@article{10.11648/j.crj.20200804.16,
  author = {Asmaa Hamoda and Hanaa Rashad and Ola Ahmad and Hala Reda and Iman Zaki and Naglaa Elkinaai and Mohamed Sedki and Alaa El Hadad and Samah Semary},
  title = {Castleman Disease in Children from Histopathology to Therapy},
  journal = {Cancer Research Journal},
  volume = {8},
  number = {4},
  pages = {100-103},
  doi = {10.11648/j.crj.20200804.16},
  url = {https://doi.org/10.11648/j.crj.20200804.16},
  eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.crj.20200804.16},
  abstract = {Background: Castleman disease (CD) describes a group of rare lymphoproliferative disorder with characteristic histopathology. It presents with heterogeneous clinical features whether unicentric (UC) or multicentric disease (MCD). The aim of the work was to describe clinic-pathological characteristics, management and outcome of different types of castleman disease in CCHE. It is a retrospective study, all children with (CD) treated from July 2007 till end of 2017 were analyzed as regard diagnosis, management and outcome. The results showed that, twelve patients with a median age of 11.5 years (ranging from 4 - to 17 years) were enrolled. Eight of them (66.6%) were males and 4 (33.3%) were females (33.3%). Histopathology was either hyaline vascular in 8 patients (66.6%) or plasma cell variant in 4 patients (33.3%). Nine patients (75%) had uni-centric disease (UC) with lymphadenopathy. Three patients (25%) were multi-centric disease (MCD); out of them one patient had immune bi-cytopenia, small intestinal thickening and splenomegaly and another one had extensive pulmonary involvement and respiratory distress, while the third patient had pleural effusion and ascites. HIV antibody was negative for all patients. Regarding the three MCD patients, IL-6 was normal in one patient, and extremely high in second one, while it hasn’t been done for the third patient. All UC patients underwent surgical excision and are alive with no active disease till now. All MCD patients received systemic steroids as initial therapy, followed by R- CHOP (Rituximab-cyclophosphamide-doxorubicin-vincristine-prednisone) as salvage therapy, except for one patient with pulmonary involvement who didn’t achieved clinical response and received single agent Rituximab with Anti IL-6. Unfortunately this patient is still having uncontrolled systemic manifestation, while the other two patients showed partial response. In conclusion, Uni-centric castleman disease is a localized surgically cured disease. MCD treatment remains challenging, and the outcome is controversial, so Uniform treatment guidelines are mandatory.},
 year = {2020}
}

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TY  - JOUR
T1  - Castleman Disease in Children from Histopathology to Therapy
AU  - Asmaa Hamoda
AU  - Hanaa Rashad
AU  - Ola Ahmad
AU  - Hala Reda
AU  - Iman Zaki
AU  - Naglaa Elkinaai
AU  - Mohamed Sedki
AU  - Alaa El Hadad
AU  - Samah Semary
Y1  - 2020/12/22
PY  - 2020
N1  - https://doi.org/10.11648/j.crj.20200804.16
DO  - 10.11648/j.crj.20200804.16
T2  - Cancer Research Journal
JF  - Cancer Research Journal
JO  - Cancer Research Journal
SP  - 100
EP  - 103
PB  - Science Publishing Group
SN  - 2330-8214
UR  - https://doi.org/10.11648/j.crj.20200804.16
AB  - Background: Castleman disease (CD) describes a group of rare lymphoproliferative disorder with characteristic histopathology. It presents with heterogeneous clinical features whether unicentric (UC) or multicentric disease (MCD). The aim of the work was to describe clinic-pathological characteristics, management and outcome of different types of castleman disease in CCHE. It is a retrospective study, all children with (CD) treated from July 2007 till end of 2017 were analyzed as regard diagnosis, management and outcome. The results showed that, twelve patients with a median age of 11.5 years (ranging from 4 - to 17 years) were enrolled. Eight of them (66.6%) were males and 4 (33.3%) were females (33.3%). Histopathology was either hyaline vascular in 8 patients (66.6%) or plasma cell variant in 4 patients (33.3%). Nine patients (75%) had uni-centric disease (UC) with lymphadenopathy. Three patients (25%) were multi-centric disease (MCD); out of them one patient had immune bi-cytopenia, small intestinal thickening and splenomegaly and another one had extensive pulmonary involvement and respiratory distress, while the third patient had pleural effusion and ascites. HIV antibody was negative for all patients. Regarding the three MCD patients, IL-6 was normal in one patient, and extremely high in second one, while it hasn’t been done for the third patient. All UC patients underwent surgical excision and are alive with no active disease till now. All MCD patients received systemic steroids as initial therapy, followed by R- CHOP (Rituximab-cyclophosphamide-doxorubicin-vincristine-prednisone) as salvage therapy, except for one patient with pulmonary involvement who didn’t achieved clinical response and received single agent Rituximab with Anti IL-6. Unfortunately this patient is still having uncontrolled systemic manifestation, while the other two patients showed partial response. In conclusion, Uni-centric castleman disease is a localized surgically cured disease. MCD treatment remains challenging, and the outcome is controversial, so Uniform treatment guidelines are mandatory.
VL  - 8
IS  - 4
ER  - 

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