Multicentric Castleman's disease in a renal allograft recipient: a case report and literature review

Abstract

Giant lymph node hyperplasia is the main symptom of Castleman’s disease (CD), which is a rare and easily overlooked lymphoproliferative disease that mimics both benign and malignant lesions. Several cases of CD after renal transplantation have been reported in the literature. A 43-year-old man was admitted to our medical center with high serum globulin levels after receiving a living donor kidney transplant. A lymph node biopsy raised suspicion for multicentric CD. Because of the poor therapeutic effect of reduction of immunosuppression, the patient was treated with CHOP chemotherapy comprising four cycles of monthly cyclophosphamide (750 g/m², day 1), vincristine (1.4 g/m², day 1), doxorubicin (50 g/m², day 1), and prednisone (60 mg/m², daily). Following the treatment, his serum globulin levels decreased to the normal range. At the 2-year follow-up examination, the abdominal, axillary, and inguinal lymph nodes had significantly decreased in size. Without a pathological diagnosis, multicentric CD after renal transplantation can be easily ignored and misdiagnosed. If the clinical signs cannot be relieved by minimizing the dose of common immunosuppressants, the CHOP regimen may be a better option. Biological agents may be added in patients with positive immunohistochemistry staining and good economic conditions.

Keywords: Castleman’s disease; Transplantation; hyperglobulinemia; lymphadenopathy; post-transplant lymphoproliferative disorders; renal transplantation.

Figure 1.

Immunohistochemical staining of lymph nodes from biopsy. (a) Reactive lymphoid tissue hyperplasia in the left inguinal lymph nodes. (b) Plasma cell proliferation in the abdominal lymph nodes. (c–h) Positive stains in the abdominal lymph nodes, including CD3, CD20, CD38, CD138, kappa light chains, and lambda antibodies.

Figure 2.

Size of the abdominal lymph nodes detected by ultrasound examination. (a) An enlarged left inguinal lymph node with a diameter of 46 × 18 mm. (b) The left enlarged inguinal lymph node shrank after treatment, decreasing to a diameter of 15 × 9 mm.

Figure 3.

Dynamic changes of serum immunoglobulins before and after treatment. Ig, immunoglobulin