Haemophagocytosis in hereditary fructose intolerance: a diagnostic dilemma

Mandel, Hanna; Gozal, David; Aizin, A.; Tavori, S.; Jaffe, Michael

doi:10.1007/bf01799369

Cited by 6 publications

(4 citation statements)

References 6 publications

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“…A clinical picture resembling HLH has also been described for several inborn metabolic diseases (Mandel et al , 1990; Ikeda et al , 1998; Duval et al , 1999).…”

Section: Hlh As Genetic Diseasementioning

confidence: 79%

Modern management of children with haemophagocytic lymphohistiocytosis

Janka¹,

Schneider²

2003

Br J Haematol

212

152

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“…A clinical picture resembling HLH has also been described for several inborn metabolic diseases (Mandel et al , 1990; Ikeda et al , 1998; Duval et al , 1999).…”

Section: Hlh As Genetic Diseasementioning

confidence: 79%

Modern management of children with haemophagocytic lymphohistiocytosis

Janka¹,

Schneider²

2003

Br J Haematol

212

152

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“…No metabolic disease-related HLH conditions such as lysinuric protein intolerance, hereditary fructose intolerance, neonatal haemochromatosis, or multiple sulphatase deficiency [6,14,24,26] were identified. Neither Chediak-Higashi syndrome nor Griscelli syndrome was diagnosed.…”

Section: Resultsmentioning

confidence: 99%

Occurrence of haemophagocytic lymphohistiocytosis at less than 1 year of age: analysis of 96 patients

et al. 2005

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“…Such a condition does not occur as a primary event. Potential precipitants of acute haemophagocytic syndrome include viral infection,29-34 bacterial or fungal infection,35-38 metabolic disturbance,39inflammatory arthropathy,40 and pregnancy41in addition to leukaemias and lymphoproliferative disease 42-44. The possibility of lymphoma as a cause of the acute haemophagocytic syndrome in the two patients without a clear precipitant aetiology seems very unlikely, since investigation during life and postmortem examination failed to provide evidence of such disease.…”

Section: Discussionmentioning

confidence: 99%

Acute liver failure secondary to hepatic infiltration: a single centre experience of 18 cases

Rowbotham

Wendon

Williams

1998

Gut

200

155

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Background-Acute liver failure (ALF) secondary to malignant infiltration of the liver is rare and is diagnosed often only after death. Aims-To determine diagnostic factors and particular clinical patterns of illness. Methods-Review of case notes from all patients with ALF secondary to hepatic infiltration admitted to this unit over an 18 year period (1978)(1979)(1980)(1981)(1982)(1983)(1984)(1985)(1986)(1987)(1988)(1989)(1990)(1991)(1992)(1993)(1994)(1995). Results-From a total of 4020 admissions, 18 patients were identified with ALF attributable to hepatic infiltration. Mean age was 40.7 years. Aetiology was nonHodgkin's lymphoma in nine patients, Hodgkin's disease in three, infiltrative metastatic carcinoma in four, and haemophagocytosis with no precipitant cause in two cases. Prodromal symptoms were non-specific, but occurred at least two to four weeks before onset of ALF, making the presence of such symptoms of value in diVerential diagnosis of the cause of ALF. Clinical examination and investigations were unhelpful in distinguishing these cases from more usual causes of ALF. Usually, the clinical course was of rapid deterioration and death from multiorgan failure, and only one patient survived. Diagnosis was made during life in 15 patients. Histology showed evidence of widespread hepatocellular necrosis, with diVuse infiltration by tumour cells rather than focal cellular aggregation. Conclusions-Only with accurate histological diagnosis from liver biopsy and institution of specific therapy early in the management of such patients will the best chance of recovery be achieved. In every case of ALF with prodromal symptoms or abnormal imaging, hepatic histology should be obtained by liver biopsy as soon as possible to diagnose infiltrative hepatic disease. (Gut 1998;42:576-580)

show abstract

Haemophagocytosis in hereditary fructose intolerance: a diagnostic dilemma

Cited by 6 publications

References 6 publications

Modern management of children with haemophagocytic lymphohistiocytosis

Modern management of children with haemophagocytic lymphohistiocytosis

Occurrence of haemophagocytic lymphohistiocytosis at less than 1 year of age: analysis of 96 patients

Acute liver failure secondary to hepatic infiltration: a single centre experience of 18 cases

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