| Sub-title | |
| Authors | D Pace R Parascandalo |
| Abstract | Destruction of neutrophils by granulocyte specific autoantibodies characteristically occurs in primary autoimmune neutropenia (AIN). AIN is often observed in infants and has an incidence rate of 1:100,000. Despite severe neutropenia, AIN usually follows a benign course. This is a case report of AIN of infancy presenting, quite unusually, at 26 months. A 26 month old girl was found to have isolated neutropenia after she was investigated for a Pseudomonas aeruginosa wound infection complicating a herniotomy. Following resolution of the infection she was noted to remain persistently neutropenic, with absolute neutrophil counts <1.0x109/l. On repeated physical examination she was always well, was growing normally and did not develop any fever, enlargement of the liver or spleen, lymphadenopathy or rashes. Her blood picture was normal. Immunoglobulin levels were normal for her age and she did not have any serological evidence of a connective tissue disorder. Examination of the bone marrow revealed hypercellularity with no signs of malignancy. Granulocyte-specific human neutrophil antigen-1 (HNA-1a) antibodies were detected in her serum. Furthermore she was genotyped as HNA-1a(+) suggesting that the antibodies were autoimmune in nature. She has remained well except for the occasional upper respiratory tract and superficial skin infections. In the majority of cases AIN resolves spontaneously over 7 to 24 months. Most children do not require any treatment except for antibiotics to treat infections; however prophylactic antibiotics may be considered in those with frequent infections. Remission of neutropenia, in children with severe infections or in those scheduled for surgery, can be achieved with granulocyte colony stimulating factor (G-CSF). |
Published in: | |
| Journal | Malta Medical Journal |
| Volume | 15 Issue 1-2/suppl. 2003 |
| Page | |
| Date | |
| Link to journal | |
| Key words | case report, infancy, autoimmune neutropenia |