The woman arrived at an outpatient clinic in New York having experienced dizziness and a rash for the past week. Two weeks prior to this she had a viral respiratory tract infection, a case report published in NEJM notes.
“On physical examination, she had a generalized, macular, non-blanching rash in a reticular pattern with purplish discoloration consistent with livedo reticularis,” the report said. A non-blanching rash is where the rash does not fade when pressed with a glass.
Livedo reticularis, according to the Mayo Clinic, is a condition that causes the skin to appear mottled and purple in a “net-like” pattern. It is thought to be related to spasms of the blood vessels or circulation problems.
Doctors treating the woman tested her blood and diagnosed cold agglutinin disease. The initial sample showed the blood had clumped. After warming it in a saline bath, it returned to a normal consistency.
“Cold agglutinin disease is a rare form of autoimmune hemolytic anemia—meaning [the] body’s own immune system starts to act on its own red blood cells membrane,” Konika Sharma and Anush Patel, from the Bassett Medical Center in New York, told Newsweek in an email. Both Sharma and Patel worked on the woman’s case.
Agglutination, she explained, is where red blood cells clump together, preventing the free flow of red cells. “It is called ‘cold’ because when patients are exposed to cold environments, symptoms get triggered, which causes red cell agglutination,” Sharma and Patel said.
It is a rare disease, affecting around one person in a million, and affects around 15 percent of people with autoimmune hemolytic anemia.
The team say her condition was likely exacerbated by the viral infection the woman had suffered with and the cold weather in upstate New York at the time. The woman was warmed and given blood transfusions along with rituximab—a drug that targets the immune system. “The patient we described had a severe exacerbation, based on profound anemia,” Sharma and Patel. “She had symptoms because of anemia, we had to give her multiple transfusions of warm blood.”
She was discharged after her dizziness had lessened. The rash, however, persisted.
“Usually the rash is seen in a localized area, unlike our lady who had an extensive rash all over her body…This extensive rash was due to the fact that all the blood vessels in her body were under low oxygen tension due to exposure to cold causing agglutination,” Sharma and Patel said.
“Her rash slightly improved with warm environment but as we live in the extreme north, where winters are harsh, [the] rash didn’t go away completely. She was placed on rituximab, usually medical treatment takes two to four weeks to suppress the immune activation. During that time we continued supportive treatments like blood transfusions and keeping warm.”
