Pediatric Splenomegaly Workup
- Author: Alexander Gozman, MD; Chief Editor: Robert J Arceci, MD, PhD more...
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- Splenomegaly is usually the result of systemic disease and not the result of primary splenic disease. Therefore, diagnostic studies are not directed at the spleen itself. Instead, they are oriented at diagnosing disease states that result in splenomegaly. The most useful initial laboratory tests include the CBC count with differential, peripheral blood smears, and liver function tests.
- The CBC count may be revealing.
- Pancytopenia may be present because of bone marrow infiltration and hypersplenism.
- The WBC count may reveal atypical lymphocytes (eg, neutropenia, or neutrophilia (eg, due to infection or leukemia).
- Hemoglobin concentrations, RBC smears, and reticulocyte counts may reveal anemia, abnormal erythrocyte morphology, reticulocytosis (eg, due to hemolysis), or malarial parasites.
- The platelet count may indicate thrombocytopenia due to decreased production (eg, due to bone marrow infiltration), increased destruction (eg, due to immunologic causes, drug reactions, or viral infections), or sequestration or hypersplenism.
- Liver function tests may demonstrate the following abnormal values:
- Hypoalbuminemia, prolonged prothrombin time, indirect and direct hyperbilirubinemia (eg, due to liver dysfunction) (see the Medscape Reference Laboratory Medicine articles Albumin, Prothrombin Time, and Bilirubin)
- Isolated indirect hyperbilirubinemia (eg, due to hemolysis)
- Elevated aspartate aminotransferase (AST) and alanine aminotransferase (ALT) levels (eg, due to liver damage)
- Elevated gamma glutamyltransferase (GGT) and alkaline phosphatase levels (eg, due to biliary obstruction)
- Obtain an antinuclear antibody titer to screen for systemic lupus erythematosus.
- Measure immunoglobulin levels, neutrophil function, and T-cell subclasses (e.g., due to immunodeficiency).
- Obtain viral-antibody titers to detect EBV, CMV, Toxoplasma gondii, and HIV.
- Cultures may reveal bacterial, fungal, or other infections.
- Examine the bone marrow to screen for leukemia, lymphoma, storage diseases, and disseminated fungal or mycobacteria infections.
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- Ultrasonography can confirm the presence of the enlarged spleen or space-occupying lesions (eg, cyst, abscess), provide accurate dimensions, and help in distinguishing between splenic enlargement and other causes of a left subchondral mass (eg, kidney). Confirming or excluding splenomegaly in patients with obesity, in whom palpation can be very challenging, is useful. Often, a single craniocaudal measurement is used to report spleen size; awareness of the normal values for age is important. Collateral blood vessels develop secondary to portal hypertension, and reversal of portal vein blood flow direction may be visualized with Doppler ultrasonography.
- CT scanning and MRI of the left upper quadrant can help in further clarifying abnormalities in size and shape and in defining parenchymal pathology. The "splenic index" is the product of the length, width, and thickness of the spleen and has limited value.[24, 25]
- Radioisotopic scanning with a technetium-99m sulfur colloid (spleen scan) can provide functional information about the spleen that other radiologic studies do not provide.
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- Biopsy of the spleen may be performed. The results are of limited value in common diagnoses, and the procedure is associated with a notable risk, particularly bleeding.
- The diagnosis is occasionally recognized after splenectomy.
- Examples of disease that might be examined with biopsy include infiltrative diseases, such as Gaucher disease, Niemann-Pick disease, amyloidosis, Tangier disease, and glycogen-storage diseases. Other diseases that may be diagnosed with splenic tissue include Langerhans cell histiocytosis, sarcoidosis, systemic lupus erythematosus, and Hodgkin disease. In Hodgkin disease, biopsy samples were often obtained in the past with staging laparotomy, but this is no longer performed because of improved imaging and systemic therapy.
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