Castleman-Kojima disease in a South Asian adolescent.

Angiofollicular lymph node hyperplasia, with the eponym Castleman disease (CD) is a histomorphologic entity uniting a group of diseases with related and occasionally overlapping pathogenesis. Histologic variants of CD include the hyalinevascular, plasmacytic, and mixed types and each of these may be clinically unicentric or multicentric. Recent reports describe a variant of idiopathic multicentric CD (MCD) in Japan provisionally labeled Castleman-Kojima disease. The characteristic features of the syndrome include thrombocytopenia, ascites, microcytic anemia, myelofibrosis, renal dysfunction and organomegaly (TAFRO syndrome); other distinguishing features of the syndrome include a lack of association with human herpes virus (HHV)-8, good response to corticosteroid therapy and an indolent clinical course in many affected patients. We report the case of an adolescent boy with Castleman-Kojima disease treated with thalidomide. A 14-years-old boy was seen in consultation at Mediciti Hospital in September 2012 with the chief complaints of recurrent episodes of fever, ascites, and anemia (Fig. 1). He had been in excellent health until September 2010 when he was seen elsewhere with the insidious onset of low-grade fever and distention of abdomen of three weeks’ duration. Physical examination showed pallor, marked ascites and splenomegaly. His hemoglobin (Hb) was 6.0 g/dL, mean corpuscular volume (MCV) 78 fL, white blood cell 10.8 × 10/L and the platelets 360 × 10/L. The serum bilirubin was 0.3 mg/ dL, total serum proteins 6.7 g/dL, albumin 3.8 g/dL, serum iron 15 μg/dL; total iron binding capacity 266 μg/dL and the serum ferritin 560 ng/mL. A bone marrow biopsy showed normal morphology, adequate marrow iron and no ring sideroblasts; the reticulin stain showed grade I reticulin fibrosis (Modified Bauermeister scale). A Montoux test resulted in 3 mm induration. He was transfused a single unit of packed erythrocytes and a three-liter paracentesis was done. The fever and ascites resolved spontaneously over two weeks. Six months later he was admitted to a separate hospital in March 2011 with abdominal distention, anasarca, and fever of several weeks’ duration. He had fever, bilateral cervical and axillary lymphadenopathy and tense ascites. A computed tomographic scan of abdomen showed splenomegaly and multiple retroperitoneal lymph nodes, the largest measuring 18 mm. A right axillary lymph node biopsy showed hyaline vascular type of CD (Fig. 2). Immunohistochemistry showed no evidence of clonal restriction. He was treated with diuretics and was given a single unit of packed erythrocyte transfusion and two units of human serum albumin. A two-liter paracentesis was done. The ascitic fluid was a transudate and a cytospin preparation showed mesothelial cells and a rare neutrophil. He received six cycles of CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) chemotherapy for reasons that are not clear, between March 2011 and July 2011. Following the chemotherapy there was resolution of ascites and anasarca but the organomegaly and lymphadenopathy remained unchanged. He had several episodes of fever and abdominal distention in the subsequent eight months that were treated symptomatically at different Clinics. In October 2012 he was hospitalized with fever, abdominal distention and anasarca. His Hb was 4.5 g/dL, MCV 77 fL, leukocytes 5.4 × 10/L and platelets 31 × 10/L. Serum creatinine was 1.3 mg/dL, total bilirubin 0.3 mg/dL, total serum proteins 5.1 g/dL, albumin 1.3 g/dL, aspartate amino-