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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sadaf Sharfaei M.D.[2], Gichoya Judy Wawira [3],

Synonyms and Keywords: Spleen enlargement; enlarged spleen; spleen swelling




The causes of splenomegaly vary widely and range from increased splenic function (such as hemoglobinopathies), splenic congestion due to abnormal blood flow (such as venous obstruction), and splenic infiltration (such as storage disorders and malignancies).

Causes by Pathogenesis

Mechanism Pathogenesis Example
Increased function Removal of defective RBCs
Immune hyperplasia Response to infection (viral, bacterial, fungal, parasitic)
Disordered immunoregulation
Drug reactions
Extramedullary hematopoiesis
Abnormal blood flow Organ Failure
Infiltration Metabolic diseases
Benign and malignant infiltrations

Differentiating Splenomegaly from Other Diseases

Category Disease Etiology Clinical manifestations Para−clinical findings Gold standard Associated findings
Demography History Symptoms Signs Lab Findings Imaging
Fever Fatigue Weight Abdominal pain Bleeding BP JVP Jaundice Ascites Other CBC Iron LFT PT/PTT BUN/Cr UA Histopathology
WBC Hb Plt
Congestive Cirrhosis[1] Any age, more common in elderly + ↑↓ + + Nl + + Nl Liver biopsy
Heart failure[2] Elderly + ↑↓ ± + Nl Nl NA Echocardiography
Thrombosis of portal, hepatic, or splenic veins[3] Any age + + + + Nl ± + Nl NA Doppler ultrasonography
Malignancy Lymphoma[4] Any age, most common malignant splenic tumor + + + + Nl Nl + High ferritin Nl Lymph node biopsy
Leukemia[5] Elderly, male>female + + + + Nl Nl ± + High ferritin Nl
  • Leukemic blasts
  • Leukoerythroblastic features
  • Giant platelets
NA Bone marrow examination + clinical manifestation
Polycythemia Vera[6] Mean age >60 years old
  • Positive family history
+ Nl + Nl Nl Nl to ↑ Nl to ↑ High ferritin Nl Nl Nl Nl
  • Elevated normochromic, normocytic RBCs
  • Thrombocytosis
  • Rarely immature cells
  • Leukoerythroblastic picture
NA WHO criteria for PV
Disease Etiology Demography History Fever Fatigue Weight loss Abdominal pain Bleeding BP JVP Jaundice Ascites Other WBC Hb Plt Iron LFT PT/PTT BUN/Cr UA Histopathology Imaging Gold standard Associated findings
Multiple myeloma[7] Any age, more common in adults + + + + Nl Nl + High ferritin Nl
  • Blast cells
Bone marrow examination
Essential thrombocythemia[8]
  • Acquired mutation of JAK2, CALR, or MPL
Mean age >60 years old, female > male ± + Nl + + Nl Nl ↑↑ Nl Nl Nl Nl Nl Bone marrow biopsy
Primary myelofibrosis[9] Mean age >60 years old, male> female + Nl + + Nl Nl + ↑/↓ Low iron Nl Nl Bone marrow biopsy
Primary splenic tumors[10] Very rare
  • Positive family history
+ + + Nl Nl Low iron, low ferritin
  • Different histopathologic features depends on the type of tumor
Metastatic solid tumors[11] Any age, more in adults + + + + Nl ± ± Low iron, high ferritin
  • Blast cells
Disease Etiology Demography History Fever Fatigue Weight Abdominal pain Bleeding BP JVP Jaundice Ascites Other WBC Hb Plt Iron LFT PT/PTT BUN/Cr UA Histopathology Imaging Gold standard Associated findings
Infection Viral Hepatitis[12]
  • Any age
  • High risk behaviors
  • Close contact
+ + + + Nl Nl + + Nl NA Clinical manifestation + lab tests
Infectious mononucleosis[13]
  • More common in children and young adults
  • Close contact
+ + Nl + Nl Nl + Nl Nl Nl Nl Nl NA NA Clinical manifestation + heterophile antibody test
  • More common in:
    • Immunocompromised patients
    • During pregnancy
  • Close contact
+ + Nl + Nl Nl + Nl Nl Nl Nl Nl NA NA ELISA
Bacterial Salmonella[15]
  • Any age
  • Eating contaminated meat, eggs, or milk
+ Nl + Nl
  • Diarrhea
  • Abdominal cramps
  • Vomiting
  • Dehydration
Nl Nl Nl Nl NA NA Stool test
  • Typhoid fever
  • Reactive arthritis
  • Irritable bowel syndrome
  • Brucella
  • Any age
  • Ingesting contaminated food such as unpasteurized milk products
  • Direct contact with an infected animal
  • Inhalation of aerosols
+ + Nl + Nl +
  • Headache
  • Vomiting
  • Diarrhea
  • Constipation
  • Arthralgia
  • Night sweats
  • Anorexia
  • Foul-smelling perspiration
Nl Nl Nl Nl NA NA Blood culture
  • Arthritis
  • Epididymo-orchitis
  • Spondylitis
  • Neurobrucellosis
  • Liver abscess formation
  • Endocarditis
  • Mycobacterium tuberculosis
  • Any age
  • More common in developing countries and among immunocompromised patients
  • Close contact
  • HIV
+ + + Nl +
  • Chronic cough with blood-containing sputum
  • Night sweats
  • Anorexia
  • Chest pain
Nl Nl
  • Acid-fast bacillus
  • Cavity on chest x ray
Identifying M. tuberculosis in a clinical sample
  • Tuberculous meningitis
  • Urogenital tuberculosis
  • Pott disease
  • Miliary tuberculosis
Parasitic Malaria[18]
  • Plasmodium
  • Worldwide
  • More common in tropical and subtropical regions
  • Bitten by Anopheles mosquito
+ + Nl + + Nl + Nl Nl NA
  • Any age
  • Bitten by sandflies
+ + + + Nl + + Nl
  • Visualization of the characteristic amastigote in smears or tissue
NA Culture
  • Could be fatal
Disease Etiology Demography History Fever Fatigue Weight Abdominal pain Bleeding BP JVP Jaundice Ascites Other WBC Hb Plt Iron LFT PT/PTT BUN/Cr UA Histopathology Imaging Gold standard Associated findings
Inflammation Sarcoidosis[20]
  • Positive family history
+ + + + Nl Nl Nl Nl Nl Nl Nl NA Diagnosis of exclusion
Serum sickness[21]
  • Any age
  • Consumption of certain medications
+ + Nl + Nl ↓ or ↑


Nl Nl Nl NA Clinical manifestation + recent medication consumption
Systemic lupus erythematosus[22]
  • More common in young females
  • Positive family history
+ + + + Nl to ↓ Nl + + Clinical findings + laboratory studies
  • Multi organ damage
Felty syndrome[23] + + + + Nl to ↓ Nl + + Nl Nl Nl Nl Nl Clinical findings + laboratory studies
Infiltrative Disease Etiology Demography History Fever Fatigue Weight Abdominal pain Bleeding BP JVP Jaundice Ascites Other WBC Hb Plt Iron LFT PT/PTT BUN/Cr UA Histopathology Imaging Gold standard Associated findings
Gaucher disease[24]
  • Positive family history
- + + + Nl to ↓ Nl + + Nl Nl NA Enzyme testing
Niemann-Pick disease[25]
  • Positive family history
- + + + Nl to ↓ Nl + + Nl NA Genetic testing
Langerhans cell histiocytosis[26]
  • Clonal proliferation of Langerhans cells
  • Any age
  • More common in children between 5-10 years old
  • Boys>girls
- - + + - Nl Nl + - Nl
  • Osteolytic lesions on imaging
  • Lung nodules on imaging
Tissue biopsy
Category Disease Etiology Demography History Fever Fatigue Weight Abdominal pain Bleeding BP JVP Jaundice Ascites Other WBC Hb Plt Iron LFT PT/PTT BUN/Cr UA Histopathology Imaging Gold standard Associated findings



If the splenomegaly underlies hypersplenism, a splenectomy is indicated and it will correct the problem. After splenectomy, patients have an increased risk for infectious diseases. After splenectomy, patients should be vaccinated against Haemophilus influenzae and Streptococcus pneumoniae. They should receive annual influenza vaccination. Long-term prophylactic antibiotic should be given.


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