Splenomegaly with Hypersplenism in Sickle Cell Anemia Treated by Radiation—Case Report

PEDIATRICS ◽  
1971 ◽  
Vol 48 (3) ◽  
pp. 457-458
Author(s):  
Annemarie Sommer ◽  
Stella B. Kontras
Keyword(s):  
Cell Survival ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Marked Improvement ◽  
Costal Margin ◽  
Red Cell ◽  
Older Children ◽  
Before And After ◽  
Red Cell Survival ◽  
Survival Studies

The incidence of splenomegaly in sickle cell anemia (defined as a spleen easily palpated below the costal margin in quiet respiration) appears to be around 10% after 10 years of age. Persistent splenomegaly in older children is rare and frequently is associated with hypersplenism. Splenectomy has been the treatment of choice in several reported cases based on red cell survival studies before and after splenectomy.1-3 Removal of the spleen has been found to be associated with marked improvement of previously very shortened red cell survival. We want to report the case of a 12-year-old boy with sickle cell anemia, splenomegaly, and sickle cell heart disease who was treated by radiation therapy for his enlarged spleen because of hypersplenism.

scholarly journals Reticulocyte Survival in Sickle Cell Anemia: Effect of Cyanate

Blood ◽  
1972 ◽  
Vol 40 (5) ◽  
pp. 733-739 ◽  
Author(s):  
Blanche P. Alter ◽  
Yuet Wai Kan ◽  
David G. Nathan
Keyword(s):  
Cell Survival ◽  
Sickle Cell ◽  
Fetal Hemoglobin ◽  
Red Cells ◽  
Red Cell ◽  
Hemoglobin Molecule ◽  
Red Cell Survival ◽  
And Control

Abstract Cyanate prevents sickling in vitro and apparently prolongs the survival of 51Cr-tagged sickle erythrocytes in vivo. Cautious interpretation is required because the effects of cyanate on 51Cr binding to sickle and fetal hemoglobin-containing red cells are unknown, and comparison of the effect of cyanate on sickle red cell survival to control red cell survival must be performed sequentially. We have studied the survival of sickle reticulocytes utilizing radioactive amino acids that are incorporated into hemoglobin. Two informed adult patients with sickle cell disease were studied. In each study, two 50-ml samples of blood were incubated separately with 14C- and 3H-leucine for 2 hr, after which 50 mM cyanate was added to one aliquot for 1 hr. The cells were then washed and reinfused. Frequent venous samples were obtained, and the specific activities of 14C and 3H in the hemoglobin were followed. The t ½ of the carbamylated cells was tripled, but remained below normal. This method provides a generally useful measurement of the influence of drugs bound to red cells on reticulocyte lifespan. The labels are incorporated into the hemoglobin molecule of the reticulocyte, and simultaneous comparison of the survivals of the same cohort of drug-treated and control cells is achieved.

Short survival of phosphatidylserine-exposing red blood cells in murine sickle cell anemia

Blood ◽  
2001 ◽  
Vol 98 (5) ◽  
pp. 1577-1584 ◽  
Author(s):  
Kitty de Jong ◽  
Renee K. Emerson ◽  
James Butler ◽  
Jacob Bastacky ◽  
Narla Mohandas ◽  
...  
Keyword(s):  
Red Blood Cells ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Blood Cells ◽  
Cell Model ◽  
Red Cell ◽  
Sickle Cells ◽  
Red Cell Survival ◽  
Transgenic Murine Models

Several transgenic murine models for sickle cell anemia have been developed that closely reproduce the biochemical and physiological disorders in the human disease. A comprehensive characterization is described of hematologic parameters of mature red blood cells, reticulocytes, and red cell precursors in the bone marrow and spleen of a murine sickle cell model in which erythroid cells expressed exclusively human α, γ, and βS globin. Red cell survival was dramatically decreased in these anemic animals, partially compensated by considerable enhancement in erythropoietic activity. As in humans, these murine sickle cells contain a subpopulation of phosphatidylserine-exposing cells that may play a role in their premature removal. Continuous in vivo generation of this phosphatidylserine-exposing subset may have a significant impact on the pathophysiology of sickle cell disease.

Polycythaemia Vera Results of Repeated Radioisotope Studies in 53 Patients During a Five-Year Period

1963 ◽  
Vol 03 (01) ◽  
pp. 1-19
Author(s):  
J. E. Varela ◽  
E. M. Rochna Viola ◽  
A. O. Carmena ◽  
M. A. Etcheverry ◽  
S. Kremenchuzky
Keyword(s):  
Cell Survival ◽  
Correct Diagnosis ◽  
Sufficient Information ◽  
Polycythaemia Vera ◽  
Red Cell ◽  
Plasma Iron ◽  
Before And After ◽  
Red Cell Survival ◽  
Iron Pool ◽  
Plasma Half Life

Summary53 patients with a confirmed diagnosis of polycythaemia vera have been studied by means of radioisotope techniques before and after P32 therapy for a five-year period.Results of measurements of the plasma iron pool, the Fe59 plasma half-life, plasma iron turnover, Fe59 incorporation and red cell survival are reported. Various indices were used to compare individuals of different sex and weight and to compare untreated patients, patients in remission after therapy and in relapse. These are discussed in detail.It is demonstrated that the routine haematological tests alone do not provide sufficient information to arrive at a correct diagnosis of the haematological status of an individual patient.

The effect of oral urea administration on red cell survival in sickle cell disease

1972 ◽  
Vol 264 (4) ◽  
pp. 283-287 ◽  
Author(s):  
THOMAS A. BENSINGER ◽  
LAVIZA MAHMOOD ◽  
MARCEL E. CONRAD ◽  
PAUL R. McCURDY
Keyword(s):  
Sickle Cell Disease ◽  
Cell Survival ◽  
Sickle Cell ◽  
Red Cell ◽  
Cell Disease ◽  
Red Cell Survival
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