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Hypersplenism

By Harry S. Jacob, MD, DHC, George Clark Professor of Medicine and Laboratory Medicine (Emeritus);Founding Chief Medical Editor, University of Minnesota Medical School;HemOnc Today

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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 RBCs and often of 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

  • CBC

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, 50,000 to 100,000/μL; WBC counts, 2500 to 4000/μL with normal WBC differential count). RBC morphology is generally normal except for occasional spherocytosis. Reticulocytosis is usual.

Treatment

  • Possibly splenic ablation (splenectomy or radiation therapy)

  • Vaccination 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 vaccinationagainst infections caused by Streptococcus pneumoniae, Neisseria meningitidis, and Haemophilus influenzae.

After splenectomy, patients are particularly susceptible to severe sepsis with encapsulated microorganisms and are often given daily prophylactic antibiotics such as penicillin or erythromycin. Patients who develop fever should receive empiric antibiotics.

Indications for Splenectomy or Radiation Therapy in Hypersplenism

Indication

Examples

Hemolytic syndromes in which splenomegaly further shortens the survival of intrinsically abnormal RBCs

Severe pancytopenia associated with massive splenomegaly

Hairy cell leukemia

Lipid-storage diseases*

Vascular insults affecting the spleen

Recurrent infarctions

Bleeding esophageal varices associated with excessive splenic venous return

Mechanical encroachment on other abdominal organs

Stomach with early satiety

Calyceal obstruction in left kidney

Excessive bleeding

Hypersplenic thrombocytopenia

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

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