Showing papers by "Luke Y.C. Chen published in 2011"

Dysmetabolic hyperferritinemia is associated with normal transferrin saturation, mild hepatic iron overload, and elevated hepcidin.

Abstract: Hyperferritinemia is common in individuals with the metabolic syndrome (dysmetabolic hyperferritinemia), but its pathophysiology and the degree to which it reflects tissue iron overload remains unclear. We conducted a cross-sectional study evaluating ten cases with dysmetabolic hyperferritinemia for liver iron overload and compared their serum iron indices and urine hepcidin levels to healthy controls. Seven out of ten cases had mild hepatic iron overload by magnetic resonance imaging (MRI) (median, 75 μmol/g dry weight). Cases had higher serum ferritin than controls (median, 672 μg/L vs. 105 μg/L, p < 0.001), but the median transferrin saturation was not significantly different (38% vs. 36%, p = 0.5). Urinary hepcidin was elevated in dysmetabolic hyperferritinemia (median; 1,584 ng/mg of creatinine vs. 799 ng/mg of creatinine, p = 0.05). Dysmetabolic hyperferritinemia is characterized by hyperferritinemia with normal transferrin saturation, elevated hepcidin levels, and mild liver iron overload in a subset of patients.

Atypical autoimmune hemolytic anemia

Abstract: 9/L (10-90), bilirubin 1.5 mg/dL (0-1.0), lactate dehydrogenase 1715 U/L (normal < 210) and ferritin 697 ng/mL. The blood film showed microcytosis and coarse basophilic stippling consistent with β-thalassemia trait. Only very occasional spherocytes were noted and there was no agglutination. A bone marrow biopsy revealed mild erythroid hyperplasia and otherwise unremarkable morphology. The direct antiglobulin test (DAT) was repeatedly negative for IgG but weakly positive for C3d. Special DAT assays by gel method, cold saline wash and IgM/IgA substrates, were negative. Weak red cell autoantibodies were detectable eventually by polyethylene glycol (PEG) method through a reticulocyte enriched blood specimen, aiming to increase the sensitivity of the DAT assay by reducing the contamination of more aged transfused red cells. Weak red cell autoantibodies with no demonstrable specificity were also detected in the patient’s serum by PEG. An AHA with IgG autoantibody directed against reticulocytes or a mature RBC precursor was suspected based upon the reticulocytopenia and IgG positive DAT restricted to the reticulocyte enriched RBC fraction. 2 Prednisone 1.5 mg/kg/day, i.v. cyclophos phamide (~900 mg/m 2 monthly for five doses) and ritux imab (1,000 mg/m 2 for two doses) were administered with subsequent cessation of transfusion requirements and return to her baseline hemoglobin of 10.5 g/dL. AHA with atypical features, as illustrated above, raises challenges in diagnosis and management. Anemia can be multifactorial and peripheral blood morphology with coexisting conditions such as thalassemia trait 3 may be misleading. Importantly, 5-10% of patients with AHA are “DAT-negative”. A laboratory approach to such cases has been suggested but definitive proof of autoimmune hemolysis is often difficult to establish. 4

Hemophagocytic syndromes in adult intensive care units: response to Okabe et Al.

Abstract: A 19-year-old Japanese woman presented with fever, coryza, and abdominal pain progressing to respiratory, hepatic, and renal failure with shock requiring intensive care unit (ICU) admission. Her platelets were 20 giga/L, hemoglobin (Hb) 87 g/L, international normalized ratio (INR) 1.5, partial thromboplastin time (PTT) 80 seconds (normal 24-40 seconds), fibrinogen 2.3 g/L (normal 1.5-4.5), and ferritin 3312 mg/L (normal 20-300) with Epstein-Barr virus (EBV) DNA levels >100 000 copies/mL. Spleen size was on the upper limit of normal and a bone marrow biopsy revealed hemophagocytosis and increased T cells with absent natural killer (NK) cells on immunophenotyping. She received a modified Hemophagocytic Lymphohistiocytosis 2004 (HLH-2004) regimen consisting of high-dose dexamethasone and reduced-dose etoposide, cyclosporine, and 1 dose of rituximab. One dose of recombinant factor VIIa was given for severe intra-abdominal bleeding with immediate hemostasis. She improved and was able to return to Tokyo for further treatment.