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2014, Number 2

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Rev Cubana Hematol Inmunol Hemoter 2014; 30 (2)

Morbility and mortality of hemoglobinopathy sc at the Institute of Hematology and Immunology. Experience of 36 years

Machín GS, Cutiño MM, Svarch E, Arencibia NA, Menéndez VA, Hernández PC, Gutiérrez DA, Lam DRM

Language: Spanish
References: 33
Page: 125-136
PDF size: 263.38 Kb.


ABSTRACT

Introduction: hemoglobinophatySC (HSC) is the second most common variant of sickle cell disease in Cuba and the world; nevertheless, there are few studies aimed in this field.
Objective: to make the characterization of the natural history of HSC.
Methods: an observational, descriptive, retrospective and longitudinal study was performed in 148 patients with HSC followed for at least two years at the Institute of Hematology and Immunology in the period 1973-2009. Hematological events according to Cuban procedures in sickle cell disease were determined and complementary studies were performed.
Results: there was a predominance of females (56.1 %). Vasocclusive painful crises (91.2 %) and acute chest syndrome (35.1 %) were the most frequent clinical events. Ophthalmology affections were present in 10,8 % (hemovitreous, retinopathy, retinal detachment and cataract). Splenomegaly was predominant in patients under 40 years and hepatomegaly was found in all ages. There were 36 women with pregnancies without maternal or perinatal mortality. From 26 abortions (65.4 % were spontaneous). Anemia was mild but more pronounced in females. Liver and kidney functions showed deterioration with age. Overall survival at 50 years was 79 %. The main cause of death was chronic renal failure.
Conclusions: increasing the quality of life and life expectancy of HSC in Cuba is the result of multidisciplinary comprehensive care and easy access to emergency services.


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