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Disorders of Granulocyte Functions

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Benign Hematologic Disorders in Children

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Abstract

Functional phagocyte disorders occur due to intrinsic neutrophil defects resulting from abnormal granulocyte development, defects in phagocytosis, or defects in chemotaxis. Abnormal phagocytic function can also originate from external causes that lead to suppression of neutrophil functions. Phagocyte disorders generally manifest with increased susceptibility to bacterial and fungal infections, but not viral infections. Most patients present during infancy, although the diagnosis may sometimes be delayed until late childhood depending on the severity of the disorder and the availability of diagnostic tests. The key to the successful management of phagocyte disorders include early diagnosis and early initiation of antibiotic to prevent and treat infections. In some disorders, treatments such as prophylactic antibiotics, granulocyte colony-stimulating factor infusions, and hematopoietic cell transplantation maybe necessary.

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Abbreviations

AD:

Autosomal dominant

AR:

Autosomal recessive

CGD:

Chronic granulomatous disease

CHS:

Chediak-Higashi syndrome

DOCK8:

Dedicator of cytokinesis 8

G-CSF:

Granulocyte colony-stimulating factor

HIES:

Hyperimmunoglobulin E syndrome, hyper IgE syndrome

HSCT:

Hematopoietic cell transplantation

LAD:

Leukocyte adhesion defect

PG:

Pyoderma gangrenosum

PGM3:

Phosphoglucomutase 3

SDS:

Shwachman-Diamond syndrome

SGD:

Specific granule deficiency

SLeX:

Sialyl Lewis X

Tyk2:

Tyrosine kinase 2

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Authors and Affiliations

  1. Children’s Hospital of Michigan, Division of Allergy/Immunology, Department of Pediatrics, Central Michigan University, Detroit, MI, USA

    Divya Seth & Pavadee Poowuttikul

Authors
  1. Divya Seth
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  2. Pavadee Poowuttikul
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Correspondence to Pavadee Poowuttikul .

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Editors and Affiliations

  1. Department of Pediatrics, University of Texas Health Science Center, San Antonio, TX, USA

    Deepak M. Kamat

  2. Department of Pediatrics, Division of Hematology-Oncology, The University of Texas Health Science Center, San Antonio, TX, USA

    Melissa Frei-Jones

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Seth, D., Poowuttikul, P. (2021). Disorders of Granulocyte Functions. In: Kamat, D., Frei-Jones, M. (eds) Benign Hematologic Disorders in Children. Springer, Cham. https://doi.org/10.1007/978-3-030-49980-8_15

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  • DOI: https://doi.org/10.1007/978-3-030-49980-8_15

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