Management of Hereditary Spherocytosis in a Public Health Care Provider in a Developing Country.

Hereditary Spherocytosis (HS) is the most common cause of non-immune hemolytic anemia in Argentine people of European ancestry, it is characterized by chronic hemolysis, which is reduced or abrogated by splenectomy.. Because of the risk of postsplenectomy sepsis, especially among children under 5 years old, partial splenectomy has been advocated as an alternative. This procedure seems to ensure prolonged reduction but not complete elimination of hemolysis and it is possible some regrowth of the splenic remnant and a secondary total splenectomy must be performed in some patients (pts). This problem is worse especially when pts. live in rural areas and a close observation is necessary. We analyzed retrospectively the evolution of 83 pediatric pts with HS with low resources, other health priorities and in some cases an underling nutritional deficit. Diagnostics was made in a public hospital between October 1971 and December 2002 and the last control was in December 2004. The pts. Median age at diagnostic was 4.7 years (14 days to 14 years). Severe forms were observed in 18%, mild in 20% and moderate in 62%. Twenty four pts were phenotypically sporadic. Biochemical analysis (SDS-PAGE) were performed in 30 pts and/or family members, combined spectrin- ankirin deficiency were observed in most of them. Sixty-nine hemolytic crisis was observed in 30 pts (37.5%). and 17.5% developed an aplastic crisis. Fifty-two pts. (65%) received 249 Red Blood Cell Transfusions (Trs), 36% between 1 and 2, 22.5% 3 to10, and 6% between 10 to 34 Trs. Gallstones were observed in 4 PTs (5%). Total splenectomy was performed in 46 of 83 pts (55%), median age: 7 years (4–15) 42 pts had conventional and 4 have undergone laparoscopyc procedure. Ninety six per cent of pts were splenectomized after age of 5. The median time from diagnostic to splenectomy was 1 year (0.2–8.9). All pts were immunized against Pneumococcus, Haemophilus, and Meningococus and received prophylactic antibiotics. With a median follow up of 18.5 years (3–30 y) after splenectomy all pts showed a significant clinical improvement and no further transfusion requirement. In all cases the hemoglobin increased (median pre and post splenectomy 8.6/13gr/dl) and reticulocyte counts decreased (8 / 1.4%). We do not observe procedure relate mortality, sepsis, thrombosis, and aplastic or hemolytic crisis post-splenectomy. On the basis of our experience we favor the total splenectomy performed by a training team in countries with low resources and in pts with deficient hygienic and housing conditions that inhabit in places with long distances from centers to ensure wider access to training health care providers.