Castleman Disease: Insights from a Case Series and Literature Review
Castleman disease (CD), a rare lymphoproliferative disorder with unicentric (UCD) and multicentric (MCD) subtypes, presents significant diagnostic and therapeutic challenges due to its clinical heterogeneity. This case series of four patients highlights rare and diverse clinical presentations of CD,...
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| Format: | Article |
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Thieme Medical and Scientific Publishers Pvt. Ltd.
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| Series: | Indian Journal of Medical and Paediatric Oncology |
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| Online Access: | http://www.thieme-connect.de/DOI/DOI?10.1055/s-0045-1810074 |
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| author | Jerin James Sanudev Sadanandan Vadakke Puthiyottil Johan George Arathi Edayattil Vivek Mathew James Swapna Dominic Supriya N. K. |
| author_facet | Jerin James Sanudev Sadanandan Vadakke Puthiyottil Johan George Arathi Edayattil Vivek Mathew James Swapna Dominic Supriya N. K. |
| author_sort | Jerin James |
| collection | DOAJ |
| description | Castleman disease (CD), a rare lymphoproliferative disorder with unicentric (UCD) and multicentric (MCD) subtypes, presents significant diagnostic and therapeutic challenges due to its clinical heterogeneity. This case series of four patients highlights rare and diverse clinical presentations of CD, including atypical anatomical sites and paraneoplastic pemphigus. Diagnostic challenges included differentiating CD from malignancies or autoimmune disorders, emphasizing the critical role of histopathology. UCD pathogenesis may involve follicular dendritic cell mutations, whereas MCD subtypes are driven by human herpesvirus (HHV)-8-associated viral interleukin-6 or idiopathic cytokine dysregulation. Treatment strategies varied, with surgery preferred for UCD and immunomodulators (siltuximab, rituximab) for MCD. Challenges such as limited standardized protocols in resource-constrained settings and biomarker variability underscore the need for individualized therapy. Emerging approaches, including cytokine-targeted therapies and bortezomib, show promise for refractory cases. This study reinforces the importance of multidisciplinary collaboration, early histopathological diagnosis, and long-term monitoring to optimize outcomes. By integrating clinical experiences with literature, it advocates for refined diagnostic criteria and context-specific therapeutic algorithms, urging further research to address gaps in managing this complex disease. |
| format | Article |
| id | doaj-art-27c38fcdb2354d3ba0f68d3d9ebe4e0d |
| institution | Kabale University |
| issn | 0971-5851 0975-2129 |
| language | English |
| publisher | Thieme Medical and Scientific Publishers Pvt. Ltd. |
| record_format | Article |
| series | Indian Journal of Medical and Paediatric Oncology |
| spelling | doaj-art-27c38fcdb2354d3ba0f68d3d9ebe4e0d2025-08-20T03:51:29ZengThieme Medical and Scientific Publishers Pvt. Ltd.Indian Journal of Medical and Paediatric Oncology0971-58510975-212910.1055/s-0045-1810074Castleman Disease: Insights from a Case Series and Literature ReviewJerin James0https://orcid.org/0009-0004-2999-0391Sanudev Sadanandan Vadakke Puthiyottil1https://orcid.org/0000-0002-0105-2705Johan George2Arathi Edayattil3Vivek Mathew James4Swapna Dominic5Supriya N. K.6Department of Medical Oncology, Government Medical College, Kozhikode, Kerala, IndiaDepartment of Medical Oncology, Government Medical College, Kozhikode, Kerala, IndiaDepartment of Medical Oncology, Government Medical College, Kozhikode, Kerala, IndiaDepartment of Medical Oncology, Government Medical College, Kozhikode, Kerala, IndiaDepartment of Nuclear Medicine, Government Medical College, Kozhikode, Kerala, IndiaDepartment of Dermatology, Government Medical College, Kozhikode, Kerala, IndiaDepartment of Pathology, Government Medical College, Kozhikode, Kerala, IndiaCastleman disease (CD), a rare lymphoproliferative disorder with unicentric (UCD) and multicentric (MCD) subtypes, presents significant diagnostic and therapeutic challenges due to its clinical heterogeneity. This case series of four patients highlights rare and diverse clinical presentations of CD, including atypical anatomical sites and paraneoplastic pemphigus. Diagnostic challenges included differentiating CD from malignancies or autoimmune disorders, emphasizing the critical role of histopathology. UCD pathogenesis may involve follicular dendritic cell mutations, whereas MCD subtypes are driven by human herpesvirus (HHV)-8-associated viral interleukin-6 or idiopathic cytokine dysregulation. Treatment strategies varied, with surgery preferred for UCD and immunomodulators (siltuximab, rituximab) for MCD. Challenges such as limited standardized protocols in resource-constrained settings and biomarker variability underscore the need for individualized therapy. Emerging approaches, including cytokine-targeted therapies and bortezomib, show promise for refractory cases. This study reinforces the importance of multidisciplinary collaboration, early histopathological diagnosis, and long-term monitoring to optimize outcomes. By integrating clinical experiences with literature, it advocates for refined diagnostic criteria and context-specific therapeutic algorithms, urging further research to address gaps in managing this complex disease.http://www.thieme-connect.de/DOI/DOI?10.1055/s-0045-1810074case reportCastleman diseaseunicentric CDmulticentric CDhuman herpesvirus 8 |
| spellingShingle | Jerin James Sanudev Sadanandan Vadakke Puthiyottil Johan George Arathi Edayattil Vivek Mathew James Swapna Dominic Supriya N. K. Castleman Disease: Insights from a Case Series and Literature Review Indian Journal of Medical and Paediatric Oncology case report Castleman disease unicentric CD multicentric CD human herpesvirus 8 |
| title | Castleman Disease: Insights from a Case Series and Literature Review |
| title_full | Castleman Disease: Insights from a Case Series and Literature Review |
| title_fullStr | Castleman Disease: Insights from a Case Series and Literature Review |
| title_full_unstemmed | Castleman Disease: Insights from a Case Series and Literature Review |
| title_short | Castleman Disease: Insights from a Case Series and Literature Review |
| title_sort | castleman disease insights from a case series and literature review |
| topic | case report Castleman disease unicentric CD multicentric CD human herpesvirus 8 |
| url | http://www.thieme-connect.de/DOI/DOI?10.1055/s-0045-1810074 |
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