Castle man disease: a case report and review of the literature

Abstract

The Castleman disease (CD) is a rare disease of unknown etiology, characterized histologically by angiofollicular lymphoid hyperplasia. It comes in two forms, unicentric and multicentric. We report a case of Castleman disease in a 58 year old man, who had consulted for chronic cervical lymphadenopathy. This case was a multicenteric and rapidly fatal despite aggressive treatment with corticosteroids, and chemotherapy

Multicentric Plasma-Cell Type Castleman Disease Masquerading As Hodgkin Lymphoma: A Case Report

“… 6 9 18 24 These constitutional symptoms are considered a consequence of elevated chemical mediators of inflammation, namely: IL6, interleukin-2 (IL2), and vascular endothelial growth factor (VEGF). 1 6 8 9 18 20 25 These constitutional symptoms include: pallor, chronic diarrhea, asthenia, fever, weight loss, generalized lymphadenopathy, edema (sometimes ascites and/or anasarca), and hepatosplenomegaly. 1 6 8 9 18 The deranged laboratory findings include: anemia, thrombocytosis or thrombocytopenia, hypoalbuminemia, hypocholesterolemia, hypergammaglobulinemia, proteinuria, increased acute phase reactants (such as serum C-reactive protein), and increased chemical mediators of inflammation levels (such as IL6, IL2, and VEGF).…”

“…Interestingly, it is of note that though MCD is associated with HIV and HHV-8 infection, with some studies even demonstrating the presence of the HHV-8 sequence in approximately 60 to 100% of patients infected with HIV and 20 to 41% in those who were not, our index patient was HIV negative and her IHC evaluation was also negative for HHV-8 expression antibodies through the PAX-5 surrogate marker (even though direct serum HHV-8 antibodies could not be done for her). 1 14 15 25 The gold standard for our diagnosis in this case was a combination of routine histopathological and IHC evaluations of her cervical lymph node biopsy. The application of IHC as an ancillary technique in this case was especially critical in deciphering this case given the diagnostic dilemma initially presented by her overlapping clinical features and the initial Hodgkin’s lymphoma pathologic diagnosis; thus IHC evaluation in combination with routine histopathological evaluations has been recommended as a standard method for lymph node examination especially where routine histopathological evaluation is nonspecific.…”

Complete resection of unicentric Castleman disease in the superior mediastinum: A case report

“…UCD characteristically develops in the third and fourth decades of life [3] and is usually is asymptomatic with a benign course [2] , [4] . Imaging examinations such as CT, magnetic resonance imaging, and positron emission tomography/CT are usually performed; however, it is difficult to achieve a definitive diagnosis by imaging.…”

“…MCD commonly presents in the sixth decade of life; however, individuals with HIV infection tend to develop it at a younger age [3] . MCD is usually associated with an immunodeficient states such as those associated with HIV or human herpes virus 8 infection [3] , [4] , [8] . Patients with MCD characteristically develop various symptoms, such as fever and weight loss.…”

“…Treatment generally involves chemotherapy, antiviral medication, or antiproliferative regimens such as anti-interleukin 6 receptor or IL-6 therapy [2] . However, despite these treatments, 70% of patients with MCD die of infective complications and 30% of cancer [4] . The median survival following a diagnosis of MCD reportedly ranges from 14 to 30 months [8] .…”