Marked hepatomegaly due to AA type amyloidosis in a case with Castleman's disease.

Hepatic amyloidosis complicated with Castleman's disease is quite rare. A 48-year-old woman was referred to our hospital with general fatigue, low-grade fever, anemia, thrombocythemia, and liver dysfunction. Physical examination revealed anemia and hepatomegaly and abdominal computed tomography showed marked hepatomegaly and right upper abdominal masses. Technetium-99m pyrophosphate (99mTc-PYP) scintigraphy revealed the diffuse abnormal uptake of the enlarged liver, suggesting amyloid deposition. Liver biopsy showed destruction of the liver structure and the massive deposition of AA type amyloid protein. Surgical resection was performed on the abdominal masses. Histological examination of the masses showed Castleman's disease (plasma cell type). After resection, her fever resolved and the liver size gradually decreased to within the normal range. This case shows that surgical resection of the main lesion is effective for hepatomegaly due to AA type amyloidosis associated with Castleman's disease.

[Myelodysplastic syndrome accompanied by Evans syndrome].

Various cases of myelodysplastic syndrome (MDS) with diverse immunological disorders have been reported by many investigators. In this case report, we present a 70-year-old woman who had been diagnosed as having MDS and liver cirrhosis (LC) type C for 20 months, and who finally developed autoimmune phenomena against autologous blood cells. She was admitted to our hospital in order to evaluate her advanced anemia, thrombocytopenia and fatigue. The laboratory data at admission were as follows: hemoglobin 5.3 g/dl, red blood cell count 109 x 10(4)/microliter, white blood cell count 1,760/microliter, platelet count 4.3 x 10(4)/microliter and reticulocyte count 1.3%. The direct Coombs test was positive. In addition, anti-platelet antibody was positive, using the MPHA method. With these results, Evans syndrome secondary to MDS or LC was diagnosed. The mechanisms for the development of immunological disorders in patients with MDS or LC have not been fully elucidated. To our knowledge, this patient is one of the rare cases with MDS and Evans syndrome reported in the literature. During the generation process of autoantibodies, the role of HCV antigen in the pathogenesis of Evans syndrome was of interest in this patient.

Ranimustine, ifosfamide, procarbazine, dexamethasone, and etoposide therapy for central nervous system recurrence of diffuse large B-cell lymphoma in patients with poor performance status: a pilot study.

The prognosis of patients with diffuse large B-cell lymphoma with central nervous system (CNS) involvement is still poor. We performed a pilot study to establish treatment for patients who had refractory or recurrent CNS involvement without employing high-dose chemotherapy or stem cell support. Eight patients with diffuse large B-cell lymphoma and CNS disease after first-line chemotherapy were enrolled. They were treated with MIND-E therapy (ranimustine, ifosfamide, procarbazine, dexamethasone, and etoposide) every 4 weeks. Three patients achieved complete remission, two patients achieved partial remission, and three patients did not respond. One patient received an autologous peripheral stem cell transplant after MIND-E therapy. Three patients are still alive. In conclusion, MIND-E therapy was effective for CNS disease in patients with B-cell lymphoma who were judged to be poor candidates for intensive chemotherapy. Its toxicity was tolerable. A prospective study should be done to confirm the efficacy of this regimen.