Castleman's disease (CD) is a
rare disease that has clinical and pathological similarities to
lymphoma and is characterized by a high frequency of associated immunological dysfunction. ImmunoglobulinG4-related disease (IgG4-RD) is a collection of systemic disorders that affect numerous organs and are also referred to as IgG4-associated sclerosing diseases. CD and
IgG4-RD are difficult to separate because they may manifest similar commin clinical features.
CASE PRESENTATION: This case describes a 53-year-old female who, during routine medical check-up, exhibited a progressive increase in
serum globulin levels and a simultaneous worsening of
anemia symptoms, raising concern for a clonal plasma cell disease such as myeloma. However, bone marrow
punctures did not reveal any abnormal plasma cells. Also, serum and urine immunofixation electrophoresis demonstrated no abnormal monoclonal
protein bands. In addition, several laboratory findings excluded chronic
liver disease,
chronic infections caused by bacteria or viruses. Later, we found elevated serum
IgG4 levels (10,700 mg/L), and identified multiple enlarged lymph nodes throughout the patient's body. Axillary lymph node aspiration revealed no abnormal lymphocytes, ruling out the possibility of
lymphoma. Pathological morphology of the axillary lymph revealed a large number of plasma cells in the lymphatic follicles. In addition, there was a reduction in lymphatic follicle size and apoptosis of the germinal centres. Immunohistochemistry revealed
IgG4+/IgG + in > 40% of cells, and more than 100 IgG4 + cells per high powered field (HPF) of specimen. As of now, finding strongly suggested
IgG4-RD. This patient was treated with
glucocorticoids and various immunosuppressive drugs, such as
prednisone,
cyclosporine,
methotrexate,
cyclophosphamide,
mycophenolate mofetil,
azathioprine and
hydroxychloroquine. Unfortunately, the patient did not recover. Ultimately, idiopathic multicentric
Castleman disease (iMCD) was diagnosed in relation to the patient's clinical presentation and laboratory tests, and after
combination chemotherapy (VCD:
Bortezomib,
Cyclophosphamide and
Dexamethasone), durable remission was achieved without serious adverse effects. During the follow-up period of one year and ten months, the patient remained stable.
CONCLUSION: