Pediatric Splenomegaly Workup

  • Author: Vikramjit S Kanwar, MD, MBA, MRCP(UK), FAAP; Chief Editor: Robert J Arceci, MD, PhD   more...
 
Updated: Dec 5, 2011
 

Laboratory Studies

  • 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.[20]
  • 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)
    • 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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Imaging Studies

  • 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.[22] 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.[23, 24]
  • Radioisotopic scanning with a technetium-99m sulfur colloid (spleen scan) can provide functional information about the spleen that other radiologic studies do not provide.[7]
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Histologic Findings

  • 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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Contributor Information and Disclosures
Author

Vikramjit S Kanwar, MD, MBA, MRCP(UK), FAAP  Associate Professor of Pediatric Hematology and Oncology, Department of Pediatrics, Albany Medical Center; Faculty, Alden March Bioethics Institute

Vikramjit S Kanwar, MD, MBA, MRCP(UK), FAAP is a member of the following medical societies: American Academy of Pediatrics, American Society of Pediatric Hematology/Oncology, Children's Oncology Group, and Royal College of Physicians of the United Kingdom

Disclosure: Nothing to disclose.

Coauthor(s)

Richard H Sills, MD  Professor of Pediatrics, Upstate Medical University

Richard H Sills, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Society of Hematology, and American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

Mundeep K Kainth, DO  Resident Physician, Department of Pediatrics, The Children's Hospital at Albany Medical Center

Mundeep K Kainth, DO is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

Specialty Editor Board

J Martin Johnston, MD  Associate Professor of Pediatrics, Mercer University School of Medicine; Director of Hematology/Oncology, The Children's Hospital at Memorial University Medical Center; Consulting Oncologist/Hematologist, St Damien's Pediatric Hospital

J Martin Johnston, MD is a member of the following medical societies: American Academy of Pediatrics and American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

James L Harper, MD  Associate Professor, Department of Pediatrics, Division of Hematology/Oncology and Bone Marrow Transplantation, Associate Chairman for Education, Department of Pediatrics, University of Nebraska Medical Center; Assistant Clinical Professor, Department of Pediatrics, Creighton University School of Medicine; Director, Continuing Medical Education, Children's Memorial Hospital; Pediatric Director, Nebraska Regional Hemophilia Treatment Center

James L Harper, MD is a member of the following medical societies: American Academy of Pediatrics, American Association for Cancer Research, American Federation for Clinical Research, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Council on Medical Student Education in Pediatrics, and Hemophilia and Thrombosis Research Society

Disclosure: Nothing to disclose.

Helen SL Chan, MBBS, FRCP(C), FAAP  Senior Scientist, Research Institute; Professor, Division of Hematology/Oncology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Canada

Helen SL Chan, MBBS, FRCP(C), FAAP is a member of the following medical societies: American Academy of Pediatrics, American Association for Cancer Research, American Society of Hematology, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Chief Editor

Robert J Arceci, MD, PhD  King Fahd Professor of Pediatric Oncology, Professor of Pediatrics, Oncology and the Cellular and Molecular Medicine Graduate Program, Kimmel Comprehensive Cancer Center at Johns Hopkins University School of Medicine

Robert J Arceci, MD, PhD is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Pediatric Society, American Society of Hematology, and American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author Wayne Hioe, MD, to the development and writing of this article.

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