The first thing the 59-year-old man saw when he opened his eyes was the blood. His pillow was drenched in gooey reds and browns. He touched his nose tentatively; the drying crust flaked onto his fingers. He texted a doctor friend. “Go to the E.R.,” was the immediate reply.
His wife drove him to Manhattan’s Mount Sinai West from their apartment in Brooklyn. The emergency room was already busy, but he was soon telling first a nurse, then a doctor, about his early morning shock.
He’d had a nosebleed the day before — the worst of his adult life. It was early December 2021, and he was shopping for Hanukkah dinner when he felt something wet at the base of his nose. The slow ooze of blood took forever to stop, maybe an hour. Then, that night, his brother noticed a rash on his leg. The tiny red spots dotting his calf and ankle didn’t hurt or itch; he hadn’t known they were there until his brother asked about them.
In the E.R., the explanation for both the rash and the bleeding came quickly. His platelets — the part of the blood that triggers clotting — had practically disappeared. Normally we have over 150,000 platelets per microliter of blood. He had a scant 3,000. Without platelets, any injury can cause bleeding that is hard to stop. The tiny red dots, known as petechiae, are leaks from capillaries injured by minor trauma or just the increased venous pressure of walking.
The man considered himself healthy, or did until he caught Covid three months earlier. It wasn’t a bad case, but it lingered long enough for him to develop a sinus infection, which required antibiotics. He saw his doctor the next month for an annual exam. Routine blood work showed then that his platelets were low, at 51,000. A repeated test was even lower, so she sent him to the E.R. and referred him to a hematologist, Lawrence Cytryn.
When the man saw the blood specialist the following week, a recent test showed a normal platelet count. His exam was unremarkable. Cytryn had some theories about his transient thrombocytopenia, the medical term for a drop in platelets. Covid can rarely cause an autoimmune disorder known as immune thrombocytopenia, or ITP, in which the body’s white cells mistakenly hunt and destroy platelets. Another possibility: The antibiotic he had been treated with, Levaquin, can infrequently cause low platelets.
But when Cytryn rechecked his blood that day, his platelet count wasn’t normal. He now had too many: 890,000, more than twice the upper limit of normal. Cytryn wondered if this were a robust rebound from his transient low, and was reassured when the numbers were coming down a few days later.
But then he had the worst nosebleed of his life and ended up in the E.R.
A Cyclical Ordeal
In the hospital, doctors assumed the patient was having a recurrence of his ITP, and he was treated with steroids and intravenous immune globulins (IVIG), a treatment made from thousands of donors’ antibodies. With the steroids, this would temporarily quell the improper attacks of an immune system gone a little wild. Almost immediately, the patient’s platelet count began to rise. He was discharged on a tapering dose of steroids.
A month later, the rash and bloody nose returned. He called Cytryn’s office and was told to go to the E.R. Again, his platelet count was low. Again, he was given steroids and IVIG and his platelets began to rise. At the next check, his platelets had again shot well past normal. Cytryn now suggested a new diagnosis. This wasn’t ITP; it was an even rarer disorder called cyclic thrombocytopenia.
Medicine knows little about cyclic thrombocytopenia beyond what is clear in the name: Patients have repeated episodes of platelets falling and rising. There have been fewer than 100 cases reported.
Over time, the man recognized a pattern. His platelets would drop over the course of two weeks, stay low for a week and then shoot up for the next two weeks. When they were low, the man would find himself covered with bruises and the petechial rash. Sometimes he would get strange welts in his mouth. Once his urine was red with blood. The whole cycle usually lasted 37 days. He joked sometimes that he had a monthly period.
Cytryn contacted experts around the country. He tried treatments to suppress the patient’s immune system and others to promote platelet production. One drug caused liver inflammation. Another was hard on his kidneys. None changed the 37-day cycle.
The patient shaped his life around his disease. When his platelets were at their lowest, he’d stay home, often in bed. It wasn’t that he felt so awful, but he wanted to make sure he didn’t do anything to cause bleeding. He was especially worried about his head. He knew that hemorrhage inside the skull could be devastating.
He had test after test. Two bone-marrow biopsies were unrevealing. Imaging of his brain, his abdomen and his pelvis showed no abnormality that could be linked to his platelets. Blood tests were likewise uninformative.
An Answer in an Unexpected Place
A couple of years into this odyssey, he found a new primary care doctor, Edward S. Goldberg. Goldberg focused on medical issues beyond his fluctuating platelet count. Most concerning was that his cholesterol was high. And he had other risk factors for coronary-artery disease: He was male; he was over 60; he had high blood pressure.
Heart disease is still the No. 1 killer of men in this country, Goldberg reminded the patient. He proposed starting a statin to lower his cholesterol. The patient was hesitant. His father had taken one and hated it. Another possibility was to get a CT angiogram of his chest to see how much plaque had formed in and around the blood vessels of his heart. If he had clean coronaries, they could give diet and exercise more of a chance. If not, the statin.
It was barely an hour after the scan when Goldberg called him. His coronaries were fine. But there was a large mass in his chest. It was nearly the size of a deck of cards, and it looked as if it were growing from his thymus gland.
The thymus is an essential component of the immune system. It trains one population of white blood cells to distinguish invaders from normal tissue. It is most active in childhood; by middle age the gland has stopped working and has turned to fat.
Goldberg recommended he see a surgeon to remove the mass, which was probably cancer.
Several scans later, an oncologic surgeon at Memorial Sloan Kettering assured the patient that the cancer had not spread and that removal of the tumor would probably be the end of the matter. The man asked if the surgery might also fix his platelets. The surgeon didn’t know; he’d never seen a case like this.
There have been a handful of documented cases of immune thrombocytopenias linked to thymic tumors, known as thymomas. In some, treating the tumor stopped the thrombocytopenia. Regardless, the tumor would have to come out. The surgeon and patient plotted the best time to schedule the surgery, when his platelets were neither too high nor too low. The operation was scheduled for early last August. The tumor was large, right at the limit to allow a minimally invasive laparoscopic surgery. He’d try for that, the surgeon told him, but if it didn’t work, they’d have to cut his sternum open.
Waking after the surgery, the man looked at his chest. It was shaved, and a dark Magic Marker line traced the middle of his sternum, but there were no sutures. Just three small incisions over his rib cage.
As September approached, the patient waited anxiously to see if his platelets would plunge on schedule. He brought a packed bag to the lab for his next round of blood work, in case he had to check in to the hospital again. But this time, his platelets were normal. Employees at the lab cheered when they delivered the news. In October, then November, there were some ups and downs, though not as dramatic as before the surgery. His doctors remain on alert. But from the man’s point of view, he’s moving in the right direction. He is optimistic that his ordeal is finally over.
Lisa Sanders, M.D., is a contributing writer for the magazine. Her latest book is “Diagnosis: Solving the Most Baffling Medical Mysteries.” If you have a solved case to share, write to her at [email protected].
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