Splenomegaly

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)
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
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

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

Lymphadenopathy
Abdominal Exam
1. Perform general abdominal exam supine with bent knees and relaxed Abdomen
2. See Splenomegaly exam below
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

Perform Spleen Exam with patient in right lateral decubitus position
1. Best position to examine enlarged Spleen
Note splenic size in cm below left costal margin
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
Massive Splenomegaly
1. Lower pole in left lower quadrant or right Abdomen
Splenic Tenderness
1. Consider infection or splenic infarction

Complete Blood Count with Platelets and differential
Comprehensive Metabolic Panel
Monospot (for Mononucleosis)
Peripheral Smear
1. Howell Jolly bodies (seen in Asplenism)
2. Thrombocytopenia (seen in splenic hyperfunction)
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)

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
Abdominal CT
1. Evaluate splenic masses or other malignancy, Portal Vein Thrombosis
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)

Step 1: Confirm Splenomegaly
1. Select imaging study (usually Ultrasound or CT)
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
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
Step 3: Evaluate for autoimmune and Connective Tissue Disorders
1. Consider sedimentation rate, C-Reactive Protein, ANA, RF
2. Causes
Step 4: Evaluate histology
1. Consider Bone Marrow Biopsy with culture
2. Consider splenic biopsy

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

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