Background

Cold Agglutinin Disease (CAD) is a rare entity of Autoimmune Hemolytic Anemia (AHAI) type immunoglobulin M characterized by destruction of red blood cells at low temperature. It affects one person in a million. Etiological evaluation is essential for managing the cause in addition to symptomatic treatment. A distinction is made between idiopathic forms and forms secondary to viral infections, auto immune diseases and hemopathies [1].

The clinical symptomatology is generally acute and the FSM can be responsible for complications whose evolution is dramatic without treatment, such as revealing digital necrosis raising problems of differential diagnosis with Raynaud’s syndrome, arterialis chemia and necrotizing vasculitis [2].

We report a case of a 68-year-old woman who presented with acute development of acrocyanosis of right foot toes within 48 hours with cutaneous-mucosalpallor on clinical examination.

The blood test showed anemia at 9 g/dl with reticulocytes at 155 g/L. Direct Coombs test was positive for anti C3 anti bodies with a titre of 1:1024 at temperature of 4°C. Etiological investigation was negative and therefore diagnosis of idiopathic cold agglutinin disease was retained. Patient was treated with weekly infusion of Rituximab in combination with preventive measures. Evolution was marked by a good clinical recovery.

Figure 1: acrocyanosis of right foot toes.

Consent for publication : Written informed consent was obtained from the patient for publication of this case report and anyac companying images.

Competing interests : The author declare that he had no competing interests.

References

1. Berentsen S, Röth A, Randen U, Jilma B, Tjønnfjord GE, et al. Cold agglutinin disease: Current challenges and future prospects. J Blood Med. 2019; 10: 93-103.
2. Nakagawa H, Kato C, Miyata Y. Necrosis secondary to cold agglutinin disease. CMAJ. 2022; 194: E528.