Documentation of Crucial Information Relating to Critically Ill Patients.

BACKGROUND: Medical decision-making has evolved to the modern model of shared decision-making among patients, surrogate decision-makers, and medical providers. As such, informed consent discussions with critically ill patients often should include larger discussions relating to values and goals of care. Documentation of care options and prognosis serves as an important component of electronic communication relating to patient preferences among care providers. OBJECTIVE: This retrospective chart review study sought to evaluate the prevalence of documentation of critical data, care options, prognosis, and medical plan, within primary team and palliative care consult team documentation. RESULTS: Three hundred two electronic medical records were reviewed. There was a significant difference in documentation between palliative care and primary teams for prognosis (83% vs 32%, P < .001), care options (82% vs 50%, P < .001), and care plan (82% vs 46%, P < .001). CONCLUSIONS: Our retrospective chart review study demonstrated a significant difference in documentation between primary and palliative care teams. We acknowledge that review of documentation cannot be extrapolated to the presence or absence of conversations between providers and patients and/or surrogates. Additional studies to evaluate this connection would be advantageous.

Personalized Prognostic Risk Score for Long-Term Survival for Children with Acute Leukemia after Allogeneic Transplantation.

We studied leukemia-free (LFS) and overall survival (OS) in children with acute myeloid (AML, n = 790) and acute lymphoblastic leukemia (ALL, n = 1096) who underwent transplantation between 2000 and 2010 and who survived for at least 1 year in remission after related or unrelated donor transplantation. Analysis of patient-, disease-, and transplantation characteristics and acute and chronic graft-versus-host disease (GVHD) was performed to identify factors with adverse effects on LFS and OS. These data were used to develop risk scores for survival. We did not identify any prognostic factors beyond 4 years after transplantation for AML and beyond 3 years for ALL. Risk score for survival for AML includes age, disease status at transplantation, cytogenetic risk group, and chronic GVHD. For ALL, the risk score includes age at transplantation and chronic GVHD. The 10-year probabilities of OS for AML with good (score 0, 1, or 2), intermediate (score 3), and poor risk (score 4, 5, 6, or 7) were 94%, 87%, and 68%, respectively. The 10-year probabilities of OS for ALL were 89% and 80% for good (score 0 or 1) and poor risk (score 2), respectively. Identifying children at risk for late mortality with early intervention may mitigate some excess late mortality.

Hematopoietic Stem Cell Transplantation Activity in Pediatric Cancer between 2008 and 2014 in the United States: A Center for International Blood and Marrow Transplant Research Report.

This Center for International Blood and Marrow Transplant Research report describes the use of hematopoietic stem cell transplantation (HSCT) in pediatric patients with cancer, 4408 undergoing allogeneic (allo) and3076 undergoing autologous (auto) HSCT in the United States between 2008 and 2014. In both settings, there was a greater proportion of boys (n = 4327; 57%), children < 10 years of age (n = 4412; 59%), whites (n = 5787; 77%), and children with a performance score ≥ 90% at HSCT (n = 6187; 83%). Leukemia was the most common indication for an allo-transplant (n = 4170; 94%), and among these, acute lymphoblastic leukemia in second complete remission (n = 829; 20%) and acute myeloid leukemia in first complete remission (n = 800; 19%) werethe most common. The most frequently used donor relation, stem cell sources, and HLA match were unrelated donor (n = 2933; 67%), bone marrow (n = 2378; 54%), and matched at 8/8 HLA antigens (n = 1098; 37%) respectively. Most allo-transplants used myeloablative conditioning (n = 4070; 92%) and calcineurin inhibitors and methotrexate (n = 2245; 51%) for acute graft-versus-host disease prophylaxis. Neuroblastoma was the most common primary neoplasm for an auto-transplant (n = 1338; 44%). Tandem auto-transplants for neuroblastoma declined after 2012 (40% in 2011, 25% in 2012, and 8% in 2014), whereas tandem auto-transplants increased for brain tumors (57% in 2008 and 77% in 2014). Allo-transplants from relatives other than HLA-identical siblings doubled between 2008 and 2014 (3% in 2008 and 6% in 2014). These trends will be monitored in future reports of transplant practices in the United States.

Transplant Outcomes for Children with T Cell Acute Lymphoblastic Leukemia in Second Remission: A Report from the Center for International Blood and Marrow Transplant Research.

Survival for children with relapsed T cell acute lymphoblastic leukemia (T-ALL) is poor when treated with chemotherapy alone, and outcomes after allogeneic hematopoietic cell transplantation (HCT) is not well described. Two hundred twenty-nine children with T-ALL in second complete remission (CR2) received an HCT after myeloablative conditioning between 2000 and 2011 and were reported to the Center for International Blood and Marrow Transplant Research. Median age was 10 years (range, 2 to 18). Donor source was umbilical cord blood (26%), matched sibling bone marrow (38%), or unrelated bone marrow/peripheral blood (36%). Acute (grades II to IV) and chronic graft-versus-host disease occurred in, respectively, 35% (95% confidence interval [CI], 27% to 45%) and 26% (95% CI, 20% to 33%) of patients. Transplant-related mortality at day 100 and 3-year relapse rates were 13% (95% CI, 9% to 18%) and 30% (95% CI, 24% to 37%), respectively. Three-year overall survival and disease-free survival rates were 48% (95% CI, 41% to 55%) and 46% (95% CI, 39% to 52%), respectively. In multivariate analysis, patients with bone marrow relapse, with or without concurrent extramedullary relapse before HCT, were most likely to relapse (hazard ratio, 3.94; P = .005) as compared with isolated extramedullary disease. In conclusion, HCT for pediatric T-ALL in CR2 demonstrates reasonable and durable outcomes, and consideration for HCT is warranted.

Transplantation conditioning regimens and outcomes after allogeneic hematopoietic cell transplantation in children and adolescents with acute lymphoblastic leukemia.

Relapse is common after hematopoietic stem cell transplantation (HSCT) for acute lymphoblastic leukemia (ALL). Although 1200 cGy total body irradiation (TBI) and cyclophosphamide (Cy) is the standard conditioning regimen, attempts to reduce relapse have led to the addition of a second chemotherapeutic agent and/or higher dose of TBI. We examined HSCT outcomes in patients age <18 years with ALL, in second or subsequent remission or in relapse at transplantation. Most transplantations were performed with the patient in remission. Patients received grafts from an HLA-matched sibling or unrelated donor. Four treatment groups were created: (1) Cy + TBI ≤ 1200 cGy (n = 304), (2) Cy + etoposide + TBI ≤ 1200 cGy (n = 108), (3) Cy + TBI ≥ 1300 cGy (n = 327), and (4) Cy + etoposide + TBI ≥ 1300 cGy (n = 26). Neither TBI > 1200 cGy nor the addition of etoposide resulted in fewer relapses. The 5-year probability of relapse was 30% for group 1, 28% for group 2, 35% for group 3, and 31% for group 4. However, transplantation-related mortality was higher (35% versus 25%, P = .02) and overall survival lower (36% versus 48%, P = .03) in group 4 compared with group 3. Our findings indicate that compared with the standard regimen, neither TBI > 1200 cGy nor the addition of etoposide improves survival after HSCT for ALL.

Comparison of outcomes after HLA-matched sibling and unrelated donor transplantation for children with high-risk acute lymphoblastic leukemia.

We compared outcomes after 94 HLA-matched sibling, 168 unrelated donor bone marrow (BM; n = 81 matched and n = 88 mismatched), and 86 cord blood transplantations in patients age 1 to 15 years with acute lymphoblastic leukemia (ALL) in second complete remission (CR). All patients had their first BM relapse within 3 years from diagnosis. Cox regression models were constructed to examine for differences in transplant outcome by donor source. Risks of grade 2 to 4 acute graft-versus-host disease (aGVHD) and chronic graft-versus-host disease (cGVHD), when compared to HLA-matched sibling transplants, were higher after matched unrelated donor BM (relative risk [RR], 2.42; P = .001; RR, 5.12; P < .001, respectively), mismatched BM (RR, 3.24; P < .001; RR, 5.19; P < .001, respectively), and cord blood (RR, 2.67; P < .001; RR, 2.54; P = .024, respectively) transplants. Although nonrelapse mortality was higher after transplantation of mismatched unrelated donor BM and cord blood, there were no differences in leukemia-free survival (LFS) between HLA-matched sibling and any of the unrelated donor transplantations. The 3-year probabilities of LFS were 50% after HLA-matched sibling and 44% after matched unrelated BM, and 44% after mismatched unrelated BM and 43% after cord blood transplantation. Our observations support transplantation of BM or cord blood from a suitably matched unrelated donor or cord blood for patients without an HLA-matched sibling with ALL in second CR.

A composite pheochromocytoma/ganglioneuroblastoma of the adrenal gland.

A 9-year-old female presented with a large abdominal mass. At surgery, the mass was noted to arise from the right adrenal gland. As the mass was manipulated, the patient developed severe hypertension. The final diagnosis was a cystic composite-pheochromocytoma/ganglioneuroblastoma. This compound adrenal tumor is only the fourth case reported in a child. Because composite pheochromocytomas are rare in the pediatric population, the management, optimal surveillance schedule and outcomes have not been characterized.