A case report of a patient diagnosed with cold agglutinin disease (CAD) following varicella infection has been published in the Indian Journal of Pathology & Microbiology.
A 24-year-old woman presented with a 5-day history of maculopapular rash which evolved into vesicles followed by crusting. The lesions appeared on the face, trunk, and extremities of the patient, consistent with chickenpox (varicella-zoster virus) infection.
The patient was pale and jaundiced and reported having a 1-day history of fever and yellowish discoloration of the eyes. Laboratory investigations revealed that she had elevated bilirubin (5.1 mg/dL), low hemoglobin (7.9 g/dL), and elevated white blood cells (16,500/mm3).
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The patient was empirically started on antibiotics. A peripheral blood smear revealed normocytic, normochromic erythrocytes with red cell agglutination, as well as neutrophilic leukocytosis with neutrophils showing erythrophagocytosis. In addition, she was found to have markedly high lactate dehydrogenase (250 IU/L).
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Abdomen ultrasound and chest x-ray were both normal. Blood and urine cultures were negative; a malaria antigen test and dengue/hepatotoxic virus serology were likewise negative. However, a direct Coombs test was positive for C3d—sufficient to confirm a diagnosis of CAD secondary to varicella-zoster virus infection.
During admission, the patient was administered packed red cells using a warmer in response to her falling hemoglobin levels. On day 6, she was started on oral prednisolone 50 mg daily, after which her hemoglobin levels began to rise steadily, accompanied by a reduction in bilirubin levels.
The patient was discharged on day 12 after her hemoglobin levels improved to 7.2 g/dL from a low of 3.8 g/dL recorded during her second day of admission. A week later, the patient was asymptomatic during follow-up and her hemoglobin levels rose to 9.2 g/dL. Her total bilirubin and lactate dehydrogenase levels returned to normal. A repeat direct Coombs test was negative.
This case study is among very few in medical literature to report a diagnosis of autoimmune hemolytic anemia following varicella infection.
Reference
Carvalho G, D’silva A, Chandran P, Manuel K, Kinglsey, Basheer A. Cold antibody autoimmune hemolytic anemia following varicella infection. Indian J Pathol Microbiol. 2023;66:188-90. doi:10.4103/ijpm.ijpm_728_21