Hemoglobin Electrophoresis Pattern Research Articles

Treatment of hypoplastic anemia in mice with placental transplants

A genetic mutation in mice (W/Wv) causes an autosomal recessive disease characterized by hypoplastic anemia which lasts throughout life. Double- dominant W/Wv anemic mice were sublethally irradiated to facilitate repopulation of marrow with transplanted cells and were injected intravenously with suspensions of 5–10 million placental cells of 15 days gestation derived from normal, isogeneic donors. Red cell counts fell promptly after irradiation and then rose progressively over a period of weeks, reaching normal levels of the nonmutant. Mean corpuscular volume and hemoglobin electrophoresis patterns of red cells in recipient W/Wv mice resembled those of normal donor animals. The therapeutic effect lasted for the duration of the observation period, in some instances over 9 mo. W/Wv mice that were administered Hanks' solution or fetal blood, instead of placental transplants, remained anemic. Late gestation placentas (18 days) were also ineffective.

TREATMENT OF HYPOPLASTIC ANEMIA WITH PLACENTAL TRANSPLANTS

A genetic mutation in mice (W/Wv) causes an autosomal recessive disease characterized by hypoplastic anemia which lasts throughout life. Homozygous W/Wv anemic mice were sublethally irradiated to facilitate repopulation of marrow with transplanted cells and injected IV with suspensions of 5-10 million placental cells of 15 days gestation derived from normal, isogeneic donors. RBC fell promptly after irradiation and then rose progressively over a period of weeks reaching normal levels for the non-mutant. Mean corpuscular volume and hemoglobin electrophoresis pattern of RBC in recipient W/Wv mice resembled those of normal donor animals. The therapeutic effect lasted for the duration of the observation period, in some instances over 7 months. W/Wv mice that were administered Hanks' solution or fetal blood, instead of placental transplants, remained anemic. Late gestation placentos (13 days) were also ineffective. The placental cells with hemopoietic potential could not be identified by routine histological examination.

A hospital based study of Hereditary Hemolytic Anaemias in Davanagere district of Karnataka, India

Background: Hereditary hemolytic anaemias constitute important cause of mortality and morbidity in developing countries next only to infection and malnutrition.These group of anaemias have various clinical presentations starting from their age of onset of symptoms, failure to thrive, anaemia, prostration, jaundice, splenomegaly, cholelithiasis, cardiomegaly, congestive cardiac failure, severe life threatening infections and chronic disabilities leading to distress in the families. Methodology: An analysis of 40 cases of hereditary hemolytic anaemia in the age group of 2 months to 12 years was done in the present study. On the basis of clinical presentations, physical findings, routine hematological investigations and hemoglobin electrophoresis pattern in hemoglobin defects were carried out to identify the type of hemolytic anaemias. Results: This clinocohematological study of hereditary hemolytic anaemia showed membrane defects- Hereditary spherocytosis in 4 cases (10%). The remaining 36 cases were having diseases affecting hemoglobin molecule which included Sickle cell anaemia-5 cases (12.5%), Sickle cell trait- 1 case (2.5%), Sickle cell/β thalassemia-1 case (2.5%), β thalassemia major- 23 cases (57.5%) and β thalassemia trait 6 cases(15%). Hereditary hemolytic anaemia with enzyme defects were not observed in this study. Majority of these cases presented with progressive pallor and hepatosplenomegaly. Peripheral blood smear examination showed microcytic hypochromic anaemia (87.5%) in majority of the cases. All cases were associated with reticulocytosis. Hemoglobin electrophoresis confirmed the diagnosis. Conclusion: Inspite of advanced diagnostic inestigations, the basic hematological investigation remains first panel or step towards the approach to diagnose hereditary hemolytic anaemia and hemoglobin electrophoresis will help in confirming the diagnosis. Keywords: hereditary hemolytic anaemia; clinoco-hematological study; hemoglobin electrophoresis DOI: 10.3329/bjms.v9i3.6471Bangladesh Journal of Medical Science Vol.09 No.3 July 2010, pp.154-160