Splenectomy for hereditary spherocytosis: complete, partial or not at all?

Abstract

Hereditary spherocytosis (HS) is an hematologic disorder, common in the Northern European population, which is characterized by hemolytic anemia and splenomegaly. The pathophysiological bedrock of HS consists of the retention and damage of abnormal red cells, caused by an intrinsic erythrocyte membrane defect. Hemolysis within the spleen is the main detrminant of erythrocyte destruction in patients with HS. Splenectomy removes the primary ‘graveyard’ for spherocytes and, thus, eliminates anemia and hyperbilirubinemia and lowers the high reticulocyte number to nearly normal levels. Spleen removal is an effective therapeutic option but it is not without risks. The incidence and public health impact of spleen surgery in children with HS is substantial. Unfortunately, splenectomy is sometimes recommended erroneously for young children who have a mild clinical phenotype or is performed without relying on careful assessment of the risks and benefits. This article aims to present criticism and address controversy about HS management. Unclear and still controversial issues are tackled according to the author’s personal viewpoint, because strong evidence in different aspects of HS management is lacking.

Keywords::

• deferasirox
• hereditary spherocytosis
• IgM memory
• immunization
• infection
• laparoscopic splenectomy
• open splenectomy
• oral chelator
• partial splenectomy
• total splenectomy
• vascular event

Acknowledgements

The authors sincerely thank Dr Kiren for text editing.

Financial & competing interests disclosure

This work was supported in part by grants from Progetti di Rilevante Interesse Nazionale, Regione Campania and ‘Francesco Fede’ Department of the Second University of Naples (Grant on Normal and Pathological Hematopoiesis). The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

No writing assistance was utilized in the production of this manuscript.

Notes

MCHC: Mean corpuscular hemoglobin concentration; RDW: Red blood cell distribution width.

Modified from [2].