Abstract
Castleman disease is a rare lymphoproliferative disorder. Contrary to its closest differential of lymphoma, the disease tends to run a benign course. However the multicentric variety, which is usually of the plasma cell type, has a more sinister prognosis. It is commonly associated with HIV infection. We report a case of a 65 year old man presenting with recurrent episodes of swelling of the whole body and diarrhoea for 3 years. Physical examination revealed generalized lymphadenopathy with hepato-splenomegaly and ascites. Lymph node biopsy revealed histopathological changes consistent with hyaline-vascular type of Castleman disease (multicentric). He was HIV negative. Patient was treated with high dose corticosteroids and discharged with follow up advice. DOI: http://dx.doi.org/10.3329/jom.v16i1.22402 J MEDICINE 2015; 16 : 48-50
Highlights
Castleman disease, known as giant lymph node hyperplasia and angiofollicular lymph node hyperplasia, was first described in 1956 by Dr Benjamin Castleman.[1]. It is a rare lymphoproliferative disorder of unknown aetiology but has specific risk factors, e.g. HIV and HHV-8. It has been classified clinically as unicentric and multicentric types and histologically as hyaline-vascular, plasma cell and mixed types.2Unicentric hyaline-vascular types run a more benign course in comparison to multicentric plasma cell types which often result in fatalities from infectious complications, multi-organ failure and development of malignancies such as lymphoma (Hodgkin and non-Hodgkin) and Kaposi’s sarcoma.3,4,5Multicentric Castleman disease is usually of the plasma cell variant[6] and associated with HIV as well as HHV8
Surgical treatment is curative for localized disease but the multicentric variety has been approached with a combination of steroids, chemotherapy, radiotherapy, immunotherapy and newer drugs
We report a case of multicentric hyaline vascular type of Castleman disease in an HIV negative 65 year old man due to rarity of the condition
Summary
Known as giant lymph node hyperplasia and angiofollicular lymph node hyperplasia, was first described in 1956 by Dr Benjamin Castleman.[1]. We report a case of multicentric hyaline vascular type of Castleman disease in an HIV negative 65 year old man due to rarity of the condition. Multicentric Hyaline-Vascular Castleman Disease respiratory or abdominal symptoms, skin rashes, joint pains or tingling numbness of the extremities. He had no history of any blood transfusion or jaundice. A lymph node biopsy (from the axillary lymph nodes) revealed histopathological changes consistent with hyaline vascular type of Castleman disease. To elucidate the risk factors and complications, a serological test for HIV antibody and protein electrophoresis were carried out
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