Castleman Disease in an HIV-Infected Patient With Kaposi Sarcoma
Multicentric Castleman disease (MCD) is a heterogeneous lymphoproliferative disorder, characterized by systemic symptoms, generalized lymphadenopathy, hepatosplenomegaly, proteinuria, and rash. The clinical course is variable and may range from indolent to aggressive, fulminating in a rapidly fatal illness. Mortality is usually from infective complications and less commonly from malignancies, such as lymphoma or Kaposi sarcoma. The association of concurrent or preceding Castleman disease with Kaposi sarcoma is well documented. Castleman disease developed in a 51-year-old patient with AIDS about 10 months after diagnosis of Kaposi sarcoma. MCD was found to be associated with human herpesvirus 8/Kaposi sarcoma-associated herpesvirus.
In 1956, Benjamin Castleman and his colleagues first described a condition of mediastinal lymphoid hyperplasia with hyalinization of follicles and interfollicular vascular proliferation (now termed the "hyaline-vascular type of angiofollicular lymph node hyperplasia"). The authors later described another type, a plasma cell type, characterized by either hyalinization or hyperplasia of follicles and interfollicular hypervascularity, with sheets of plasma cells in the interfollicular region. The hyaline-vascular type of Castleman disease is usually localized to the mediastinum or, rarely, to the peripheral lymph nodes, with no systemic symptoms. The plasma cell type can be generalized, with a constitutional reaction, anemia, and monoclonal or polyclonal hyperglobulinemia. The plasma cell type of Castleman disease is a systemic immunologic process that can be fatal.
The pathophysiology of Castleman disease was previously related to the dysregulation of the immune system associated with Epstein-Barr virus infection; later investigations showed that the pathophysiology of the disease can be variable. Localized Castleman disease (LCD), usually the plasma cell type, may be associated with HIV infection. Castleman disease is now known to be nearly always associated with the presence of human herpesvirus 8 (HHV-8)/Kaposi sarcoma-associated herpesvirus (KSHV). Also, the association of preceding or concurrent Castleman disease with Kaposi sarcoma has been well documented. Therefore, the relationship among Castleman disease, Kaposi sarcoma, and KSHV/HHV-8 brings new insights into the pathophysiology of this disease. Multicentric Castleman disease (MCD) is usually variable in etiology and generally not the plasma cell type.
Multicentric Castleman disease (MCD) is a heterogeneous lymphoproliferative disorder, characterized by systemic symptoms, generalized lymphadenopathy, hepatosplenomegaly, proteinuria, and rash. The clinical course is variable and may range from indolent to aggressive, fulminating in a rapidly fatal illness. Mortality is usually from infective complications and less commonly from malignancies, such as lymphoma or Kaposi sarcoma. The association of concurrent or preceding Castleman disease with Kaposi sarcoma is well documented. Castleman disease developed in a 51-year-old patient with AIDS about 10 months after diagnosis of Kaposi sarcoma. MCD was found to be associated with human herpesvirus 8/Kaposi sarcoma-associated herpesvirus.
In 1956, Benjamin Castleman and his colleagues first described a condition of mediastinal lymphoid hyperplasia with hyalinization of follicles and interfollicular vascular proliferation (now termed the "hyaline-vascular type of angiofollicular lymph node hyperplasia"). The authors later described another type, a plasma cell type, characterized by either hyalinization or hyperplasia of follicles and interfollicular hypervascularity, with sheets of plasma cells in the interfollicular region. The hyaline-vascular type of Castleman disease is usually localized to the mediastinum or, rarely, to the peripheral lymph nodes, with no systemic symptoms. The plasma cell type can be generalized, with a constitutional reaction, anemia, and monoclonal or polyclonal hyperglobulinemia. The plasma cell type of Castleman disease is a systemic immunologic process that can be fatal.
The pathophysiology of Castleman disease was previously related to the dysregulation of the immune system associated with Epstein-Barr virus infection; later investigations showed that the pathophysiology of the disease can be variable. Localized Castleman disease (LCD), usually the plasma cell type, may be associated with HIV infection. Castleman disease is now known to be nearly always associated with the presence of human herpesvirus 8 (HHV-8)/Kaposi sarcoma-associated herpesvirus (KSHV). Also, the association of preceding or concurrent Castleman disease with Kaposi sarcoma has been well documented. Therefore, the relationship among Castleman disease, Kaposi sarcoma, and KSHV/HHV-8 brings new insights into the pathophysiology of this disease. Multicentric Castleman disease (MCD) is usually variable in etiology and generally not the plasma cell type.
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