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Hypersplenism

By

Harry S. Jacob

, MD, DHC, University of Minnesota Medical School

Last full review/revision Dec 2019| Content last modified Dec 2019
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Hypersplenism is cytopenia caused by splenomegaly.

Hypersplenism is a secondary process that can arise from splenomegaly of almost any cause (see table Common Causes of Splenomegaly). Splenomegaly increases the spleen’s mechanical filtering and destruction of red blood cells (RBCs) and often of white blood cells (WBCs) and platelets. Compensatory bone marrow hyperplasia occurs in those cell lines that are reduced in the circulation.

Symptoms and Signs

Splenomegaly is the hallmark; spleen size correlates with the degree of cytopenia. Other clinical findings usually result from the underlying disorder.

Diagnosis

  • Physical examination, sometimes ultrasonography

  • Complete blood count

Hypersplenism is suspected in patients with splenomegaly and anemia or cytopenias. Evaluation is similar to that of splenomegaly.

Unless other mechanisms coexist to compound their severity, anemia and other cytopenias are modest and asymptomatic (eg, platelet counts, 50 to 100 × 103/mcL [50 to 100 × 109/L]; white blood cell counts, 2500 to 4000/mcL [2.5 to 4 × 109/L] with normal white cell differential count). Red blood cell morphology is generally normal except for teardrop forms and occasional spherocytosis. Reticulocytosis is usual.

Treatment

  • Possibly splenic ablation (splenectomy or radiation therapy)

  • Vaccination and prophylactic antibiotics for splenectomized patients

Treatment is directed at the underlying disorder. However, if hypersplenism is the only serious manifestation of the disorder (eg, Gaucher disease), splenic ablation by splenectomy or radiation therapy may be indicated. The indications for splenectomy or radiation therapy in hypersplenism are detailed below (see table Indications for Splenectomy or Radiation Therapy in Hypersplenism).

Because the intact spleen protects against serious infections with encapsulated bacteria, splenectomy should be avoided whenever possible, and patients undergoing splenectomy require prior vaccination against infections caused by Streptococcus pneumoniae, Neisseria meningitidis, and Haemophilus influenzae. Patients should also receive the influenza vaccine and may need other vaccinations according to their clinical situation.

After splenectomy, patients are particularly susceptible to severe sepsis with encapsulated microorganisms and are often given daily prophylactic antibiotics such as penicillin, amoxicillin, or erythromycin, particularly when they have regular contact with children. Patients who develop fever should receive empiric antibiotics because rapidly fatal sepsis can sometimes occur.

Table
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Indications for Splenectomy or Radiation Therapy in Hypersplenism

Indication

Examples

Excessive bleeding

Hypersplenic thrombocytopenia

Hemolytic syndromes in which splenomegaly further shortens the survival of intrinsically abnormal red blood cells

Mechanical encroachment on other abdominal organs

Calyceal obstruction in left kidney

Stomach with early satiety

Severe pancytopenia associated with massive splenomegaly

Hairy cell leukemia

Lipid-storage diseases*

Vascular insults affecting the spleen

Bleeding esophageal varices associated with excessive splenic venous return

Recurrent infarctions

* The spleen may be up to 30 times larger than normal.

Drugs Mentioned In This Article

Drug Name Select Trade
ERY-TAB, ERYTHROCIN
AMOXIL
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