Mohamad Cherry

Haematopoietic cell transplantation for blastic plasmacytoid dendritic cell neoplasm: a North American multicentre collaborative study

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is incurable with conventional therapies. Limited retrospective data have shown durable remissions after haematopoietic cell transplantation (HCT) [allogeneic (allo) or autologous (auto)]. We conducted a multicentre retrospective study in BPDCN patients treated with allo‐HCT and auto‐HCT at 8 centres in the United States and Canada. Primary endpoint was overall survival (OS). The population consisted of 45 consecutive patients who received an allo‐HCT (n = 37) or an auto‐HCT (n = 8) regardless of age, pre‐transplant therapies, or remission status at transplantation. Allo‐HCT recipients were younger (50 (14–74) vs. 67 (45–72) years, P = 0·01) and had 1‐year and 3‐year OS of 68% [95% confidence interval (CI) = 49–81%] and 58% (95% CI = 38–75%), respectively. Allo‐HCT in first complete remission (CR1) yielded superior 3‐year OS (versus not in CR1) [74% (95% CI = 48–89%) vs. 0, P < 0·0001]. Allo‐HCT outcomes were not impacted by regimen intensity [3‐year OS for myeloablative conditioning = 61% (95% CI = 28–83%) vs. reduced‐intensity conditioning = 55% (95% CI = 28–76%)]. One‐year OS for auto‐HCT recipients was 11% (95% CI = 8–50%). These results demonstrate efficacy of allo‐HCT in BPDCN, especially in patients in CR1. Pertaining to auto‐HCT, our results suggest lack of efficacy against BPDCN, but this observation is limited by the small sample size. Larger prospective studies are needed to better define the role of HCT in BPDCN.

Outcome of Acute Myeloid Leukemia and High-Risk Myelodysplastic Syndrome According to Health Insurance Status

BACKGROUNDnAge, cytogenetic status, and molecular features are the most important prognostic factors in acute myeloid leukemia (AML). This study aimed to analyze the outcomes of patients with AML or high-risk myelodysplastic syndrome (MDS) according to insurance status.nnnPATIENTS AND METHODSnA retrospective chart review was performed, covering all patients with AML and high-risk MDS evaluated and treated at Akron General Medical Center between 2002 and 2012. A Cox regression model was analyzed to account for survival over time, adjusted for insurance type, while controlling for patient age at diagnosis and patient risk of mortality.nnnRESULTSnA total of 130 adult patients (age ≥ 18 years) were identified. Insurance information was available for 97 patients enrolled in the study; 3 were excluded because of self-pay status. Cox regression analysis with insurance type as the predictor found that overall survival declines over time and that the rate of decline may be influenced by insurance type (χ(2)(2) = 6.4; P = .044). The likelihood of survival in patients with Medicaid or Medicare without supplemental insurance was .552 (95% CI, .338-.903; P = .018) times the likelihood in patients who had Medicare with supplemental insurance. To explain the difference, variables of age, gender, and risk of mortality were added to the model. Age and risk of mortality were found to be significant predictors of survival. The addition of insurance type to the model did not significantly contribute (χ(2)(3) = 3.83; P = .147).nnnCONCLUSIONnNo significant difference in overall survival was observed when patients with AML or high-risk MDS were analyzed according to their health insurance status. The overall survival was low in this study compared with the national average. Early referral to a specialized center or possible clinical trial enrollment may be a good alternative to improve outcome.

Extranodal Marginal Zone Lymphoma of the Central Nervous System

Abstract Extranodal marginal zone lymphoma of the central nervous system (CNS EMZBL) is a rare disease. We present a review of the literature and describe its presentation, differential diagnosis, treatment options, and outcomes. Systematic search of PubMed, Medline, and Embase databases via the Ovid engine for primary articles and case reports yielded 37 unduplicated peer‐reviewed articles of CNS EMZBL. We identified 69 cases in these articles and 1 unreported case at our institution, which were included for this reviews analysis. Median age at diagnosis was 55 years (range, 18‐78 years), with a female preponderance of 77% (n = 54). Most common presenting symptoms were headache in 43% (n = 30), seizures in 31% (n = 22), and visual defects in 27% (n = 19). The most common treatment modalities were localized therapies, which were provided to 67% (n = 47) of cases. These included radiotherapy in 27% (n = 19), radiotherapy with surgery in 24% (n = 17), and surgery alone in 16% (n = 11). Ninety percent (n = 63) of patients had a median follow‐up of 23 months. Complete remission was experienced by 77% (n = 49) patients, and 22% (n = 14) were alive with disease. Three patients had evidence of relapse, and one patient died. CNS EMZBL is an indolent, low‐grade, radiosensitive lymphoma with good treatment outcomes and prognosis. It is an important differential to consider in extra‐axial dural‐based masses. Individualized management plans, with preference given to localized treatment options, should be considered after factoring in the site and extent of disease, its resectability, and the expected adverse effects of systemic therapy.

Impact of Health Care Insurance Status on Treatment Outcomes of Acute Myeloid Leukemia

Micro‐Abstract Insurance status has been found to influence treatment outcomes in various solid tumors, but limited data is available for patients with acute myeloid leukemia (AML). A total of 161 patients were included in this retrospective study to examine the impact of insurance source in AML outcomes in the state of Oklahoma. Insurance source at diagnosis was found to have no impact on AML treatment outcomes and receipt of bone marrow transplant. The consistency of our results with some, but not all, studies is probably driven primarily by access‐to‐care requirements among different states. Introduction: Insurance status has been found to influence treatment outcomes in various solid tumors. Limited data with conflicting results are available in patients with acute myeloid leukemia (AML). We examined the impact of health insurance at diagnosis on AML treatment outcomes. Patients and Methods: All consecutive adult patients (≥ 18 years of age) diagnosed with AML between 2002 and 2011 and followed through August 2013 were included. Survival estimates were calculated by Kaplan‐Meier survival curves. Logistic regression and multivariate Cox proportional hazards methods were used to explore the influence of multiple baseline covariates on treatment outcomes. Results: A total of 217 patients with complete medical records were identified. Of these, 161 patients had complete cytogenetic/molecular data for risk stratification and were included in the final efficacy analyses. Most patients (45.8%) were publicly insured, 36.3% were privately insured, and 17.3% were uninsured. No significant association was found between insurance source and cytogenetic/molecular risk status. Transplantation information was available for 157 patients, with no significant association found between transplant receipt and insurance source. After adjustment for age, cytogenetic/molecular risk, and transplant receipt, we found no statistically significant association between the insurance source and either event‐free or overall survival. Conclusion: Insurance source at diagnosis has no impact on AML treatment outcomes. The consistency of our results with some, but not all, studies is probably driven primarily by access‐to‐care eligibility requirements among different states. Further efforts to better understand such disparities are warranted.

Efficacy of High-Dose Therapy and Autologous Hematopoietic Cell Transplantation in Gray Zone Lymphoma: A US Multicenter Collaborative Study

High-dose therapy (HDT) and autologous hematopoietic cell transplantation (auto-HCT) has been anecdotally prescribed in gray zone lymphoma (GZL), showing encouraging efficacy. We conducted a multicenter retrospective study aimed at assessing outcomes after auto-HCT in 32 patients with GZL treated at 9 transplantation centers in the United States. The median age of patients at transplantation was 38 years (range, 18 to 70 years), and the majority were male (nu2009=u200921; 66%). The median number of lines of therapy before transplantation was 2 (range, 1 to 4). BEAM was the most commonly prescribed regimen (nu2009=u200923; 72%). The median duration of follow-up for surviving patients was 34 months (range, 1 to 106 months). Median overall survival (OS) was not reached. The 3-year progression-free survival (PFS) and OS for all patients were 69% and 78%, respectively. Three-year PFS and OS were 100% for patients who received only 1 line of therapy before auto-HCT versus 65% (PFS, Pu2009=u2009.25) and 75% (OS, Pu2009=u2009.39) for those receiving >1 line. The cumulative incidence of relapse/progression was 4% at 1 year post-transplantation and 31% at 3 years post-transplantation. The 3-year nonrelapse mortality was 0%. These findings suggest that HDT and auto-HCT is an effective treatment in patients with GZL. Our findings ideally require confirmation in a larger cohort of patients, preferably in the setting of large prospective multicenter randomized controlled trials. However, we acknowledge that such studies could be difficult to conduct in patients with GZL owing to the diseases rarity. Alternatively, a multicenter prospective study that includes tissue banking and a data registry is warranted to help better understand the biology and natural history of the disease.

Non-seminomatous mediastinal germ cell tumor and acute megakaryoblastic leukemia

The association between mediastinal germ cell tumors (MGCT) and acute megakaryoblastic (M7) leukemia has been known for many years. We hereby present this review to better characterize the coexistence of these entities as well as the salient features, the treatment options, and the overall prognosis. A search of PUBMED, Medline, and EMBASE databases via OVID engine for primary articles and case reports under keywords “germ cell tumors” and “acute myeloid leukemia” revealed a total of 26 cases in English that reported MGCT and M7 leukemia. The median age at diagnosis of MGCT was 24 (13–36) years. All cases were stage III. All cases of MGCT were of non-seminomatous origin and one case was unclassified. MGCT occurred prior to the diagnosis of leukemia in 46% of cases and concomitantly in 31% of cases. M7 leukemia was never reported prior to the appearance of MGCT. Complex cytogenetics and hyperdiploidy were the most commonly reported cytogenetic abnormalities. In the 23 cases where the treatment regimen was available, platinum-based chemotherapy directed towards management of the germ cell tumors was used initially in 21 cases and leukemia-directed treatment was used initially in 2 cases only. The median time from diagnosis of MGCT to development of M7 leukemia was 5 (2.25–39) months. Median time to death from the initial diagnosis of MGCT was 6xa0(0.5–60)xa0months. Patients with a history of MGCT are at higher risk of developing M7 leukemia. They need long-term follow-up with a particular attention to the development of hematological malignancies. The overall prognosis remains poor.

Autoimmune hemolytic anemia and thrombocytopenia attributed to an intrauterine contraceptive device.

Evans syndrome is a rare condition manifested by combined autoimmune hemolytic anemia (AIHA) and thrombocytopenia or neutropenia. It is often associated with other autoimmune disorders, immunodeficiencies, and non‐Hodgkins lymphoma.

Central venous catheter-related complications in acute myeloid leukemia patients

Central venous catheter-related complications in acute myeloid leukemia patients all cases. DVT rates were 22% in patients with PICC, 6% with IP and none with Hickman (p=0.002). When divided by age group, thrombosis rates were 9% in patients 55 years of age or older and 17% in those <55 years of age (p=0.29). In conclusion, among AML patients referred to our institution, PICCs were associated with significantly higher rate of DVT compared to IPs and Hickman. There was no statistically significant difference in BSI across the different catheter types. Prospective studies exploring catheterrelated complications in AML patients are warranted.