Bacterial lung infection causes CAD, tissue death in nose for girl, 9
Child's condition didn't respond to treatment for M. pneumoniae: Report
A young girl developed cold agglutinin disease (CAD) secondary to a bacterial lung infection, due to Mycoplasma pneumoniae (M. pneumoniae), that resulted in progressive tissue death in her nose and limb extremities, according to a case report from Iran.
The child’s condition did not respond to typical therapies aimed at addressing the underlying infection or problems with circulation, the researchers noted. As these conditions progressed, tissues in her extremities, notably her fingers, began to die.
After establishing a CAD diagnosis, the girl’s clinicians began treatments that targeted CAD and modulated immune system activity more directly. Although this stopped the spread of tissue death, the child’s fingers ultimately required amputation, the team noted.
Though noting that tissue death is not commonly reported with M. pneumoniae infections, the researchers wrote that “the importance of the current report lies in the requirement for quick and effective management to prevent these complications.”
The study, “Mycoplasma pneumoniae Infection and Progressive Cold Agglutinin Syndrome in a Child: Catastrophic Multiple Cutaneous Necrosis,” was published in the journal Clinical Case Reports.
CAD is caused by self-targeting antibodies, called cold agglutinins, that bind to red blood cells at low temperatures. This results in red blood cell clumping, or aggregation, and death, which can disrupt oxygen flow throughout the body.
The disease can lead to symptoms including fatigue and pain, as well as acrocyanosis and Raynaud’s phenomenon — two conditions marked by skin discoloration of the fingers or toes when exposed to cold.
Bacterial lung infections, like from M. pneumoniae, can cause secondary CAD
CAD can occur on its own or secondary to an underlying disease or infectious agent, such as M. pneumoniae. The bacterium typically causes mild upper respiratory tract infections, but in severe cases, it can lead to pneumonia, marked by inflammation and fluid buildup in small air sacs in the lungs.
Secondary CAD, also known as cold agglutinin syndrome (CAS), has been reported in association with M. pneumoniae infection in both children and adults. In rare cases, this has led to more severe blood flow blockage that can result in periods of low oxygen supply, particularly in the body’s extremities. If circulatory problems persist, oxygen-poor tissues may die in a process called necrosis.
Now, a team of researchers from Isfahan University of Medical Sciences described the case of a 9-year-old girl who developed tissue necrosis in the extremities due to CAD secondary to a M. pneumoniae lung infection.
The girl had a history of a seizure condition, heart defects, and cerebral palsy, a group of lifelong disorders that affect movement. She was admitted to the hospital with pneumonia after 10 days of experiencing fever, respiratory distress, and symptoms of a common cold.
The child was treated with standard antibiotics for 10 days, but her fever continued, and she experienced a seizure. On day 10, her nose tip and some of her fingers and toes began to turn red or purple. A day later, her fingers and toes became cold and swollen, and showed acrocyanosis. The skin abnormalities continued to spread and progressed into necrosis.
At this time, certain laboratory tests showed no signs of hemolysis, or the breakdown of red blood cells.
Her skin abnormalities were consistent with microvascular occlusion, in which small blood vessels become blocked. Many conditions can cause microvascular occlusion, so the clinical team began investigating several possibilities.
In their diagnostic workup, the clinicians found evidence of red blood cell clumping, elevated cold agglutinins, and M. pneumoniae infection.
“Therefore, based on clinical examination and laboratory results, the possibility of CAS secondary to [M. pneumoniae] infection was considered for the child,” the researchers wrote.
Following treatment, child underwent surgery to amputate her fingers
Several medications were administered to increase the girl’s blood flow, but her symptoms persisted.
“The lesions at the tip of the nose, right wrist, right heel, and fingers of both hands progressed to gangrene,” in which dead tissue begins to decay, the team wrote.
This prompted the physicians to try an approach more focused on targeting CAD. The girl underwent plasmapheresis, a blood-cleaning procedure, and received intravenous, or into-the-vein, immunoglobulin (IVIG). Plasmapheresis is sometimes used for severe CAD with the goal of removing cold agglutinins from circulation. IVIG delivers a set of healthy antibodies, and is thought to help in CAD by neutralizing cold agglutinins and boosting the immune system’s disease-fighting skills.
After five days of plasmapheresis, the girl’s fever broke and tissue damage stopped progressing. After three weeks in intensive care, she was transferred to outpatient care.
Symptoms [of tissue death in an M. pneumoniae infection, while rarely reported] may lead to severe complications if not managed appropriately, highlighting the need for clinicians to remain vigilant and provide proper supportive care.
About three months later, the girl was referred to undergo surgery for the amputation of her necrotic fingers.
“Skin necrosis, progressive acrocyanosis, and gangrene formation are rarely reported manifestations of [M. pneumoniae] infection, especially in children,” the researchers wrote.
Altogether, the team identified seven cases in the scientific literature of skin manifestations in the extremities in individuals with an M. pneumoniae infection.
“In a few cases, gangrene and necrosis in their extremities healed without complications, but in some of them, including our case, surgical amputation was performed,” the team wrote. “These symptoms may lead to severe complications if not managed appropriately, highlighting the need for clinicians to remain vigilant and provide proper supportive care.”
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