ALBERT

Description: Background: The utility of bone marrow biopsy (BMB) in patients with Hodgkin lymphoma (HL) has been a controversial topic. Clinical stage 4 (CS4) has been shown to be an independent poor prognostic marker and is also included in the International Prognostic Scoring (IPS) index for predicting both progression-free survival (PFS) and overall survival (OS). The current guidelines still recommend performing a BMB in patients with stage IIB (with unfavorable risk factors), III and IV HL, mainly for staging purposes. Though bone marrow involvement (BMI) upstages the HL to stage IV, the prognostic significance of BMI remains unclear. The study was aimed to determine the prognostic significance of BMI in underserved patients with HL and to determine the prognostic importance of other blood parameters. Methods: The study was conducted at John H. Stroger Hospital of Cook County, an inner city tertiary care hospital providing care to the underserved population of Chicago. Charts of 241 patients diagnosed with HL were screened from tumor registry. Patients with incomplete charts were not included in the study. Socio-demographic, clinical and pathologic factors were recorded at the time of diagnosis. For comparative purpose, CS4 disease did not include patients with BMI. Kaplan-Meier and bivariate analyses were performed. Cox regression analyses were conducted to explore predictors of OS and PFS. Hazard ratios (HR) with 95% confidence intervals (CI) were obtained. Results: The study included 192 patients of which 41% were Afro-Americans, 34% were Hispanics and 21% were Caucasians. Median age was 34 years with 25.5% patients being older than 45 years and 68% patients being women. Seventeen percent patients were positive for HIV. Nodular sclerosis was the most common histologic subtype (55%), bulky disease was recorded in 19% patients and 61.5% patients had B-symptoms. CS4 disease was seen in 12% patients while 28% patients were stage III, 47% were stage II and 13% were stage I. Single-site BMB was done in 96% patients. BMB was positive for involvement with HL in 19% patients (n=37). Out of these 37 patients, 84% (n=31) had advanced stage (III & IV) HL. BMI was seen in 5% patients with early-stage HL (stages I-II) and 41% patients with advanced-stage HL. Median IPS score was 2 (range 0-6). Median values for clinical factors were: hemoglobin-11.8 g/dL, platelets 314.5 x103/uL, leukocytes 8.3 x103/uL, neutrophils 6x103/uL, lymphocytes 1.2 x103/uL and albumin 3.7 g/dL. Mean OS was 143 months (95% CI 126-160) with 5-year OS of 89%. Significant correlates of OS included: age 45 years or older (HR 2.83, 95%CI 1.25-6.43, P =0.013), HIV (HR 2.80, 95%CI 1.19-6.61, P =0.019), nodular sclerosistype (HR 0.29, 95%CI 0.12-0.71, P =0.006), CS4 disease (HR 3.05, 95%CI 1.20-7.77, P =0.019), BM positive for involvement with HL (HR 5.76, 95%CI 2.56-12.98, P

Description: Wiskott Aldrich syndrome (WAS) is a rare disease and hematopoietic stem cell transplant (HCT) is considered the treatment modality of choice for WAS. We conducted a cross-sectional analysis on the KIDS’ pediatric inpatient database and compared hospitalization rates, complications and healthcare utilizations in the transplant and non-transplant arms. Of the 383 pediatric admissions with diagnosis of WAS between 2006–2012, 114 underwent transplant and 269 did not. The non-transplant arm included older children, female patients and more African Americans. Death rates, income and payer source were similar in both arms, however the total charge for each admission was higher in the transplant arm. Emergency room visits were similar but non-elective admissions were more in the non-transplant arm. Length of stay was prolonged in the transplant arm. When comparing morbidities, lymphomas, ulcerative colitis and autoimmune complications of WAS were seen only in the non-transplant arm. Our study shows that transplant is the largest contributor to healthcare utilization in WAS patients. We identified healthcare disparities based on race and socioeconomic status and found that this rare disease is being appropriately directed to centers with HCT expertise. We noted a change in practice moving away from splenectomy in WAS patients.