title: Hemophagocytic histiocytosis diagnosed by fine needle aspiration cytology of the spleen. A case report.
abstract: BACKGROUND
  Hemophagocytic histiocytosis (HPS) is an idiopathic, familial or secondary syndrome characterized by mature histiocytes causing intensive erythrophagocytosis.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Dipartimento di Anatomia Patologica, Facoltà di Medicina e Chirurgia, Università-di Napoli Federico II, Via Pansini n. 5, 80131 Naples, Italy.
authors.forename: Pio
authors.initials: P
authors.lastname: Zeppa
authors.forename: Antonio
authors.initials: A
authors.lastname: Vetrani
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Ciancia
authors.forename: Antonio
authors.initials: A
authors.lastname: Cuccuru
authors.forename: Lucio
authors.initials: L
authors.lastname: Palombini
mesh_terms: D000911:Antibodies, Monoclonal / Q000378:metabolism; D018951:Antigens, CD20 / Q000378:metabolism; D044963:Biopsy, Fine-Needle; D003581:Cytodiagnosis; D003937:Diagnosis, Differential; D054747:Histiocytic Sarcoma / Q000175:diagnosis; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis; D015616:Histiocytosis, Non-Langerhans-Cell / Q000175:diagnosis* / Q000473:pathology; D015618:Histiocytosis, Sinus / Q000175:diagnosis; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D008297:Male; D013048:Specimen Handling; D013154:Spleen / Q000473:pathology
chemical_list: D000911:Antibodies, Monoclonal; D018951:Antigens, CD20