演題詳細

ポスター / Poster

ポスター 14 (Poster 14) :MPN:臨床 1

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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
藤 重夫 (Shigeo Fuji):1
1:国立がん研究センター中央病院 移植科
 
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Systemic infiltration with mature neutrophils in a patient with BCR-ABL-negative MPN

演題番号 : PS-1-99

山田 布沙絵 (Fusae Yamada):1、佐川 森彦 (Morihiko Sagawa):1、高橋 康之 (Yasuyuki Takahashi):1、木村 勇太 (Yuta Kimura):1、富川 武樹 (Tatsuki Tomikawa):1、根本 朋恵 (Tomoe Nemoto):1、多林 孝之 (Takayuki Tabayashi):1、渡部 玲子 (Reiko Watanabe):1、得平 道英 (Michihide Tokuhira):1、森 茂久 (Shigehisa Mori):1、木崎 昌弘 (Masahiro Kizaki):1

1:Dept. Hematology, Saitama Medical Center, Saitama Medical Univ., Kawagoe, Japan

 

According to the 2008 WHO criteria of myeloproliferative neoplasms (MPN), they are classified according to genetic and karyotypic analyses, although it is often difficult to diagnose cases lacking BCR-ABL fusion gene because there are no definite criteria for the diagnosis of atypical CML, MPN-U (unclassifiable) and chronic neutrophilic leukemia (CNL). These rare subtypes of MPN show increased numbers of mature neutrophils and often present with extramedullary disease. We herein report the first case of MPN with systemic infiltration of mature neutrophils presenting with multiple brain masses. A 68-year-old male was admitted to our hospital because of neutrophilic leukocytosis, anemia and thrombocytopenia. Bone marrow analysis revealed hypercellular marrow with a vast majority of mature neutrophils, without an increase in blast cells and dysplastic changes. Karyotype was normal, with no BCR-ABL fusion gene and JAK2 mutations. Hence, he was diagnosed as either atypical CML, MPN-U or CNL, and managed for anemia and thrombocytopenia with frequent transfusions and leukocytes with hydroxyurea. Two years after diagnosis, his leukocyte count progressively increased to 170 x 103/μl and he was hospitalized due to loss of consciousness. MRI revealed multiple brain masses with signs of bleeding. He died 20 days after hospitalization due to disease progression. Autopsy showed systemic infiltration of mature neutrophils in his bone marrow, spleen, liver, lungs, and brain masses. This is the first case report of a patient with MPN showing systemic infiltration with mature neutrophils.

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