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Approach to the child with an enlarged spleen

Author
Kenneth L McClain, MD, PhD
Section Editor
Donald H Mahoney, Jr, MD
Deputy Editor
Carrie Armsby, MD, MPH

INTRODUCTION

This topic reviews the approach to the child whose spleen is enlarged on physical examination and/or is more than minimally enlarged on abdominal imaging. The clinical or diagnostic significance of a spleen that is not palpable on physical examination and is only minimally enlarged on imaging is uncertain.

The approach to the adult patient with splenomegaly and other splenic disorders is discussed separately. (See "Approach to the adult patient with splenomegaly and other splenic disorders".)

OVERVIEW OF SPLENIC FUNCTION

The spleen is a hematopoietic organ, which, at various times during gestation and/or extrauterine life, is capable of supporting elements of the erythroid, myeloid, megakaryocytic, lymphoid, and monocyte-macrophage (ie, reticuloendothelial) systems. In certain disease states (eg, beta thalassemia major, primary myelofibrosis), it may become the site of extramedullary hematopoiesis and contain developing erythroid, myeloid, and megakaryocytic precursors. (See "Clinical manifestations and diagnosis of the thalassemias".)

The white pulp of the spleen is a major part (up to 25 percent) of the lymphoid tissue in the body. Like lymph nodes, it has germinal centers where early B-lymphocytes predominate, along with plasma cells. T-lymphocytes are the major population around periarteriolar sheaths. When antigens are present in the circulation, the spleen plays a key role in providing an environment for the immunologic response. Thus, in the absence of the spleen, antibody production may be significantly diminished. As an example, when pneumococcal vaccine is given intramuscularly to asplenic individuals, the IgG and IgM antibody titers are less than those noted in normal individuals [1]. Likewise, the titers of pneumococcal antibodies decline more rapidly in asplenic patients [2].

The largest component of the spleen is the red cell mass known as the "red pulp." It consists of the red blood cells surrounding endothelial cords of Billroth and interdigitating splenic sinusoids, which are lined with macrophages. Because of the anatomic arrangement of blood vessels, red cells are relatively concentrated in the terminal splenic arteries, as plasma with potentially antigenic material is shunted to the white pulp. The high concentration of red cells in these arterioles and sinusoids explains how relatively mild degrees of hypoxia can cause transformation of sickle cells to the irreversibly hardened variety and promote infarction in this organ (autosplenectomy). (See "Overview of the clinical manifestations of sickle cell disease".)

                   

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Literature review current through: Nov 2016. | This topic last updated: Tue Apr 28 00:00:00 GMT+00:00 2015.
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