Wednesday, June 4, 2014

President's Message May 2014

Dear Friends and Colleagues,

It is with great pleasure and honor that I assumed the position of President of the Hans Popper Hepatopathology Society (HPHS) during the United States and Canadian Academy of Pathology (USCAP) Annual Meeting this past March. I took over this position from Matthew Yeh who remains a member of HPHS Executive Committee. We thank Matthew for having ably led the HPHS for the last two years and particularly for having organized two excellent companion meetings at the USCAP. The attendance during these sessions was among the highest in the society’s history. Recruitment of new members also increased during Matthew’s stint as president.

Beth Brunt has stepped down from her position of Secretary-Treasurer, a position she has adeptly held for many years. Beth was instrumental in increasing the society membership and was key in keeping our society in an extremely sound financial state. I would like to express my sincere thanks not only for her past accomplishments and dedication but also for her continued support of the society. Grace Kim has now stepped into Beth’s shoes as our Secretary-Treasurer. She has enthusiastically taken on this challenging task from the get go. Lastly, Michael Torbenson has been elected as Vice President during the USCAP meeting. He will assume the position of HPHS president in 2016.

I expect that 2014 will be a busy one for our society. The HPHS will be participating in the International Academy of Pathology 2014 Congress to be held in Bangkok on October 5-10, 2014. The society was invited through the USCAP’s Bruce Smoller with the help of Beth Brunt and Matthew Yeh. The Scientific Committee of the IAP has requested the HPHS to conduct a two and a half hour seminar on liver pathology on Thursday, October 9.  

The Executive Committee is in the process of applying for a Non-Profit Organization status for the HPHS. Subsequent to this, we will be able to apply for a 5013C tax-exempt status with the IRS. This will enable the members to claim the annual HPHS dues as a tax deduction.

The website is up and running, courtesy of the Newsletter Committee. Details as to how to access the website are found in this newsletter. Cases that are interesting, challenging and have educational value will be regularly posted. The Education Committee, chaired by Oyedele Adeyi, is actively recruiting and encouraging submission of such cases. In addition, applications for membership will be made available in the website. We are working on having the ability to pay member dues via PayPal as well, and for payment to go directly into a secure web server that can be accessed from the website.

The number of liver specimens we all see has steadily declined because fewer biopsies are being performed by clinicians for hepatitis C staging. This is primarily due to the newly approved medications for hepatitis C wherein treatment can be initiated regardless of the stage of fibrosis. However, the role of liver pathology has shifted to evaluating biopsies from patients with metabolic liver diseases and drug-induced liver injury, and other more challenging cases. Additionally, molecular diagnostic modalities are being incorporated into the work up of liver tumors. We will try to continue to disseminate advances in the diagnosis of such entities via the website, newsletter and future companion meetings. The Executive Committee is also planning to propose an increased involvement and greater visibility of liver pathology in the American Association for the Study of Liver Diseases (AASLD). In the next few months, we will reach out to the AASLD Governing Board and the Education Committee to add a new liver pathology session during its Annual Meeting. When these meetings  were held in previous years they were well-attended not just by pathologists but also by clinicians. Having a liver pathology session at national meetings such as the AASLD will increase awareness and further promote liver pathology.

Lastly, I encourage you to advocate for greater membership and foster education and interest amongst your junior hepatopathology colleagues.

Thank you for your continued support of the HPHS. I look forward to seeing you at the Companion Meeting during the USCAP Annual Meeting in Boston on March 22, 2015.


M. Isabel Fiel, M.D.
Hans Popper Hepatopathology Society

Tuesday, June 3, 2014

Interesting Case May 2014

Clinical History 
This 23 year old woman is status post allogeneic BMT for Fanconi anemia 15 years prior. She was generally doing well, but was lost to follow up at our institution since 5 years ago. She presented to an outside pediatrician with complaint of fatigue, malaise, abdominal pain and pruritus for the past couple of months.

At the time of presentation her alkaline phosphatase >700 U/L, AST 100-200 U/L, ALT 200-300 U/L and total bilirubin 18-20 mg/dL.

Microscopic Images
Figure 1

Figure 2 

Figure 3

Figure 4

Figure 5

Figure 6
Chronic graft versus host disease

Figure 1 shows a large portal area with several vascular profiles and lack of interlobular bile duct. Figure 2 shows the only bile duct identified on multiple levels. This duct shows features of injury including loss of nuclear polarity and nuclear hyperchromasia.
Figure 3 shows marked canalicular cholestasis.
Figure 4 is the keratin 19 stain confirming a lack of interlobular bile duct and essentially no ductular reaction.
Figures 5 and 6 show portal-portal bridging fibrosis at least.

The paucity of interlobular bile ducts and lack of ductular reaction raises several possibilities. The top of the list in this setting would be chronic graft versus host disease (GVHD). However, advanced fibrosis with chronic GVHD is rarely described. From the available clinical records, this patient has a history of chronic skin and liver GVHD that has responded well to immunosuppression in the past. Furthermore, the patient intermittently electively takes herself off immunosuppressive therapy with resultant flares, as happened in this case.

The differential diagnosis would also include drug/medication induced “vanishing bile duct syndrome”. The lack of a history of drug induced cholestatic hepatitis, the presence of what appears to be well documented chronic cutaneous GVHD, along with prompt symptomatic and laboratory improvement with reestablishment of immunosuppression support chronic liver GVHD. In this age group, the differential diagnosis would also include primary sclerosing cholangitis. Although no cholangiogram was performed, the patient underwent abdominal MRI with and without contrast which showed no evidence of intra- or extrahepatic biliary ductal dilation and she has no history of inflammatory bowel disease.

As the outcomes for BMT patients continue to improve and the duration of patient survival is extended, perhaps we will see more cases of chronic GVHD with advanced fibrosis.

Contributed by
Cynthia D. Guy, MD