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Splenomegaly

by Harry S. Jacob, MD

Splenomegaly is almost always secondary to other disorders. Its causes are myriad, as are the many possible ways of classifying them (see Table: Common Causes of Splenomegaly*). In temperate climates, the most common causes are

  • Myeloproliferative disorders

  • Lymphoproliferative disorders

  • Storage diseases (eg, Gaucher disease)

  • Connective tissue disorders

In the tropics, the most common causes are

  • Infectious diseases (eg, malaria, kala-azar)

If splenomegaly is massive (spleen palpable 8 cm below the costal margin), the cause is usually chronic lymphocytic leukemia, non-Hodgkin lymphoma, chronic myelocytic leukemia, polycythemia vera, myelofibrosis with myeloid metaplasia, or hairy cell leukemia.

Splenomegaly can lead to cytopenia (see Hypersplenism).

Evaluation

History

Most of the presenting symptoms result from the underlying disorder. However, splenomegaly itself may cause early satiety by encroachment of the enlarged spleen on the stomach. Fullness and left upper quadrant abdominal pain are also possible. Severe pain suggests splenic infarction. Recurrent infections, symptoms of anemia, or bleeding manifestations suggest cytopenia and possible hypersplenism.

Physical examination

The sensitivity for detection of ultrasound-documented splenic enlargement is 60 to 70% for palpation and 60 to 80% for percussion. Up to 3% of normal, thin, people have a palpable spleen. Also, a palpable left upper quadrant mass may indicate a problem other than an enlarged spleen.

Other helpful signs include a splenic friction rub that suggests splenic infarction and epigastric and splenic bruits that suggest congestive splenomegaly. Generalized adenopathy may suggest a myeloproliferative, lymphoproliferative, infectious, or autoimmune disorder.

Common Causes of Splenomegaly*

Type

Examples

Congestive

Cirrhosis

External compression or thrombosis of portal or splenic veins

Certain malformations of the portal venous vasculature

Infectious and inflammatory

Acute infections (eg, infectious mononucleosis, infectious hepatitis, subacute bacterial endocarditis, psittacosis)

Chronic infections (eg, miliary TB, malaria, brucellosis, kala-azar, syphilis)

Sarcoidosis

Secondary amyloidosis

Connective tissue disorder (eg, SLE, Felty syndrome)

Myeloproliferative and lymphoproliferative

Myelofibrosis with myeloid metaplasia

Lymphomas

Leukemias, especially chronic lymphocytic, large granular lymphocytic, and chronic myelocytic

Polycythemia vera

Primary thrombocythemia

Chronic hemolytic anemia

RBC shape abnormalities (eg, hereditary spherocytosis, hereditary elliptocytosis)

Hemoglobinopathies, including thalassemias, sickle cell hemoglobin variants (eg, hemoglobin S-C disease), and congenital Heinz body hemolytic anemias

RBC enzymopathies (eg, pyruvate kinase deficiency)

Storage diseases

Lipoid (eg, Gaucher, Niemann-Pick, Hand-Schüller-Christian, and Wolman diseases)

Nonlipoid (eg, Letterer-Siwe disease)

Structural

Splenic cysts, usually caused by resolution of previous intrasplenic hematoma

*In order of clinical frequency.

Usually congenital.

Adapted from Williams WJ et al: Hematology. New York, McGraw-Hill Book Company, 1976.

Testing

If confirmation of splenomegaly is necessary because the examination is equivocal, ultrasonography is the test of choice because of its accuracy and low cost. CT and MRI may provide more detail of the organ’s consistency. MRI is especially useful in detecting portal or splenic vein thromboses. Nuclear scanning is accurate and can identify accessory splenic tissue but is expensive and cumbersome.

Specific causes suggested clinically should be confirmed by appropriate testing (see elsewhere in T he M anual ). If no cause is suggested, the highest priority is exclusion of occult infection, because early treatment affects the outcome of infection more than it does most other causes of splenomegaly. Testing should be thorough in areas of high geographic prevalence of infection or if the patient appears to be ill. CBC, blood cultures, and bone marrow examination and culture should be considered. If the patient is not ill, has no symptoms besides those due to splenomegaly, and has no risk factors for infection, the extent of testing is controversial but probably includes CBC, peripheral blood smear, liver function tests, and abdominal CT. Flow cytometry of peripheral blood is indicated if lymphoma is suspected.

Specific peripheral blood findings may suggest underlying disorders (eg, small-cell lymphocytosis in chronic lymphocytic leukemia and large granular lymphocytosis in T-cell granular lymphocyte [TGL] hyperplasia or TGL leukemia; leukocytosis and immature forms in other leukemias). Excessive basophils, eosinophils, or nucleated or teardrop RBCs suggest myeloproliferative disorders. Cytopenias suggest hypersplenism. Spherocytosis suggests hypersplenism or hereditary spherocytosis. Liver function test results are diffusely abnormal in congestive splenomegaly with cirrhosis; an isolated elevation of serum alkaline phosphatase suggests hepatic infiltration, as in myeloproliferative and lymphoproliferative disorders and miliary TB.

Some other tests may be useful, even in asymptomatic patients. Serum protein electrophoresis identifying a monoclonal gammopathy or decreased immunoglobulins suggest lymphoproliferative disorders or amyloidosis; diffuse hypergammaglobulinemia suggests chronic infection (eg, malaria, kala-azar, brucellosis, TB) or cirrhosis with congestive splenomegaly, sarcoidosis, or connective tissue disorders. Elevation of serum uric acid suggests a myeloproliferative or lymphoproliferative disorder. Elevation of WBC alkaline phosphatase suggests a myeloproliferative disorder, whereas decreased levels suggest chronic myelocytic leukemia.

If testing reveals no abnormalities other than splenomegaly, the patient should be reevaluated at intervals of 6 to 12 mo or when new symptoms develop.

Treatment

  • Treatment of underlying disorder

Treatment is directed at the underlying disorder. The enlarged spleen itself needs no treatment unless severe hypersplenism is present. Patients with palpable or very large spleens probably should avoid contact sports to decrease the risk of splenic rupture.

Hypersplenism

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 anemia. The spleen can be expected to extend about 2 cm beneath the costal margin for each 1-g decrease in Hb. 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 (see 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 (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 vaccination against infections caused by Streptococcus pneumoniae, Neisseria meningitidis, and Haemophilus influenzae. After splenectomy, patients are particularly susceptible to severe sepsis 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

Hereditary spherocytosis

Thalassemia

Severe pancytopenia associated with massive splenomegaly

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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