Researchers found a wide range of demographic, clinical, and laboratory characteristics when studying eight patients with cold agglutinin disease (CAD).
The findings revealed that treatment with corticosteroids, surgical removal of the spleen, and the use of CellCept (mycophenolate mofetil) — an antirejection medicine given to transplant patients — led to partial or complete responses in people with the acquired autoimmune disorder.
The study, “Report on Cold Agglutinin Disease Patients Clinical Characteristics,” was presented at the 25th European Hematology Association( EHA) Virtual Congress, held online.
CAD is characterized by an autoimmune response induced by exposure to cold temperatures. Antibodies called cold agglutinins bind to and destroy red blood cells, resulting in anemia. People with CAD may have primary disease, when the cause of the condition is unknown, or secondary disease, in which CAD is due to an underlying condition, such as a viral infection or lymphoid cancers.
To better understand the range of characteristics associated with CAD, a research team at the National and Kapodistrian University of Athens, in Greece, studied eight patients with the rare condition.
Five participants were women and three were men; their median age at diagnosis was 62. All eight had cold agglutinins, and one also had cryoglobulins, another type of cold-sensitive antibodies.
As for symptoms, the patients’ fatigue ranged from none to extreme, but all had pallor, or a pale appearance, and jaundice, which is caused by high bilirubin levels. None of the participants had lymph node enlargement or splenomegaly, which is an enlarged spleen.
The patient with cryoglobulins also had skin necrosis, or dead skin tissue, which was caused by blood vessel blockage.
Laboratory findings showed that the patients’ median hemoglobin level was 8.9 g/dL, which is below the normal range of 13.5–17.5 g/dL for men and 12–15.5 g/dL for women.
All of the participants tested positive on the direct Coombs test, which assesses the attachment of antibodies to red blood cells and is common when analyzing the presence of CAD.
Low levels of IgM antibodies — the first type of antibody made in response to an infection — were found in half of the patients, and most had hypogammaglobulinemia, or not enough gamma globulin antibodies in the blood.
All patients were initially treated with corticosteroids.
Three of the individuals with primary CAD responded either partially or completely to the combination of corticosteroids, splenectomy, or spleen removal, and CellCept, a medication that reduces immune system activity.
Other patients underwent approaches such as plasma exchange, or plasmapheresis.
After a median 42.5 months (just over 3.5 years) of follow-up, two patients had completely recovered and two had died from infections and sepsis. The remaining four participants had varying degrees of relapse and remission.
The findings demonstrate a wide array of symptoms and laboratory findings in CAD patients.
“Although it is a small series, it highlights the wide range of CAD clinical behavior,” the researchers wrote. “Further and larger studies are needed to better understand and manage this rare entity.”
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