Castleman Disease in a Patient with Common Variable Immunodeficiency

Area of Science:

• Immunology
• Hematology
• Oncology

Background:

• Common variable immunodeficiency (CVID) is a primary immunodeficiency characterized by hypogammaglobulinemia, leading to increased infection susceptibility.
• Noninfectious inflammatory conditions, including autoimmunity and lymphoproliferative disorders, are also associated with CVID.
• Castleman disease (CD) is a systemic lymphoproliferative disorder presenting with lymphadenopathy, splenomegaly, anemia, and systemic inflammation, often mimicking other conditions.

Observation:

• A novel case of multicentric Castleman disease (MCD) with splenomegaly is described in a middle-aged woman with CVID.
• The authors propose that inadequate management of hypogammaglobulinemia, with immunoglobulin G (IgG) levels outside normal ranges, contributed to the onset of MCD and splenomegaly.
• This represents the first reported association of MCD with an adult case of CVID.

Findings:

• Optimal management of hypogammaglobulinemia in CVID patients is essential for preventing complications.
• Effective management of hypogammaglobulinemia enabled successful splenectomy without infectious complications.
• The case underscores the potential link between CVID, its management, and the development of MCD.

Implications:

• Timely diagnosis and appropriate treatment of CVID are critical to avoid severe complications such as MCD and splenomegaly.
• This case highlights the importance of maintaining normal IgG levels in CVID patients to mitigate risks.
• Further research may elucidate the pathogenic mechanisms linking CVID and Castleman disease.