Castleman's disease is a rare lymphoproliferative disorder of unknown aetiology. The presentation is varied, diagnosis is difficult, and optimum management is still unknown. We report our experience with a case of Castleman's disease in a 34-year old woman who presented with pallor, hepatosplenomegaly, and a right iliac fossa mass that was 5 cm in diameter. this was initially diagnosed as a soft tissue sarcoma and preoperative tumour embolization was planned before excision. Mesenteric arteriogram revealed that the feeder arteries arose from the superior mesenteric artery and embolization was aborted for fear of causing bowel ischaemia. On laparotomy, lymphoid enlargement was found between the leaves of the jejunal mesentery. The tumour was relatively avascular and the overlying mesenteric vessels contributed to teh duplex ultrasound and computerized tomography appearance of hypervascularity. The tumour with the mesentery and the overlying segment of jejunum was excised completely. Histopathology confirmed Castleman's disease. The purpose of this report is to present this rare case that caused a diagnostic dilemma and to review the management of this disorder.
Angiofollicular lymphoid hyperplasia (Castleman's disease) is a lymphoproliferative process thought to be mediated by overexpression of II interleukin-6. Castleman's disease has two variants: Castleman's disease has two variants: Hyaline vascular type and plasma cell variant (multicentric Castleman's disease). The hyaline vascular type tends to be localized, and the plasma cell variant shows more systematic signs and carriers a worse clinical prognosis. Castleman's disease is associated with B-cell lymphoma, Kaposi sarcoma, Human herpes virus 8 (HHV-8), and Epstein-Barr virus. Castleman's disease have been described thrice post kidney transplant. In this report, we document the course of a renal recipient who developed the plasma cell variant of Castleman's disease at 16 months after failure of his allograft and return to dialysis. He displayed clinical resolution of this complication after graft nephrectomy. To our knowledge, this is the first case where the disease manifestations disappeared after graft removal. Our patient experienced chronic renal allograft rejection which may have driven all the systematic manifestations of multicentric castleman's disease and possibly reactivated a latent HHV-8 infection. In this case immunohistochemical testing for HHV-8 was not available to prove a role for this agent.
Peripheral neuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes are often the presenting features of the POEMS syndrome. Approximately 50% of these are associated with osteosclerotic myeloma, a rare variant of multiple myeloma and some with Castleman's disease, an unusual lympho-proliferative disorder. The underlying pathogenetic mechanisms have not been elucidated but amelioration of symptoms with the disappearance of the M-component suggests possible etiopathogenic mechanisms. We present a 40-year-old woman with manifestations of all three disorders.
To evaluate humoral immune response to influenza vaccine and polysaccharide pneumococcal vaccine in patients with rheumatoid arthritis (RA) or Castleman's disease (CD) during tocilizumab therapy.
Currently, of less than 50 cases of head and neck follicular dendritic cell (FDC) sarcoma reported in the literature, 5 have been found to occur in the background of Castleman disease. We report another case of head and neck FDC sarcoma with emphasise on its associated lesions and review the outcome of treatment from the existing cases in the literature.
Castleman's disease is an uncommon disease and the histopathogenesis is poorly understood. This study aims to investigate their clinicopathological and immunophenotypic profile.