Die angiofollikuläre Lymphadenopathie Castleman

Zusammenfassung

Der Morbus Castleman, bekannt auch als angiofollikuläre Lymphknotenhyperplasie, ist eine seltene Erkrankung. Er manifestiert sich mit einer pathologischen Proliferation von B-Lymphozyten und Plasmazellen in den Lymphorganen. Die Krankheit ist erstmals als pathologische Einheit 1954 und als eigenständiges Krankheitsbild 1956 von Castleman u. Mitarb. beschrieben worden. Der Morbus Castleman ist durch eine Lymphknotenhyperplasie, Gefäßproliferation und Endothelhyperplasie gekennzeichnet. Histologisch werden eine hyalin-vaskuläre und eine Plasmazellvariante unterschieden. Der hyalin-vaskuläre Typ (90 % der Fälle) ist grundsätzlich benigne und tritt am häufigsten bei jungen Patienten als isolierter mediastinaler Tumor, bei dem die thoraxchirurgische Therapie einen kurativen Ansatz hat, auf. Die Plasmazellvariante (10 %) ist selten lokalisiert, häufiger multizentrisch, grundsätzlich aggressiv und tritt in der Regel bei älteren Patienten auf. Sie kann mit einer generalisierten Lymphadenopathie, Hepatosplenomegalie, systemischen Symptomen, Anämie, erhöhten Entzündungszeichen oder polyklonaler Immunoglobulinämie assoziiert sein.

Wir berichten über zwei Patientinnen mit der unizentrischen Form des Morbus Castleman vom hyalin-vaskulären Typ. Sie wurden durch die chirurgische Resektion kurativ behandelt.

Abstract

The Castleman Disease, also known as angiofollicular lymph node hyperplasia is a rare disorder that results in abnormal proliferation of B-lymphocytes and plasma cells in lymphoid organs. It was first described as a pathologic entity in 1954 and later defined by Castleman et al. in 1956.

The Castleman's disease is characterised by lymph node hyperplasia with vascular proliferation and endothelial hyperplasia. On the basis of histologic criteria it has been divided into hyaline-vascular and plasma cell variant. The hyaline-vascular type (90 % of cases), generally benign, occurs in younger patients with a localised mediastinal mass - surgical excision is curative. The plasma cell variant (10 % of cases), localised or multicentric, is generally aggressive, and occurs in older patients with generalised lymphadenopathy, hepatosplenomegaly, systemic symptoms, anaemia, elevated inflammatory indices, and a polyclonal rise in immunoglobulins. The prognosis is poor despite the Chemotherapy. Surgery is not indicated.

We present two cases of unicentric Castleman Tumor of hyaline-vascular type. The patients were cured by surgical resection.

Literatur

• 1 Castleman B, Iverson L, Mendez V. Lokalised mediastinal lymph node hyperplasia resembling thymona.  Cancer. 1956;  9 822-830
• 2 Gaba A R, Stein R S, Sweet D L. Multicentric giant follicular lymph node hyperplasia.  Am J Clin Pathol. 1978;  69 86-90
• 3 Nishimoto N, Sasai M, Shima Y. et al . Improvement in Castleman's disease by humanized anti-interleukin-6 receptor antibody therapy.  Blood. 2000;  95 56-61
• 4 Emile C, Danon F, Ferman J P. et al . Castleman disease in POEMS syndrome with elevated interleukin-6.  Cancer. 1993;  71 874
• 5 Ioachim H L, Ratech H. Castleman Lymphadenopathy. In: “Ioachims Lymph Node Pathology” (Hrsg: Ioachim HL). Lippinkott: Williams & Wilkins 2002: 246-253
• 6 Frizzera G. Castleman's disease: more questions than answers.  Hum Pathol. 1985;  16 202-205
• 7 Frizzera G, Banks P M, Massarelli G. et al . A systemic lymphoproliferative disorder with morphologic features of Castleman's disease. Pathological findings in 15 patients.  Am J Surg Pathol. 1983;  7 211-231
• 8 Oksenhendler E, Boulanger E, Galicier L. et al . High incidende of Kaposi sarcoma-associated herpesvirus-related non-Hodgkin lymphoma in patients with HIV infection and multicentric Castleman disease.  Blood. 2002;  99 2331-2336
• 9 Bollen J M, Polstra A M, Kuyl A C van der. et al . Multicentric Castleman's disease and Kaposi's sarcoma in a cyclosporin treated, HIV-1 negative patient: case report.  BMC Blood Disorders. 2003;  3: 3, doi: 10.1186/1471-2326-3-3, http://www.biomedcentral.com/1471-2326/3/3
• 10 Ioachim H L. “Pathology of AIDS” (Hrsg: Ioachim HL). JB Lippinkott Co 1989
• 11 Coty P C, Astrow A, Gallinson D. et al .A single institution's experience treating castlemans disease in HIV positive patients. Abstract 2485, 39^th ASCO. Chicago, IL/USA 2003
• 12 Oksenhendler E, Duarte M, Soulier J. et al . Multicentric Castleman's disease in HIV infection: a clinical and pathological study of 20 patients.  AIDS. 1996;  10 61-67
• 13 Zietz C, Bognertein J R, Goebel F D. et al . An unusual cluster of cases of Castleman's disease during HAART for AIDS.  N Engl J Med. 1999;  340 1923-1924
• 14 Abdel-Reheim F A, Koss W, Rappaport E S. et al . Coexistence of Hodgkin's disease and giant lymph node hyperplasia of the plasma-cell type (Castleman's disease).  Arch Pathol Lab Med. 1996;  120 91-96
• 15 Soubrier M J, Dubost J-J, Sauvezie B JM. POEMS syndrome: a study of 25 cases and a review of the literatur.  Am J Med. 1994;  97 543-553
• 16 Rehmus W, Kimball A B. POEMS Syndrome. http://www.emedicine.com/derm/topic771.htm#section∼introduction
• 17 Hinaut R, Elhadad R, Galet B. et al . Castleman's disease with thoracic localization. Apropos of a case with x-ray computed tomography. Review of the literature.  J Radiol. 1987;  68 555-558
• 18 Khan J, Sinner W von, Akhtar M. et al . Castleman's disease of the chest. Magnetic resonance imaging features.  Chest. 1994;  105 1608-1610
• 19 Bragg D G, Chor P J, Murray K A. Lymphoproliferative disorders of the lung: histopathology, clinical manifestations, and imaging features.  Am J Roentgenol. 1994;  163 273-281
• 20 Johkoh T, Muller N L, Ichikado K. Intrathoracic multicentric Castleman disease: CT findings in 12 patients.  Radiology. 1998;  209 477-481
• 21 Ferrozzi F, Tognini G, Spaggiari E. Focal Castleman disease of the lung: MRI findings.  Clin Imaging. 2001;  25 400-402
• 22 Frizzera G, Banks P M, Massarelli G. A systemic lymphoproliferative disorder with morphologic features of Castleman's disease. Pathological findings in 15 patients.  Am J Surg Pathol. 1983;  7 211-231
• 23 Epstein D M, Glickstein M F. Pulmonary lymphoproliferative disorders.  Radiol Clin North Am. 1989;  27 1077-1084
• 24 Saito K, Morita M, Enomoto K. Bronchiolitis obliterans with pemphigus vulgaris and Castleman's disease. of hyaline-vascular type: an autopsy case analyzed by computer-aided 3-D reconstruction of the airway lesions.  Hum Pathol. 1997;  28 1310-1312
• 25 Chin A C, Stich D, White F V. et al . Paraneoplastic pemphigus and bronchiolitis obliterans associated with a mediastinal mass: A rare case of Castleman's disease with respiratory failure requiring lung transplantation.  J Pediatr Surg. 2001;  36 E22
• 26 Sun H, Wang R, Bin H. Localized Castleman disease with paraneoplatic pemphigus and pulmonary involvement: clinical features and histopathology.  Zhonghua Yi Xue Za Zhi. 2002;  82 530-533
• 27 Iyonaga K, Ichikado K, Muranaka H. Multicentric Castleman's disease manifesting in the lung: clinical, radiographic, and pathologic findings and successful treatment with corticosteroid and cyclophosphamide.  Intern Med. 2003 Feb;  42 182-186
• 28 Nicolosi A C, Almassi G H, Komorowski R. Cardiac tamponade secondary to giant lymph node hyperplasia (Castleman's disease).  Chest. 1994;  105 637-639
• 29 Hsiao C J, Hsu M M, Lee J Y. et al . Paraneoplastic pemphigus in association with a retroperitoneal Castleman's disease presenting with a lichen planus pemphigoides-like eruption. A case report and review of literature.  Br J Dermatol. 2001;  144 372-376
• 30 Begemann H, Rastetter J. Benignes angiofollikuläres Lymphom. In: “Klinische Hämatologie” (Hrsg: Begemann H, Rasteter J). Thieme-Verlag 1993: S. 661
• 31 Keller A R, Hochholzer L, Castleman B. Hyaline-vascular and plasma-cell types of giant lymph node hyperplasia of the mediastinum and other mediastinum and other locations.  Cancer. 1972;  29 670-683
• 32 Ng S-H, Ko S-F, Lin J-W. et al . Paracardiac pleural Castleman disease: radiographic and MR findings.  Br J Radiol. 2004;  77 433-435
• 33 Diebold J, Bientz M, Daussy M. Hyperplasie lymphoide angiofolliculaire de localisation mediastinale. Étude en immunofluorescence directe.  Rev Fr Mal Respir. 1976;  4 373-378
• 34 Iglesias L, Fuentes J, Ruiz M. et al . The heterogeneity of Castleman disease. Report of treatment and outcome of five cases.  Proc Am Soc Clin Oncol. 2003;  22 605
• 35 Shahidi H, Myers J L, Kvale P A. Castleman's disease.  Mayo Clin Proc. 1995;  70 969-977
• 36 Sarrot-Reynauld F. Castleman's disease. Orphanet Encyclopedia. http://www.orpha.net/data/patho/GB/uk-castleman.pdf August 2001
• 37 Muller A, Franzen C, Klussmann P. et al . Human herpesvirus type 8 in HIV-infected patients with interstitial pneumonitis.  J Infect. 2000;  40 242-247
• 38 Akimoto Y, Ishiyama T, Kawakami K. et al . Multicentric Castleman's disease with lymphoid interstitial pneumonia died of aggressive course with adult respiratory distress syndrome.  Rinsho Ketsueki. 1995;  36 742-748
• 39 Mizuno I, Murayama T, Obayashi C. et al . Multicentric Castleman's disease with lymphoid interstitial pneumonia and polyneuropathy.  Rinsho Ketsueki. 1998;  39 593-599
• 40 Advani R, Warnke R, Rosenberg S. et al . Treatment of multicentric Castleman's disease complicated by the development of non-Hodgkin's lymphoma with high-dose chemotherapy and autologous peripheral stem-cell support.  Ann Onc. 1999;  10 1207-1209
• 41 Bowne W B, Lewis J J, Filippa D A. et al .The management of unicentric and multicentric Castleman's disease. A report of 16 cases and a review of the literature. 50th Annual Cancer Symposium of the Society of Surgical Oncology. Chicago, Illinois 21 March 1997
• 42 Nishimoto N, Sasai M, Shima Y. et al . Improvement in Castleman's disease by humanized anti-interleukin-6 receptor antibody therapy.  Blood. 2000;  1 56-61
• 43 Straus D, Krown S, Hennesey N P. Kaposi's Sarcoma and Multicentric Castleman's Disease: An Update on KSHV/HHV-8 and Targeted Therapies.  The PRN Notebook. 1999;  4 5-9