Iranian Journal of Pathology

Leukocyte Adhesion Deficiency Type 1 Presenting as Leukemoid Reaction

Document Type : Case Reports

Authors

Sharique Ahmad 1
Sufia Ahmad Khan 1
Shivam Shingla 2
Shirish Bhatnagar 2
Kabeer Ahmad Khan 2

1 Dept. of Pathology, Era’s Lucknow Medical College and Hospital, RML Avadh University Lucknow, India

2 Dept. of Paediatrics, Era’s Lucknow Medical College and Hospital, RML Avadh University Lucknow, India

Abstract
The hallmarks of leukocyte adhesion deficiency (LAD) are defects in the leukocyte adhesion process, marked leukocytosis and recurrent infections. These molecular and clinical manifestations result from an impaired step in the inflammatory process, namely, the emigration of leukocytes from the blood vessels to sites of infection, which requires adhesion of leukocytes to the endothelium. Over last 20 years, three distinctive defects in the leukocyte adhesion cascade, involving several precise ordered steps such as rolling, integrin activation and firm adhesion of the leukocytes have been described. While LAD I and II are clearly autosomal recessive disorders, the mode of inheritance of LAD III is still not clear. LAD I is due to structural defects in the integrin molecule, preventing firm adhesion to occur. We present a case of a male neonate referred with continuation of leukemoid reaction and multiple non healing ulcers, gingivitis and periodontitis in spite of good antibiotic therapy.
 

Keywords

Leukocyte Adhesion Deficiency Syndrome
Leukemoid reaction
neonate
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Iranian Journal of Pathology
Volume 8, Issue 3 - Serial Number 3
Summer 2013
Pages 204-208

  • Receive Date 08 July 2012
  • Accept Date 03 March 2013

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