CD, firstly described by Benjamin Castleman in 1956, is a lymphoproliferative disorder of unknown etiology . It is also known as angiomatous lymphoid hamartoma, giant lymph node hyperplasia, angiofollicular lymph node hyperplasia or lymphoreticuloma [2, 8, 9]. The generation of IL-6 in germinal centers of hyperplastic lymph nodes, which results in induction of B-cell differentiation, seems to be implicated in the etiopathogenesis of the disease . Thoracic, cervical, abdominal, and axillary involvements are reported to be 60, 14, 11, 4 percent, respectively . The outcomes of the hyaline vascular variant of CD generally remain favorable. Symptoms associated with the presence of a space-occupying lesion, including cough, dyspnea, dysphagia, hemopthysis, angina, upper respiratory system infections and back pain may be observed . On the other hand, patients with the multicentric, plasma cell variant of CD present with fever, which is nearly universal, as well as night sweats, weight loss, weakness or fatigue and sometimes with polyneuropathies. Additionally, anemia, leucocytosis, hypergammaglobulinemia, hypoalbuminemia, elevated sedimentation rate and increased levels of CRP may also be observed . This case had a two year history of weight loss and night sweating but routine investigations such as complete blood count and biochemical profile were found to be within normal range. It has been shown that there is a strong association between the multicentric, plasma cell variant of CD and HHV-8. It is assumed that this virus increases the risk of extranodal B cell lymphoma and Kaposi's sarcoma by inducing the expression of IL-6 which is found to stimulate vascular endothelial growth factor expression [2, 12]. This case, the serologic tests for HIV and HHV-8 infections were negative. Imaging techniques may provide information in identifying and determining the extent of the lymphadenopathy, but have little impact on definite diagnosis. The most critical diagnostic test is an accurate histopathologic evaluation. Immunohistochemical assessment is important in diagnosis. Although the diagnosis can be suggested by routine radiologic studies and fine needle aspiration, this case was confirmed with immunohistochemical assessment of the resected nodes. The available literature suggests that progression of the multicentric, variant of CD to B cell lymphoma is more frequent. Additionally, the progression of hyaline vascular type of CD to malignant lymphoma is very rare and only small number of cases has been identified in the literature . Because of progressive enlargement of the lymph nodes and the persistence of the systemic symptoms, since the initial evaluation of the patient, excisional biopsy was repeated. Histopathologic evaluation of the resected nodes revealed associated with B cell lymphoma. Prognosis of CD strongly depends on the precise histologic subtype, the extent and sites of disease. Mortality rates have been reported to be more than 50 percent in multicentic CD due to infectious complications and related malignancies . The median survival time is 29 months and 26 percent of the patients die in a year after the initial diagnosis. Surgical resection is sufficient in the treatment of localized CD, it generally does not have a role in the treatment of multicentric CD, and therefore a multidiscipliner approach must be provided. Chemotherapy, radiotherapy, interferon alpha and steroids may be used as treatment [2, 6, 13, 14]. At admission to our clinic, the patient presented with night sweats, weight loss and progressively enlarged cervical lymph nodes within a 2-year period. No viral infection was detected. It is difficult to establish an accurate estimate, whether the development of lymphoma occurred de novo or was secondary to CD in this case. Although CD was histopathologic confirmed by lymph node biopsy, we did not perform further immunohistochemical assessment and we do not really know if it was initially associated with lymphoma. Although receiving no treatment over a 2-year period until the day before diagnosis and having less aggressive clinical course, as usually not expected in patients with lymphoma, it is highly likely that Multicentric CD associated with lymphoma. The incidence of lymphoma is increased in patients with CD compared with the general population. In the literature, progression to malignant lymphoma in Multicentric CD associated with HIV is frequent, and within a prospective cohort study of 60 HIV-infected patients with Multicentric CD, and a follow-up period of 20 months, 14 patients (23%) are reported to develop HHV8-associated non-Hodgkin lymphoma. The five-year survival rate in Multicentric CD is 82% and this prognosis appears to be far better than that encountered with malignant lymphomas. Multicentric CD runs a more aggressive course and can progress to non-Hodgkin's lymphoma. Multicentric CD, whether or not HHV8- associated, is generally treated with combination chemotherapy (CHOP) [13, 14].
In this case, we assumed malignant lymphoma developed two years later after the diagnosis of the multicentric, hyaline vascular variant of CD. Following the treatment with radiotherapy and combination chemotherapy a durable complete response was achieved.