Korean J Hematol.  2012 Sep;47(3):163-177. 10.5045/kjh.2012.47.3.163.

Castleman disease

• Saeed-Abdul-Rahman I
• Al-Amri AM

• KMID: 2251950
• DOI: http://doi.org/10.5045/kjh.2012.47.3.163

Abstract

Castleman and Towne described a disease presenting as a mediastinal mass resembling thymoma. It is also known as "giant lymph node hyperplasia", "lymph node hamartoma", "angiofollicular mediastinal lymph node hyperplasia", and "angiomatous lymphoid hyperplasia". The pathogenesis is unknown, but the bulk of evidence points toward faulty immune regulation, resulting in excessive B-lymphocyte and plasma-cell proliferation in lymphatic tissue. In addition to the mediastinal presentation, extrathoracic involvement in the neck, axilla, mesentery, pelvis, pancreas, adrenal gland, and retroperitoneum also have been described. There are 2 major pathologic variations of Castleman disease: (1) hyaline-vascular variant, the most frequent, characterized by small hyaline-vascular follicles and capillary proliferation; and (2) the plasma-cell variant, in which large lymphoid follicles are separated by sheets of plasma cells. The hyaline-vascular cases usually are largely asymptomatic, whereas the less common plasma-cell variant may present with fever, anemia, weight loss, and night sweats, along with polyclonal hypergamma-globulinemia. Castleman disease is a rare lymphoproliferative disorders. Few cases have been described world widely. In this article we reviewed the classification, pathogenesis, pathology, radiological features and up to date treatment with special emphasis on the role of viral stimulation, recent therapeutic modalities and the HIV-associated disease.

Keyword

Castleman disease; Hyaline vascular variant; Plasma cell variant; Unicentric; Multicentric Castleman disease; Human immunodeficiency virus

MeSH Terms

Adrenal Glands
Anemia
Axilla
B-Lymphocytes
Capillaries
Fever
Giant Lymph Node Hyperplasia
HIV
Lymph Nodes
Lymphoid Tissue
Lymphoproliferative Disorders
Mesentery
Neck
Pancreas
Pelvis
Plasma Cells
Sweat
Thymoma
Weight Loss

Figure

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  Fig. 1 Distribution of Castleman variants in patients with Castleman disease. Abbreviations: U-HV: unicentric-hyaline vascular variant; U-PC: unicentric plasma cell variant; M-PC: multicentric plasma cell variant.

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  Fig. 2 Castleman disease, hyaline vascular type. B-cell follicle with typical expanded mantle zone showing "onion skin" pattern and depleted, hyalinized germinal center with increased vascularity. Original magnification ×200.

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  Fig. 3 Castleman disease, plasma cell variant. Lymph node biopsy with reactive follicle, hyperplastic germinal center, interfollicular plasma cell infiltrate and lack of vascular proliferation. Original magnification ×200.

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  Fig. 4 Castleman disease, multicentric. It shows diffuse plasma cell proliferation in the interfollicular region. The image shows a small follicle in the center with eosinophilic deposits of fibrin and immune complexes and dilated sinuses. Hyaline-vascular changes are absent. Original magnification ×200.

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  Fig. 5 Castleman disease, plasmablastic variant with large plasmablasts (immunoblasts) in the mantle zone. Original magnification ×1,000.

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  Fig. 6 Castleman disease. Posteroanterior chest radiograph shows widening of upper mediastinum, aortopulmonary and azygos adenopathy, and enlargement of hila (arrows) with extension of lesion below right hilum.

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  Fig. 7 Thoracic Castleman disease. Posteroanterior chest radiograph shows incomplete upper border of mass (arrows) over right lower lung field suggestive of pleural lesion abutting fissure.

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  Fig. 8 Intrapulmonary Castleman disease. Posteroanterior chest radiograph reveals solitary lung mass (solid arrows) in right upper lobe with inferior border contacting right minor fissure (open arrow).

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  Fig. 9 Castleman disease. Unenhanced CT scan using bone window setting reveals posterior mediastinal mass (arrow) with typical "arborizing" pattern of intralesional calcifications.

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  Fig. 10 Castleman disease. Contrast-enhanced chest CT scan shows matted lymphadenopathy (arrows) formed by confluence of inhomogeneously enhancing enlarged lymph nodes confined to anterior mediastinum.

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  Fig. 11 Castleman disease. Enhanced abdominal CT scan shows well-defined mesenteric mass (arrows) with homogeneously intense enhancement.

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  Fig. 12 Castleman disease. Contrast-enhanced pelvic CT scan shows well-defined pelvic mass with areas of focal necrosis (arrows) and erosion of left sacral bone.

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  Fig. 13 Castleman disease. Abdominal CT scan at level of lower pole of left kidney (white open arrow) shows heterogeneously enhanced retroperitoneal mass (black open arrow) with multiple hypodense areas and peripherally located calcifications (solid arrows).

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  Fig. 14 Schematic presentation of the treatment plan in Castleman disease. Abbreviations: pred, prednisolone; Comb. Chem, combined chemotherapy.

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