The first reported use of red blood cell exchange to treat hemoglobin Evans with secondary methemoglobinemia.

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.

Depomedroxyprogesterone acetate therapy for hot flashes in survivors of breast cancer: no unfavorable impact on recurrence and 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.

Palliative radiotherapy for hepatobiliary obstruction caused by colorectal metastases.

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.

What happens in vagus: a case of recurrent paraganglioma with malignant transformation and an updated treatment algorithm†.

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.