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Article: Chronic benign neutropenia among Chinese children

TitleChronic benign neutropenia among Chinese children
Authors
KeywordsAutoimmune diseases
Chinese
Immunoglobulins
Neutropenia
Issue Date2004
PublisherHong Kong Medical Association. The Journal's web site is located at http://www.hkmj.org/resources/supp.html
Citation
Hong Kong Medical Journal, 2004, v. 10 n. 4, p. 231-236 How to Cite?
AbstractObjective. To delineate the clinical behaviour of chronic benign neutropenia in Chinese children in Hong Kong. Design. Retrospective study. Setting. University teaching hospital, Hong Kong. Patients. All infants and children with absolute neutrophil count of 1.5 × 109 /L or lower for more than 3 months. Main outcome measures. Development of significant infection, and achievement of remission. Results. Twenty-four children with chronic benign neutropenia were identified between 1992 and 2001. Their median age of diagnosis was 9 months. The mean (standard deviation) initial absolute neutrophil count was 0.28 × 109 /L (0.24 × 109 /L). Twenty-three patients presented with infection. Of the 19 patients tested, four (21%) were positive for anti-neutrophil antibodies. Bone marrow examination was performed in 17 patients: nine had normal results, but six showed evidence of peripheral consumption, one showed late maturation arrest at band stage, and one showed phagocytosis of myeloid cells by histiocytes. The overall hospitalised infection rate was 51.6 episodes per 1000 patient-months. Ten percent of cases were considered 'significant' infections and required hospital admission with either surgical intervention or intravenous therapy (antibiotics or fluid replacement). In the first year of diagnosis, more than 80% of patients had their lowest absolute neutrophil count (mean, 0.16 × 109 /L; standard deviation, 0.11 × 109 /L). Granulocyte-colony stimulating factor was used to treat three patients and induced transient elevation of absolute neutrophil count in all three. The projected remission rate was 55.4% at 3 years. Even for those with persistent disease, there was significant recovery in absolute neutrophil count to a mean of 0.5 × 109 /L (P<0.01). Conclusions. Patients with chronic benign neutropenia experienced a relatively benign clinical course regardless of their remission status. Only a small proportion of patients developed significant infections. A multi-centre prospective study may help identify predictive factors of remission.
Persistent Identifierhttp://hdl.handle.net/10722/53454
ISSN
2015 Impact Factor: 0.887
2015 SCImago Journal Rankings: 0.279
References

 

DC FieldValueLanguage
dc.contributor.authorChung, BHYen_HK
dc.contributor.authorChan, GCFen_HK
dc.contributor.authorLee, TLen_HK
dc.contributor.authorKwok, JSYen_HK
dc.contributor.authorChiang, AKSen_HK
dc.contributor.authorHo, HKen_HK
dc.contributor.authorHa, SYen_HK
dc.contributor.authorLau, YLen_HK
dc.date.accessioned2009-04-03T07:20:13Z-
dc.date.available2009-04-03T07:20:13Z-
dc.date.issued2004en_HK
dc.identifier.citationHong Kong Medical Journal, 2004, v. 10 n. 4, p. 231-236en_HK
dc.identifier.issn1024-2708en_HK
dc.identifier.urihttp://hdl.handle.net/10722/53454-
dc.description.abstractObjective. To delineate the clinical behaviour of chronic benign neutropenia in Chinese children in Hong Kong. Design. Retrospective study. Setting. University teaching hospital, Hong Kong. Patients. All infants and children with absolute neutrophil count of 1.5 × 109 /L or lower for more than 3 months. Main outcome measures. Development of significant infection, and achievement of remission. Results. Twenty-four children with chronic benign neutropenia were identified between 1992 and 2001. Their median age of diagnosis was 9 months. The mean (standard deviation) initial absolute neutrophil count was 0.28 × 109 /L (0.24 × 109 /L). Twenty-three patients presented with infection. Of the 19 patients tested, four (21%) were positive for anti-neutrophil antibodies. Bone marrow examination was performed in 17 patients: nine had normal results, but six showed evidence of peripheral consumption, one showed late maturation arrest at band stage, and one showed phagocytosis of myeloid cells by histiocytes. The overall hospitalised infection rate was 51.6 episodes per 1000 patient-months. Ten percent of cases were considered 'significant' infections and required hospital admission with either surgical intervention or intravenous therapy (antibiotics or fluid replacement). In the first year of diagnosis, more than 80% of patients had their lowest absolute neutrophil count (mean, 0.16 × 109 /L; standard deviation, 0.11 × 109 /L). Granulocyte-colony stimulating factor was used to treat three patients and induced transient elevation of absolute neutrophil count in all three. The projected remission rate was 55.4% at 3 years. Even for those with persistent disease, there was significant recovery in absolute neutrophil count to a mean of 0.5 × 109 /L (P<0.01). Conclusions. Patients with chronic benign neutropenia experienced a relatively benign clinical course regardless of their remission status. Only a small proportion of patients developed significant infections. A multi-centre prospective study may help identify predictive factors of remission.en_HK
dc.languageengen_HK
dc.publisherHong Kong Medical Association. The Journal's web site is located at http://www.hkmj.org/resources/supp.htmlen_HK
dc.relation.ispartofHong Kong Medical Journalen_HK
dc.rightsCreative Commons: Attribution 3.0 Hong Kong License-
dc.rightsHong Kong Medical Journal. Copyright © Hong Kong Medical Association.en_HK
dc.subjectAutoimmune diseasesen_HK
dc.subjectChineseen_HK
dc.subjectImmunoglobulinsen_HK
dc.subjectNeutropeniaen_HK
dc.subject.meshAutoimmune Diseases - epidemiology - ethnologyen_HK
dc.subject.meshBacterial Infections - epidemiology - ethnologyen_HK
dc.subject.meshNeutropenia - epidemiology - ethnologyen_HK
dc.subject.meshHospitalization - statistics & numerical dataen_HK
dc.subject.meshRetrospective Studiesen_HK
dc.titleChronic benign neutropenia among Chinese childrenen_HK
dc.typeArticleen_HK
dc.identifier.openurlhttp://library.hku.hk:4550/resserv?sid=HKU:IR&issn=1024-2708&volume=10&issue=4&spage=231&epage=236&date=2004&atitle=Chronic+benign+neutropenia+among+Chinese+childrenen_HK
dc.identifier.emailChung, BHY:bhychung@hku.hken_HK
dc.identifier.emailChan, GCF:gcfchan@hkucc.hku.hken_HK
dc.identifier.emailChiang, AKS:chiangak@hkucc.hku.hken_HK
dc.identifier.emailLau, YL:lauylung@hkucc.hku.hken_HK
dc.identifier.authorityChung, BHY=rp00473en_HK
dc.identifier.authorityChan, GCF=rp00431en_HK
dc.identifier.authorityChiang, AKS=rp00403en_HK
dc.identifier.authorityLau, YL=rp00361en_HK
dc.description.naturepublished_or_final_versionen_HK
dc.identifier.pmid15299167-
dc.identifier.scopuseid_2-s2.0-4043090569en_HK
dc.identifier.hkuros91145-
dc.relation.referenceshttp://www.scopus.com/mlt/select.url?eid=2-s2.0-4043090569&selection=ref&src=s&origin=recordpageen_HK
dc.identifier.volume10en_HK
dc.identifier.issue4en_HK
dc.identifier.spage231en_HK
dc.identifier.epage236en_HK
dc.publisher.placeHong Kongen_HK
dc.identifier.scopusauthoridChung, BHY=7203043997en_HK
dc.identifier.scopusauthoridChan, GCF=16160154400en_HK
dc.identifier.scopusauthoridLee, TL=24483772800en_HK
dc.identifier.scopusauthoridKwok, JSY=7006208874en_HK
dc.identifier.scopusauthoridChiang, AKS=7101623534en_HK
dc.identifier.scopusauthoridHo, HK=7401465257en_HK
dc.identifier.scopusauthoridHa, SY=7202501115en_HK
dc.identifier.scopusauthoridLau, YL=7201403380en_HK

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