Background: A low level of L-glutamine, a precursor of nicotinamide adenine dinucleotide (NAD) in red blood cells (RBCs), is identified as an underlying mechanism for the potential decrement of the NAD redox and the incidence of pain crisis in sickle cell anemia (SCA). The aim of this study is to assess the impact of oral L-glutamine therapy on pain crisis reduction in patients with SCA and sic...

CLINICAL MANIFESTATIONS OF INHERITED ABNORMAL HEMOGLOBINS. I. THE / \. INTERACTION OF HEMOGLOBIN-S WITH HEMOGLOBIN-D. II. INTERACTION / OF HEMOGLOBIN-E AND THALASSEMIA TRAIT Phillip Sturgeon, Harvey A. Itano and William R. Bergren 389 STUDIES ON ABNORMAL HEMOGLOBINS. XI. SICKLE CELL-THALASSEMIA DISEASE IN THE NEGRO. THE SIGNIFICANCE OF THE S + A + F AND S -4A PATTERNS OBTAINED BY HEMOGLOBIN ANA...

Spinal cord compression due to extramedullary hematopoiesis (EMH) is a rare complication of thalassemia and generally presents as paraparesis with sensory impairment.1 Complete paraplegia is extremely rare in EMH due to thalassemia although it is known to occur in polycythemia vera and sickle cell anemia.2 Cases presenting with paraparesis have been treated with either surgery or radiotherapy3 ...

Spinal cord compression due to extramedullary hematopoiesis (EMH) is a rare complication of thalassemia and generally presents as paraparesis with sensory impairment. Complete paraplegia is extremely rare in EMH due to thalassemia although it is known to occur in polycythemia vera and sickle cell anemia. Treatment options mostly include surgery and/or radiotherapy. Whereas cases presenting with...

Several human genetic disorders of hemoglobin have risen in frequency because of the protection they offer against death from malaria, sickle-cell anemia being a canonical example. Here we address the issue of why this highly protective mutant, present at high frequencies in subSaharan Africa, is uncommon in Mediterranean populations that instead harbor a diverse range of thalassemic hemoglobin...

In regions highly endemic for Plasmodium falciparum malaria, red cell polymorphisms that confer resistance to severe disease are widespread. Sickle cell trait, alpha-thalassemia, glucose-6-phosphate dehydrogenase deficiency, and blood groups were determined in 100 children from Gabon with severe malaria who were matched with 100 children with mild malaria and followed up for evaluation of reinf...

The red blood cell (RBC) is historically the original parent cell of microparticles (MPs). In this overview, we describe the discovery and the early history of red cell-derived microparticles (RMPs) and present an overview of the evolution of RMP. We report the formation, characteristics, effects of RMP and factors which may affect RMP evaluation. The review examines RMP derived from both norma...

By W. N. POWELL, M.D., J. G. RODARTE, M.D., AND J. V. NEEL, PH.D., M.D. A LTHOUGH in recent years the blood disorders sickle cell disease and thalassemia have both been studied extensively from the genetic standpoint, thus far the simultaneous occurrence within a single family group of the genes responsible for these two diseases has not been reported in the medical literature of the United Sta...

PURPOSE Evaluation of contrast sensitivity in patients with beta-thalassemia major and sickle cell disease which undergo regular transfusions and chelation therapy with desferrioxamine (DFX). MATERIALS AND METHODS We studied contrast sensitivity in 48 eyes (24 patients) with beta-thalassemia major (group A) and in 42 eyes (21 patients) with sickle cell disease (group B), compared to 60 eyes o...

A B S T R A C T Human hemoglobin messenger RNA was isolated by sucrose gradient centrifugation from reticulocytes of patients having various hemolytic anemias. Using a messenger RNA-dependent cell-free system derived entirely from rabbit reticulocytes, the human hemoglobin messenger RNA has been translated and the products analyzed by carboxymethylcellulose column chromatography. Normal messeng...