![]() To our knowledge, this is the first reported use of concomitant ruxolitinib and ibrutinib in pediatric patients. This cGVHD was steroid-refractory and resolved with the administration of concomitant ibrutinib and ruxolitinib. The patient was transplanted under a haploidentical protocol from the mother but developed bronchiolitis obliterans organizing pneumonia (BOOP) and pathology-confirmed GVHD. Patient 2 was a one-year-old male with sickle cell anemia. This cGVHD was refractory to steroids and ibrutinib but improved with the administration of concomitant ibrutinib and ruxolitinib. Patient 1 was a four-year-old female diagnosed with natural killer (NK) cell dysfunction who underwent alloHSCT with cells from a 9/10 National Marrow Donor Program (NMDP) donor and subsequently developed chronic GVHD (cGVHD) of the skin and gut. The first-line treatment of GVHD is typically corticosteroids, but steroid-refractory chronic GVHD (cGVHD) has led to the Food and Drug Administration (FDA) approval of ruxolitinib (Jakafi), ibrutinib (Imbruvica), and belumosudil (Rezurock). Our analysis showed that early initiation of corticosteroid therapy is associated with long-term survival in NIPCs.Īllogeneic hematopoietic stem cell transplant (alloHSCT) can be a life-saving treatment for patients with hematological disorders but far too often carries the feared complication of graft-versus-host disease (GVHD). We found that the ≤ 7 days group were more likely to survive after their NIPC diagnosis compared to the ≥ 8 days group (p = 0.06). We further divided these cases into those who received prednisolone within seven days and after 8 days. The median time between NIPC diagnosis and first administration of ≥ 1 mg/kg/day corticosteroids (prednisolone dose equivalent) was significantly longer in the Dead group than the Alive group, at 9 days versus 4 days (p = 0.01). Among these, 18 died (Dead group) while 19 remained alive (Alive group) during the study period. Thirty-seven were diagnosed with NIPCs, which consisted of idiopathic pneumonia syndrome, bronchiolitis obliterans, and interstitial lung disease including cryptogenic organizing pneumonia. We analyzed data from 393 patients who underwent allogeneic HSCT during a 10-year period. It is important to maximize the effectiveness of primary treatment because secondary treatment has not been established. Non-infectious pulmonary complications (NIPCs) after allogeneic hematopoietic stem cell transplantation (HSCT) are associated with poor outcomes. The characteristics of COP with spontaneous resolution and factors that determine the patients in whom steroid therapy might be avoided is detailed in this study. Conversely, 4 patients in the steroid therapy group showed recurrence and were treated by an additional course of steroids. Only 1 patient in the spontaneous resolution group showed recurrence but did not require steroid therapy. When we arbitrarily determined the cutoff values, including CRP levels of ≤3.79 mg/dL, the sensitivity, specificity, and odds ratio were 73.9%, 93.8%, and 39.8 (95% confidence interval: 4.51-1968.9), respectively. The area under the receiver operating characteristic (ROC) curve was 0.859 (95% confidence interval : 0.741-0.978) in CRP. Within 2 weeks, all patients in the spontaneous resolution group showed relief of symptoms and alleviated radiographic findings. 009) than those in the steroid therapy group. 002), and a longer duration from symptom onset to diagnosis of COP (median 51.5 days vs 23.0 days, P =. 001), a higher lymphocyte ratio (median 21.7% vs median 13.3%, P =. Patients in the spontaneous resolution group showed a lower C-reactive protein (CRP) concentration (median 0.93 mg/dL 0.46-1.91] vs median 10.42 mg/dL, P <. Sixteen patients who improved without steroid therapy (the spontaneous resolution group) and 24 patients who required steroid therapy (the steroid therapy group) were compared. We retrospectively collected data from 40 adult patients who were diagnosed with COP through bronchoscopic examination at Fukujuji Hospital from May 2016 to June 2022. Therefore, we investigated the characteristics of patients with spontaneous resolution. However, evidence supporting the need for COP treatment is poor. Cryptogenic organizing pneumonia (COP) is an idiopathic interstitial pneumonia generally requiring steroid therapy, and spontaneous resolution has been reported in patients with mild disease.
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