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, more in elderly + ↑↓ + + Nl + + Nl Liver biopsy
Heart failure[2] Elderly + ↑↓ ± + Nl Nl NA Echocardiography
Thrombosis of portal, hepatic, or splenic veins[3] Any + + + + Nl ± + Nl NA Doppler ultrasonography
Malignancy Lymphoma[4] Any, 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, more 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]
  • Lymphoid neoplasms
  • Vascular neoplasms
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
  • Poor prognosis
Metastatic solid tumors[11] Any, 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]
  • Infection
  • Autoimmune
Any
  • High risk behaviors
  • Close contact
+ + + + Nl Nl + + Nl Clinical manifestation + lab tests
Infectious mononucleosis[13]
  • EBV
More in children and young adults
  • Close contact
+ + Nl + Nl Nl +
  • Sore throat
  • Enlarged tonsils
  • Lymphadenopathy
  • Headache
  • Maculopapular rash on the arms or trunk
Nl Nl Nl Nl Nl NA Clinical manifestation + Heterophile antibody test
  • Splenic rupture
  • Lymphocytosis
  • Seizures
Cytomegalovirus[14]
  • CMV
More in immunocompromised patients and during pregnancy
  • Close contact
+ + Nl + Nl Nl +
  • Exudative pharyngitis
  • Symmetrical cervical adenopathy
Nl Nl Nl Nl Nl NA ELISA
Bacterial Salmonella[15]
Brucella[16]
Tuberculosis[17]
Parasitic Malaria[18]
Schistosomiasis[19]
Toxoplasmosis[20]
Kala-azar[21] Visceral leishmaniasis
Infective endocarditis[22]
Fungal[23]
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
Inflammation Sarcoidosis[24] Autoimmune disease, more in young African American women
  • Positive family history
+ + + + Nl Nl Nl Nl Nl Nl Nl NA Diagnosis of exclusion
Serum sickness[25]
  • Immune complex hypersensitivity (type III) to certain medications
Any
  • Consumption of certain medications
+ + Nl + Nl
  • Rashes
  • Itching
  • Facial swelling
  • Arthralgia
  • Lymphadenopathy
  • Dyspnea
↓ or ↑

Eosinophilia

Nl Nl Nl
  • Proteinuria
  • Hematuria
  • Plasma cells on peripheral blood smear
  • Numerous histological changes including inflammation of arterial wall or fibrosis
NA Clinical manifestation + recent medication consumption
  • Glomerulonephritis
  • Shock
Systemic lupus erythematosus[26] More in young females
  • Family history
+ + + + Nl to ↓ Nl + + Clinical findings + laboratory studies
  • Multi organ damage
Felty syndrome[27] Rare autoimmune disease, more 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[28]
  • Accumulation of glucocerebroside
Autosomal recessive hereditary deficiency of the enzyme glucocerebrosidase, more in Ashkenazi Jews
  • Family history
- + + + Nl to ↓ Nl + +
  • Painful bone lesions
  • Lymphadenopathy
  • Yellowish-brown skin pigmentation
Nl Nl
  • Proteinuria
  • Hematuria
NA Enzyme testing
  • The most common of the lysosomal storage diseases
  • Severe neurological complications
  • Infections
  • Osteoporosis
Niemann-Pick disease[29]
  • Mutations in the SMPD1 gene
  • Deficiency in the activity of the lysosomal enzyme acid sphingomyelinase
  • Accumulation of sphingomyelin
Autosomal recessive lysosomal storage disease, more in Ashkenazi Jews
  • Family history
- + + + Nl to ↓ Nl + +
  • Unsteady gait
  • Slurred speech
  • Dysphagia
  • Dystonia
Nl
  • Proteinuria
  • Hematuria
NA Genetic testing
  • CNS involvement
  • Supranuclear gaze palsy
  • Dementia
  • Seizure
  • Gelastic cataplexy
  • Pulmonary infections
  • Splenic rupture
  • Coronary artery or valvular heart disease
Amyloidosis[30]
  • Protein misfolding
Langerhans cell histiocytosis[31]
Hemophagocytic lymphohistiocytosis[32]
Rosai-Dorfman disease[33]
Hematologic (hypersplenic) states Hemolytic anemia[34]
Sickle cell disease[35]
Granulocyte colony-stimulating factor[36]
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

References

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