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

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Last updated: 01 Jan 2025

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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.

DOI

10.21608/resoncol.2023.179779.1187

Keywords

Castleman disease, Idiopathic, Mediastinal lymphadenopathy, Multicentric, Rituximab

Authors

First Name

Yasmine

Last Name

Shaaban

MiddleName

-

Affiliation

Clinical Hematology Unit, Internal Medicine Department, Oncology Center of Mansoura University (OCMU), Faculty of Medicine, Mansoura University, Mansoura, Egypt

Email

yasmineessam@mans.edu.eg

City

-

Orcid

0000-0002-0523-7651

First Name

Shaimaa

Last Name

El-Ashwah

MiddleName

-

Affiliation

Clinical Hematology Unit, Internal Medicine Department, Oncology Center of Mansoura University (OCMU), Faculty of Medicine, Mansoura University, Mansoura, Egypt

Email

shaimaasaber@mans.edu.eg

City

-

Orcid

-

First Name

Alaa

Last Name

Elshamy

MiddleName

-

Affiliation

Clinical Hematology Department, El Sheikh Zayed Specialized Hospital, Giza, Egypt

Email

2laanabil@gmail.com

City

-

Orcid

-

First Name

Manal

Last Name

Atef

MiddleName

-

Affiliation

Clinical Hematology Department, Dar El Salam Cancer Center, Cairo, Egypt

Email

dr.manalatif@hotmail.com

City

-

Orcid

-

First Name

Maged

Last Name

Saafan

MiddleName

-

Affiliation

Clinical Hematology Unit, Internal Medicine Department, Oncology Center of Mansoura University (OCMU), Faculty of Medicine, Mansoura University, Mansoura, Egypt

Email

maged.ebrahim90@yahoo.com

City

-

Orcid

-

First Name

Ahmed

Last Name

E. Eladl

MiddleName

-

Affiliation

Pathology Department, Faculty of Medicine, Mansoura University, Mansoura, Egypt

Email

ah_eladl@hotmail.com

City

-

Orcid

-

First Name

Sarah

Last Name

El-Sherpiny

MiddleName

-

Affiliation

Pathology Department, Faculty of Medicine, Mansoura University, Mansoura, Egypt

Email

sara_ah_123@yahoo.com

City

-

Orcid

-

First Name

Shaimaa

Last Name

Hendawy

MiddleName

R.

Affiliation

Clinical Pathology Department, Oncology Center of Mansoura University (OCMU), Faculty of Medicine, Mansoura University, Mansoura, Egypt

Email

shoshooo_dok@yahoo.com

City

-

Orcid

-

First Name

Aya

Last Name

Elbogdady

MiddleName

-

Affiliation

Radiology Department, Faculty of Medicine, Mansoura University, Mansoura, Egypt

Email

ayabogdady@gmail.com

City

-

Orcid

-

First Name

Mohamed

Last Name

El Bogdady

MiddleName

-

Affiliation

Clinical Hematology Unit, Internal Medicine Department, Oncology Center of Mansoura University (OCMU), Faculty of Medicine, Mansoura University, Mansoura, Egypt;

Email

m_elbogdady@hotmail.com

City

-

Orcid

-

First Name

Basma

Last Name

Atef

MiddleName

-

Affiliation

Clinical Hematology Unit, Internal Medicine Department, Oncology Center of Mansoura University (OCMU), Faculty of Medicine, Mansoura University, Mansoura, Egypt;

Email

basma.atef@mans.edu.eg

City

-

Orcid

-

Volume

19

Article Issue

1

Related Issue

42511

Issue Date

2023-06-01

Receive Date

2022-12-26

Publish Date

2023-06-01

Page Start

13

Page End

19

Print ISSN

2357-0687

Online ISSN

2357-0695

Article File

RESONCOL_Volume 19_Issue 1_Pages 13-19.pdf

PDF . 437.3kB

Link

https://resoncol.journals.ekb.eg/article_289726.html

Detail API

https://resoncol.journals.ekb.eg/service?article_code=289726

Order

3

Type

Case reports

Type Code

287

Publication Type

Journal

Publication Title

Research in Oncology

Publication Link

https://resoncol.journals.ekb.eg/

MainTitle

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

Details

Type

Article

Created At

30 Dec 2024