Tag: gamma-glutamyl transferase

High ALP + High GGT + High IgM + AMA = PBC

So how did I end up getting diagnosed with primary biliary cirrhosis (PBC) three years ago? It wasn’t easy, believe me.

Stop 1: Family doctor, July 2006

My complaint was that I was more tired than usual. The previous year I had had iron deficiency anemia due to female problems 😦 leading to a D&C. So anemia was what I expected as this year’s verdict as well.

Wrong. My family doctor, Dr. Cindy McAdams, paid close attention to my lab work (in another post I will explain why I think that worth mentioning. Suffice it to say, I have had doctors who haven’t).

My serum alkaline phosphatase (alk phos or ALP) was 410 (normal is 25-153), AST 74 and ALT 91 (0-40 norm).

Explain.

These are liver enzymes. Abnormal  AST and ALT are linked to a number of conditions and frequently occur in connection with the use or misuse of scores of medications. A mild to moderate elevstion, like I had, is seen in PBC, but is not a crucial bit of information.

Markedly elevated alkaline phosphatase is, and a score of 410 when 153 is top normal is not good. From MedicineNet:

Abnormally high blood levels of alkaline phosphatase may indicate disease in bone or liver, bile duct obstruction, or certain malignancies. The enzyme is often elevated in the leukemic cells in chronic myelogenous leukemia.

A repeat of the bloodwork with additional tests added yielded more bad marks, including a 68 Westergren sedimentation rate (upper norm of 20) and low platelets and white blood count, and I was packed off to a haematologist (specialist in blood disorders).

Stop 2: Hematologist, August 2006

This stop proved useful to rule some things out. The hematologist started with an ultrasound, and that revealed a much enlarged spleen — and enlarged lymph nodes. Things had taken a grim turn indeed, and a CAT scan was ordered to establish whether there was activity in these enlarged lymph nodes. Now I am not a medico, but I know what activity in the lymph nodes could have indicated: cancer.

I was lucky, however, and not sorry to be sent along from that office; the hematology and oncology units were housed together, and it was a place I didn’t want to belong.

I did leave with some new findings:

  • Markedly elevated GGT (585 when normal is 7-32). GGT stands for gamma-glutamyl transferase. High ALP with normal GGT means that the trouble is a bone disease; high ALP and high GGT narrow it down to bile ducts or liver.
  • Immunoglobulin IgM was 810 (norm 40-230). This is what I found out: “Increased serum immunoglobulin concentrations occur due to polyclonal or oligoclonal immunoglobulin proliferation in hepatic disease (hepatitis, liver cirrhosis), connective tissue diseases…”

Stop 3: Rheumatologist, September 2006

I had enough findings to make lupus or rheumatoid arthritis a possible if not exclusive diagnosis, and so I visited the rheumatologist and left behind who knows how many vials of my blood. My ALP was now 481, and other bad marks were added to my lengthening list:

  • Actin (Smooth Muscle) Antibody of 115 units when norm is 0-19. Enter a new possible diagnosis: autoimmune hepatitis.
  • Presence of antimitochondrial antibodies (AMA), and “between 95 and 98% of patients with primary biliary cirrhosis (PBC) have autoantibodies (antibodies to self) in their blood that react with the inner lining of mitochondria.”

Stops 4 & 5: Gastroenterologists, October to November 2006

At this point I should have seen a hepatologist, a specialist in liver diseases, but since there isn’t one locally, a gastroenterologist was the fallback.

Two stops here because the first GI I visited ordered his own bloodwork, gave me an appointment date two weeks later, and when I arrived, he was not. Yes, I know emergencies happen. But when his staff offered to reschedule me in the new year, his first available appointment since he would be out of the country in December, I said no way, just give me my records and I’ll find someone else. They told me to hold on a minute, and came back with the news that another doctor in the practice would see me in an hour.

His approach was to pursue the possibility of autoimmune hepatitis (AIH). We were pretty much down to two choices: that or PBC. And 7 of 10 people with AIH go into remission after 3 years’ treatment. People with PBC don’t.

So the plan was to give me prednisone, and if I responded to this steroid, it was likely I had AIH. If not, then we were left with PBC.

So I took prednisone for 6 weeks.

Maybe you’ve seen it in old British movies: back when that country still had capital punishment, if the judge was going to announce a sentence of hanging,  he entered the court he wearing something like a black piece of cloth atop his white wig. GI2 may as well have been wearing one of those when he came into the examining room. My scores were worse.

Then he did the best thing and the dumbest thing he could.

The best was that he set me up an appointment with a hepatologist at the University of Alabama in Birmingham Medical School. The dumbest was he simply stopped the prednisone.

When you’ve taken 40 mg of prednisone daily for 6 weeks, it is not a good idea to go cold turkey, to abruptly entirely withdraw it. I spent the next month practicing being dead.

Stop 6: Hepatologist, December 2006

I first visited the hepatologist the week before Christmas. All the puzzle pieces were ready to put into place:

An elevation of the aminotransferases alanine aminotransferase (ALT) and aspartate aminotransferase (AST) may be identified in most patients with primary biliary cirrhosis, but significant elevations of the alkaline phosphatase (ALP), g -glutamyl transpeptidase (GGTP), and immunoglobulin levels (mainly immunoglobulin M [IgM]) are usually the most prominent findings….AMAs can be found in 90-95% of patients with primary biliary cirrhosis, and they have a specificity of 98% for this disease.

  • Elevated ALP √
  • Elevated GGT √
  • Elevated IgM √
  • Elevated ALT √
  • Elevated AST √
  • Presence of AMA √
  • The hepatologist started me on 1000 mg of Urso Forte a day.

    January 2007

    A month later my ALP, which at its highest was 481, had returned to the upper limit of normal (on this lab’s scale [they vary]): 117.

    November 2009

    My ALP is still 117. Just how meaningful this is remains to be seen.

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