%0 Journal Article
%T Multicentric Castleman¡¯s Disease, Associated with Idiopathic Thrombocytopenic Purpura
%A Ruchi Sood
%A Harris C. Taylor
%A Hamed Daw
%J Case Reports in Hematology
%D 2013
%I Hindawi Publishing Corporation
%R 10.1155/2013/269268
%X The most common cause of a neck mass in young adults is hyperplastic lymphadenopathy consequent to infection and inflammation. Castleman¡¯s disease (CD), an unusual benign lymphoproliferative disorder, infrequently causes neck masses. It occurs in unicentric (UCD) and multicentric (MCD) forms and is associated with human immunodeficiency virus (HIV), human herpes virus 8 (HHV-8), and Kaposi's sarcoma. We present the third known association between MCD and previous immune thrombocytopenia in the absence of HIV and HHV-8 infection and review its association with other autoimmune disorders and attendant implications for pathogenesis. Finally, we summarize the current approach to therapy. 1. Case Presentation A 27-year-old female with a history of asthma, presented with diffuse ecchymoses and nosebleeds in 2006. Her platelet count was as low as 3 ¡Á 109/L having decreased from 112 ¡Á 109/L in 2005. Her white cell count was 7.5 ¡Á 109/L and hemoglobin was 15.2£¿g/dL. HIV testing was negative, and no new medications had been started. Because of the severe isolated decline in platelet count, her thrombocytopenia was thought to be immune thrombocytopenia. She was begun on intravenous steroids and immunoglobulins. All signs of bleeding resolved but the platelet count started to drop while she was on the prednisone taper. She eventually required splenectomy after which her platelets increased to 461 ¡Á 109/L. Her histological findings were compatible with immune thrombocytopenia. On follow-up visit 5 months later, she presented with significant episodes of dental bleeding and bruising and her platelet count had decreased to as low as 2 ¡Á 109/L. She was started on rituximab only chemotherapy after which she remained in complete remission. In March 2012, she presented with a three-month history of a growing, right lower neck mass. She denied any history of bleeding, fever, chills, night sweats, or weight loss. Examination revealed an afebrile female with hard, fixed, painless, right cervical lymphadenopathy measuring 4 ¡Á 2£¿cm. There were no other palpable lymph nodes. WBC was 11.23 ¡Á 109/L (34% segmented neutrophils, 49% lymphocytes, 5% reactive lymphocytes, 7% monocytes, 5% eosinophils, and 0% basophils), Hgb 12.7£¿g/dL, and platelets 415 ¡Á 109/L. Her comprehensive metabolic panel, serum protein electrophoresis, and lactate dehydrogenase were unremarkable. Pathology of the excised node revealed marked angiofollicular hyperplasia consistent with the hyaline vascular variant of CD (Figure 1). Contrast enhanced CT scan of the chest and abdomen showed bilateral axillary
%U http://www.hindawi.com/journals/crihem/2013/269268/