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Chronic GVHD

Оглавление

cGVHD pathogenesis in children is not fully understood but no major differences from adults have emerged from the few pediatric‐specific studies [27]. Children experience lower rates of cGVHD than adults [28–31] that vary widely with stem‐cell source, graft manipulations (ATGs, naïve or other forms of T‐cell depletion), and posttransplant cyclophosphamide. The NIH consensus reclassification also lowered cGVHD incidence by only considering classic cGVHD ± overlap subtype, effectively excluding isolated late acute GVHD (> day 100) from the definition. One prospective study that examined NIH cGVHD in children found an overall 21% and a 24.7% incidence of late acute GVHD. NIH global severity at onset was >80% moderate‐to‐severe [31]. Major risk factors for cGVHD in children are past acute GVHD, peripheral blood grafts, age ≥12 years, while only the first of these two factors are risks for late acute GVHD. While the frequency of organ involvement appears to parallel that seen in adults, the intersection of normal childhood development with morbid forms of cGVHD can contribute to failure‐to‐thrive, linear growth delay, skeletal deformities, as well as increased risk for late deaths due to infections even after IST has been discontinued [32].

Sclerotic forms of GVHD occur in 7% of children but it is unclear if this is truly lower than in adults given shorter follow‐up in the pediatric study [31], as well as heterogeneities in the incidence of peripheral blood grafts and TBI >450 cGy between adult and pediatric studies [30,31,33] given that these were the two major risk factors for sclerosis established from an earlier study [33]. Because this form of cGVHD is difficult to treat when advanced, irreversible manifestations have developed, early detection of joint limitation may be screened using the validated, easy to administer, Photographic Range of Motion (P‐ROM) scale [34]. P‐ROM is very sensitive to change in either direction and can also be used to follow therapeutic responses [35]. Chronic lung GVHD can be difficult to diagnose in children (see Pulmonary section).

Blood and Marrow Transplantation Long Term Management

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