Splenomegaly

Aka: Splenomegaly, Spleen Enlargement

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II. Mechanisms

  1. Hyperplasia and hypertrophy (Increased splenic function, functional Splenomegaly)
    1. Immune mediated Platelet destruction (e.g. Immune Thrombocytopenic Purpura)
    2. Immune mediated Red Blood Cell destruction (e.g. Autoimmune Hemolytic Anemia)
    3. Splenic Sequestration (e.g. Sickle Cell Anemia, Beta Thalassemia)
    4. Infection (e.g. Infectious Mononucleosis, HIV, Malaria)
    5. Connective Tissue Diseases (e.g. Systemic Lupus Erythematosus, Rheumatoid Arthritis)
  2. Infiltration
    1. Malignant cell accumulation (one third of Splenomegaly cases, esp. Leukemia, Lymphoma)
    2. Abnormal cell accumulation (e.g. Amyloidosis, Sarcoidosis)
    3. Glycogen Storage Disease
  3. Venous pooling or congestion
    1. Cirrhosis or Portal Hypertension (one third of Splenomegaly cases)
    2. Congestive Heart Failure
    3. Renal Failure
    4. Splenic vein thrombosis

III. Causes: Infectious

  1. Bacterial Infection
    1. Subacute Bacterial Endocarditis
    2. Brucellosis
    3. Syphilis
    4. Typhoid
    5. Tuberculosis
    6. Rocky Mountain Spotted Fever
    7. Lyme Disease
    8. Actinomycosis
    9. Babesiosis
    10. Bartonella
    11. Splenic Abscess
  2. Fungal Infection
    1. Toxoplasmosis
    2. Histoplasmosis
  3. Parasitic Infection
    1. Malaria
    2. Visceral Leishmaniasis
    3. Schistosomiasis
  4. Viral Infection
    1. Epstein-Barr Virus Infection (Mononucleosis)
    2. Cytomegalovirus
    3. HIV Infection
    4. Viral Hepatitis (Hepatitis A, Hepatitis B, Hepatitis C)
    5. Malaria

IV. Causes: Malignancy

  1. Leukemia
  2. Lymphoma
  3. Melanoma
  4. Sarcoma
  5. Pancreatic Cancer
  6. Myelofibrosis
  7. Multiple Myeloma
  8. Polycythemia Vera

V. Causes: Hematologic

  1. Hemolytic Anemia
  2. Beta Thalassemia Major
  3. Hereditary Spherocytosis
  4. Sickle Cell Anemia and other Hemoglobinopathy
  5. Megaloblastic Anemia (e.g. Pernicious Anemia)
  6. Polycythemia Vera
  7. Immune Thrombocytopenic Purpura

VI. Causes: Miscellaneous

  1. Liver disease with secondary Portal Hypertension
  2. Congestive Heart Failure
  3. Systemic Lupus Erythematosus
  4. Rheumatoid Arthritis (Felty's Syndrome)
  5. Langerhan's Cell Histiocytosis
  6. Gaucher's Disease
  7. Hyperthyroidism
  8. Sarcoidosis
  9. Intravenous Drug Abuse

VII. History

  1. Travel history from tropical regions
    1. Parasitic Infections (Malaria, Leishmaniasis, Schistosomiasis)
    2. Patients immigrating from endemic regions have up to 80% Prevalence of Splenomegaly
  2. Infectious Symptoms (e.g. Sore Throat, fever, Fatigue)
    1. Mononucleosis Exposure
      1. College Freshman in dormitories (3% Prevalence of acute Mononucleosis)
  3. Malignancy Symptoms
    1. Weight loss
    2. Night Sweats
  4. Hematologic Symptoms or Signs
    1. Bleeding, Bruising or Petechiae
    2. Pallor
  5. Family History
    1. Malignancy
    2. Liver Disease
    3. Lipid Storage Disorders
    4. Hematologic Disorders
  6. Lifestyle
    1. Alcohol Abuse
    2. Intravenous drug use
    3. Sexually Transmitted Infection exposures or risks
  7. Past Medical History
    1. Cancer (esp. Leukemia, Lymphoma)
    2. Hematologic disorders
    3. Congestive Heart Failure
    4. Cirrhosis

VIII. Symptoms

  1. Early satiety
  2. Left upper quadrant fullness

IX. Exam

  1. Lymphadenopathy
  2. Abdominal Exam
    1. Perform general abdominal exam supine with bent knees and relaxed Abdomen
    2. See Splenomegaly exam below
  3. Specific cause related findings
    1. Liver disease signs (e.g. Hepatomegaly, caput medusa, Ascites)
    2. Congestive Heart Failure signs (e.g. Peripheral Edema, pulmonary rales)
    3. Petechiae

X. Signs: Splenomegaly

  1. Perform Spleen Exam with patient in right lateral decubitus position
    1. Best position to examine enlarged Spleen
  2. Note splenic size in cm below left costal margin
  3. Castell's Point percussion (best Negative Likelihood Ratio, Test Sensitivity 82%)
    1. Percuss point at anterior axillary line at last intercostal space
    2. Dull to percussion in cases of Splenomegaly (if hollow sound then rules-out diagnosis)
    3. Palpate below costal margin to confirm
  4. Massive Splenomegaly
    1. Lower pole in left lower quadrant or right Abdomen
  5. Splenic Tenderness
    1. Consider infection or splenic infarction

XI. Labs

  1. Complete Blood Count with Platelets and differential
  2. Comprehensive Metabolic Panel
  3. Monospot (for Mononucleosis)
  4. Peripheral Smear
    1. Howell Jolly bodies (seen in Asplenism)
    2. Thrombocytopenia (seen in splenic hyperfunction)
  5. Additional testing to consider if indicated by history or exam
    1. NT-BNP
    2. Sexually Transmited Infection Tests (e.g. HIV, RPR/VDRL, Hepatitis Serology)
    3. C-Reactive Protein
    4. Rheumatoid Factor
    5. Blood smears or PCR for specific infectious organisms (e.g. Babesia)
    6. Tuberculin Skin Test (or Quantiferon-TB)

XII. Imaging

  1. Abdominal Ultrasound
    1. First-line study to confirm Splenomegaly on exam
    2. Splenic diameter >10 cm (>3.9 in) is considered Splenomegaly
    3. Normal Spleen is larger in tall patients and male gender
    4. Consider CT Imaging (e.g. CT chest, Abdomen and Pelvis with contrast) if concerns for malignancy
  2. Abdominal CT
    1. Evaluate splenic masses or other malignancy, Portal Vein Thrombosis
  3. Other imaging
    1. MRI Abdomen
    2. Gallium Scan (suspected Lymphoma or infection)
    3. Technetium liver-Spleen scan (comorbid liver disease)
    4. Consider chest imaging (e.g. Tuberculosis, Sarcoidosis, malignancy)

XIII. Evaluation

  1. Step 1: Confirm Splenomegaly
    1. Select imaging study (usually Ultrasound or CT)
  2. Step 2: Evaluate for hematologic or infectious cause
    1. Consider Complete Blood Count and Peripheral Smear, Monospot
    2. Consider specific testing directed by symptoms, signs and risks
  3. Step 3: Evaluate for splenic congestion
    1. Consider Liver Function Tests and Renal Function tests
    2. Consider Echocardiogram
    3. Causes
      1. Liver disease with Portal Hypertension
      2. Congestive Heart Failure
      3. Subacute Bacterial Endocarditis
      4. Renal Failure
  4. Step 3: Evaluate for autoimmune and Connective Tissue Disorders
    1. Consider sedimentation rate, C-Reactive Protein, ANA, RF
    2. Causes
      1. Systemic Lupus Erythematosus
      2. Rheumatoid Arthritis
      3. Sarcoidosis
  5. Step 4: Evaluate histology
    1. Consider Bone Marrow Biopsy with culture
    2. Consider splenic biopsy

XIV. Management

  1. Consultation based on underlying cause
    1. Consider hematology consult
    2. Consider hepatology consult
    3. Consider infectious disease consult
  2. Spleen Reduction Measures (symptomatic Splenomegaly)
    1. Irradiation
    2. Chemotherapy
    3. Transfusion
    4. Splenectomy
  3. Functional Asplenia, Hyposplenia or Asplenia
    1. See Asplenia for infection prevention and febrile illness management

XV. Complications

  1. Splenic Rupture
    1. Complicates 0.5% of Mononucleosis cases
    2. Atraumatic Splenic Rupture may occur (esp. malignancy and infectious causes)
  2. Acute Infections
    1. See Functional Asplenia
  3. Anemia

XVI. Resources

  1. Splenomegaly (Stat Pearls)
    1. https://www.ncbi.nlm.nih.gov/books/NBK430907/

XVII. References

  1. Armitage in Goldman (2000) Cecil Medicine, p. 960-2
  2. Degowin (1987) Diagnostic Examination, p. 508-11
  3. Ferri (2004) Clinical Advisor, p. 1173 and p. 1330
  4. Aldulaimi (2021) Am Fam Physician 104(3): 271-6 [PubMed]

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