Figure 2.
Clinical complications in the clinic cohort. (A) Development of clinical complications over time in 91 patients (clinic cohort). Red shaded areas show number of participants with clinical features (indicated on the y-axis) at time of evaluation at the National Institutes of Health. Blue shaded areas indicate the additional number of patients who developed these complications during follow-up. Mucocutaneous triad features include nail dysplasia, lacy reticular skin pigmentation, oral leukoplakia. Severe BMF: ANC <500/mm3, platelets <20 000/mm3, and/or Hb <8.0 g/dL OR cytopenia requiring treatment (HCT, regular platelet or red blood cell transfusions, or androgen treatment) and/or diagnosis of myelodysplastic syndrome or leukemia. (B) Clinical complications occurring with no prior hematopoietic cell transplantation. (C) Clinical complications occurring following HCT. AVN, avascular osteonecrosis of hip(s), knees, and/or shoulder(s); GI, gastrointestinal; PAVM, pulmonary arteriovenous malformation. *Esophageal strictures in 2 cases were diagnosed following HCT without history of gut GvHD.

Clinical complications in the clinic cohort. (A) Development of clinical complications over time in 91 patients (clinic cohort). Red shaded areas show number of participants with clinical features (indicated on the y-axis) at time of evaluation at the National Institutes of Health. Blue shaded areas indicate the additional number of patients who developed these complications during follow-up. Mucocutaneous triad features include nail dysplasia, lacy reticular skin pigmentation, oral leukoplakia. Severe BMF: ANC <500/mm3, platelets <20 000/mm3, and/or Hb <8.0 g/dL OR cytopenia requiring treatment (HCT, regular platelet or red blood cell transfusions, or androgen treatment) and/or diagnosis of myelodysplastic syndrome or leukemia. (B) Clinical complications occurring with no prior hematopoietic cell transplantation. (C) Clinical complications occurring following HCT. AVN, avascular osteonecrosis of hip(s), knees, and/or shoulder(s); GI, gastrointestinal; PAVM, pulmonary arteriovenous malformation. *Esophageal strictures in 2 cases were diagnosed following HCT without history of gut GvHD.

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