Fairy circles: liver by any other name

Shahar Madjar, MD

In my last article, I told you that as all doctors, mine, too, is the duty to notice patterns, and to detect disturbances in patterns. Here is a story about a patient, his doctors, and one disturbed pattern that was a key to the correct diagnosis:

Adam was 58 when he came to the emergency department. He told the doctors that he had, for the past 2 weeks, high fevers and drenching night sweats. Adam’s eyes were yellow and his urine was dark.

Before I tell you more about Adam, there is something you need to know about the liver. It weighs about 3.5 lb. It is a seemingly dull, unsexy organ that sits, motionless and without undue drama, in the upper right side of the abdomen. The liver is, nonetheless, a kingdom of activity: its cells, the hepatocytes, perform more than 500 different functions including processing of carbohydrates, proteins and lipids.

More relevant to Adam’s case, when aged red-blood-cells die, the cells within the liver break down the hemoglobin that was in them (hemoglobin is a red molecule that carries oxygen to the tissues. It gives blood it’s red color. Each red-blood-cell contains millions of molecules of hemoglobin).

One of the products of this breakdown of hemoglobin by the liver is bilirubin, a very yellow compound. Once formed in the liver, bilirubin drains, in a dark-green fluid called bile, via a system of delicate tubes within the liver, and then by larger tubes outside of the liver. Some of the bile is stored in the gallbladder, and some drains into the intestine.

Adam’s yellow eyes, his dark urine, and a dramatic increase in the bilirubin level in his blood, all indicated that something was rotten in the kingdom of Adam’s liver.

But what?

The fevers and the night sweats might have been the result of an infection, perhaps a malignant condition that blocked the tubes draining the liver, or an autoimmune disease where cells of the immune system erroneously attacked the cells within Adam’s liver, or along the tubes that drained his bile.

But blood tests showed no evidence of any infection that commonly affects the liver and the markers of autoimmune disease were inconclusive. An ultrasound of the liver and the biliary system showed no blockage along the biliary system outside the liver. What is the cause for Adam’s symptoms, then? It was time to take a closer look.

The doctors prepped and draped the skin along Adam’s neck. They inserted a long sheath that passed through the jugular vein, through his heart and all the way into a vein that drained his liver. They then passed a small-caliber biopsy forceps and took a tiny sample of his liver, a liver biopsy.

Under the microscope, Adam’s doctors saw the typical structure of a normal liver, with hepatocytes and blood vessels, and tiny bile ducts. They also noticed a pattern not seen in a normal liver (it reminded me of the pattern of the Fairy Circles in the Namibian Desert I told you about in my last article): cells called histiocytes arranged themselves, forming a ring around tiny spaces filled with fat. The ring of cells was further surrounded by another flimsy ring of scar tissue. This pattern, or a disturbance in the usual normal pattern of the liver, is the pattern seen in Q fever, a disease caused by bacteria called Coxiella burnetii.

Looking back, the doctors could retrace the events that led to Adam’s illness: several weeks prior to becoming sick, Adam made a trip to California. Not very far from the place he stayed in, there was a farm in which cattle, sheep, perhaps goats infected with C. burnetii lived. The infected animals excreted the spores of C. burnetii in their stool. The spores were then carried by the wind, for a distance as far as 10 miles away. Adam inhaled the spores. He could have remained asymptomatic, suffer from headaches, or developed a pneumonia. Instead, he had an infection of the liver, also known as hepatitis.

Adam was treated with doxycycline, an antibiotic treatment that is very effective against C. burnetii. His symptoms completely resolved and he quickly returned to his normal patterns of health.

When faced with patterns in nature, one can see order, and beauty. A pattern — and, at times, a disturbance in pattern — is, for the curious mind, a call for new questions, an invitation to discover.

Editor’s note: Dr. Shahar Madjar is a urologist working in several locations in the Upper Peninsula. Contact him at smadjar@yahoo.com or at DrMadjar.com.