Approach to the child with an enlarged spleen
- Kenneth L McClain, MD, PhD
Kenneth L McClain, MD, PhD
- Professor of Pediatrics
- Texas Children's Cancer Center/Hematology Service
- Baylor College of Medicine
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 . Likewise, the titers of pneumococcal antibodies decline more rapidly in asplenic patients .
●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".)
- Hosea SW, Burch CG, Brown EJ, et al. Impaired immune response of splenectomised patients to polyvalent pneumococcal vaccine. Lancet 1981; 1:804.
- Giebink GS, Le CT, Schiffman G. Decline of serum antibody in splenectomized children after vaccination with pneumococcal capsular polysaccharides. J Pediatr 1984; 105:576.
- Heath HW, Pearson HA. Thrombocytosis in pediatric outpatients. J Pediatr 1989; 114:805.
- McMillan J, Nieburg P, Oski FA. The whole pediatrician catalogue, WB Saunders, Philadelphia 1977. p.21.
- Giouleme O, Theocharidou E. Management of portal hypertension in children with portal vein thrombosis. J Pediatr Gastroenterol Nutr 2013; 57:419.
- Ali N, Anwar M, Ayyub M, et al. Hematological evaluation of splenomegaly. J Coll Physicians Surg Pak 2004; 14:404.
- Swaroop J, O'Reilly RA. Splenomegaly at a university hospital compared to a nearby county hospital in 317 patients. Acta Haematol 1999; 102:83.
- O'Reilly RA. Splenomegaly in 2,505 patients at a large university medical center from 1913 to 1995. 1963 to 1995: 449 patients. West J Med 1998; 169:88.
- O'Reilly RA. Splenomegaly at a United States County Hospital: diagnostic evaluation of 170 patients. Am J Med Sci 1996; 312:160.
- Sneller MC, Wang J, Dale JK, et al. Clincal, immunologic, and genetic features of an autoimmune lymphoproliferative syndrome associated with abnormal lymphocyte apoptosis. Blood 1997; 89:1341.
- Parez N, Bader-Meunier B, Roy CC, Dommergues JP. Paediatric Castleman disease: report of seven cases and review of the literature. Eur J Pediatr 1999; 158:631.
- Aricò M, Janka G, Fischer A, et al. Hemophagocytic lymphohistiocytosis. Report of 122 children from the International Registry. FHL Study Group of the Histiocyte Society. Leukemia 1996; 10:197.
- Tamayo SG, Rickman LS, Mathews WC, et al. Examiner dependence on physical diagnostic tests for the detection of splenomegaly: a prospective study with multiple observers. J Gen Intern Med 1993; 8:69.
- McIntyre OR, Ebaugh FG Jr. Palpable spleens in college freshmen. Ann Intern Med 1967; 66:301.
- Barkun AN, Camus M, Green L, et al. The bedside assessment of splenic enlargement. Am J Med 1991; 91:512.
- Paterson A, Frush DP, Donnelly LF, et al. A pattern-oriented approach to splenic imaging in infants and children. Radiographics 1999; 19:1465.
- Rosenberg HK, Markowitz RI, Kolberg H, et al. Normal splenic size in infants and children: sonographic measurements. AJR Am J Roentgenol 1991; 157:119.
- Gielchinsky Y, Elstein D, Hadas-Halpern I, et al. Is there a correlation between degree of splenomegaly, symptoms and hypersplenism? A study of 218 patients with Gaucher disease. Br J Haematol 1999; 106:812.
- Patel U, Gandhi G, Friedman S, Niranjan S. Thrombocytopenia in malaria. J Natl Med Assoc 2004; 96:1212.
- Ladhani S, Khatri P, El-Bashir H, Shingadia D. Imported malaria is a major cause of thrombocytopenia in children presenting to the emergency department in east London. Br J Haematol 2005; 129:707.
- Maroushek SR, Aguilar EF, Stauffer W, Abd-Alla MD. Malaria among refugee children at arrival in the United States. Pediatr Infect Dis J 2005; 24:450.
- Harris NL. The patient with splenomegaly and lymphocytosis: A diagnostic dilemma. In: Hematology 1998: The American Society of Hematology education program book, p.99.
- OVERVIEW OF SPLENIC FUNCTION
- CAUSES OF SPLENOMEGALY
- Causes of a massively enlarged spleen
- Physical examination
- - Examination of the spleen
- Palpation method
- - Other exam findings
- - Spleen size
- Laboratory evaluation
- - Complete blood count and peripheral blood smear
- Congenital hemolytic anemias
- Evidence for infection
- Evidence for widespread bone marrow invasion
- Evidence for malignant disease
- Additional studies