Splenomegaly

The spleen is the largest lymphoid organ in the body. One of its primary functions is to filter defective and/or foreign cells. Splenomegaly is usually caused by systemic disease and not by primary splenic disease. Splenomegaly is usually caused by infection (excessive antigen stimulation), autoimmune disorders (disordered immunoregulation), or hemolysis (excessive destruction of abnormal blood components). Because of exposure below the protective rib cage, splenomegaly results in increased risk of splenic injury or rupture.

Differential Diagnosis

• Normal variants
  –Palpable spleen tip due to thinner abdominal musculature
  –15–30% of neonates
  –10% of healthy children
  –5% of adolescents
• Infection/inflammation
  –Acute hepatitis (B or C)
  –Viral (EBV, CMV, HIV)
  –Bacterial (SBE, cat-scratch disease, TB, histoplasmosis, toxoplasmosis, Salmonella)
  –Systemic lupus erythematosus (SLE)
  –Rheumatoid arthritis
  –Inflammatory bowel disease
  –Celiac disease
  –Acidosis
  –Chronic granulomatous disease
  –Serum sickness
  –Protozoal infection (malaria and schistosomiasis are rare in the U.S.)
  • Hemolytic anemias
    –Hereditary spherocytosis
    –Hemoglobinopathies
    –Thalassemia major
    –Nonspherocytic hemolytic anemias (pyruvate kinase deficiency)
  • Malignancy
    –Leukemia, 50% of children with ALL
    –Hodgkin disease, non-Hodgkin lymphoma
    –Metastatic disease
  • Extramedullary hematopoiesis
    –Thalassemia major
    –Osteopetrosis (rare)
    –Myelofibrosis
  • Storage/infiltrative disorders
    –Histiocytosis
    –Lipidoses (e.g., Niemann-Pick, Gaucher)
    –Mucopolysaccharidoses (e.g., Hurler, Hunter)
  • Congestive
    –Chronic congestive heart failure
    –Portal hypertension
         –Portal or splenic venous thrombosis
    –Hepatic fibrosis
    –Cirrhosis
  • Structural
    –Hematoma (trauma)
    –Cysts or pseudocysts
  • Wandering spleen

  Workup and Diagnosis

  • History
    –Fever, LUQ pain, abdominal trauma
    –Ingestion of hepatotoxic substances
    –Acute illness, dyspnea, fatigue, diarrhea
    –Signs of malignancy
    –Pruritus, travel, sexual history
    –Developmental milestones
    –Medical history: NICU stay, umbilical catheter, jaundice, failure to thrive, anemia, heart disease
    –Family history: Early cholecystectomy, gallstones, anemias, ethnic heritage
  • Physical exam
    –Vitals, growth parameters
    –Pallor, jaundice, purpura, petechiae, ecchymoses, excoriated skin, rashes
    –Scleral icterus, cherry red retinal spots, uveitis/iritis
    –New murmur, edema
    –Abdominal distension, ascites, prominent veins, spleen size, tenderness, liver size and texture
    –Lymph node, joint pain or swelling
    • Labs
      –CBC/peripheral smear, ESR or CRP, LFT, PT/PTT
      –EBV/CMV, viral titers, blood culture
      –Autoimmune or rheumatologic tests (ANA, RF)
      –CXR, bone marrow exam
      –immunodeficiency workup, thrombophilia screen
    • Ultrasound: Exclude retroperitoneal tumors or masses, evaluate portal vein flow with Doppler

    Treatment

    • Therapy is directed at treatment of underlying disease
    • Splenectomy benefits need to be balanced with risk of postsplenectomy sepsis
    • If splenectomy is performed, immunize at least 10 days prior
      –Pneumococci
      –Haemophilus influenzae, if under 5
      –Meningococcal vaccine
      –Postsurgical penicillin prophylaxis required
    • Febrile illness in patients postsplenectomy is a life-threatening emergency
      –Major risk is overwhelming sepsis from encapsulated bacteria (Streptococcus pneumoniae, H. influenzae, Neisseria meningitidis)
    • Sepsis most frequent in first 5 years after splenectomy

Book Source Details

• Book Title: In A Page: Pediatric Signs and Symptoms
• Author(s): Jonathan E. Teitelbaum, Kathleen O. Deantonis, Scott Kahan
• Year of Publication: 2007
• Copyright Details: In A Page: Pediatric Signs and Symptoms, Copyright © 2007 Lippincott Williams & Wilkins.

Other Book Chapters Related to Swollen spleen

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Copyright Details: In A Page: Pediatric Signs and Symptoms, Copyright © 2008 Williams & Wilkins.

More About Causes of Swollen spleen

• Back to symptom: Swollen spleen: Introduction (review 1237 causes)
• Next Book Extract About Swollen spleen: Abdominal Masses (In A Page: Pediatric Signs and Symptoms)
• All Book Extracts: All Online Book Extracts for Swollen spleen

More About This Book: Title: In A Page: Pediatric Signs and Symptoms Authors: Jonathan E. Teitelbaum, Kathleen O. Deantonis, Scott Kahan Publisher: Lippincott Williams & Wilkins Copyright: 2007 ISBN: 1-4051-0427-9

 » Next page: Abdominal Masses (In A Page: Pediatric Signs and Symptoms)

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• Abdominal mass (Handbook of Signs & Symptoms (Third Edition))

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