CAD secondary to Epstein-Barr virus leads to blood clots in teenager

Condition was complicated by blocked vein in girl's arm

Margarida Maia, PhD avatar

by Margarida Maia, PhD |

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A woman rolls up her pants leg to reveal problems with her blood vessels.

Cold agglutinin disease (CAD) secondary to an infection with the Epstein-Barr virus led to a blocked vein due to blood clots in a young girl in Turkey, prolonging her hospital stay, a case study shows.

The report, “A Rare Case Presentation: EBV-related Cold Agglutinin Disease and Venous Thrombosis,” was published as a letter to the editor of The Pediatric Infectious Disease Journal. The Epstein-Barr virus can cause infectious mononucleosis, or mono.

CAD occurs when self-reactive antibodies called cold agglutinins bind to red blood cells at low temperatures, causing them to clump together and be marked for destruction. People with CAD develop hemolytic anemia, which occurs when red blood cells are destroyed faster than they can be replaced. Having fewer red blood cells means less oxygen will be carried to tissues, causing symptoms such as tiredness and weakness.

When CAD is triggered by an underlying condition, such as an infection, it’s called secondary CAD. While it often causes mild or no symptoms, an Epstein-Barr infection is a common cause of secondary CAD, with several cases reported in the literature.

Here, researchers in Turkey report the case of a 13-year-old girl whose CAD secondary to an infection with the Epstein-Barr virus was complicated by a vein blockage in her left arm due to a blood clot. CAD-associated clumping of red blood cells can increase the risk of blood clots, which can lead to thrombosis, that is, clots that block blood flow, which can be life-threatening.

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Secondary CAD and blood clots

The girl was admitted to the hospital due to fever, jaundice, or yellowing of the skin and whites of the eyes, and vomiting that had lasted for 10 days. She also had a sore throat with pus and swollen lymph nodes in her neck.

Blood testing revealed a reduced proportion of red blood cells, immature red blood cells, and mild anemia, which was reflected by slightly lower levels of hemoglobin, the protein that carries oxygen in red blood cells. Her liver enzyme levels were elevated, a sign the liver may be damaged. An abdominal scan showed no localized lesions in the liver or spleen.

An infection with the Epstein-Barr virus was detected. A direct Coombs test was positive, indicating the presence of antibodies bound to red blood cells, and a blood sample analysis under a microscope showed roll-like clumps of red blood cells, finding that supported a diagnosis of CAD secondary to Epstein-Barr virus infection.

Treatment involved adding fluids and closely monitoring the girl’s hemolytic anemia. Her hemoglobin levels improved after about a week and her liver function returned to normal.

Pharmacological therapy for cold agglutinin disease is not indicated in patients with mild anemia or compensated hemolysis without troublesome clinical symptoms,” the researchers wrote.

Swelling, redness, and increased temperature were observed on her left arm where a tube was placed to access a blood vessel to deliver fluids. An ultrasound scan revealed thrombophlebitis, or inflammation in a vein near the skin due to a blood clot, as well as venous thrombosis that didn’t respond to compression.

The girl was started on enoxaparin, a blood thinner, and the antibiotics ceftriaxone and clindamycin. These helped resolve her thrombophlebitis and venous thrombosis and ease any complications.

“Our patient’s onset of thrombophlebitis and venous thrombosis led to a protracted hospital stay,” the researchers wrote. “Although cases of cold agglutinin hemolytic anemia from [Epstein-Barr virus infection] … are uncommon, they serve to illustrate the variety of manifestations that this virus can cause.” The researchers said “prompt prevention and treatment“ are important in this indication.