Hypersplenism

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Basics

Description

  • Hypersplenism is defined as overactivity of the spleen and presents as the following:
    • Splenomegaly (commonly but not always)
    • Cytopenias with respective bone marrow hyperplasia of precursors
    • Resolution of cytopenias with splenectomy
  • Splenomegaly is not synonymous with hypersplenism. Overactivity of the spleen can occur without enlargement, as is seen in immune thrombocytopenic purpura (ITP) and autoimmune hemolytic anemia. Similarly, splenomegaly is not always associated with hypersplenism.

Epidemiology

May be as common as 30–70% in patients with cirrhosis and portal hypertension (HTN)

Etiology and Pathophysiology

  • Enlargement of the spleen results in sequestration of formed blood elements, leading to peripheral cytopenias and concomitant bone marrow precursor hyperplasia.
  • Many of the common etiologies are listed below. Almost any process involving the spleen or the hematologic system can result in hypersplenism:
    • Infectious
      • Tuberculosis
      • Brucellosis
      • Malaria
      • Leishmaniasis
      • Ehrlichiosis
      • Schistosomiasis
      • Histoplasmosis
      • Candidiasis
      • Viral
      • Syphilis
      • Infective endocarditis
    • Hematologic
      • Myeloproliferative disorders
      • Polycythemia vera
      • Primary hypersplenism
      • ITP
      • Hemolytic anemias
    • Neoplastic
      • Hematologic malignancies
      • Melanoma
      • Various carcinomas
      • Metastatic cancers
    • Storage diseases
      • Gaucher disease
      • Niemann-Pick disease
      • Amyloidosis
      • Glycogen storage disease
    • Inflammatory
      • Sarcoidosis
      • Systemic lupus erythematosus
      • Felty syndrome
    • Congestive
      • Cirrhosis
      • Heart failure
      • Portal or splenic vein thrombosis
      • Congenital malformations of the portal vein

-- To view the remaining sections of this topic, please or --

Basics

Description

  • Hypersplenism is defined as overactivity of the spleen and presents as the following:
    • Splenomegaly (commonly but not always)
    • Cytopenias with respective bone marrow hyperplasia of precursors
    • Resolution of cytopenias with splenectomy
  • Splenomegaly is not synonymous with hypersplenism. Overactivity of the spleen can occur without enlargement, as is seen in immune thrombocytopenic purpura (ITP) and autoimmune hemolytic anemia. Similarly, splenomegaly is not always associated with hypersplenism.

Epidemiology

May be as common as 30–70% in patients with cirrhosis and portal hypertension (HTN)

Etiology and Pathophysiology

  • Enlargement of the spleen results in sequestration of formed blood elements, leading to peripheral cytopenias and concomitant bone marrow precursor hyperplasia.
  • Many of the common etiologies are listed below. Almost any process involving the spleen or the hematologic system can result in hypersplenism:
    • Infectious
      • Tuberculosis
      • Brucellosis
      • Malaria
      • Leishmaniasis
      • Ehrlichiosis
      • Schistosomiasis
      • Histoplasmosis
      • Candidiasis
      • Viral
      • Syphilis
      • Infective endocarditis
    • Hematologic
      • Myeloproliferative disorders
      • Polycythemia vera
      • Primary hypersplenism
      • ITP
      • Hemolytic anemias
    • Neoplastic
      • Hematologic malignancies
      • Melanoma
      • Various carcinomas
      • Metastatic cancers
    • Storage diseases
      • Gaucher disease
      • Niemann-Pick disease
      • Amyloidosis
      • Glycogen storage disease
    • Inflammatory
      • Sarcoidosis
      • Systemic lupus erythematosus
      • Felty syndrome
    • Congestive
      • Cirrhosis
      • Heart failure
      • Portal or splenic vein thrombosis
      • Congenital malformations of the portal vein

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