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IDIOPATHIC MULTICENTRIC CASTLEMAN DISEASE: TWO CASE REPORTS AND RETROSPECTIVE MEDICAL LITERATURE
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Abstract
Castleman disease (CD) is a rare benign lymph node hyperplasia first described by Benjamin Castleman in the 1950s. CD is characterized by increased numbers of lymphoid follicles with germinal center regression and marked capillary proliferation, including intrafollicular and midfollicular hyperplasia. In 1972, Keller and colleagues classified histopathology Castleman's disease into two types: (1) Hyaline-vascular type and (2) plasma-cell type. Clinically, CD is divided into Unicentric Castleman disease (UCD) and Multicentric Castleman disease (MCD). MCD includes HHV-8-associated MCD and idiopathic MCD (iMCD). iMCD is subclassified into iMCD-TAFRO and iMCD–not otherwise specified (iMCD-NOS). The clinical manifestations of Castleman disease are diverse and non-characteristic, which makes it difficult to diagnose before there is histopathology. In this article, we report 2 iMCD cases. The first case was a 40-year-old female patient who initially showed left migraine, dysphagia and “B symptoms”. After she had CT scanning, tumor in left carotid space and neck lymph nodes were seen. Then she was admitted to the hospital for cervical lymph node biopsy. The second case was a 65-year-old female patient with persistent cough and rash accompanied with cervical, axillary, inguinal lymph nodes and underwent an inguinal lymph node biopsy. Conclusion: iMCD is a major challenge in medicine with its complex clinical features and many differential diagnoses. Excisional lymph nodes biopsy and histopathology is the gold standard for accurate diagnosis. The laboratory test and diagnostic imaging will help to sub-classify the disease.
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Keywords
Castleman disease, lymph nodes, lymphocyte hyperplasia, human herpes virus 8.
References
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