Summary Tissue accumulation of advanced glycation end‐products (AGEs) has been implicated in the oxidant‐induced vascular pathology of diabetes and other diseases. Because homozygous sickle cell anaemia (SCA) is a state of oxidative stress, we tested the hypothesis that circulating AGE levels are elevated in SCA. Blood was obtained from age‐ and race‐matched children classified as either non‐sickle cell controls, SCA without vaso‐occlusive crisis (SCA − VOC), or SCA with vaso‐occlusive crisis (SCA + VOC). Plasma and red blood cell (RBC) AGE levels were measured by immunoassay. RBC levels of reduced (GSH) and oxidized (GSSG) glutathione were measured by capillary electrophoresis as an indicator of endogenous antioxidant status. The results showed that plasma AGE levels and the rate of RBC AGE accumulation were significantly higher in patients with SCA compared with controls. GSH was not different between groups but was significantly inversely correlated with plasma AGEs in both controls and patients with SCA. GSSG was significantly lower and GSH/GSSG higher in SCA + VOC patients, suggesting that GSH/GSSG might be an objective indicator of acute VOC or a risk factor for VOC. We conclude that circulating AGE levels are strongly influenced by endogenous antioxidant status and may play a role in the vascular pathology of SCA.

This report describes a rare case of multiple intracranial, extradural chloromas. A five year old African American male presented with headache, fever, and vomiting. The peripheral blood smear showed myeloblasts with Auer rods. The CTscan of the brain showed three intracranial, epidural lesions as well as soft tissue masses in the retroorbital region and sphenoid sinuses. CTscan of the chest showed two paraspinal epidural thoracic masses. Pathology of the epidural intracranial mass revealed a granulocytic sarcoma. Cytogenetic analysis showed simultaneous occurrence of t(8;21) and trisomy 8. Following induction therapy, he is now in complete remission.

Ovarian metastasis of childhood tumors is rare despite their aggressive nature. The childhood tumor that spreads to the ovary most frequently is the neuroblastoma. The clinical features and frequent bilaterality of ovarian metastatic tumors are helpful diagnostic features in many cases, but when the ovarian tumor is the presenting manifestation of the disease, differentiation from various primary ovarian tumors may be difficult. In this paper, a rare case of metastatic ovarian neuroblastoma is reported.

This article reports a case of cystic nephroma to bring awareness about the benign nature of this condition. The patient presented with a painless abdominal mass. Computed tomography showed a homogeneous, multicystic tumor of the superolateral portion of the left kidney with thin septa without solid parts. Histology confirmed the diagnosis of cystic nephroma.

Hb Iowa is a rare hemoglobin (Hb) variant with a Gly --> Ala substitution at position 119 of beta-globin. It was previously reported only in an African American infant who was also heterozygous for Hb S [beta6(A3)Glu --> Val] and her mother (Hb A/Iowa). Here we describe the second report of Hb Iowa, the first in conjunction with Hb C [beta6(A3)Glu --> Lys]. The patient was an African American girl, originally diagnosed as homozygous Hb C during neonatal screening. When seen in our clinic, hematological data for both the child and her mother (Hb C trait) indicated mild anemia with slightly low mean corpuscular volume (MCV) but normal red blood cell (RBC) count. Analysis of blood from the child by capillary isoelectric focusing (cIEF) identified Hb C and an unknown Hb variant with an isoelectric point (pI) intermediate to that of Hbs F and A. The unknown variant was identified as Hb Iowa by DNA sequence analysis of the beta-globin gene (GGC --> GCC). Both reported cases of Hb Iowa indicated that there are no abnormal hematological manifestations associated with this rare Hb variant. In both cases, however, Hb Iowa was mistaken for Hb F during routine neonatal screening by high performance liquid chromatography (HPLC) and/or gel IEF. Neonatal misidentification of Hb Iowa led to misdiagnosis of sickle cell disease and Hb C disease, respectively. In our patient, Hb Iowa was also misidentified as Hb A at 2 years of age by a commercial reference laboratory using cellulose acetate and citrate agar gel electrophoresis. This led to an incorrect diagnosis of Hb C trait. These results show that commonly used analytical methods can easily misidentify Hb Iowa as Hbs F or A in neonates, or older individuals, resulting in incorrect identification of the Hb phenotype. We conclude that the presence of Hb Iowa, or other variants with similar pIs, should be considered in cases where the results of follow-up testing conflict with neonatal diagnosis of sickle cell or Hb C disease, or where clinical presentation does not agree with diagnosis of either homozygous or heterozygous Hb S or Hb C.