[Accessory spleens after splenectomy in a patient with common variable immunodeficiency]

Area of Science:

• Immunology
• Hematology
• Surgical Pathology

Background:

• Autoimmune cytopenia involves destruction of blood cells, often necessitating splenectomy.
• Splenectomy, while therapeutic, can lead to disease relapse due to accessory spleens or splenosis.
• Common variable immunodeficiency (CVID) can present with autoimmune cytopenia, complicating treatment decisions.

Observation:

• An 18-year-old male with CVID and immune thrombocytopenia achieved remission post-splenectomy.
• Relapse of thrombocytopenia was observed, attributed to splenosis given the absence of Jolly bodies on peripheral blood smear.
• Laparoscopic splenectomy of identified accessory spleens resulted in long-term remission.

Findings:

• Accessory spleens are implicated in 15-20% of autoimmune cytopenia relapses following splenectomy.
• Absence of Jolly bodies on blood smear can indicate splenosis or accessory spleens as a cause of relapse.
• Surgical ablation of accessory spleens offers a curative option for relapsed autoimmune cytopenia.

Implications:

• Identifying and removing accessory spleens is crucial for managing post-splenectomy autoimmune cytopenia relapse.
• This approach provides a potential cure for patients with relapsed autoimmune cytopenia, including those with CVID.
• Further research into the prevalence and management of accessory spleens in autoimmune cytopenia is warranted.