Against the Odds

The problem had been going on for weeks: Extreme abdominal pain, chills, sweats, nausea, endless, sleepless nights, weight loss, and, most puzzling, a strange rash on his thigh.

He had been to the clinic, and even the emergency center—more than once—to no avail.

But now things were clearly worse. He had sporadic headaches. His vision was blurred. And he was getting confused, the way stroke victims often do. Something was definitely, seriously wrong.

Luckily for this young man, his local health care facility in Royal Oak, Mich. was one of the best in the area.

While the physical examination was being conducted, lab results poured in. The hematology lab reported a dangerously low platelet level despite no active bleeding. Urinalysis was positive for blood, yet coagulation studies were normal. In the chemistry department, liver function tests were negative. Immunology reported he did not have hepatitis or HIV.

A number of diagnoses were considered, including gastrointestinal ailments, Epstein-Barr virus, and a rare autoimmune disease known as Evans Syndrome. But the lab data suggested a more dire possibility: Thrombotic Thrombocytopenic Purpura, or TTP. TTP is a rare disorder of the blood coagulation system that often leads to stroke, heart attack, or kidney failure. The doctors knew that TTP typically occurs in females around the age of 40, not young men in their twenties. But with stakes so high—TTP has a 90 percent fatality rate if not quickly diagnosed and treated—they could take no chances. The patient was admitted for further study, and the hospital pathologist was called in.

Additional lab results, combined with the ongoing physical symptoms, ultimately led the pathologist to a diagnosis: the patient indeed suffered from TTP. The pathologist ordered an additional test that uncovered the cause – the patient was producing auto-antibodies to a very important enzyme known as ADAMTS13.

The patient began a daily regimen of complete plasma exchange. In this procedure, blood is slowly extracted from the body and spun down, separating out the plasma. The patient's own red blood cells are then re-infused with fresh, recently-thawed plasma. It's a highly specialized procedure that takes hours, and this Royal Oak hospital is one of the few area hospitals that can perform it.

The treatments continued for a week, at which time the patient's platelet count had risen and his symptoms were gone. Five more days of plasma exchange followed, allowing the platelet count to stabilize. The patient was discharged from the hospital with a steroid prescription and instructions to return for plasma exchanges, which tapered in frequency.

He also left with tremendous gratitude for the quick thinking of the pathologist, the efforts of the laboratorians in each department, and the expertise of the blood bank clinical laboratory scientists. Without them, he knew, he wouldn't have survived his ordeal.