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2015, Number 3

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Rev Cubana Hematol Inmunol Hemoter 2015; 31 (3)

Morbidity and mortality of sickle cell anemia: thirty-six years of observational study

Machín GS, Álvarez MI, Svarch E, Menéndez VA, Hernández PC, Sosa PO

Language: Spanish
References: 37
Page: 265-276
PDF size: 155.71 Kb.


ABSTRACT

Introduction: sickle cell anemia (SS) is the most common congenital hemolytic anemia worldwide. The sickle cell trait (AS) comprises a range between 5 to 15 % of the world population. In Cuba it is 3,08 %, which represents a public health problem.
Objective: identify morbidity and mortality in sickle cell anemia at the Institute of Hematology and Immunology (IHI).
Methods: all patients followed at least two years at the IHI between January 1973 and December 2009 were studied.
Results: 411 patients with sickle cell anemia were studied (January, 1973 to December, 2009), 215 female. Mean follow-up was 17.8 ± 9.8 years. The average annual vasoocclusive painful crises, hospital admissions and infection were 1.8; 1.7; and 1.2, respectively. Vasoocclusive painful crises, acute chest syndrome and hepatic crises were the most frequent clinical manifestations, with slight predominance in females, but not statistically significant in the last two. The number of hospitalizations and medical consultations was higher in extreme age groups. The hematological and biochemical values were within expected ranges for the disease except leukocytes; there were no differences between sex. One hundred thirty two pregnancies in 86 patients were diagnosed. There were no maternal deaths and only 5 perinatal deaths. Overall survival was 55 years. The most frequent causes of death were liver complications, strokes and heart failure.
Conclusions: Patients were similar clinical and hematologically as described in the literature, except the frequency of liver complications which was higher. The probability of survival was high. This study confirms the effectiveness of national programs implemented in Cuba in the diagnosis, monitoring and treatment of the disease.


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