Clinical features of disorders of neutrophil function.
Clinical Features . | Associated Neutrophil Disorders* . |
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*Disorders not discussed in detail in the text are listed with accession numbers for Online Mendelian Inheritance in Man (OMIM; http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=OMIM) | |
†Hereditary atypical mycobacteriosis represents a defect in monocyte and macrophage,16 rather than neutrophil, function. It is included because of the overlap in presentation with congenital disorders of neutrophil function, most of which also affect monocyte/macrophage function. | |
Recurrent bacterial and/or fungal infections | All |
Single opportunistic infection | All |
Organisms associated with specific disorders: | |
Aspergillus | CGD |
Atypical mycobacteria | Atypical mycobacteriosis† (OMIM 209950) |
Bacillus Calmette-Guerin (BCG) – disseminated | CGD, atypical mycobacteriosis† |
Burkholderia cepacia | CGD |
Candida – invasive | CGD |
Candida – mucocutaneous | Hyper-IgE syndrome (OMIM 147060) |
Serratia marcescens | CGD |
Marked leukocytosis with neutrophilia (absolute neutrophil count 30,000–100,000/mm3) | Leukocyte adhesion disorder, type 1 (OMIM 116920); congenital disorder of glycosylation, type IIc (OMIM 266265) |
Delayed separation of the umbilical cord (> 30 d) | Leukocyte adhesion disorder, type 1 |
Partial oculocutaneous albinism | Chédiak-Higashi syndrome (OMIM 214500) |
Impaired resorption of primary teeth; coarse facies | Hyper-IgE syndrome |
Clinical Features . | Associated Neutrophil Disorders* . |
---|---|
*Disorders not discussed in detail in the text are listed with accession numbers for Online Mendelian Inheritance in Man (OMIM; http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=OMIM) | |
†Hereditary atypical mycobacteriosis represents a defect in monocyte and macrophage,16 rather than neutrophil, function. It is included because of the overlap in presentation with congenital disorders of neutrophil function, most of which also affect monocyte/macrophage function. | |
Recurrent bacterial and/or fungal infections | All |
Single opportunistic infection | All |
Organisms associated with specific disorders: | |
Aspergillus | CGD |
Atypical mycobacteria | Atypical mycobacteriosis† (OMIM 209950) |
Bacillus Calmette-Guerin (BCG) – disseminated | CGD, atypical mycobacteriosis† |
Burkholderia cepacia | CGD |
Candida – invasive | CGD |
Candida – mucocutaneous | Hyper-IgE syndrome (OMIM 147060) |
Serratia marcescens | CGD |
Marked leukocytosis with neutrophilia (absolute neutrophil count 30,000–100,000/mm3) | Leukocyte adhesion disorder, type 1 (OMIM 116920); congenital disorder of glycosylation, type IIc (OMIM 266265) |
Delayed separation of the umbilical cord (> 30 d) | Leukocyte adhesion disorder, type 1 |
Partial oculocutaneous albinism | Chédiak-Higashi syndrome (OMIM 214500) |
Impaired resorption of primary teeth; coarse facies | Hyper-IgE syndrome |