Induction bortezomib in Al amyloidosis followed by high dose melphalan and autologous stem cell transplantation: a single institution retrospective study.

Introduction/background: High-dose melphalan (HDM) followed by autologous stem cell transplant (ASCT) for light chain amyloidosis (AL) was performed in 31 patients at Oregon Health and Science University between 2005 and 2012. Fifteen patients had cardiac involvement.

Patients And Methods: Patients received melphalan 200 mg/m(2) or dose-adjusted HDM (100-140 mg/m(2)) depending on high risk features.

High levels of soluble serum hemojuvelin in patients with congenital dyserythropoietic anemia type I.

Objective: Congenital dyserythropoietic anemia (CDA) is a rare group of red blood cell disorders with ineffective erythropoiesis and secondary hemochromatosis. Inappropriate suppression of hepcidin and high levels of growth differentiation factor 15 (GDF15) have been described in CDA I and II patients, probably contributing to secondary hemochromatosis. Hemojuvelin (HJV) is an important regulator of serum hepcidin, while soluble form of HJV (s-HJV) competitively down-regulates hepcidin.

Left ventricular geometric abnormality screening in hypertensive patients using a hand-carried ultrasound device.

The authors assessed the validity of a hand-carried cardiac ultrasound device operated by an internal medicine resident for left ventricular geometric abnormalities (LVGAs) in mild hypertensive patients. LVGAs were diagnosed when at least one of the following was present: left ventricular mass index exceeding 125 g/m(2) and 110 g/m(2) for men and women, respectively; intraventricular septum thickness > or = 10 mm; posterior wall thickness > or = 10 mm; and left ventricular end-diastolic diameter > or = 5.3 mm.

Agranulocytosis at first presentation of autoimmune hepatitis type-1.

Autoimmune hepatitis (AIH) is a progressive, chronic disease of unknown cause with varying presenting symptoms, ranging from no symptoms through nonspecific symptoms to fulminant hepatic failure. Although nonspecific hematologic abnormalities in AIH may occur, a case of agranulocytosis (<100 neutrophils/microL) associated with a flare of AIH and suspected to be of autoimmune origin was recently reported. Increased levels of suppressing cytokines had been previously reported in bone marrow samples of patients with AIH type-1 (AIH-1).

Elevated growth differentiation factor 15 expression in patients with congenital dyserythropoietic anemia type I.

Congenital dyserythropoietic anemia (CDA) is a rare group of red blood cell disorders characterized by ineffective erythropoiesis and increased iron absorption. To determine whether growth differentation factor 15 (GDF15) hyper-expression is associated with the ineffective erythropoiesis and iron-loading complications of CDA type I (CDA I), GDF15 levels and other markers of erythropoiesis and iron overload were studied in blood from 17 CDA I patients. Significantly higher levels of GDF15 were detected among the CDA I patients (10 239 +/- 3049 pg/mL) compared with healthy volunteers (269 +/- 238 pg/mL).

Pregnancy outcome in congenital dyserythropoietic anemia type I.

Objectives: Congenital dyserythropoietic anemia type I (CDA I) is a rare inherited disease characterized by moderate to severe macrocytic anemia and abnormal erythroid precursors with nuclear chromatin bridges and spongy heterochromatin. Moderate to severe maternal anemia is a recognized independent risk factor for low birth weight (LBW) and complicated delivery. The aim of the study was to review the outcome of pregnancies in women with CDA I.

Fiber-optic biosensor to assess circulating phagocyte activity by chemiluminescence.

We describe herein the construction of a novel computerized multi-sample temperature-controlled luminometer for a fiber array-based biosensor to monitor circulating phagocyte activity. It can perform simultaneously integral measurements of chemiluminescence emitted from up to six samples containing less than 0.5 microl whole blood while the samples and detector do not change their position during the measurement cycle.