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conferenceseries.com
Volume 8, Issue 2 (Suppl) J Blood Disord Transfus
ISSN: 2155-9864 JBDT, an open access journal Hematologists 2017
May 08-09, 2017
7th World Hematologists Congress
May 08-09, 2017 Barcelona, Spain
Zaza Mtvarelidze et al., J Blood Disord Transfus 2017, 8:2(Suppl)
Congenital dyserythropoietic anemia type II revealed in Georgia
Zaza Mtvarelidze1, Kvezereli-Kopadze A 2, Kvezereli-Kopadze M2 and D Rexviashvili1
1Children New Clinic, Georgia
2G. Zhvania Pediatric Academic Clinic, Georgia
Objective: The congenital dyserythropoietic anemia (CDA) is a rare hereditary disorder, characterized by ineffective erythropoiesis
and distinct morphologic abnormalities of erythroblasts in the bone marrow. This study was carried out to investigate infant with
CDA type-II and make long-term observat on.
Methods: A full term infant, aged seven month, presented with pallor and jaundice of the skin, moderate splenomegaly and severe
anemia was enrolled in the study. Pallor appeared in the five month of life, which was not interpreted correctly. Profound anemia
revealed by sevenmonth of age, at the time of admission. Investigations include: detailed history and physical examination, information
about used medications, complete blood count with red cell indices, reticulocyte count, iron metabolism, bilirubin, liver and kidney
function tests, bone marrow examination, abdominal ultrasound, parvovirus B19 antibodies, measurement of hemoglobin F, folic
acid and vitamin B12 levels and acidified serum lyses test. We made packed red cell transfusions each month as a result of profound
anemia.
Results: Based on clinical and para clinical data analyses and catamnestic observation, this case was diagnosed with CDA type-II.
Diagnostic criteria were: Evidence of congenital chronic anemia with law reticulocyte count for the degree of anemia; increased
serum iron and ferritin levels, indirect hyper-bilirubinemia and; typical morphologic abnormalities of the erythroblasts (CDA
erythroblasts-30%) in the bone marrow. Acidified serum lyses test was positive.
Conclusion: We had a chance to observe infant with CDA type-II. This rare diagnosis was raised for the first time in Georgia. Anemia
was non-responsive to iron, folic acid and vitamin B12. The diagnosis of CDA should be suspected in case of refractory anemia of long
duration with a low reticulocyte count for the severity of anemia, features of iron overloud, bi-nucleated normoblasts. Early diagnosis
of the disease will allow us to prevent iron abundance caused by multiple blood transfusions. Only curative treatment is allogeneic
bone marrow transplantation which is currently not possible due to the absence of HLA-compatible donor. The observation on this
patient and blood transfusion has been continued per month.
Biography
Zaza Mtvarelidze is a Pediatric Hematologist of Children's New Clinic.Tbilisi.Georgia. For 20 years he has been working on the problems of inherited and acquired
anemias in children. He has published 58 articles on this problem in Georgian and European medical Journals. He is the author of two Monographs: Anemia in
Children and Iron Deficiency Anemia in Children. In recent years he had become interested in the beta-Thalassemia Major