Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content +++ ACUTE GRAFT VS. HOST DISEASE (GVHD)1,2 ++ Definition: Pro-inflammatory syndrome affecting the skin, liver, and gastrointestinal (GI) tract, typically within 100 days of hematopoietic stem cell transplant; immunocompetent transplanted cells mount an inflammatory response to cells in an immunosuppressed host. Skin: Most commonly affected organ, commonly maculopapular eruption, can progress to bulla formation and epidermal necrosis Gastrointestinal: Symptoms include watery diarrhea, severe abdominal pain, GI bleeding or ileus (endoscopy and biopsy are recommended for diagnosis when possible) Hepatic: Cholestatic jaundice most common, but can progress to hepatic failure See Table 58-1 for staging and grading of GVHD Therapy: First-line therapy is corticosteroids (decrease inflammatory cytokines that propagate acute GVHD) Skin GVHD typically responds most quickly to steroid therapy Variety of second-line therapies include polyclonal (ATG) and monoclonal antibodies, TNF-blockade (infliximab, etanercept), and calcineurin inhibitors Supportive care: Organ specific, including wound care, antimotility agents, bowel rest, ursodeoxycholic acid, and antimicrobial prophylaxis ++ ++Table Graphic Jump LocationTABLE 58-1Staging and Grading of Graft vs. Host Disease (GVHD) View Table||Download (.pdf) TABLE 58-1 Staging and Grading of Graft vs. Host Disease (GVHD) Skin Gastrointestinal (GI) Liver Stage 1 <25% skin surface Diarrhea 10–19.9 mL/kg/day or persistent nausea, vomiting or anorexia, with a positive upper GI biopsy Bilirubin 2–3 mg/dL 2 25%–50% skin surface Diarrhea 20–30 mL/kg/day Bilirubin 3.1–6 mg/dL 3 >50% skin surface Diarrhea >30 mL/kg/day Bilirubin 6.1–15 mg/dL 4 Generalized erythroderma with bullae formation Severe abdominal pain with or without ileus Bilirubin >15 mg/dL Grade I Stages 1–2 None None II Stage 3 or Stage 1 or Stage 1 III - Stages 2–4 or Stages 2–3 IV Stage 4 - Stage 4 Grade II requires Stage 3 Skin or Stage 1 GI or Stage 1 Liver Grade III requires Stage 2–4 GI or Stage 2–3 Liver +++ VASOOCCLUSIVE DISEASE (VOD)/SINUSOIDAL OBSTRUCTION SYNDROME (SOS)1–3 ++ Definition: Clinical syndrome of tender hepatomegaly, fluid retention, weight gain, and elevated serum bilirubin Reported incidence varies from 5% to 60% of pediatric patients undergoing stem cell transplant Poor prognosis with mortality rates as high at 90% in severe disease Diagnosis: Clinical diagnosis based on physical exam and laboratory findings. Liver ultrasound with Doppler is an adjunctive test but may be normal. Treatment: Supportive care: Focus on fluid balance and preservation of renal blood flow, using fluid restriction, diuretics, dialysis Defibrotide: Polydisperse mixture of single-stranded oligonucleotide with antithrombotic and fibrinolytic effects on microvascular endothelium Bleeding is serious complication and requires frequent monitoring of platelet count and markers of coagulation +++ IDIOPATHIC PNEUMONIA SYNDROME1,2 ++ Definition: Widespread alveolar injury following hematopoietic stem cell transplant in the absence of an active lower respiratory tract infection or cardiogenic causes Spectrum of disease that includes interstitial pneumonitis, diffuse alveolar hemorrhage, peri-engraftment respiratory distress syndrome, and bronchiolitis obliterans organizing pneumonia Widespread ... Your MyAccess profile is currently affiliated with '[InstitutionA]' and is in the process of switching affiliations to '[InstitutionB]'. Please click ‘Continue’ to continue the affiliation switch, otherwise click ‘Cancel’ to cancel signing in. Get Free Access Through Your Institution Learn how to see if your library subscribes to McGraw Hill Medical products. Subscribe: Institutional or Individual Sign In Username Error: Please enter User Name Password Error: Please enter Password Forgot Username? Forgot Password? Sign in via OpenAthens Sign in via Shibboleth