Leukocyte adhesion deficiency

Clinical and postmortem observations

Hal K. Hawkins, Sue C. Heffelfinger, Donald C. Anderson

Research output: Contribution to journalArticle

77 Citations (Scopus)

Abstract

The clinical and autopsy findings in a patient with the severe form of leukocyte adhesion deficiency are presented. An 18-month-old Hispanic female had a history of delayed umbilical cord separation, recurrent necrotizing skin lesions, and gingivitis. Her neutrophils were found to lack detectable CD11/CD18 adhesion glycoproteins and were deficient in adhesion-dependent functions. She succumbed to necrotizing enterocolitis, peritonitis, and pneumonia following sudden cardiorespiratory collapse. Postmortem examination revealed multiple regions of mucosal ulceration and bacterial and fungal overgrowth with complete lack of an acute inflammatory response. Impaired neutrophil emigration from blood vessels into injured tissue appears to have been the basis of this patient's disease. Some of the many foci of bronchopneumonia, in contrast, contained numerous neutrophils. Lymphoid tissue, including the thymus, was severely depleted of lymphocytes. These findings support the concepts that neutrophils can emigrate in response to certain stimuli via CD18-independent mechanisms and that severe deficiency of CD18 is associated with compromised function of lymphocytes in vivo..

Original languageEnglish (US)
Pages (from-to)119-130
Number of pages12
JournalFetal and Pediatric Pathology
Volume12
Issue number1
DOIs
StatePublished - 1992
Externally publishedYes

Fingerprint

Neutrophils
Leukocytes
Autopsy
Lymphocytes
Bronchopneumonia
Necrotizing Enterocolitis
Gingivitis
Umbilical Cord
Emigration and Immigration
Lymphoid Tissue
Peritonitis
Hispanic Americans
Thymus Gland
Blood Vessels
Glycoproteins
Skin

Keywords

  • CD18
  • Infection
  • Inflammation
  • Mac-1
  • Neutrophils

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Pediatrics, Perinatology, and Child Health

Cite this

Leukocyte adhesion deficiency : Clinical and postmortem observations. / Hawkins, Hal K.; Heffelfinger, Sue C.; Anderson, Donald C.

In: Fetal and Pediatric Pathology, Vol. 12, No. 1, 1992, p. 119-130.

Research output: Contribution to journalArticle

Hawkins, Hal K. ; Heffelfinger, Sue C. ; Anderson, Donald C. / Leukocyte adhesion deficiency : Clinical and postmortem observations. In: Fetal and Pediatric Pathology. 1992 ; Vol. 12, No. 1. pp. 119-130.
@article{f0754d23a6bf4ddf970c505bec981f92,
title = "Leukocyte adhesion deficiency: Clinical and postmortem observations",
abstract = "The clinical and autopsy findings in a patient with the severe form of leukocyte adhesion deficiency are presented. An 18-month-old Hispanic female had a history of delayed umbilical cord separation, recurrent necrotizing skin lesions, and gingivitis. Her neutrophils were found to lack detectable CD11/CD18 adhesion glycoproteins and were deficient in adhesion-dependent functions. She succumbed to necrotizing enterocolitis, peritonitis, and pneumonia following sudden cardiorespiratory collapse. Postmortem examination revealed multiple regions of mucosal ulceration and bacterial and fungal overgrowth with complete lack of an acute inflammatory response. Impaired neutrophil emigration from blood vessels into injured tissue appears to have been the basis of this patient's disease. Some of the many foci of bronchopneumonia, in contrast, contained numerous neutrophils. Lymphoid tissue, including the thymus, was severely depleted of lymphocytes. These findings support the concepts that neutrophils can emigrate in response to certain stimuli via CD18-independent mechanisms and that severe deficiency of CD18 is associated with compromised function of lymphocytes in vivo..",
keywords = "CD18, Infection, Inflammation, Mac-1, Neutrophils",
author = "Hawkins, {Hal K.} and Heffelfinger, {Sue C.} and Anderson, {Donald C.}",
year = "1992",
doi = "10.3109/15513819209023288",
language = "English (US)",
volume = "12",
pages = "119--130",
journal = "Fetal and Pediatric Pathology",
issn = "1551-3815",
publisher = "Informa Healthcare",
number = "1",

}

TY - JOUR

T1 - Leukocyte adhesion deficiency

T2 - Clinical and postmortem observations

AU - Hawkins, Hal K.

AU - Heffelfinger, Sue C.

AU - Anderson, Donald C.

PY - 1992

Y1 - 1992

N2 - The clinical and autopsy findings in a patient with the severe form of leukocyte adhesion deficiency are presented. An 18-month-old Hispanic female had a history of delayed umbilical cord separation, recurrent necrotizing skin lesions, and gingivitis. Her neutrophils were found to lack detectable CD11/CD18 adhesion glycoproteins and were deficient in adhesion-dependent functions. She succumbed to necrotizing enterocolitis, peritonitis, and pneumonia following sudden cardiorespiratory collapse. Postmortem examination revealed multiple regions of mucosal ulceration and bacterial and fungal overgrowth with complete lack of an acute inflammatory response. Impaired neutrophil emigration from blood vessels into injured tissue appears to have been the basis of this patient's disease. Some of the many foci of bronchopneumonia, in contrast, contained numerous neutrophils. Lymphoid tissue, including the thymus, was severely depleted of lymphocytes. These findings support the concepts that neutrophils can emigrate in response to certain stimuli via CD18-independent mechanisms and that severe deficiency of CD18 is associated with compromised function of lymphocytes in vivo..

AB - The clinical and autopsy findings in a patient with the severe form of leukocyte adhesion deficiency are presented. An 18-month-old Hispanic female had a history of delayed umbilical cord separation, recurrent necrotizing skin lesions, and gingivitis. Her neutrophils were found to lack detectable CD11/CD18 adhesion glycoproteins and were deficient in adhesion-dependent functions. She succumbed to necrotizing enterocolitis, peritonitis, and pneumonia following sudden cardiorespiratory collapse. Postmortem examination revealed multiple regions of mucosal ulceration and bacterial and fungal overgrowth with complete lack of an acute inflammatory response. Impaired neutrophil emigration from blood vessels into injured tissue appears to have been the basis of this patient's disease. Some of the many foci of bronchopneumonia, in contrast, contained numerous neutrophils. Lymphoid tissue, including the thymus, was severely depleted of lymphocytes. These findings support the concepts that neutrophils can emigrate in response to certain stimuli via CD18-independent mechanisms and that severe deficiency of CD18 is associated with compromised function of lymphocytes in vivo..

KW - CD18

KW - Infection

KW - Inflammation

KW - Mac-1

KW - Neutrophils

UR - http://www.scopus.com/inward/record.url?scp=0026520089&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0026520089&partnerID=8YFLogxK

U2 - 10.3109/15513819209023288

DO - 10.3109/15513819209023288

M3 - Article

VL - 12

SP - 119

EP - 130

JO - Fetal and Pediatric Pathology

JF - Fetal and Pediatric Pathology

SN - 1551-3815

IS - 1

ER -