Splenomegaly

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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.

* Common.

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.

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.