Chapter 480 Splenomegaly
Differential Diagnosis
Specific causes of splenomegaly are listed in Table 480-1. A thorough history with a focus on systemic complaints (fever, night sweats, malaise, weight loss) in combination with a complete blood count and careful review of the peripheral smear can help guide diagnosis. Unique problems are discussed next.
Table 480-1 DIFFERENTIAL DIAGNOSIS OF SPLENOMEGALY BY PATHOPHYSIOLOGY
ANATOMIC LESIONS
HYPERPLASIA CAUSED BY HEMATOLOGIC DISORDERS
Acute and Chronic Hemolysis*
Chronic Iron Deficiency
Extramedullary Hematopoiesis
INFECTIONS†
Bacterial
Viral*
Spirochetal
Rickettsial
Fungal/Mycobacterial
Parasitic
IMMUNOLOGIC AND INFLAMMATORY PROCESSES*
MALIGNANCIES
STORAGE DISEASES
CONGESTIVE*
G6PD, glucose-6-phosphate dehydrogenase; HHV-6, human herpesvirus 6.
† Chronic or recurrent infection suggests underlying immunodeficiency.
From Kliegman RM, Greenbaum LA, Lye PS: Practical strategies in pediatric diagnosis and therapy, ed 2, Philadelphia, 2004, Elsevier, p 347.
Pseudosplenomegaly
Abnormally long mesenteric connections may produce a wandering or ptotic spleen. An enlarged left lobe of the liver, a left upper quadrant mass, or a splenic hematoma may be mistaken for splenomegaly. Splenic cysts may contribute to splenomegaly or mimic it; these may be congenital (epidermoid) or acquired (pseudocyst) after trauma or infarction. Cysts are usually asymptomatic and are found on radiologic evaluation. Splenosis after splenic rupture or an accessory spleen (present in 10% of normal individuals) may also mimic splenomegaly; most are not palpable. The syndrome of congenital polysplenism includes cardiac defects, left-sided organ anomalies, bilobed lungs, biliary atresia, and pseudosplenomegaly (Chapter 425.11).
Congestive Splenomegaly (Banti Syndrome)
Splenomegaly may result from obstruction in the hepatic, portal, or splenic veins leading to hypersplenism. Wilson disease (Chapter 349.2), galactosemia (Chapter 81.2), biliary atresia (Chapter 348), and α-1-antitrypsin deficiency (Chapter 349.6) may result in hepatic inflammation, fibrosis, and vascular obstruction. Congenital abnormalities (absence or hypoplasia) of the portal or splenic veins may cause vascular obstruction. Septic omphalitis or thrombophlebitis (spontaneous or as a result of umbilical venous catheterization in neonates) may result in secondary obliteration of these vessels. Splenic venous flow may be obstructed by masses of sickled erythrocytes leading to infarction. When the spleen is the site of vascular obstruction, splenectomy cures hypersplenism. However, since obstruction usually is in the hepatic or portal systems, portacaval shunting may be more helpful, because both portal hypertension and thrombocytopenia contribute to variceal bleeding.
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