This manuscript describes a novel approach for treating patients with long‐term sequelae from hemoglobin Evans (Hb Evans). After instituting conservative therapies for approximately 2 years, our ...patient's symptoms continually worsened. Therefore, we performed red blood cell exchange (RBCx) to reduce his Hb Evans percentage and his co‐existing elevation of methemoglobin. Our assumptions of clinical benefit were based on our collective experience performing RBCx for patients with sickle cell disease. After the first exchange, pre‐ and post‐laboratory results supported our approach and the patient experienced marked improvement in his clinical signs and symptoms. This report provides preliminary proof of principle for the use of RBCx to treat Hb Evans and other non‐Hb S hemoglobinopathies.
AIM To review microbiome alterations associated with pancreatic cancer, its potential utility in diagnostics, risk assessment, and influence on disease outcomes.METHODS A comprehensive literature ...review was conducted by allinclusive topic review from PubM ed, MEDLINE, and Web of Science. The last search was performed in October 2016.RESULTS Diverse microbiome alterations exist among several body sites including oral, gut, and pancreatic tissue, in patients with pancreatic cancer compared to healthy populations.CONCLUSION Pilot study successes in non-invasive screening strategies warrant further investigation for future translational application in early diagnostics and learn modifiable risk factors relevant to disease prevention. Pre-clinical investigations exist in other tumor types that suggest microbiome manipulation provides opportunity to favorably transform cancer response to existing treatment protocols and improve survival.
Purpose
Survivors of estrogen receptor–expressing breast cancer generally do not receive estrogen-based therapy for menopausal symptoms due to concern for provoking recurrence of disease. Single-dose ...depomedroxyprogesterone acetate has been shown to be among the most effective non-estrogen strategies for treatment of menopausal hot flashes, but long-term evidence for safety in survivors is lacking.
Methods
We conducted an institutional review board approved, retrospective, case-control cohort study at a tertiary, academic referral center. Patients with estrogen receptor-expressing early-stage operable breast cancer who received depomedroxyprogesterone acetate for hot flashes between January 2005 and December 2012 were identified. We confirmed 75 patients who met strict inclusion criteria who were matched 1:1 with controls for age, stage of disease, HER2 status, and year of diagnosis. Overall survival, loco-regional recurrence-free survival, and progression-free survival assessments for cases were compared with controls.
Results
Median follow-up duration was 68.4 months in cases and 57.6 months in controls. Estimated local-regional recurrence-free survival at 10 years was 97% (95% CI, 92–100%) in cases and 98% (95% CI, 95–100%) in controls. Estimated progression-free survival at 10 years was 89% (95% CI, 80–100%) in cases and 83% (95% CI, 73–95) in controls. The majority (75%) of case patients experienced satisfactory relief of hot flashes from depomedroxyprogesterone injection.
Discussion
In this retrospective case-control study, we were unable to identify a detrimental effect of depomedroxyprogesterone acetate therapy for hot flashes in survivors of estrogen receptor–expressing breast cancer. Depomedroxyprogesterone acetate may be acceptable for management of hot flashes in this population.
Based on the current WHO Classification of Myeloid Neoplasms, cytogenetic findings play a central role in the diagnostic classification of the myeloid malignancies. Cytogenetic abnormalities detected ...at primary diagnosis may change over time. Karyotype changes can be characterized as cytogenetic evolution, cytogenetic regression or a combination of both. While the exact mechanism of cytogenetic evolution is not completely understood, the process of cytogenetic evolution is not random, but follows different, and often disease-specific patterns during progression and relapse of myeloid neoplasms. Important lessons were learned from the cytogenetic evolution pathways observed over the course of chronic myelogenous leukemia (CML), progressing through chronic phase into accelerated phase and blast crisis. Cytogenetic evolution pathways of CML are divided into major and minor route abnormalities. The major route changes include an extra Ph chromosome (+Ph) trisomy 8 (+8) and the occurrence of an i(17q). The six most common minor route abnormalities include -7, -17, +17, +21 and -Y and one structural change, t(3;21). Recently an increased number of CML cases with karyotype abnormalities in Ph-negative cells have been reported in patients treated with imatinib. These abnormalities include trisomy 8, abnormalities of chromosome 7, and chromosome 20. The significance of the Ph-negative karyotype changes in subsequent development of myelodysplastic syndrome (MDS) or acute myeloid leukemia (AML) is unclear. Nevertheless, the occurrence of clonal abnormalities in Ph-negative cells underlines the importance of conventional cytogenetic studies in monitoring of CML patients. In AML, karyotype changes commonly occur between diagnosis and relapse status post chemotherapy. Karyotype evolution seems more common in patients who had unfavorable aberrations at diagnosis as compared to patients with favorable or intermediate abnormalities. Karyotype evolution results in shortened remission duration as compared to cases without cytogenetic change. Recent studies on cytogenetic evolution at relapse after allogeneic hematopoietic cell transplantation (allo-HCT) were similar to the data observed in chemotherapy-treated AML. Serial bone marrow evaluations after allo-HCT offer insights into the dynamics of karyotype evolution, notably, they demonstrated that a detection of karyotype abnormalities is usually followed by a relapse within the next 90 days. As a contrast, karyotype abnormalities were not observed in patients who do not relapse in the next 3 months. CGE at relapse was associated with significantly decreased postrelapse and post-transplantation survival compared with the non-CGE group. Very few data exist regarding a potential association between the dose or certain types of chemotherapy and cytogenetic evolution. Based on the results of a single study conducted recently, no specific chemotherapy regimen emerged to predispose for cytogenetic evolution. Further studies are necessary to evaluate the impact of the altered bone marrow environment and immunosuppression on karyotype stability.
Hyperbilirubinemia in the setting of stent-intolerant biliary obstruction is a challenging problem and can prevent cancer patients from pursuing additional treatments such as further systemic ...therapies. We report a case of a 75-year-old female who underwent treatment with palliative radiotherapy (RT) for relieving persistent biliary obstruction secondary to liver metastases from colorectal disease, despite prior appropriate stent placement. Prior to RT, the patient's total bilirubin was 14.6 mg/dL, and she experienced fatigue, diarrhea, nausea, vomiting, and severe jaundice. After treatment with 37.5 Gy in 15 once daily fractions, total bilirubin decreased to 3.9 mg/dL, with resolution of previous symptoms including jaundice and pruritus. The patient did not experience any significant treatment-related toxicities. This case, along with a succinct literature review, demonstrates that palliative RT can be successful in relieving biliary obstruction unrelieved by biliary stent. Further research is required to evaluate the efficacy of RT in palliating biliary obstruction for liver metastases in a general population.
Purpose of Review
In this review article, we will highlight ethical issues faced by hematologists due to a growing constellation of expensive diagnostics and therapeutics in hematology. We outline ...the important issues surrounding this topic including stakeholders, cost considerations, and various ethical challenges surrounding access to care, communication about costs, and individual vs. societal responsibilities. We review available tools to navigate these ethical themes and offer potential solutions.
Recent Findings
We identified several gaps in the literature on the topic of ethical issues in hematology treatment and supplement by non-hematological cancer and general medical literature.
Summary
We propose proactive solutions to address these problems to include cost transparency, utilization of evidence-based decision making tools, application of the four quadrant approach to ethical care, and advanced systems-based practice curriculum for physician trainees.
Paragangliomas (PGLs) are rare, extra-adrenal tumors, originating from neural crest cells and can occur anywhere from the skull base to the pelvic floor. Although these tumors are often benign, a ...fraction of malignant cases exist. Few isolated cases of malignant head and neck PGL are reported in the literature. Treatment algorithms rely heavily on retrospective case studies and institutional experience. We report an unusual case of an extensive, hereditary PGL, with invasive characteristics, that was refractory to radiation therapy. An operative approach was selected for recurrent disease in the setting of critical neurovascular structure compromise. Six months postoperatively, the patient was recovering as expected and had no evidence of recurrent disease. We propose a modified treatment algorithm based on an updated literature review that encompasses the spectrum of PGL, from benign and asymptomatic to invasive and malignant disease.
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