Beta-thalassemia

Beta-thalassemia (BT) is characterized by deficiency (Beta+) or absence (Beta0) of synthesis of the beta globin chains of hemoglobin (Hb).

Asplenia

Absence (aplasia) of the spleen.


Total: 2

                       


(per page)
PMID (PMCID)
15545849
 MALE Adult
Intrathoracic masses due to extramedullary hematopoiesis.
Castelli R, Graziadei G, Karimi M, Cappellini MD.
Am J Med Sci. 2004;328(5):299-303.
We describe massive intrathoracic extramedullary hematopoiesis in a 41-year-old man with compound heterozygosis for beta-thalassemia and sickle cell anemia and functional asplenia.
988745
 FEMALE Child
Sickle beta-thalassemia: identical twins differing in severity implicate nongenetic factors influencing course.
Joishy SK, Griner PF, Rowley PT.
Am J Hematol. 1976;1(1):23-33.
We conclude that in sickle beta-thalassemia: (1) genotype alone does not determine the clinical course; (2) significant differences in clinical course can occur with almost identical hemoglobin composition and globin synthetic rates; (3) cellular deformability changes do not correlate exactly with clinical course; and (4) functional asplenia and leanness may be advantageous.