver acini, and proteins of liver derivation are carried into the cardiac/systemic circulation. Liver sinusoid structures are lined by endothelial cells unique to their location, and Kupffer cells. Multifunctional hepatic stellate cells and various immune active cells are localized within the space of Disse between the sinusoid and the adjacent hepatocytes. Flow within the sinusoids can be compromised by physical or pressure blockage in their lumina as well as obstructive processes within the space of Disse. The intimate relationship of the liver sinusoids to neighbouring hepatocytes is a significant factor affecting the health of hepatocytes, or transmission of the effects of injury within the sinusoidal space. Pathologists should recognize several patterns of injury involving the sinusoids and surrounding hepatocytes. In this review, injury, alterations and accumulations within the liver sinusoids are illustrated and discussed.

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“…After radiation one patient had progression of his CNS JXG, while the other only had stabilization of his disease [17]. Careful continous surveillance for skin infiltrates is important, as metachronous skin involvement is possible even under treatment [18]. …”

mentioning

confidence: 99%

Use of Radiation in Treatment of Central Nervous System Juvenile Xanthogranulomatosis

Vijapura¹,

Fulbright²

2012

Pediatric Hematology and Oncology

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Juvenile xanthogranulomatosis (JXG) represents a subset of non-Langerhan cell histiocytosis that typically manifests in younger children with skin lesions. Unresectable central nervous system (CNS) disease is difficult to treat. We describe the case of a 13-year-old successfully treated with adjuvant radiation therapy for symptomatic intracranial and leptomeningeal JXG. An extensive literature review was performed to identify all previous CNS JXG cases utilizing radiation, of which six of eight total patients demonstrated temporary or long-term improvement of neurologic disease. This suggests that radiation should be considered in cases unresponsive to conventional treatment options.

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A Rare Case of Neonatal Systemic Xanthogranulomatosis With Severe Hepatic Disease and Metachronous Skin Involvement

Cited by 21 publications

References 12 publications

Successful treatment of systemic juvenile xanthogranulomatosis with cytarabine and 2‐chlorodeoxyadenosine: case report and review of the literature

Successful treatment of systemic juvenile xanthogranulomatosis with cytarabine and 2‐chlorodeoxyadenosine: case report and review of the literature

Pathology of the liver sinusoids

Use of Radiation in Treatment of Central Nervous System Juvenile Xanthogranulomatosis

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