Castleman's disease

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Raviteja Guddeti, M.B.B.S. [2]

Castleman’s disease is a rare disorder characterized by non-cancerous growths (tumors) that may develop in the lymph node tissue throughout the body. It involves hyperproliferation of certain B cells that often produce cytokines.

de:Morbus Castleman

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Raviteja Guddeti, M.B.B.S. [2]

In 1954 Dr. Benjamin Castleman, a pathologist described an unusual histopathology of a lymph node in a patient with mediastinal mass.

de:Morbus Castleman

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Raviteja Guddeti, M.B.B.S. [2]

Two types of classifications exist for Castleman’s disease. They are:

• Clinical and Radiologic classification:
  • Unicentric – single lymph node is involved, most commonly in the mediastinum or the mesentery.
  • Multicentric – wide spread involvement of lymph nodes and also liver and spleen in some cases

• Histopathologic classification:
  • Hyaline vascular
  • Plasmacytic
  • Mixed cellularity – has features of both hyaline vascular type and plasmacytic type.

de:Morbus Castleman

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Raviteja Guddeti, M.B.B.S. [2]

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In all cases, Castleman’s disease is likely due to hypersecretion of the cytokine IL-6. In KSHV positive tumors, this is most likely due to expression of the a virus-encoded cytokine, vIL-6, while KSHV negative tumors appear to be the result of over secretion of human IL-6.^[1]

Castleman’s disease is sometimes associated with:

• POEMS syndrome
• Paraneoplastic pemphigus
• Thrombotic thrombocytopenic purpura (TTP)

de:Morbus Castleman

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Raviteja Guddeti, M.B.B.S. [2]

• About 50% of Multicentric Castleman’s disease (MCD) is caused by Kaposi’s sarcoma-associated herpesvirus (KSHV), a gammaherpesvirus that is also the cause of Kaposi’s sarcoma and primary effusion lymphoma, while the remainder of MCD are of unknown cause.
• The form of MCD most closely associated with KSHV is the plasmacytic form of Castleman’s disease while another pathologic form, the hyaline-vascular form, is generally negative for this virus.

de:Morbus Castleman

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Raviteja Guddeti, M.B.B.S. [2]

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Castleman’s disease should be differentiated from other conditions presenting with fever, fatigue, weight loss, arthralgia, myalgia, rash and soft tissue swelling. The differentials include the following:^{[1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25]}

de:Morbus Castleman

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Raviteja Guddeti, M.B.B.S. [2]

• Estimated prevalence per 100,000 is 1 case.

• Average age of the patients with unicentric disease is 30 – 40 yrs
• For multicentric disease it is 50 – 60 yrs

• No gender differentiation is seen in the occurrence of the disease.

de:Morbus Castleman

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Raviteja Guddeti, M.B.B.S. [2]

• HIV/AIDS can act as a risk factor for this disease. The course of the disease is worse in these patients.

de:Morbus Castleman

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Raviteja Guddeti, M.B.B.S. [2]

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• Life threatening infections
• Multi organ failure
• Kaposi sarcoma
• Lymphoma
• Death

• Prior to 1996 MCD carried a poor prognosis of about 2 years, due to autoimmune hemolytic anemia and non-Hodgkin’s lymphoma which may arise as a result of proliferation of infected cells. The timing of diagnosis, with particular attention to the difficulty of determining the cause of B symptoms without a CT scan and lymph node biopsy, may impact significantly on the prognosis and risk of death. Left untreated, MCD usually gets worse and becomes increasingly difficult and unresponsive to current treatment regimens.
• HIV patients with multicentric disease have a grave prognosis. They have a less favorable clinical course and tend to develop Kaposi sarcoma and even plasmablastic non-Hodgkin lymphoma.

de:Morbus Castleman

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