Multicentric vs. Unresectable Unicentric Castleman Disease with Active Presentation: An Orphan Rare Disease in a Young Egyptian Female Patient. A Case Report
Shaaban, Y., El-Ashwah, S., Elshamy, A., Atef, M., Saafan, M., E. Eladl, A., El-Sherpiny, S., Hendawy, S., Elbogdady, A., El Bogdady, M., Atef, B. (2023). Multicentric vs. Unresectable Unicentric Castleman Disease with Active Presentation: An Orphan Rare Disease in a Young Egyptian Female Patient. A Case Report. EKB Journal Management System, 19(1), 13-19. doi: 10.21608/resoncol.2023.179779.1187
Yasmine Shaaban; Shaimaa El-Ashwah; Alaa Elshamy; Manal Atef; Maged Saafan; Ahmed E. Eladl; Sarah El-Sherpiny; Shaimaa R. Hendawy; Aya Elbogdady; Mohamed El Bogdady; Basma Atef. "Multicentric vs. Unresectable Unicentric Castleman Disease with Active Presentation: An Orphan Rare Disease in a Young Egyptian Female Patient. A Case Report". EKB Journal Management System, 19, 1, 2023, 13-19. doi: 10.21608/resoncol.2023.179779.1187
Shaaban, Y., El-Ashwah, S., Elshamy, A., Atef, M., Saafan, M., E. Eladl, A., El-Sherpiny, S., Hendawy, S., Elbogdady, A., El Bogdady, M., Atef, B. (2023). 'Multicentric vs. Unresectable Unicentric Castleman Disease with Active Presentation: An Orphan Rare Disease in a Young Egyptian Female Patient. A Case Report', EKB Journal Management System, 19(1), pp. 13-19. doi: 10.21608/resoncol.2023.179779.1187
Shaaban, Y., El-Ashwah, S., Elshamy, A., Atef, M., Saafan, M., E. Eladl, A., El-Sherpiny, S., Hendawy, S., Elbogdady, A., El Bogdady, M., Atef, B. Multicentric vs. Unresectable Unicentric Castleman Disease with Active Presentation: An Orphan Rare Disease in a Young Egyptian Female Patient. A Case Report. EKB Journal Management System, 2023; 19(1): 13-19. doi: 10.21608/resoncol.2023.179779.1187

Multicentric vs. Unresectable Unicentric Castleman Disease with Active Presentation: An Orphan Rare Disease in a Young Egyptian Female Patient. A Case Report

Research in Oncology
Article 3, Volume 19, Issue 1, June 2023, Page 13-19  XML PDF (427.04 K)
Document Type: Case reports
DOI: 10.21608/resoncol.2023.179779.1187
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Authors
Yasmine Shaaban email orcid 1; Shaimaa El-Ashwah1; Alaa Elshamy2; Manal Atef3; Maged Saafan1; Ahmed E. Eladl4; Sarah El-Sherpiny4; Shaimaa R. Hendawy5; Aya Elbogdady6; Mohamed El Bogdady7; Basma Atef7
1Clinical Hematology Unit, Internal Medicine Department, Oncology Center of Mansoura University (OCMU), Faculty of Medicine, Mansoura University, Mansoura, Egypt
2Clinical Hematology Department, El Sheikh Zayed Specialized Hospital, Giza, Egypt
3Clinical Hematology Department, Dar El Salam Cancer Center, Cairo, Egypt
4Pathology Department, Faculty of Medicine, Mansoura University, Mansoura, Egypt
5Clinical Pathology Department, Oncology Center of Mansoura University (OCMU), Faculty of Medicine, Mansoura University, Mansoura, Egypt
6Radiology Department, Faculty of Medicine, Mansoura University, Mansoura, Egypt
7Clinical Hematology Unit, Internal Medicine Department, Oncology Center of Mansoura University (OCMU), Faculty of Medicine, Mansoura University, Mansoura, Egypt;
Abstract
Background: Castleman disease (CD) is a rare disorder that affects lymph nodes and has a wide range of associated symptoms. The affected lymph nodes show characteristic histological picture. Most of the unicentric Castleman disease (UCD) cases can be cured by complete surgical removal or radiotherapy, while multicentric CD (MCD) is much more complicated and have several subtypes and requires more effort to reach a precise diagnosis and management.
Case presentation: A 17-years old female presented with sever fatigue and abdominal pain. Massive mediastinal lymphadenopathy was detected on radiological studies. Pathology confirmed a plasma cell variant of MCD. Autoimmune disorders, overlapping IgG4-related disease, TAFRO (Thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly) syndrome and other malignancies were excluded after a series of investigations. She was HIV-negative, and the human herpes virus-8 status was unknown. The final diagnosis of idiopathic MCD-not otherwise specified (iMCD-NOS) was reached. She showed a very good response to corticosteroids and monoclonal antibody course of treatment. Radiological investigations showed marked regression of the lymph node mass, and there was complete resolution of her symptoms and normalization of the hematological and biochemical parameters.
Conclusion: The diagnosis and management of MCD remain very challenging, and the exclusion of infectious, autoimmune, and neoplastic disorders is necessary.
Keywords
Castleman disease; Idiopathic; Mediastinal lymphadenopathy; Multicentric; Rituximab
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