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Chronic graft-versus-host disease in Infant/Neonate
See also in: Nail and Distal Digit,Oral Mucosal Lesion
Other Resources UpToDate PubMed

Chronic graft-versus-host disease in Infant/Neonate

See also in: Nail and Distal Digit,Oral Mucosal Lesion
Contributors: Youssef M. Salem, Lorena A. Mija, Philip I. Song MD, Ivy Lee MD, Jonathan Cotliar MD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Graft-versus-host disease (GVHD) refers to multiorgan dysfunction resulting from the introduction of foreign immunocompetent lymphocytes or bone marrow tissue (the graft) into an immunologically defective host. Most GVHD occurs in individuals who have undergone allogeneic bone marrow transplants, but it may occur in solid organ transplant and blood transfusion recipients.

Cutaneous GVHD has both an acute and a chronic form. Acute GVHD normally occurs within 2-4 weeks of stem cell infusion and typically presents as a morbilliform eruption that may progress to erythroderma or, rarely, a toxic epidermal necrolysis-like picture.

Chronic cutaneous GVHD usually presents with mucocutaneous manifestations a mean of 4 months after transplantation. The incidence of chronic GVHD is estimated to be 60%-70% in recipients of allogeneic stem cell transplants with mismatched and unrelated donors and approximately 30% in recipients of fully histocompatible sibling donor transplants. Chronic GVHD is occasionally triggered by exposure to ultraviolet (UV) light, physical trauma, varicella zoster virus, or Borrelia infection. Activation of host antigen-presenting cells (APC) triggers a cytotoxic donor T-effector response. Thymic injury with subsequent autoreactive and alloreactive T-lymphocyte proliferation and a dysregulated TH17 response lead to macrophage and fibroblast activation with altered diffuse tissue repair and fibrosis.

Almost all chronic GVHD patients will have skin involvement. Oral mucosal, hepatic, and ocular involvement is also common. The gastrointestinal (GI) tract and the lungs, as well as the peripheral nervous and musculoskeletal systems, may also be affected. Chronic GVHD increases the overall risk of systemic and recurrent bacterial infections.

GVHD is a major source of morbidity and mortality among transplant recipients. The risk of developing chronic GVHD increases with advancing age, prior acute GVHD, history of splenectomy, and donor or recipient cytomegalovirus seropositivity. On the other hand, chronic GVHD is associated with beneficial graft-versus-tumor effects and reduced risk of leukemia relapse, especially with more severe chronic GVHD.

Codes

ICD10CM:
D89.811 – Chronic graft-versus-host disease

SNOMEDCT:
402356004 – Chronic graft-versus-host disease

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Last Reviewed:11/15/2022
Last Updated:01/16/2023
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Chronic graft-versus-host disease in Infant/Neonate
See also in: Nail and Distal Digit,Oral Mucosal Lesion
A medical illustration showing key findings of Chronic graft-versus-host disease : Alkaline phosphatase elevated, ALT elevated, AST elevated, Oral white plaque, Arthralgia, Dry eyes
Clinical image of Chronic graft-versus-host disease - imageId=6320846. Click to open in gallery.  caption: 'Sclerodactyly with some pink, scaly, and eroded plaques on the fingertips. Note the thinned thumbnail and scattered hyperkeratotic papules on the palm.'
Sclerodactyly with some pink, scaly, and eroded plaques on the fingertips. Note the thinned thumbnail and scattered hyperkeratotic papules on the palm.
Copyright © 2024 VisualDx®. All rights reserved.