演題詳細

ポスター / Poster

ポスター 21 (Poster 21) :LPD/免疫不全・高齢者

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日程
2013年10月11日(金)
時間
16:50 - 17:50
会場
ポスター会場 / Poster (ロイトン札幌 3F ロイトンホールABCD)
座長・司会
岡 芳弘 (Yoshihiro Oka):1
1:大阪大学大学院医学系研究科 呼吸器・免疫アレルギー内科学
 
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Hyaline-vascular type of multicentric Castleman disease with myelofibrosis and thrombocytopenia

演題番号 : PS-1-155

黒澤 光俊 (Mitsutoshi Kurosawa):1、鈴木 左知子 (Sachiko Suzuki):1、米積 昌克 (Masakatsu Yonezumi):1、笠原 耕平 (Kohei Kasahara):1、鈴木 宏明 (Hiroaki Suzuki):2

1:Department of Hematology, Hokkaido Cancer Center, Sapporo, Japan、2:Department of Diagnostic Pathology, Hokkaido Cancer Center, Sapporo, Japan

 

Most cases of multicentric Castleman disease (MCD) are the plasma cell type or mixed type in histopathological classification. We report an infrequent case of the hyaline-vascular (HV) type of MCD with thrombocytopenia and myelofibrosis in addition to systemic symptoms and laboratory abnormalities. Case: A woman in her 40's presented with a low-grade fever and prolonged menstruation in December 2012. She was admitted to a nearby hospital because of thrombocytopenia, mild anemia and elevated CRP. CT demonstrated general lymphadenopathy and hepatosplenomegaly. She was transferred to our hospital because of persistent fever. PET showed increased FDG uptake in multiple lymph nodes, spleen and bone marrow. The bone marrow aspiration could not be aspirated but the biopsy revealed a slightly hypercellular marrow with CD20-positive B cell clusters and reticulin fibrosis. Biopsy of the right inguinal lymph node showed atrophic germinal centers occasionally penetrated by hyalinized vessels, and interfollicular hyalinized vascular proliferation. The lymph node biopsy showed no rearrangements of the IgH and TCR β genes. These findings confirmed the diagnosis of HV type of MCD. The serum levels of IL-6, amyloid A and VEGF were elevated, but IgG4 and PAIgG were not elevated. She tested positive for direct Coombs test and anti-single stranded DNA antibody. She was started on prednisolone and showed the rapid improvement of symptoms and laboratory abnormalities. Thrombocytopenia and myelofibrosis were thought to be associated with splenomegaly and autoimmune abnormalities, respectively.

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