Hemophagocytic histiocytosis diagnosed by fine needle aspiration cytology of the spleen. A case report.
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.
Zeppa
Pio
P
Dipartimento di Anatomia Patologica, Facoltà di Medicina e Chirurgia, Università-di Napoli Federico II, Via Pansini n. 5, 80131 Naples, Italy.
Vetrani
Antonio
A
Ciancia
Giuseppe
G
Cuccuru
Antonio
A
Palombini
Lucio
L
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
D000911:Antibodies, Monoclonal; D018951:Antigens, CD20