Friday, March 27, 2015

Congratulations to Winners of 2015 HPHS Trainee Award!

Winner of The 2015 HPHS Best Abstract
Dr. Emily Waterhouse & Dr. David Solomon (UCSF,  faculty sponsor. Linda Ferrell):
Validation of an RT-PCR assay for detection of recurrent DNAJB1-PRKACA fusion transcripts in fibrolamellar hepatocellular carcinoma

Drs. Fiel, Waterhouse, Solomon and Adeyi


First Runner-up Abstract
Dr.  Christos Tsokos,  UCSF (faculty sponsor Gregor Krings):
Telomerase Reverse Transcriptase Promoter Mutations (mTERTp) in Combined Hepatocellular(HCC)-Cholangiocarcinoma(CC; cHCC-CC) Support Clonal and HCC-like Origin for Both Components

Drs. Fiel, Tsokos and Adeyi


Second Runner-up Abstract
Dr. Dana Balitzer,  UCSF (Faculty sponsor -  Sanjay Kakar):
Autoimmune Hepatitis: Review of Validity of Histologic Features Included in the Simplified Criteria Proposed by the International Autoimmune Hepatitis Group (IAIHG)

Drs. Fiel, Balitzer and Adeyi

Monday, March 23, 2015

Hans Popper Hepatopathology Society Companion Meeting 2015 Handouts

Hans Popper Hepatopathology Society
Sunday, March 22, 2015 - 8:30am to 12:00pm
Hynes Convention Center Ballroom B
8:30 AMCompanion Meeting Moderator- M. Isabel Fiel, MD, Mount Sinai Medical Center, New York, NY Maria Isabel Fiel, MD
8:30 AMLiver Pathology and the Clinician in 2015: At the Crossroads Thomas D. Schiano, MD
8:30 AMPractical Issues and Diagnostic Challenges in Liver Pathology Maria Isabel Fiel, MD
8:50 AMDifferential Diagnosis of Fatty Liver Disease: Not the Usual Culprits Elizabeth M. Brunt, MD
9:20 AMNoncirrhotic Portal Hypertension and Pathology of the Sinusoids Ian R. Wanless, MD
9:50 AMQuestion and Answer Session Maria Isabel Fiel, MD
10:00 AMCoffee Break Maria Isabel Fiel, MD
10:30 AMUpdate on Cholangiocarcinoma and Mixed Hepatocellular-Cholangiocarcinoma Emma E. Furth, MD
11:00 AMBiomedical Informatics 101 Arief Suriawinata, MD
11:20 AMSpecial stains, Immunohistochemistry and Genomic Testing of the Liver: A Guide for the Practicing Pathologist Michael S. Torbenson, MD
11:50 AMQuestions and Answers Maria Isabel Fiel, MD

Tuesday, March 10, 2015

President's Message March 2015

Dear Friends and Colleagues,

I would like to remind you that the HPHS Companion Society Meeting at Boston is scheduled for March 22, 2015 from 8:30am-12 noon. The title is “Diagnostic Challenges and Update in Hepatopathology”. We have created a program that will not only be very educational to our members but will hopefully be interesting to non-members as well. The topics that will be discussed include new therapeutic interventions for hepatitis and liver cancer and what this means to the pathologist, differential diagnosis of fatty liver diseases, vascular diseases and portal hypertension, the basics of biomedical informatics and its applicability in general practice, diagnosis of mixed hepatocellular-cholangiocarcinoma, and the current methods available to assist in reaching a diagnosis in challenging liver pathology cases. 

The Business meeting will immediately follow the companion meeting. Members are encouraged to attend, as we will need to vote on some modifications to the bylaws as a result of the tax-exempt status of the HPHS, as well as some changes to the duties and responsibilities of the members of the Executive Committee.

Please encourage both your junior and senior colleagues to officially become members of the HPHS. Residents and fellows can become Trainee members without having to pay any dues. They can easily transition to Regular membership once they finish their training. Application to membership is available on the website. Please email completed application to Maha Guindi (Maha.Guindi@cshs.org), chair of the membership committee.

I look forward to seeing you all in Boston.

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

Monday, March 9, 2015

Interesting Case March 2015

Clinical History
A 30 year old man was referred for liver transplantation workup for presumed liver cirrhosis from NASH. The clinical diagnosis of NASH cirrhosis was based on hepatosplenomegaly, low platelets and steatosis on liver imaging and the local biopsy. The only risk factor for NASH was hypercholesterolemia. 

Labs
AST 53 U/L, ALT 87 U/L, ALP 75 U/L, platelets 101,000/µL.

The patient was referred to a liver transplantation center and the biopsy was re-evaluated. Images from the liver biopsy are shown below.



Liver Biopsy Findings
The biopsy shows clusters of “sea-blue” cells that contain pale, foamy cytoplasmic material. These cells stain with the macrophage marker CD68, but not the hepatocyte marker Hep-Par1. The hepatocytes have mild microvesicular steatosis, but no features of steatohepatitis are apparent. Also there is only very little fibrosis. By electron micrograph, the macrophages contain laminated myelin figures.

Diagnosis
Sea blue histocytosis syndrome

Major Learning Points
  1. Clinical, non-invasive features of cirrhosis and portal hypertension, such as thrombocytopenia, can have other causes.
  2. Sea-blue histiocytosis (SBH) syndrome can be subtle and not always as prominent as in this case. But SBH syndrome should be kept in mind in certain clinical contexts, including unexplained hepatosplenomegaly.
  3. The published cases of SBH involving the liver are for the most part due to inherited lysosomal storage disorders. Typically the bone marrow is the most commonly affected organ, baring a few exceptions such as patients receiving fat emulsion in total parenteral nutrition.
  4. Electron microscopy, though rarely utilized in today’s clinical hepatopathology practice, could play a helpful role in defining the nature of cytoplasmic accumulations.
Discussion
Steatohepatitis is a common disease and it has become a frequent cause of referral to liver transplantation centers. In addition, the proportion of liver transplantations for NASH is projected to increase due the availability of new highly efficacious direct antiviral agents for hepatitis C. It is worth highlighting the need to carefully evaluate the clinical, non-invasive parameters of cirrhosis knowing that these assessments are surrogates and can sometimes produce false positive results. In this patient with an enlarged spleen, low platelets, hyperlipidemia and radiologic diagnosis of “fatty liver”, the clinical assessment had pointed in the direction of NASH-related end-stage liver disease. Ultimately, however, this proved to be a case of Nieman-Pick disease which manifests characteristically as hepatosplenomegaly, low platelets and hypercholesterolemia.

The accumulation of lipid material in macrophages of several organs, including the liver, spleen, bone marrow, and lymph nodes, is sometimes referred to as “sea-blue histocytosis” (SBH). The name initially originated form the appearance of these macrophages when stained with Giemsa/PAS, although in the case described above this stain was not necessary to demonstrate these cells. There are many causes of SBH, but in general, it is a feature of lipid or ceroid storage disorders, and can be primary or secondary. The term was first applied in 1941 in the Swiss literature by Dr.  Möschlin and later reported as a syndrome in 1970 by Silverstein et al (N Engl J Med 1970; 282:1-4). The syndrome was attributed to adults with Nieman-Pick disease (NPD) in 1977 by Long et al (Am J Med. 1977;62(4):627-35). NPD was ultimately diagnosed in the patient presented above. Following additional studies, he is believed to have Nieman-Pick disease type B. The SBH syndrome is however not unique to NPD and histopathology lacks the specificity to make an etiologic diagnosis in most cases. 

Determining an etiology for SBH syndrome requires the combination of clinical, histopathological, biochemical and genetic information. Some of the other primary/inherited diseases associated with the SBH are cholesterol ester storage disease, Gaucher disease, hypertriglyceridemia (with mutation in the receptor-binding region of the apoE molecule), lecithin-cholesterol acyltransferase deficiency (i.e., Norum disease), ceroid lipofuscinosis and Tangier disease. Several acquired conditions have also been associated SBH, including administration of fat emulsion in total parenteral nutrition, as well as some hematologic conditions. The bone marrow, and less likely the liver, is the usual site of SBH accumulation in these hematologic conditions, such as in myelodysplastic syndrome (J Clin Pathol. 1993 Nov;46(11):1030-2).

Contributed by:
Oyedele Adeyi, MD
Department of Pathology, Toronto General Hospital, Toronto, Ontario.