Acute autoimmune hemolytic anemia due to anti-Ena autoantibody successfully treated with rituximab.

BACKGROUND: Autoimmune hemolytic anemia (AIHA) due to anti-Ena has been previously reported in association with massive intravascular hemolysis, disseminated intravascular coagulation, and fatal outcomes. Here we report a case of successfully treated AIHA due to anti-Ena . CASE REPORT: A 69-year-old male with a past medical history of cirrhosis due to nonalcoholic steatohepatitis status post-orthotopic liver transplant presented with 1-month history of progressive anemia. At presentation, his hemoglobin (Hb) was 5.6 g/dL, hematocrit (Hct) 16%, reticulocytes 0.3%, direct bilirubin (bili) 4 g/dL, lactate dehydrogenase 533 units/L (reference, 125-220 units/L), and haptoglobin 254 mg/dL (reference, 40-273 mg/dL). Blood bank testing revealed an autoantibody present in his plasma and a direct antiglobulin test positive for immunoglobulin G (IgC) but negative for complement. He received 1 unit of an incompatible blood group O phenotypically matched red blood cell unit. RESULTS: Over the course of the next 5 days, the Hb and Hct decreased to 4.1 g/dL and 12%, respectively, direct bili increased to 12.3 mg/day, reticulocytes slightly increased to 0.9%, and haptoglobin decreased to less than 8 mg/dL. Marrow study showed a hypercellular marrow with erythroid hyperplasia. Additional workup performed at a reference laboratory identified an anti-Ena autoantibody. He received prednisone and weekly rituximab infusions and was monitored weekly. At the 2-month visit, Hb and Hct were 10 g/dL and 32%, respectively. CONCLUSION: Unlike two of the previously reported fatal cases of AIHA with anti-Ena specificity, this 69-year-old male treated with weekly rituximab infusion underwent clinical recovery and significant anemia improvement.

Diagnostic validation of three test methods for detection of cyprinid herpesvirus 3 (CyHV-3).

Cyprinid herpesvirus 3 (CyHV-3) is the aetiological agent of koi herpesvirus disease in koi and common carp. The disease is notifiable to the World Organisation for Animal Health. Three tests-quantitative polymerase chain reaction (qPCR), conventional PCR (cPCR) and virus isolation by cell culture (VI)-were validated to assess their fitness as diagnostic tools for detection of CyHV-3. Test performance metrics of diagnostic accuracy were sensitivity (DSe) and specificity (DSp). Repeatability and reproducibility were measured to assess diagnostic precision. Estimates of test accuracy, in the absence of a gold standard reference test, were generated using latent class models. Test samples originated from wild common carp naturally exposed to CyHV-3 or domesticated koi either virus free or experimentally infected with the virus. Three laboratories in Canada participated in the precision study. Moderate to high repeatability (81 to 99%) and reproducibility (72 to 97%) were observed for the qPCR and cPCR tests. The lack of agreement observed between some of the PCR test pair results was attributed to cross-contamination of samples with CyHV-3 nucleic acid. Accuracy estimates for the PCR tests were 99% for DSe and 93% for DSp. Poor precision was observed for the VI test (4 to 95%). Accuracy estimates for VI/qPCR were 90% for DSe and 88% for DSp. Collectively, the results show that the CyHV-3 qPCR test is a suitable tool for surveillance, presumptive diagnosis and certification of individuals or populations as CyHV-3 free.

Clinical characteristics, prognostic factors, and survival of 393 patients with mycosis fungoides and Sézary syndrome in the southeastern United States: a single-institution cohort.

BACKGROUND: Limited data exist on patients with mycosis fungoides (MF) and Sézary syndrome (SS) from the southeastern United States, a region with a high proportion of African Americans (AA). OBJECTIVES: We sought to determine clinical characteristics, prognostic factors, and survival of patients with MF/SS in a southeastern US cohort, compare with other cohorts, and validate proposed revisions in MF/SS staging. METHODS: This was a retrospective chart review of patients from an academic dermatology referral center (Atlanta, GA) from 1998 to 2013. Kaplan-Meier estimates were calculated for overall survival, disease-specific survival, and progression; univariate and multivariate Cox proportional hazard models were used for assessment of prognostic variables. RESULTS: Of 393 patients, 55.2% were white, 43.3% AA, and 1.5% other; 52.7% were male and 47.3% female (ratio 1.1:1). Mean age was 53.6 years; mean age among AA was 48.9 years. In all, 19.6% died of disease; 21.9% experienced disease progression. Advanced TNMB classification, presence of a circulating clone without phenotypic evidence of blood involvement, and older age were predictors of poor disease-specific survival in the multivariate analysis, whereas AA race was not. LIMITATIONS: This study was from a single academic center. CONCLUSIONS: Outcomes of our patients generally paralleled those of other geographic regions. MF/SS may affect younger patients and more women than previously recognized, particularly among AA. Survival among AA may be more favorable than that observed in prior reports. Our data support the validity of the staging criteria revisions for MF/SS.

Secretory Carcinoma of the Thyroid Gland: Report of a Highly Aggressive Case Clinically Mimicking Undifferentiated Carcinoma and Review of the Literature.

After being described in the salivary glands as a malignancy with features essentially identical to those of the breast, secretory carcinoma (SC) (formerly mammary analogue SC) has now been identified in other sites including the skin, lung, and thyroid gland. In the breast, SC has a relatively favorable prognosis. Likewise when arising in the salivary glands, it is generally considered to be a low to intermediate grade carcinoma; however, there is a range of clinical behavior with occasional patients dying of progressive disease. SCs of the thyroid gland are rare, and reports suggest a relatively aggressive behavior, at least relative to well differentiated carcinomas such as papillary carcinoma and minimally invasive follicular carcinoma. We present a patient with a highly aggressive thyroid gland SC that mimicked undifferentiated carcinoma clinically. The patient had widespread metastatic disease and died rapidly from airway compromise. We also review the literature for reported cases of thyroid gland SC in order to better establish the clinical features and expected clinical course of such tumors occurring at this site.