Engsig Frederik Neess, Møller Michael Boe, Hasselbalch Hans K, Mahdi Bassam, Obel Niels
Department of Infectious Diseases, Odense University Hospital, Denmark.
APMIS. 2007 Jun;115(6):778-83. doi: 10.1111/j.1600-0463.2007.apm_585.x.
We describe a 47-year-old male admitted with fever and extreme neutrophil granulocytosis (up to 80 x 10(9)/L). All microbiology tests and test for autoimmune disease were negative. CT scan showed pulmonary infiltrates bilaterally, mediastinal lymphadenopathy and splenomegaly. Conventional pathological examination of bone marrow and lymph node biopsies did not demonstrate malignant cells and inflammatory disease was suspected. The patient died of multiorgan failure 23 days after admission. Autopsy showed neutrophil infiltration of several organs. Immunohistochemistry and cytogenetics postmortem led to a diagnosis of anaplastic large cell lymphoma (ALCL) of T-cell lineage. Involvement of peripheral blood with leukemoid reaction is a rare manifestation of ALCL. This case emphasizes the importance of immunophenotyping in unexplained extreme granulocytosis.
我们报告一例47岁男性患者，因发热及极度中性粒细胞增多症（高达80×10⁹/L）入院。所有微生物学检查及自身免疫性疾病检测均为阴性。CT扫描显示双侧肺部浸润、纵隔淋巴结肿大及脾肿大。骨髓常规病理检查及淋巴结活检未发现恶性细胞，怀疑为炎性疾病。患者入院23天后死于多器官功能衰竭。尸检显示多个器官有中性粒细胞浸润。死后免疫组织化学和细胞遗传学检查确诊为T细胞系间变性大细胞淋巴瘤（ALCL）。外周血受累伴类白血病反应是ALCL的一种罕见表现。该病例强调了免疫表型分析在不明原因的极度粒细胞增多症中的重要性。
