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Splenectomy induced complete remission in a patient with multicentric Castleman's disease and autoimmune hemolytic
R Lerza1, G Castello, M Truini
1Cattedra di Clinica Medica R., Dipartimento di Medicina Interna, Università di Genova, Italy.
Insights
Castleman disease (CD) is a rare lymphoid disorder. Splenectomy effectively treated a patient with multicentric Castleman disease (MCD) and severe immunohemolytic anemia when other treatments failed.
Area of Science:
- Hematology
- Oncology
- Immunology
Background:
- Castleman disease (CD) is a rare lymphoid proliferative disorder.
- Multicentric Castleman disease (MCD) presents with severe symptoms and a poor prognosis, often mimicking lymphoma.
- The etiology, pathogenesis, and optimal treatment for MCD remain unclear.
Observation:
- A 57-year-old patient with MCD developed severe immunohemolytic anemia.
- Initial treatment with steroids and chemotherapy yielded only partial clinical and laboratory responses.
- The patient underwent splenectomy.
Findings:
- Splenectomy resulted in complete remission of hemolysis.
- Post-splenectomy, the patient experienced resolution of constitutional symptoms.
- Generalized lymphadenopathies resolved after splenectomy.
Implications:
- Splenectomy may be a viable therapeutic option for MCD complicated by severe immunohemolytic anemia.
- This case highlights the potential benefits of splenectomy in refractory MCD.
- Further research is warranted to elucidate the role of splenectomy in MCD management.
Abstract:
Castleman's disease (CD) is a rare disorder of the lymphoid tissue in which the clinical manifestations often mimic a malignant lymphoma. Despite the absence of monoclonality of the lymphoid proliferation, the multicentric variant of the disease (MCD) is characterized by severe symptoms and poor prognosis. Etiologic, pathogenetic, and therapeutic aspects of MCD are still uncertain. We report the case of a 57-year-old patient affected by MCD complicated by severe immunohemolytic anemia. Whereas the clinical and laboratory response to steroids and chemotherapeutic agents was only partial, splenectomy induced a complete remission of hemolysis and disappearance of the constitutional symptoms and of all generalized lymphadenopathies.
