Castleman's disease

Castleman disease: surgical cure in pediatric patients

“…Castleman disease can be subclassified into 2 clinical subtypes-multicentric and localized [5][6][7]. Castleman disease can be further classified based on histopathologic features into hyaline-vascular and plasma-cell variants [3].…”

“…It has also been generally appreciated that in children the disease is most often unicentric [3][4][5][6][7]. Patients who present with a solitary mass typically follow a more benign course.…”

“…Multicentric Castleman disease is mostly of the plasma-cell variant and more commonly presents with a systemic illness with lymphadenopathy, fatigue, fever, weight loss, autoimmune abnormalities, recurrent infections, and laboratory abnormalities such as anemia, hypoalbuminemia, hypergammaglobulinemia, and elevated ESR [3][4][5][6]. The most common location for localized Castleman disease has classically been thought to be the thorax; however, other case series have found that cervical, abdominal, and axillary lymphadenopathy are equally common [3][4][5][6][7][8]. The multicentric plasma-cell variant is the least commonly encountered (10%-20% of cases) but presents with the most systemic manifestations [3,7].…”

“…In contrast to the localized form, multicentric Castleman disease may be chronic or even malignant, rapidly progressive, and fatal. Death in multicentric cases may be caused by sepsis, systemic inflammation leading to multiorgan failure, or the development of malignancy, most commonly lymphoma [3][4][5][6][7].…”

“…Disease activity is followed by serum IgG, C reactive protein, ESR, and lactate dehydrogenase. Multicentric disease is rare in the pediatric population but carries a more favorable prognosis with a good response to steroids [4][5][6][7][8][9][10].…”