Splenomegaly

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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

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
  • Lymphadenopathy
  • Dyspnea
  • Shoulder pain
  • Constipation
  • Cachexia
  • Pleural infusion
Low iron, low ferritin
  • Different histopathologic features depends on the type of tumor
Biopsy
Metastatic solid tumors[11] Any age, more in adults + + + + Nl ± ± Low iron, high ferritin
  • Blast cells
Biopsy
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
Cytomegalovirus[14] More common in immunocompromised patients and 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[16]
  • 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
Tuberculosis[17]
  • 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 +
  • Flu-like symptoms
  • Headache
  • Shivering
  • Joint pain
  • Vomiting
Nl Nl NA
Kala-azar[19] Any age
  • Bitten by sandflies
+ + + + Nl + +
  • Asymptomatic
  • Tender spleen
  • Hepatomegaly
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] Autoimmune disease, more common in young African American women
  • 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 ↑

Eosinophilia

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] Rare autoimmune disease, more common in females 50-70 years old + + + + 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] Autosomal recessive hereditary deficiency of the enzyme glucocerebrosidase, more in Ashkenazi Jews
  • Positive family history
- + + + Nl to ↓ Nl + + Nl Nl NA Enzyme testing
Niemann-Pick disease[25] Autosomal recessive lysosomal storage disease, more common in Ashkenazi Jews
  • 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 + -
  • Painful bone swelling
  • Scaly erythematous lesions
  • Lymohadenopathy
  • Cough
  • Dyspnea
Nl
  • Birbeck granules on electron microscopy
  • 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

References

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