medigraphic.com
ISSN 0185-3252 (Print)
Revista de la Asociación Médica del Centro Médico ABC

2017, Number 4

<< Back Next >>

An Med Asoc Med Hosp ABC 2017; 62 (4)

Splenectomy in an infant with severe hereditary spherocytosis

Goldbard RD, Casaubon GPR, Baptista GH, Lamshing SP

Language: Spanish
References: 19
Page: 283-288
PDF size: 213.01 Kb.


ABSTRACT

Hereditary spherocytosis is the most common congenital erythrocyte membrane defect. In patients with severe and constant hemolytic activity, splenectomy is part of the definitive treatment. However, it is very rare for this procedure to be needed in patients under one year of age. Our objective is to present the case of a 16-day-old female newborn with severe hemolytic anemia and unconjugated hyperbilirubinemia, initially related to ABO incompatibility. She underwent several sessions of exchange transfusion without achieving a sustained hematologic response. The hemolytic crises recurred at her 31st day of life. The diagnosis of hereditary spherocytosis was established, given the persistence of spherocytosis in the peripheral blood smear, even in the absence of a family history of hemolytic disease. A positive electrophoresis of erythrocyte membrane confirmed the diagnosis. Given the persistence and intensity of hemolytic activity, at day 34 a splenectomy was performed. In the follow-up until adolescence, the patient is in good general and hematological conditions, without any new hemolytic crisis. Clinical and laboratory data of hereditary spherocytosis and considerations about total splenectomy in the first weeks of life are discussed.


REFERENCES

  1. Dorantes-Mesa S. Diagnóstico de los problemas hematológicos en pediatría. México: Ediciones Médicas del Hospital Infantil de México; 1997.

  2. Perrotta S, Gallagher PG, Mohandas N. Hereditary spherocytosis. Lancet. 2008; 372 (9647): 1411-1426.

  3. Christensen RD, Yaish HM, Gallagher PG. A pediatrician’s practical guide to diagnosing and treating hereditary spherocytosis in neonates. Pediatrics. 2015; 135 (6): 1107-1114.

  4. Aramburu-Arriaga N, Fernández-Cuesta MA, Martínez-González MJ, Astigarraga-Aguirre I, Fernández-Teijeiro-Álvarez A, Navajas-Gutiérrez A et al. Esferocitosis hereditaria neonatal: revisión de casuística. An Esp Pediatr. 2000; 52 (6): 569-572.

  5. Stevens RF, Evans DI. Congenital spherocytosis is often not hereditary. Clin Pediatr (Phila). 1981; 20 (1): 47-49.

  6. Delhommeau F, Cynober T, Schischmanoff PO, Rohrlich P, Delaunay J, Mohandas N et al. Natural history of hereditary spherocytosis during the first year of life. Blood. 2000; 95 (2): 393-397.

  7. Christensen RD, Henry E. Hereditary spherocytosis in neonates with hyperbilirubinemia. Pediatrics. 2010; 125 (1): 120-125.

  8. Da Costa L, Galimand J, Fenneteau O, Mohandas N. Hereditary spherocytosis, elliptocytosis, and other red cell membrane disorders. Blood Rev. 2013; 27 (4): 167-178.

  9. Bader-Meunier B, Gauthier F, Archambaud F, Cynober T, Miélot F, Dommergues JP et al. Long-term evaluation of the beneficial effect of subtotal splenectomy for management of hereditary spherocytosis. Blood. 2001; 97 (2): 399-403.

  10. Bolton-Maggs PH, Langer JC, Iolascon A, Tittensor P, King MJ; General Haematology Task Force of the British Committee for Standards in Haematology. Guidelines for the diagnosis and management of hereditary spherocytosis--2011 update. Br J Haematol. 2012; 156 (1): 37-49.

  11. Severin CM. Anatomy of the spleen. In: Pochedly C, Sills RH, Schwartz AD, eds. Disorders of the spleen: pathophysiology and management. New York, NY: Dekker; 1989. pp. 1-19.

  12. Holroyde CP, Oski FA, Gardner FH. The “pocked” erythrocyte. Red-cell surface alterations in reticuloendothelial immaturity of the neonate. N Engl J Med. 1969; 281 (10): 516-520.

  13. Vargas PL, Messen HS, Hubach KE, García UH. Esferocitosis hereditaria en el periodo de recién nacido. Rev Chil Pediatr. 1987; 58 (1): 61-65.

  14. Christensen RD, Yaish HM, Lemons RS. Neonatal hemolytic jaundice: morphologic features of erythrocytes that will help you diagnose the underlying condition. Neonatology. 2014; 105 (4): 243-249.

  15. Yaish HM, Christensen RD, Henry E, Baer VL, Bennett ST. A simple method of screening newborn infants for hereditary spherocytosis. J Applied Hematol. 2013: 27-32.

  16. Miraglia del Giudice E, Perrotta S, Lombardi C, Iolascon A. Decision making at the bedside: diagnosis of hereditary spherocytosis in a transfused infant. Haematologica. 1998; 83 (4): 347-349.

  17. Rubin LG, Schaffner W. Clinical practice. Care of the asplenic patient. N Engl J Med. 2014; 371 (4): 349-356.

  18. Julianov A, Palijska S, Nedkov N. Infection with unexpected micro-organisms in splenectomised patients. Lancet. 2002; 360 (9337): 949-950.

  19. Eber SW, Langendörfer CM, Ditzig M, Reinhardt D, Stöhr G, Soldan W et al. Frequency of very late fatal sepsis after splenectomy for hereditary spherocytosis: impact of insufficient antibody response to pneumococcal infection. Ann Hematol. 1999; 78 (11): 524-528.




2020     |     www.medigraphic.com