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Acute graft-versus-host disease in Child
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Acute graft-versus-host disease in Child

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Contributors: Philip I. Song MD, Susan Burgin MD, Ivy Lee MD, Jonathan Cotliar MD
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Synopsis

Graft-versus-host disease (GVHD) refers to organ dysfunction resulting from the introduction of foreign immunocompetent lymphocytes or bone marrow tissue (the graft) into an immunologically defective host. Most GVHD occurs in patients who have undergone allogeneic bone marrow transplants, but it may occur in solid organ transplant and blood transfusion recipients. Rarely, a GVHD-like syndrome can occur following autologous hematopoietic stem cell transplantation, likely due to failure to re-establish self-tolerance.

Cutaneous GVHD has both an acute and a chronic form. Acute disease normally occurs within 2-4 weeks of stem cell infusion around the time of engraftment, and typically presents as a morbilliform eruption that may progress to erythroderma or, rarely, a toxic epidermal necrolysis-like picture. Inflammation is triggered by sterile damage-associated molecular pattern (DAMP) and pathogen-associated molecular pattern (PAMP) molecules. Chronic cutaneous GVHD usually presents a mean of 4 months after transplantation with mucocutaneous manifestations; sclerotic and nonsclerotic (lichen planus-like) skin lesions are most common.

Clinical staging of acute cutaneous GVHD:
  • Stage 1 – Less than 25% of body surface area involved
  • Stage 2 – Between 25% and 50% of body surface area involved
  • Stage 3 – Greater than 50% of body surface area involved, or generalized erythroderma
  • Stage 4 – Generalized erythroderma with blister formation and desquamation
Classification of acute GVHD:
  • Hyperacute – Onset prior to day 14 following transplant
  • Classic acute – Onset prior to day 100 following transplant
  • Persistent, recurrent, late-onset acute – Onset after day 100 following transplant or donor lymphocyte infusion
Acute GVHD occurs with mild to moderate severity (stage 1-2) in about 30%-40% of patients who have received allogeneic bone marrow transplants. Organs involved most frequently with GVHD are the skin, liver, and intestinal mucosa; limited skin involvement is most common. The incidence and severity of the disease correspond with the degree of major histocompatibility antigen (MHC) mismatch between the donor and the host, but other key factors related to the development of acute GVHD include increased age of transplant recipient and sex mismatch between donor and recipient as well as the GVHD prophylaxis given to the stem cell recipient.

Hyperacute GVHD occuring within 14 days after transplantation accounts for about 27% of all cases of acute GVHD, and it involves the skin in about 88% of patients. Hyperacute GVHD manifests with high fevers and with more severe skin disease, lower response to topical steroids, and higher nonrelapse mortality compared to regular acute GVHD. Risk factors for hyperacute GVHD include mismatched related or matched unrelated donor, donor-recipient sex mismatch, a myeloablative conditioning regimen, and receipt of over 5 prior chemotherapy regimens.

GVHD is the major cause of nonrelapse morbidity and mortality among stem cell transplant recipients.

Codes

ICD10CM:
D89.810 – Acute graft-versus-host disease

SNOMEDCT:
402355000 – Acute graft-versus-host disease

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Differentiating between acute GVHD and its mimickers may be difficult, as many of the clinical and histologic features of the entities are similar. Mucositis (due to myeloablative preparative regimens, or to methotrexate given for GVHD prophylaxis) is a common early complication after bone marrow transplant and may be difficult to distinguish from severe acute GVHD with oral involvement.

Differential diagnoses for acute GVHD include:

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Therapy

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References

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Last Reviewed: 03/31/2017
Last Updated: 01/11/2018
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Acute graft-versus-host disease in Child
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Acute graft-versus-host disease : Abdominal pain, Diarrhea, Fever, Hepatomegaly, Rash, Neck, Shoulder, Hyperbilirubinemia, Anorexia, Plantar feet, Palms, Ears
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