Hemophagocytic histiocytosis diagnosed by fine needle aspiration cytology of the spleen. A case report.

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作者：

Z Pio，V Antonio，C Giuseppe，C Antonio，P Lucio

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摘要：

BACKGROUND: Hemophagocytic histiocytosis (HPS) is an idiopathic, familial or secondary syndrome characterized by mature histiocytes causing intensive erythrophagocytosis. CASE: A 2-month-old male suffering from autoimmune hemolytic anemia, fever, jaundice and hepatosplenomegalia underwent fine needle aspiration cytology of the spleen. Aspiration was performed using a 23-gauge, short needle with a subcostal approach. The smear showed a monomorphous cell population of mature histiocytes with marginal nuclei and wide, well-defined cytoplasm. The cytoplasm was microvaculated and often contained > or = 1 erythrocytes and occasional lymphocytes. Immunostaining performed on cytospin samples showed diffuse positivity for alpha-1-antichymotrypsin and S-100. Differential diagnosis with malignant histiocytosis, Langerhans histiocytosis and sinus histiocytosis with massive lymphadenopathy was established. HPS was diagnosed because of the cytologic and immunocytochemical features and clinical data. CONCLUSION: HPS may be diagnosed using fine needle aspiration of the spleen when other biopsy samples have been unsuccessful. Cytologic, diagnosis of HPS should always be considered in a specific clinical setting, because early treatment can often save the patient's life.

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关键词：

Histiocytosis, Non-Langerhans-Cell 组织细胞增多症, 非郎格尔汉斯细胞

DOI：

10.1159/000326396

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年份：

2004