Background Primary autoimmune neutropenia (AIN) is a rare and often unrecognized disorder in adults.Study Design and Methods We report the case of a patient referred to our institution for weight loss and severe chronic neutropenia with a negative personal history for severe recurrent infections.Results The patient was diagnosed with a lung infiltrate, and a bronchoalveolar lavage was positive for Pseudomonas aeruginosa. Antibiotic therapy was performed with resolution of infection, but persistence of neutropenia. Several investigations excluded the most common causes of neutropenia and a marrow trephine showed a maturation arrest of the myeloid lineage. Treatment with granulocyte-colony-stimulating factor (G-CSF) caused a transient increase in neutrophil counts. Based on the mild clinical history and the short-lived increase in neutrophil count after G-CSF, primary AIN was suspected. Intravenous immunoglobulins induced a short-lived increase in neutrophil count; primary AIN was confirmed about 5 months after discharge by direct and indirect granulocyte immunofluorescence tests. The patient was discharged and no further therapy was required for persistent severe neutropenia in the absence of recurrent infections.Conclusion Primary AIN should be considered early in the diagnostic process of isolated neutropenia, to avoid expensive and time-consuming unnecessary diagnostic procedures.

Primary autoimmune neutropenia in adults : case report and review of the literature / J. Mariotti, S. Caberlon, E. Bertinato, G. Podda, M.T. Pugliano, M. Cattaneo. - In: TRANSFUSION. - ISSN 0041-1132. - 54:11(2014), pp. 2906-2910. [10.1111/trf.12711]

Primary autoimmune neutropenia in adults : case report and review of the literature

J. Mariotti
Primo
;
S. Caberlon
Secondo
;
E.M. Bertinato;G. Podda;M. Cattaneo
Ultimo
2014

Abstract

Background Primary autoimmune neutropenia (AIN) is a rare and often unrecognized disorder in adults.Study Design and Methods We report the case of a patient referred to our institution for weight loss and severe chronic neutropenia with a negative personal history for severe recurrent infections.Results The patient was diagnosed with a lung infiltrate, and a bronchoalveolar lavage was positive for Pseudomonas aeruginosa. Antibiotic therapy was performed with resolution of infection, but persistence of neutropenia. Several investigations excluded the most common causes of neutropenia and a marrow trephine showed a maturation arrest of the myeloid lineage. Treatment with granulocyte-colony-stimulating factor (G-CSF) caused a transient increase in neutrophil counts. Based on the mild clinical history and the short-lived increase in neutrophil count after G-CSF, primary AIN was suspected. Intravenous immunoglobulins induced a short-lived increase in neutrophil count; primary AIN was confirmed about 5 months after discharge by direct and indirect granulocyte immunofluorescence tests. The patient was discharged and no further therapy was required for persistent severe neutropenia in the absence of recurrent infections.Conclusion Primary AIN should be considered early in the diagnostic process of isolated neutropenia, to avoid expensive and time-consuming unnecessary diagnostic procedures.
Hematology; Immunology; Immunology and Allergy
Settore MED/09 - Medicina Interna
Article (author)
File in questo prodotto:
Non ci sono file associati a questo prodotto.
Pubblicazioni consigliate

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/257114
Citazioni
  • ???jsp.display-item.citation.pmc??? 4
  • Scopus 11
  • ???jsp.display-item.citation.isi??? 9
social impact