Autoimmune Hemolytic Anemia Following IVIG Treatment in a Patient with CIDP and Positive Porphobilinogen Testing: A Case Report
Karlos Daniell dos Santos1, Filipi Andreão2, Filipe Ribeiro3, Fernanda Herculano4, Carolina Moura5, Diogo Haddad Santos6
1Federal University of Roraima, 2Federal University of Rio de Janeiro, 3Barão de Mauá University Center, 4Hospital Universitario Antonio Pedro, 5Hospital Universitário Antonio Pedro, 6Moema
Objective:
This report objectives to describe the clinical presentation, diagnosis, and management of AIHA following IVIG therapy in a CIDP patient, complicated by elevated porphobilinogen levels, raising the possibility of porphyria.
Background:
Intravenous immunoglobulin (IVIG) therapy is a common treatment for immune-mediated disorders such as Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP), but it can lead to complications like autoimmune hemolytic anemia (AIHA). We present a case of a patient who developed AIHA following IVIG treatment, complicated by elevated porphobilinogen levels, raising the possibility of porphyria.
Results:
A 46-year-old female presented with worsening ataxia and dysarthria since January 2021, which affected her fine motor skills and gait. Her workup revealed normal cerebrospinal fluid (CSF) cytology but elevated gamma globulins on CSF electrophoresis, suggesting an immune-mediated process. Examination showed motor strength grade IV in the left lower extremity, with axial and appendicular ataxia, dysarthria, and an ataxic gait. She was diagnosed with probable CIDP and treated with IVIG from August 15 to 19, 2024. Following treatment, her ataxia improved, but on the final day of IVIG, she noted dark reddish urine and discoloration of the lower limbs, without other symptoms. Laboratory results showed elevated lactate dehydrogenase (LDH) and bilirubin levels, with a positive direct antiglobulin test (DAT) for IgG and C3, indicating AIHA. Additionally, a positive porphobilinogen test suggested porphyria, with confirmatory testing pending. The patient was started on prednisone (80 mg daily), which led to improvement.
Conclusions:
This case highlights the potential for IVIG to induce AIHA, emphasizing the need for early recognition and prompt treatment to avoid severe consequences. The concurrent elevation in porphobilinogen introduces the possibility of porphyria, complicating the clinical picture. Further studies are warranted to explore the mechanisms underlying IVIG-induced hemolysis and its potential link to porphyria.
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