Outcomes of inpatients with and without sickle cell disease after high‐volume surgical proceduresDinan, Michaela A.; Chou, Chia‐Hung; Hammill, Bradley G.; Graham, Felicia L.; Schulman, Kevin A.; Telen, Marilyn J.; Reed, Shelby D.
doi: 10.1002/ajh.21520pmid: 19787790
In this study, we examined differences in inpatient costs, length of stay, and in‐hospital mortality between hospitalizations for patients with and without sickle cell disease (SCD) undergoing high‐volume surgical procedures. We used Clinical Classification Software (CCS) codes to identify discharges in the 2002–2005 Nationwide Inpatient Sample of the Healthcare Cost and Utilization Project for patients who had undergone either cholecystectomy or hip replacement. We limited the non‐SCD cohort to hospitals where patients with SCD had undergone the same procedure. We compared inpatient outcomes using summary statistics and generalized linear regression analysis to adjust for patient, hospital, and procedural characteristics. Overall, the median age of surgical patients with SCD was more than three decades less than the median age of patients without SCD undergoing the same procedure. In recognition of the age disparity, we limited the analyses to patients aged 18 to 64 years. Nonetheless, patients with SCD undergoing cholecystectomy or hip replacement were 12.1 and 14.4 years younger, had inpatient stays that were 73% and 82% longer, and incurred costs that were 46% and 40% higher per discharge than patients without SCD, respectively. Inpatient mortality for these procedures was low, ∼0.6% for cholecystectomy and 0.2% for hip replacement and did not differ significantly between patients with and without SCD. Multivariable regression analyses revealed that higher inpatient costs among patients with SCD were primarily attributable to longer hospital stays. Patients with SCD who underwent cholecystectomy or hip replacement required more health care resources than patients without SCD. Am. J. Hematol. 2009. © 2009 Wiley‐Liss, Inc.
HFE, SLC40A1, HAMP, HJV, TFR2, and FTL mutations detected by denaturing high‐performance liquid chromatography after iron phenotyping and HFE C282Y and H63D genotyping in 785 HEIRS Study participants Barton, James C.; LaFreniere, Susie A.; Leiendecker‐Foster, Catherine; Li, Honggui; Acton, Ronald T.; Press, Richard D.; Eckfeldt, John H.
doi: 10.1002/ajh.21524pmid: 19787796
We sought to identify mutations that could explain iron phenotype heterogeneity in adults with previous HFE genotyping to detect C282Y and H63D. HEIRS Study participants genotyped for C282Y and H63D were designated as high transferrin saturation (TS) and/or serum ferritin (SF) (high TS/SF), low TS/SF, or controls. We grouped 191 C282Y homozygotes as high TS/SF, low TS/SF, or controls, and 594 other participants by race/ethnicity as high TS/SF or controls. Using denaturing high‐performance liquid chromatography (DHPLC), we screened 20 regions of HFE, SLC40A1, HAMP, HJV, TFR2, and FTL in each participant. DHPLC analyses were successful in 99.3% of 791 participants and detected 117 different mutations. In C282Y homozygotes, 4.0% of high TS/SF participants had SLC40A1 Q248H, HAMP ‐72C>T, or HAMP R59G heterozygosity (0% Controls; P = 0.1200). In whites, 4.1% with high TS/SF and 1.3% of controls had HFE S65C or E168Q (P = 0.3049). HJV c.‐6C>G and FTL L55L frequencies were greater in whites with high TS/SF than controls (0.0811 vs. 0.0200, P = 0.0144; 0.5743 vs. 0.4400, P = 0.0204, respectively). One Hispanic with high TS/SF (1.3%) had HAMP G71D heterozygosity. In blacks, SLC40A1 Q248H frequencies did not differ significantly between high TS/SF and control participants. Among Asians, 2.8% with high TS/SF were HFE V295A heterozygotes. Mutations other than HFE C282Y and H63D reported to be pathogenic were infrequently detected in high TS/SF participants. Genetic regions in linkage disequilibrium with HJV c.‐6C>G and FTL L55L could partly explain high TS/SF phenotypes in whites. Am. J. Hematol., 2009. Published 2009 Wiley‐Liss, Inc.
Secondary clonal cytogenetic abnormalities following successful treatment of acute promyelocytic leukemiaBatzios, Crisoula; Hayes, Lachlan A.; He, Simon Z.; Quach, Hang; McQuilten, Zoe K.; Wall, Meaghan; Campbell, Lynda J.
doi: 10.1002/ajh.21528pmid: 19806661
To identify patients who developed secondary clonal cytogenetic aberrations (CCA) following therapy for acute promyelocytic leukemia (APL), we retrospectively analyzed cytogenetic results from 123 patients diagnosed with APL between 1995 and 2007, who had ongoing cytogenetic analysis undertaken in our laboratory. During follow‐up for APL we identified 12 patients (9.8%) who developed CCA, not detected at diagnosis of APL and unrelated to their original APL karyotype. All patients had received all‐trans retinoic acid (ATRA) and chemotherapy and were in complete remission for APL when secondary CCA were identified. The median latency period between diagnosis of APL and emergence of secondary CCA was 27.5 months (range: 2–54 months). To date, four patients with CCA have been diagnosed with therapy‐related myelodysplastic syndrome (t‐MDS)/acute myeloid leukemia (t‐AML), giving a median t‐MDS/AML free survival of 78 months, with follow‐up ranging between 20 and 136 months from APL diagnosis. Three patients have died: two patients died of t‐AML and another developed relapsed APL with persistence of his secondary clone but no diagnosis of t‐MDS/AML and died from transplant‐related complications. Two patients are alive with t‐MDS. Seven patients with CCA are alive with no morphological evidence of MDS at the time of their last known follow‐up; thus median survival has not been reached. The appearance of these abnormalities in the absence of morphological evidence of MDS in the majority of patients is unusual, and highlights the importance of continued cytogenetic follow‐up in these patients. Am. J. Hematol., 2009. © 2009 Wiley‐Liss, Inc.
Severe iron overload in Blackfan‐Diamond anemia: A case‐control studyRoggero, Simona; Quarello, Paola; Vinciguerra, Tiziana; Longo, Filomena; Piga, Antonio; Ramenghi, Ugo
doi: 10.1002/ajh.21541pmid: 19810012
Chronic iron overload is a serious complication in transfusion‐dependent patients. Few studies have addressed this issue in Diamond‐Blackfan anemia (DBA). We describe a retrospective analysis of iron overload, and its related complications in 31 transfusion‐dependent Italian DBA patients whose records included one or more evaluation of liver iron concentration (LIC) by means of noninvasive magnetic liver susceptometry with a superconductive quantum interference device (SQUID). This cohort is also matched with a group of transfusion‐dependent β‐thalassemia major patients to look for differences. A severe iron overload was observed in 54% patients, especially among those inadequately chelated. The DBA patients displayed a significantly higher LIC than the regularly chelated β‐thalassemics. This difference may have been attributable to nonoptimal chelation (late onset, type, dose, prescription, and compliance), or an unknown biological mechanism that lead to an early severe iron overload. We therefore suggest that all transfusion patients should have an accurate record of their iron intake, a regular monitoring of iron overload, in order to start chelation when a critical transfusion load is reached, and to test the efficacy/compliance of chelation treatment. Physicians taking care of transfusion‐dependent DBA patients must be concerned about the frequent and early complications such as cardiac toxicity. Am. J. Hematol., 2009. © 2009 Wiley‐Liss, Inc.
Short‐term and long‐term failure of laparoscopic splenectomy in adult immune thrombocytopenic purpura patients: A systematic reviewMikhael, Joseph; Northridge, Kelly; Lindquist, Karla; Kessler, Craig; Deuson, Robert; Danese, Mark
doi: 10.1002/ajh.21501pmid: 19714591
Splenectomy is a common therapy for adults with chronic idiopathic thrombocytopenic purpura (ITP). Thisstudy was designed to estimate both the short‐term surgical non‐response rate and the long‐term relapse rate after laparoscopic splenectomy. A systematic review was conducted of articles published between January 1, 1991 and January 1, 2008. Selection criteria included: chronic ITP, study enrollment in 1990 or later, ≥12 months of follow‐up, ≥15 patients with ITP, ≥75% of patients at least 14 years of age, not HIV positive, not undergoing a second splenectomy, and type of performed splenectomy clearly reported. Data were pooled across studies to estimate rates. We identified 170 articles, of which 23 met our inclusion criteria (all observational studies). These studies represent 1,223 laparoscopic splenectomies (71 or 5.6% were converted to open splenectomy during surgery). The pooled short‐term surgical non‐response rate among the 18 studies reporting data was 8.2% (95% CI 5.4–11.0). The pooled long‐term relapse rate across all 23 studies was 43.6 per 1,000 patient years (95% CI 28.2–67.2). This translates to an approximate failure rate of 28% at 5 years for all patients undergoing splenectomy. Studies with shorter durations of follow‐up had significantly higher pooled relapse rates than studies with longer follow‐up (P = 0.04). Laparoscopicsplenectomy is effective for most patients. Splenectomy may have higher initial relapse rates, particularly, in the first 2 years after surgery, and the rate may decline over time. Am. J. Hematol. 2009. © 2009 Wiley‐Liss, Inc.