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Castleman's disease

Written2003-12Antonio Cuneo, Gianluigi Castoldi
Hematology Section, Department of Biomedical Sciences, University of Ferrara, Corso Giovecca 203, Ferrara, Italy

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Atlas_Id 2123
Other namesAngiofollicular lymph node hyperplasia

Clinics and Pathology

Phenotype / cell stem origin The disease appears to be polyclonal in origin in the majority of cases, however evidence for clonal expansion was documented in some cases, possibly representing transformation into non Hodgkin's lymphoma. In approximately 1/3 of the cases studied a monoclonal IgH rearrangement was documented. A minor T-cell clone, mostly in a polyclonal background, was also documented in some cases.
Epidemiology The disease is very rare.
Clinics The disease may present as a solitary mass frequently occurring in the mediastinum or as a systemic disorder (multicentric Castleman's disease) with diffuse adenopathies, systemic symptoms and recurrent infections. Splenomegaly, hepatomegaly and neurologic symptoms may occur frequently as is the case with autoimmune manifestations.
Pathology There are two variants: the hyaline-vascular and the plasma cell subtype. In the former subtype there are shrunken germinal centres with concentric expansion of the mantle zones with eosinophils and hyalinization around the vessels; in the latter subtype an extensive infiltrate by plasma cells is seen in the interfollicular areas. Some patients may be infected by human herpervirus-8 which may induce interleuchin-6 (IL6) overproduction. IL6 is believed to play an essential role in the pathogenesis of the disease.

The hyaline-vascular type is usually diagnosed in asymptomatic patients whereas systemic symptoms are present in the majority of patients with the plasma cell subtype. Patients with "multicentric" Castleman disease show histologic features consistent with the plasma cell subtype.

Treatment The patients can be treated by surgical excision if the mass is localized. Steroid treatment is recommended in cases with disseminated disease and combination chemotherapy utilized for lymphoma must be reserved to unresponsive patients. Some patients with HIV associated Castleman's disease were successfully treated with the anti CD20 monoclonal antibody or with the antiviral agent ganciclovir targeting the HHV-8.
Prognosis The prognosis varies greatly depending on the histologic type and disease presentation. If the disease is localized, surgery with or without radiotherapy may be curative. Those patients with multicentric disease who fail to respond to steroid treatment have a serious disease.


Cytogenetics Morphological Many of the cases so far studied showed a normal karyotyope.
Occasional abnormalities were found in a few patients.
  • One case with the hyaline vascular type showed a t(1;6)(p11;p11), a del(7)(q21q22) and a del(8)(q12q22). In this patient no clonal expansion of lymphoid cells was present, suggesting that the clonal proliferation involved dysplastic stromal cells.
  • Another patient was shown to carry a clonal abnormality in CD21-positive follicular dendritic cells. Abnormal chromosomes in this patient were add(1)(q21), der(6)t(6;12)(q23;q15), add(7)(p22), -9, inv(9)p11q13), del(12)(q15).
  • One patient carried a t(7;14)(p22;q22).
  • Bibliography

    Remission of HHV-8 and HIV-associated multicentric Castleman disease with ganciclovir treatment.
    Casper C, Nichols WG, Huang ML, Corey L, Wald A
    Blood. 2004 ; 103 (5) : 1632-1634.
    PMID 14615380
    Hyaline vascular Castleman's disease with HMGIC rearrangement in follicular dendritic cells: molecular evidence of mesenchymal tumorigenesis.
    Cokelaere K, Debiec-Rychter M, De Wolf-Peeters C, Hagemeijer A, Sciot R
    The American journal of surgical pathology. 2002 ; 26 (5) : 662-669.
    PMID 11979097
    Atypical lymphoproliferative disorders.
    Frizzera G
    In Knowles DM (Ed) Neoplastic hemopathology IInd edition..
    A case of multicentric Castleman's disease associated with advanced systemic amyloidosis treated with chemotherapy and anti-CD20 monoclonal antibody.
    Gholam D, Vantelon JM, Al-Jijakli A, Bourhis JH
    Annals of hematology. 2003 ; 82 (12) : 766-768.
    PMID 12898190
    Atypical Lymphoproliferative Diseases.
    Greiner T, Armitage JO, Gross TG
    Hematology / the Education Program of the American Society of Hematology. American Society of Hematology. Education Program. 2000 : 133-146.
    PMID 11701539
    Clonal rearrangement for immunoglobulin and T-cell receptor genes in systemic Castleman's disease. Association with Epstein-Barr virus.
    Hanson CA, Frizzera G, Patton DF, Peterson BA, McClain KL, Gajl-Peczalska KJ, Kersey JH
    The American journal of pathology. 1988 ; 131 (1) : 84-91.
    PMID 2833104
    Rituximab therapy for HIV-associated Castleman disease.
    Marcelin AG, Aaron L, Mateus C, Gyan E, Gorin I, Viard JP, Calvez V, Dupin N
    Blood. 2003 ; 102 (8) : 2786-2788.
    PMID 12842986
    Lack of cytogenetic abnormalities in Castleman's disease.
    Menke DM, DeWald GW
    Southern medical journal. 2001 ; 94 (5) : 472-474.
    PMID 11372793
    [Chromosomal abnormalities in Castleman's disease with high levels of serum interleukin-6]
    Nakamura H, Nakaseko C, Ishii A, Kogure K, Kawano E, Hashimoto S, Nishimura M, Matsuura Y, Oh H, Yoshida S
    [Rinsho ketsueki] The Japanese journal of clinical hematology. 1993 ; 34 (2) : 212-217.
    PMID 8492420
    A chromosomal abnormality in hyaline vascular Castleman's disease: evidence for clonal proliferation of dysplastic stromal cells.
    Pauwels P, Dal Cin P, Vlasveld LT, Aleva RM, van Erp WF, Jones D
    The American journal of surgical pathology. 2000 ; 24 (6) : 882-888.
    PMID 10843293
    Molecular analysis of clonality in Castleman's disease.
    Soulier J, Grollet L, Oksenhendler E, Micléa JM, Cacoub P, Baruchel A, Brice P, Clauvel JP, d'Agay MF, Raphael M
    Blood. 1995 ; 86 (3) : 1131-1138.
    PMID 7620166


    This paper should be referenced as such :
    Cuneo, A ; Castoldi, GL
    Castleman's disease
    Atlas Genet Cytogenet Oncol Haematol. 2004;8(2):81-82.
    Free journal version : [ pdf ]   [ DOI ]
    On line version :

    Other genes implicated (Data extracted from papers in the Atlas) [ 1 ]

    Genes BCLAF1

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