macrophage activating syndrome (mas) is a life-threatening disease seen in autoimmune diseases including lupus erythematosus, rheumatoid arthritis, still's disease, polyarteritis nodosa. it is characterized by fever, pancytopenia, liver failure, coagulopathy, and neurologic symptoms and high serum ferritin. a 27 yr. old female patient was admitted in shahid mostafa khomeini hospital (tehra...

The POEMS syndrome, also known as Crow-Fukase syndrome, is an unusual systemic disorder described mainly in Asian individuals. It is characterized by the presence of (P)polyneuropathy, (O)organomegaly, (E)endocrinopathy, (M) M-protein, and (S) skin changes. Several other associated conditions such as sclerotic bone lesions, Castleman disease, low-grade fever, edema and hematologic disorders are...

POEMS syndrome is a rare hematological disorder associated with plasma cell dyscrasia characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes. Castleman disease is a lymphoproliferative disorder that can be present in POEMS patients, which can be defined as Castleman disease variant of POEMS syndrome. Herein, we described a 24-year-old male patient ...

Multicentric Castleman's disease (MCD) is a polyclonal lymphoproliferative disorder that manifests as marked hyper-γ-globulinemia, severe inflammation, anemia, and thrombocytosis. Recently, Takai et al. reported a new disease concept, TAFRO syndrome, named from thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly. Furthermore, Kojima et al. reported Japanese MCD cases with ef...

TAFRO syndrome is defined by the presence of thrombocytopenia, hydrops, fever, fibrosis, renal dysfunction, and organomegaly can be seen with idiopathic multicentric Castleman disease (iMCD) or as an isolated process without iMCD. The pathophysiology not well understood, but it thought to related hypercytokinemia. Subclinical hypothyroidism may a potential factor in pathogenesis symptomatology ...

We herein describe a case of an unusual multicentric castleman disease(MCD) accompanied by Thrombocytopenia, Anasarca, myeloFibrosis and Renal failure, compatible with TAFRO syndrome and associated with Sjogren disease. The treatment with corticosteroids dramatically improved the symptoms. The clinical features of this case were similar to those reported previously in Japan but this appears to ...

abstract castleman disease or giant node hyperplasia is a rare disorder of the lymphoid tissue most often involving the mediastinal lymph nodes. we report a case of localized retroperitoneal hyaline-vascular type of castleman disease. a 35-year old woman presented with mild epigastric pain with radiation to the back. amild anemia was the only abnormal associated finding. abdominal ultrasound an...

Castleman tumor, a rare lymphoproliferative disorder, is one of the associated tumors in paraneoplastic pemphigus (PNP). Castleman tumor was a frequently reported neoplasm in association with paraneoplastic pemphigus which was caused by an autoimmune reaction originating from the B lymphocytes. Castleman tumor association with PNP is a rarely reported subtype of PNP in China. Early detection an...

Macrophage Activating Syndrome (MAS) is a life-threatening disease seen in autoimmune diseases including lupus erythematosus, rheumatoid arthritis, Still's disease, polyarteritis nodosa. It is characterized by fever, pancytopenia, liver failure, coagulopathy, and neurologic symptoms and high serum ferritin. A 27 yr. old female patient was admitted in shahid Mostafa Khomeini Hospital (Tehran-Ira...