Castleman Disease in Children
Definition & Epidemiology
- Rare lymphoproliferative disorder characterized by abnormal lymph node enlargement
- Peak incidence: Adolescence and early adulthood
- No gender predilection
- Incidence: approximately 21-25 cases per million person-years
Pathophysiology
- Key pathogenic mechanisms:
- Dysregulated production of IL-6
- HHV-8 infection (in some cases)
- VEGF overexpression
- Immune system dysregulation
- Associated conditions:
- HIV infection
- Autoimmune disorders
- POEMS syndrome
Disease Classification
Histopathological Variants
- Hyaline Vascular Type (HVV)
- Most common in children (90%)
- Characterized by:
- Hyalinized follicles
- "Lollipop" formations
- Increased vascularity
- Plasma Cell Type (PCV)
- Less common in children
- Features:
- Sheets of mature plasma cells
- Preserved lymph node architecture
- Mixed Type
- Shows features of both HVV and PCV
- Intermediate prognosis
Clinical Subtypes
- Unicentric Castleman Disease (UCD)
- Single lymph node station
- Usually HVV type
- Better prognosis
- Multicentric Castleman Disease (MCD)
- Multiple lymph node regions
- Usually plasma cell or mixed type
- Subtypes:
- HHV-8 positive
- HHV-8 negative/idiopathic (iMCD)
Clinical Manifestations
Unicentric Disease
- Local symptoms:
- Single enlarged lymph node/mass
- Compression symptoms
- Usually asymptomatic
- Common locations:
- Thorax (70%)
- Neck (14%)
- Abdomen (12%)
- Axilla (4%)
Multicentric Disease
- Systemic symptoms:
- Fever
- Night sweats
- Weight loss
- Fatigue
- Growth failure
- Laboratory abnormalities:
- Anemia
- Thrombocytopenia
- Elevated inflammatory markers
- Hypergammaglobulinemia
- Organ involvement:
- Hepatosplenomegaly
- Fluid retention
- Skin manifestations
- Renal dysfunction
Diagnostic Approach
Initial Evaluation
- Laboratory studies:
- Complete blood count
- Comprehensive metabolic panel
- LDH, CRP, ESR
- IL-6 levels
- HIV testing
- HHV-8 testing
- Imaging:
- CT with contrast
- PET-CT for staging
- MRI for specific locations
Definitive Diagnosis
- Excisional lymph node biopsy
- Essential for histological classification
- Immunohistochemistry
- Flow cytometry
- Diagnostic criteria for iMCD:
- Major criteria (2 required):
- Characteristic histopathology
- Multicentric lymphadenopathy
- Minor criteria (2-3 required):
- Elevated CRP/ESR
- Anemia
- Thrombocytopenia/thrombocytosis
- Constitutional symptoms
- Organomegaly
- Major criteria (2 required):
Case-1
A male child, at the age of 13 years presented with a mass in the anterior part of the neck
CT scan showed that the neck mass, a multiple lymph node enlargement, with an high contrast-enhancement, originated in the anterior-superior mediastinum and cause a serious right dislocation of the trachea (A-D). (source)
Histology showed small germinal center depleted of follicular center B cells, and penetration by hyalinized venules (A); follicular dendritic cell were evidenced by their strong immunoreactivity for CD 35 (B); immunoreactivity for CD20 (C) and CD3 (D) was strongly positive. (source)
Treatment Approach
Unicentric Disease
- Primary treatment:
- Complete surgical excision (curative)
- Radiation therapy if unresectable
- Follow-up:
- Regular monitoring
- Imaging surveillance
Multicentric Disease
- First-line therapy:
- Anti-IL-6 therapy:
- Siltuximab
- Tocilizumab
- HHV-8 positive MCD:
- Rituximab
- Antiviral therapy
- Anti-IL-6 therapy:
- Second-line options:
- Chemotherapy:
- CHOP protocol
- CVP regimen
- Immunomodulators:
- Thalidomide
- Sirolimus
- Corticosteroids
- Chemotherapy:
Supportive Care
- Management of complications
- Growth monitoring
- Psychosocial support
- Regular assessment of disease activity
- Prevention of infections
Castleman Disease in Children
- What is another name for Castleman Disease?
Answer: Giant lymph node hyperplasia or angiofollicular lymph node hyperplasia - Who first described Castleman Disease?
Answer: Dr. Benjamin Castleman - What are the two main clinical types of Castleman Disease?
Answer: Unicentric (localized) and Multicentric - Which type of Castleman Disease is more common in children?
Answer: Unicentric (localized) Castleman Disease - What are the three main histological variants of Castleman Disease?
Answer: Hyaline vascular, plasma cell, and mixed type - Which histological variant is most common in pediatric Castleman Disease?
Answer: Hyaline vascular type - What is the most common site of involvement in unicentric Castleman Disease in children?
Answer: Mediastinum - Is Castleman Disease considered a neoplastic or reactive condition?
Answer: Generally considered a lymphoproliferative disorder, with some debate about its nature - What virus is associated with some cases of multicentric Castleman Disease?
Answer: Human Herpesvirus 8 (HHV-8) - Is HHV-8 commonly associated with unicentric Castleman Disease in children?
Answer: No, it's rarely associated with unicentric disease - What cytokine plays a key role in the pathogenesis of Castleman Disease?
Answer: Interleukin-6 (IL-6) - What are the typical symptoms of unicentric Castleman Disease in children?
Answer: Often asymptomatic, may present with a painless mass - What are common systemic symptoms in multicentric Castleman Disease?
Answer: Fever, night sweats, weight loss, fatigue - What is the first-line treatment for unicentric Castleman Disease in children?
Answer: Surgical excision - Is chemotherapy typically used for unicentric Castleman Disease in children?
Answer: No, it's rarely needed for unicentric disease - What is the role of rituximab in treating Castleman Disease?
Answer: It's used in some cases of multicentric Castleman Disease, especially HHV-8 associated cases - Can Castleman Disease transform into lymphoma?
Answer: Yes, particularly in multicentric cases - What is the prognosis for children with unicentric Castleman Disease after complete surgical excision?
Answer: Excellent, with very low recurrence rates - Is anemia a common finding in Castleman Disease?
Answer: Yes, particularly in multicentric disease - What imaging modality is typically used for initial evaluation of suspected Castleman Disease?
Answer: CT scan or MRI - Can PET-CT be useful in evaluating Castleman Disease?
Answer: Yes, particularly for assessing disease extent in multicentric cases - What is POEMS syndrome and how is it related to Castleman Disease?
Answer: POEMS is a rare paraneoplastic syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein, Skin changes) that can be associated with Castleman Disease - Is thrombocytosis or thrombocytopenia more common in Castleman Disease?
Answer: Thrombocytosis is more common - What is the typical age of onset for unicentric Castleman Disease in children?
Answer: It can occur at any age, but often presents in adolescence - Can Castleman Disease affect the spleen?
Answer: Yes, particularly in multicentric disease - What is the role of anti-IL-6 therapy in Castleman Disease?
Answer: It's used in some cases of multicentric Castleman Disease, particularly those refractory to other treatments - Is multicentric Castleman Disease associated with HIV infection?
Answer: Yes, HIV-positive individuals are at higher risk for multicentric Castleman Disease - What is the typical serum IL-6 level in active Castleman Disease?
Answer: Elevated - Can Castleman Disease present with nephrotic syndrome?
Answer: Yes, it's a rare presentation but has been reported - What is the role of corticosteroids in treating Castleman Disease?
Answer: They can be used for symptomatic relief, particularly in multicentric disease
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