Hemoglobin E disease

Abstract 1. A clinical and hematologic description is presented of a patient with sickle-cell-hemoglobin E disease in a new family. In contrast to the mild hematologic and clinical changes observed in the patients described by the author previously, the clinical and hematologic manifestations in the new patient were moderate in degree. The anemia was normocytic in type. Variation in the clinical and hematologic picture of sickle cell-hemoglobin E disease has been noted. 2. Genetic studies of two families with sickle-cell-hemoglobin E disease have been presented. According to the genetic data obtained by evaluation of the genealogies of these two families, it is strongly suggested that the genes responsible for hemoglobins S, E and A are allelomorphs or linked.

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Thrombotic thrombocytopenic purpura in a patient with hemoglobin E disease-the importance of timely examination of a blood film

American Journal of Hematology ◽

10.1002/ajh.23197 ◽

2012 ◽

Vol 87 (10) ◽

pp. 996-996 ◽

Cited By ~ 2

Author(s):

Biswadip Hazarika ◽

Barbara J. Bain

Keyword(s):

Thrombotic Thrombocytopenic Purpura ◽

Blood Film ◽

Thrombocytopenic Purpura ◽

Hemoglobin E ◽

Hemoglobin E Disease

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A Further Example of Thalassemia-Hemoglobin E Disease in Turkey

Acta Haematologica ◽

10.1159/000209460 ◽

1965 ◽

Vol 34 (6) ◽

pp. 354-360 ◽

Cited By ~ 5

Author(s):

A. Okçuoğlu ◽

Virginia Minnich ◽

A. Arcasoy

Keyword(s):

Hemoglobin E ◽

Hemoglobin E Disease

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Pregnancy Outcomes Among Women with Homozygous Hemoglobin E Disease: A Retrospective Cohort Study

Maternal and Child Health Journal ◽

10.1007/s10995-016-2063-4 ◽

2016 ◽

Vol 20 (11) ◽

pp. 2367-2371

Author(s):

Supatra Sirichotiyakul ◽

Phudit Jatavan ◽

Kuntharee Traisrisilp ◽

Theera Tongsong

Keyword(s):

Cohort Study ◽

Retrospective Cohort Study ◽

Pregnancy Outcomes ◽

Retrospective Cohort ◽

Hemoglobin E ◽

Hemoglobin E Disease ◽

Homozygous Hemoglobin E

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DISCREPANCY BETWEEN HAEMOGLOBIN, RDW, AND MEAN CORPUSCULAR VALUES IN PATIENTS WITH BETA THALASSEMIA / HEMOGLOBIN E DISEASE AND BETA THALASSEMIA TRAIT

INDONESIAN JOURNAL OF CLINICAL PATHOLOGY AND MEDICAL LABORATORY ◽

10.24293/ijcpml.v25i3.1459 ◽

2019 ◽

Vol 25 (3) ◽

pp. 343

Author(s):

Vinisia Setiadji ◽

Bidasari Lubis ◽

Adi Koesoema Aman ◽

Herman Hariman

Keyword(s):

Beta Thalassemia ◽

Hemoglobin E ◽

Thalassemia Trait ◽

Hemoglobin E Disease

Thalassemia beta / hemoglobin E adalah suatu kondisi dengan heterozigot ganda gen pembawa thalassemia beta dan hemoglobin E. Hal ini menyebabkan kondisi dengan gambaran fenotip yang berat dibandingkan trait thalassemia beta dan trait hemoglobin E. Secara logika, nilai mean corpuscular dari thalassemia beta / hemoglobin E seharusnya memburuk. Pada penelitian ini, kami meneliti sebelas kasus dari dua keluarga dengan anggota menderita thalassemia beta / hemoglobin E.Pada keluarga-1 dua anggota dengan trait thalassemia beta memiliki nilai MCV 68 fL dan 65 fL, dan nilai MCH 21 pg dan 20 pg. Pada keluarga-2 anggota dengan trait thalassemia beta memiliki nilai MCV 60,2 fL dan MCH 18,8 pg. Anak perempuan dari kedua keluarga dengan thalassemia beta / hemoglobin E memiliki nilai mean ± SD MCV 70,8 ± 4,9 fL dan MCH 22.8 ± 2.3 pg, nilai ini signifikan lebih tinggi daripada trait thalassemia beta (p<0.05). Terdapat perbedaan yang signifikan antara nilai hemoglobin dan RDW antara thalassemia beta / hemoglobin E (p=0.001).Kami juga menemukan bahwa nilai MC dari keadaan post-transfusi signifikan lebih tinggi daripada pre-transfusi (p<0.001)Kami menyimpulkan bahwa nilai MC dari thalassemia beta / hemoglobin E secara persisten lebih tinggi daripada trait thalassemia beta. Peran transfusi darah pada pasien dengan thalassemia beta / hemoglobin E tampak memainkan peran dalam diskrepansi pada kasus ini.

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