RE: Wilson's disease

From:Tony Henwood

The copper in PBC is probably easier to stain than that in Wilson's Disease. Interstingly not all Wilsons disease livers stain with the copper stains (see Pathologica. 90(6):771-5, 1998 Dec, abstract below). We routinely use PBC as control.

Tony Henwood JP, BAppSc, GradDipSysAnalys, CT(ASC)
Laboratory Manager
The Children's Hospital at  Westmead,
Locked Bag 4001, Westmead, 2145, AUSTRALIA.
Tel: (02) 9845 3306
Fax: (02) 9845 3318

Lecca S. Pilloni L. Ambu R. Flore C. Callea F. Faa G.(Pathologica. 90(6):771-5, 1998 Dec)
Conflicting results have been reported on the usefulness of histochemistry for copper in the diagnosis of Wilson's Disease (W.D.). In spite of the high number of methods proposed in the literature, no-one has shown to have the characteristics typical of a good histochemical method: high sensitivity associated with high specificity. On this basis, it seemed of interest to evaluate, in a large series of patients with W.D., the diagnostic value of the most commonly used histochemical methods for copper (rhodanine, orcein and Timm's method). To this end, 74 liver needle biopsies from patients affected by W.D., 39 males and 35 females, aged 4-60 years (mean age 28.5 years), were stained with rhodanine (R), orcein (O) and Timm's (T) methods. On the basis of the histological picture, liver biopsies were subdivided in four evolutive stages: stage I = steatosis; stage II = interface hepatitis; stage III =
bridging fibrosis; stage IV = cirrhosis. In stage I, histochemistry for copper was positive in 11 out of 21 cases: 6 cases were T+; 1 case R+ and 2 cases O+; 2 cases were T+, R+, O+. In stage II, 11 out of 14 cases were positive for copper staining: 4 T+, 2 R+ and 2 O+; 3 cases were contemporary positives for T, R, O. In stage III, 22 out of 25 cases resulted positive: 8 T+, 3 R+ and 1 O+; 10 cases were positives, in the mean time, for more of one method. In stage IV, 12 of 14 cases were positives: 5 T+, 2 R+, 2 O+ and 3 cases were contemporaneously positives for multiple methods. Our data show that: 1) the percentage of positivity obtained using three histochemical methods for copper is higher than using only one method. From a practical point of view, it is mandatory to utilize, in clinical practice, multiple histochemical stains in order to increase the diagnostic utility of histochemistry for copper; 2) the Timm's method appears to be the most sensitive method for the demonstration of copper in all stages of W.D.; 3) even though hepatic copper already abounds in the early stages of W.D., this pool of intrahepatic copper is not yet demonstrable with any of the three histochemical techniques utilized.
-----Original Message-----
From: []
Sent: Tuesday, 3 June 2003 12:01
Subject: Wilson's disease

Can anyone tell me if all copper stains require use of control tissue that is specifically diagnosed with Wilson's disease?  Historically, as was passed along to me....copper controls had to come from a liver with Wilson's disease.  One of our doctors recently told me any liver with chronic cholestasis (ie. Primary biliary cirrhrosis or Primary schlerosing cholangitis) is okay to use too.  I recently stained some fetal liver tissue and it looks great.  I am hoping the consensus from histoland confers that PBC and PSC liver tissue that stains great for copper is okay to use too.

Deb King, HT (ASCP)
Sacramento, CA

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