Leucocyte adhesion deficiency type 1 with secondary haemophagocytic lymphohistiocytosis

Halder, Ankita; Paul, Subhasish; Guha, Suparna; Mitra, Dipendra Kumar

doi:10.4038/sljch.v46i2.8279

Cited by 1 publication

(3 citation statements)

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“…LAD1 is a rare disorder of immune deficiency that occurs in 1 in 10 million births 2 integrins was recognized to be the cause of this syndrome in the early 1980s. 1 The essential function of the integrins is the integration of the cytoskeleton with the extracellular environment and they are found on most cell types.…”

Section: Discussionmentioning

confidence: 99%

“…Some of these interactions depend on cytokines for their regulation, others require firm leucocyte-cell or leucocyte-matrix contact, called adhesion. 1 Leukocyte adhesion defects (LAD) is a rare group of disorders of leucocyte function transmitted by autosomal recessive pattern of inheritance 2 . LAD syndrome is characterized by delayed umbilical cord separation, recurrent severe bacterial infection, periodontitis, and delayed wound healing and often persistent leukocytosis with absent pus formation.…”

Section: Introductionmentioning

confidence: 99%

“…. Only about 300 cases have been reported worldwide2 .Patients with LAD present with recurrent and often life threatening infections.…”

mentioning

confidence: 99%

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A patient with leukocyte adhesion defect

Gunasekara¹,

Kudagammana

2018

SL J. Med.

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During inflammation, leukocytes play a key role in maintaining tissue homeostasis by elimination of pathogens and removal of damaged tissues. Leukocytes migrate to the site of inflammation by crawling over and through the blood vessel wall, into the tissue. Leukocyte adhesion deficiencies (LAD 1, LAD 2 and LAD 3) are caused by defects in the adhesion of leukocyte to the blood vessel wall due to mutations in the genes encoding integrin, selectin and kindling-3, respectively. Patients experience recurrent none pus forming bacterial infections and neutrophilia, often preceded by delayed separation of umbilical cord. This case report describes a child who presented with umbilical sepsis followed by separation of the umbilical cord on day 15, and recurrent bacterial meningitis with high neutrophilia.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%