Castleman Disease in Children from Histopathology to Therapy

Asmaa Hamoda,Alaa El Hadad,Mohamed Sedki,Ola Ahmad,Samah Semary,Hala Reda,Hanaa Rashad,Naglaa Elkinaai,Iman Zaki

doi: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.

Highlights

IntroductionCastleman disease (CD) is described as a group of heterogeneous hematologic disorders which involve a spectrum of lymph node histopathology consisting of atrophied germinal centers with hypervascularization (hyaline vascular/hypervascular histopathological subtype) on the other hand the histopathology may consisting of hyperplastic germinal centers concomitant with polytypic plasmacytosis (plasmacytic histopathological subtype) & mixed type [1]
Castleman disease (CD) is described as a group of heterogeneous hematologic disorders which involve a spectrum of lymph node histopathology consisting of atrophied germinal centers with hypervascularization on the other hand the histopathology may consisting of hyperplastic germinal centers concomitant with polytypic plasmacytosis & mixed type [1]
Castleman disease is classified to two groups according to the affected region, Unicentric CD (UCD) affecting a single region of enlarged lymph nodes with diagnostic histopathology and causing mild symptoms, which can be treated with excision of the enlarged lymph node

Summary

Introduction

Castleman disease (CD) is described as a group of heterogeneous hematologic disorders which involve a spectrum of lymph node histopathology consisting of atrophied germinal centers with hypervascularization (hyaline vascular/hypervascular histopathological subtype) on the other hand the histopathology may consisting of hyperplastic germinal centers concomitant with polytypic plasmacytosis (plasmacytic histopathological subtype) & mixed type [1]. 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. All MCD patients received systemic steroids as initial therapy, followed by R- CHOP (Rituximab-cyclophosphamide-doxorubicinvincristine-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. This patient is still having uncontrolled systemic manifestation, while the other two patients showed partial response. MCD treatment remains challenging, and the outcome is controversial, so Uniform treatment guidelines are mandatory

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