Beta-thalassemia

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

Splenomegaly

Abnormal increased size of the spleen.


Total: 6

                      


(per page)
PMID (PMCID)
29299089
FEMALE
Hamartoma of the spleen (splenoma) with calcifications in a child with beta-thalassemia: A case report.
Giambelluca D, Picone D, Re GL, Pappalardo S, Romeo P.
J Radiol Case Rep. 2017;11(5):7-12.
We report a case of a 15-year-old girl with iron-deficiency anemia and beta-thalassemia, who had a large (10 8 7 cm) splenic lesion with calcifications, incidentally found during follow-up for splenomegaly and histologically characterized as hamartoma with calcified areas.
26881631
MALE Adult
Extramedullary hematopoiesis on 18F-FDG PET/CT in a patient with thalassemia and nasopharyngeal carcinoma: A case report and literature review.
Qiu D, Hu X, Xu L, Guo X.
J Cancer Res Ther. 2015;11(4):1034.
The patient had a long history of beta-thalassemia and a splenectomy due to splenomegaly at 12 years of age.
8037185
MIXED_SAMPLE Infant
Beta-thalassemia alleles and unstable hemoglobin types among Russian pediatric patients.
Curuk MA, Molchanova TP, Postnikov YuV , Pobedimskaya DD, Liang R, Baysal E, Kolodey S, Smetanina NS, Tokarev YuN , Rumyantsev AG, et al..
Am J Hematol. 1994;46(4):329-32.
This condition and two new variants have the characteristics of a dominant type of beta-thalassemia heterozygosity with moderate anemia, Heinz body formation, splenomegaly, etc.
7693620
FEMALE Adult
Hb Hradec Kralove (Hb HK) or alpha 2 beta 2 115(G17)Ala-->Asp, a severely unstable hemoglobin variant resulting in a dominant beta-thalassemia trait in a Czech family.
Divoky V, Svobodova M, Indrak K, Chrobak L, Molchanova TP, Huisman TH.
Hemoglobin. 1993;17(4):319-28.
Its presence results in a dominant type of beta-thalassemia in the two heterozygotes, with moderate anemia, reticulocytosis, nucleated red cells, target cells, and other red cell changes, Heinz body formation, and splenomegaly; the oldest of the two patients was splenectomized.
2434529
FEMALE Child
Hemoglobin Mississippi (beta 44ser----cys). Studies of the thalassemic phenotype in a mixed heterozygote with beta +-thalassemia.
Steinberg MH, Adams JG 3rd, Morrison WT, Pullen DJ, Abney R, Ibrahim A, Rieder RF.
J Clin Invest. 1987;79(3):826-32.
While heterozygotes for HbMS are clinically and hematologically normal and carriers of the beta +-thalassemia gene in our family had mild microcytic anemia, the proband with HbMS-beta +-thalassemia had a hemoglobin level of 7 g/dl, mean corpuscular volume (MCV) of 68 fl, reticulocytes of 2-6%, HbF of 18%, marked anisocytosis and poikilocytosis, and splenomegaly, all features of thalassemia intermedia.
2434529
FEMALE Child
Hemoglobin Mississippi (beta 44ser----cys). Studies of the thalassemic phenotype in a mixed heterozygote with beta +-thalassemia.
Steinberg MH, Adams JG 3rd, Morrison WT, Pullen DJ, Abney R, Ibrahim A, Rieder RF.
J Clin Invest. 1987;79(3):826-32.
While heterozygotes for HbMS are clinically and hematologically normal and carriers of the beta +-thalassemia gene in our family had mild microcytic anemia, the proband with HbMS-beta +-thalassemia had a hemoglobin level of 7 g/dl, mean corpuscular volume (MCV) of 68 fl, reticulocytes of 2-6%, HbF of 18%, marked anisocytosis and poikilocytosis, and splenomegaly, all features of thalassemia intermedia.