Haemophilia and the mysterious spleen: the story of a hate–love relationship

Ingram, John R.

doi:10.1046/j.1365-2516.1998.00152.x

Cited by 2 publications

(4 citation statements)

References 46 publications

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“…Obviously the contribution of the liver to FVIII synthesis can be adequately compensated by other tissues in FHF, at least in our porcine model of acute hepatic failure. Immunohistochemical analysis of pre‐ and postsurgical tissue confirmed previous studies that the spleen is a potential FVIII source [29,30]. This tissue displayed intense staining for FVIII, notably following hepatectomy.…”

Section: Discussionsupporting

confidence: 80%

“…2), probably by the kidney. It is likely that a substantial part of the endogenous FVIII present at the time of hepatectomy had also been cleared after 24 h. As at this time point FVIII levels were clearly elevated, this indicates that the increase in FVIII was due to extra‐hepatic de novo synthesis and/or release from storage pools (reviewed in [30,32]). Also the increased levels of plasma VWF (Fig.…”

Section: Discussionmentioning

confidence: 99%

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Extra-hepatic factor VIII expression in porcine fulminant hepatic failure

Hollestelle

Poyck

Hollestelle

et al. 2005

Journal of Thrombosis and Haemostasis

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To cite this article: Hollestelle MJ, Poyck PPC, Hollestelle JM, Marsman HA, van Mourik JA, van Gulik TM. Extra-hepatic factor VIII expression in porcine fulminant hepatic failure. J Thromb Haemost 2005; 3: 2274-80.Summary. In humans, fulminant hepatic failure (FHF) is frequently associated with increased factor VIII (FVIII) levels, despite widespread liver cell death. The mechanisms leading to increased FVIII levels and cellular sites of this enhanced FVIII production are poorly understood. We studied the effect of total hepatectomy in pigs, a large-animal model of FHF, on the expression of plasma and tissue FVIII during 24-hour follow-up. Tissue FVIII expression was determined before and 24 h after hepatectomy, both at the mRNA level and immunohistochemically. The expression of plasma and tissue von Willebrand factor (VWF), the natural stabilizing carrier protein of FVIII, was also measured. Total hepatectomy elicited a gradual and sustained twofold elevation of circulating FVIII, whereas FVIII mRNA levels in various organs did not increase after hepatectomy. The half-life of FVIII increased from 7.7 to 10.3 h and VWF levels were also elevated in anhepatic pigs. The increase in the half-life of FVIII and increased levels of VWF were not sufficient to explain the rise in plasma FVIII levels. At the protein level, prominent changes in the cellular distribution of FVIII were seen in spleen and kidney. These observations suggest that in this model of FHF the lack of hepatic FVIII synthesis is adequately compensated by other organs, notably spleen and kidneys.

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Section: Discussionsupporting

confidence: 80%

Section: Discussionmentioning

confidence: 99%

Extra-hepatic factor VIII expression in porcine fulminant hepatic failure

Hollestelle

Poyck

Hollestelle

et al. 2005

Journal of Thrombosis and Haemostasis

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“…Between 1950 and 1971, ®ve authors reported cases of haemophiliacs and two reported FIX cases where splenectomy had previously been carried out for other reasons, and follow-up showed no clear bene®t to the bleeding disorder [20]. Seven cases were described between 1939 and 1957, but the results were at best equivocal.…”

mentioning

confidence: 99%

“…Seven cases were described between 1939 and 1957, but the results were at best equivocal. Between 1950 and 1971, ®ve authors reported cases of haemophiliacs and two reported FIX cases where splenectomy had previously been carried out for other reasons, and follow-up showed no clear bene®t to the bleeding disorder [20]. For splenic haemorrhage 1961±65 VIII Blood, eACA, factor VIII concentrates Not stated 3/0/1 *Factor defect not de®ned, but good evidence of haemophilia-type defect; VIII, factor VIII defect 0±30% in different cases; àone case with ®brinogen Zurich, platelets 96´10 9 L )1 , leucocytes 1.5±3.0´10 9 L )1 ; eACA (epsilon amino caproic acid).…”

mentioning

confidence: 99%

Haemophilia and the forbidden abdomen

Ingram¹

2000

Haemophilia

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Abdominal surgery became routinely possible over a hundred years ago, after the introduction of general anaesthesia and sterile procedures. Abdominal surgery for haemophiliacs had to wait another 60 or 70 years for adequate control of haemostasis. This paper traces its gradual achievement from the 1920s to the 1970s through a series of reports of appendectomies, gastric and intestinal operations, gall bladder operations and splenectomies in patients with haemophilia of varying degrees of severity. A short-lived flurry of interest in splenectomy as a proposed treatment for haemophilia is also mentioned.

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Haemophilia and the mysterious spleen: the story of a hate–love relationship

Cited by 2 publications

References 46 publications

Extra-hepatic factor VIII expression in porcine fulminant hepatic failure

Extra-hepatic factor VIII expression in porcine fulminant hepatic failure

Haemophilia and the forbidden abdomen

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