eMedicine Specialties > Pediatrics: General Medicine > Hematology

Splenomegaly: Differential Diagnoses & Workup

Author: Mundeep K Kainth, DO, Resident Physician, Department of Pediatrics, The Children's Hospital at Albany Medical Center
Coauthor(s): Vikramjit S Kanwar, MBBS, MBA, MRCP(UK), FAAP, Associate Professor of Pediatric Hematology-Oncology, Department of Pediatrics, Albany Medical Center; Faculty, Alden March Bioethics Institute; Richard H Sills, MD, Professor of Pediatrics, Upstate Medical University
Contributor Information and Disclosures

Updated: Sep 4, 2008

Differential Diagnoses

Acute Lymphoblastic Leukemia
Juvenile Rheumatoid Arthritis
Acute Myelocytic Leukemia
Malaria
Chronic Granulomatous Disease
Myelofibrosis
Coccidioidomycosis
Neuroblastoma
Cytomegalovirus Infection
Pneumonia
Endocarditis, Bacterial
Salmonella Infection
Gaucher Disease
Sarcoidosis
Heart Failure, Congestive
Serum Sickness
Hepatitis B
Sickle Cell Anemia
Hepatitis C
Syphilis
Histiocytosis
Systemic Lupus Erythematosus
Histoplasmosis
Toxoplasmosis
Hodgkin Disease
Tropical Splenomegaly Syndrome
Human Immunodeficiency Virus Infection
Tuberculosis

Other Problems to Be Considered

Hepatitic cirrhosis
Portal hypertension
Cavernous transformation of the portal vein
Chronic myelocytic leukemia
Hereditary spherocytosis
Autoimmune hemolytic anemia
Chediak-Higashi syndrome
Immunodeficiency disorders
Niemann-Pick disease
Lipid storage diseases
Langerhans cell histiocytosis
Hematomas
Pseudocysts

Workup

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.19
  • 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.

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.21 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.22
  • Radioisotopic scanning with a technetium-99m sulfur colloid (spleen scan) can provide functional information about the spleen that other radiologic studies do not provide.6

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.

More on Splenomegaly

Overview: Splenomegaly
Differential Diagnoses & Workup: Splenomegaly
Treatment & Medication: Splenomegaly
Follow-up: Splenomegaly
References
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References

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  30. Goodman J, Newman MI, Chapman WC. Disorders of the spleen. In: Greer JP, Foerster J, Lukens J, et al, eds. Wintrobe's Clinical Hematology. 11th ed. Philadelphia, Pa: Lippincott Williams and Wilkins; 2004:1893-909.

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Further Reading

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Keywords

splenomegaly, splenic enlargement, enlarged spleen, palpable spleen, hypersplenism, splenic enlargement, splenectomy, mononuclear-phagocyte system, MPS, malaria, schistosomiasis, hyperreactive malarial splenomegaly, splenic rupture, noncirrhotic portal fibrosis, sickle cell disease, hepatitis, portal hypertension, abdominal trauma, splenic hematoma, diarrhea, salmonellosis, leukemia, Hodgkin disease, jaundice, sepsis, hypotension, umbilical catheter thrombosis, anemia, leishmaniasis, trypanosomiasis, splenic pseudocyst, cytomegalovirus, human immunodeficiency virus, HIV, hemolytic anemia–associated gallstones, hemolytic anemia, thalassemia, glucose-6-phosphate dehydrogenase deficiency, G6PD deficiency, hereditary pyropoikilocytosis, pyruvate kinase deficiency, hereditary spherocytosis

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Contributor Information and Disclosures

Author

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.

Coauthor(s)

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

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.

Medical Editor

J Martin Johnston, MD, Associate Professor of Pediatrics, Mercer University School of Medicine; Director of Pediatric Hematology/Oncology, Backus Children's Hospital; Consulting Oncologist/Hematologist, St Damien's Pediatric Hospital
J Martin Johnston, MD is a member of the following medical societies: American Society of Pediatric Hematology/Oncology and Idaho Medical Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

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; 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.

CME Editor

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, Department of Oncology, Division of Pediatric Oncology, 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.

 
 
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