Pediatric Graft Versus Host Disease Clinical Presentation

Updated: Oct 07, 2015
  • Author: Phillip Ruiz, Jr, MD, PhD; Chief Editor: Harumi Jyonouchi, MD  more...
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Presentation

History

See the list below:

  • Acute graft versus host disease (GVHD): The clinical presentation is often a triad of dermatitis, hepatitis, and gastroenteritis, although symptoms may occur alone or in different combinations. Other tissues that may be involved include mucous membranes, conjunctiva, exocrine glands, bronchial tree, and urinary bladder.
    • Skin: Maculopapular rash may present with the onset occurring within 5-47 days after transplantation. Pruritus involving the palms and soles may precede the rash. In the early stage, the rash is confined to the nape of the neck, shoulder, palms, or soles. It may be confluent and involve the entire integument. In severe cases, bullous lesions similar to third-degree burns may develop.
    • Liver: The liver is the second most common organ involved. GVHD first manifests as elevated liver transaminases. Cholestatic jaundice is common, but hepatic failure with encephalopathy is unusual. Hepatic and GI involvement manifest with or following skin involvement.
    • GI: GVHD of the distal bowel and colon results in profuse diarrhea; intestinal bleeding; cramping, abdominal pain; and paralytic ileus. Diarrhea is greenish mucoid, watery, and secretory in nature. Upper GI involvement without enteric manifestation has been described in 13% of adults. Common symptoms are anorexia, nausea, vomiting, and dyspepsia.
    • Grading: Acute GVHD is graded in 5 steps from 0-IV based on involvement of the skin, liver, and GI tract. Grade 0 indicates no clinical evidence of disease. Grades I-IV are graded functionally. Grade I indicates rash on less than 50% of skin and no gut or liver involvement. Grade II indicates rash covering more than 50% of skin, bilirubin level of 2-3 mg/dL, diarrhea of 10-15 mL/kg/d, or persistent nausea. Grade III or IV indicates generalized erythroderma with bullous formation, bilirubin level of more than 3 mg/dL, or diarrhea of more than 16 mL/kg/d.
  • Chronic GVHD: This is a more pleiotropic syndrome that develops after day 100. The syndrome resembles autoimmune systemic collagen vascular disease with protean manifestations, involving essentially every organ. [13] Systemic manifestations include recurrent infections with immunodeficiency, weight loss, sicca syndrome, and failure to thrive (children) or debility and weight loss (adults).
    • Chronic GVHD can present with 2 forms of skin involvement. An early phase resembles lichen planus; these lesions may be sparse and transitory, ranging from papules to more typical lesions. Poikiloderma can be present in the later phase, which is extra pigmentation of the skin demonstrating various shades and associated with telangiectasia in the affected area.
    • Hair manifestations present as alopecia.
    • The mouth may present with sicca syndrome, depapillation of the tongue with variegations, scalloping of lateral margins, lichen planus, oral ulcers, and angular tightness. See the image below.
      Oral mucosal changes in a patient with chronic gra Oral mucosal changes in a patient with chronic graft versus host disease (GVHD). Note the skin discoloration (vitiligo), which can result from GVHD. Image courtesy of Romeo A. Mandanas, MD, FACP.
    • Joints exhibit decreased range of movements with associated myositis and tendinitis.
    • Manifestations of the eyes include decreased tearing, injected sclera, and conjunctivitis.
    • The liver involvement typically presents as cholestasis and cirrhosis.
    • GI presentations include esophageal stricture, malabsorption, and chronic diarrhea.
    • Pulmonary presentations include cough, dyspnea, wheezing, rales, pneumothorax, and, finally, bronchiolitis obliterans.
    • Hematological manifestations include refractory thrombocytopenia and eosinophilia.
    • Spleen involvement may present with functional asplenia.
    • Chronic GVHD has 2 stages. Limited chronic GVHD presents with localized skin involvement, hepatic dysfunction caused by chronic GVHD, or both. Extensive chronic GVHD presents with the following:
      • Generalized skin involvement, or localized skin involvement and/or hepatic dysfunction caused by chronic GVHD
      • Liver histologic findings showing chronic aggressive hepatitis, bridging necrosis, or cirrhosis
      • Eye involvement - Schirmer test (< 5 mm wetting)
      • Involvement of minor salivary glands or oral mucosa demonstrated by buccal/labial biopsy
      • Involvement of any other target organ
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Physical

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  • See History.
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