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Splenomegaly

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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Splenomegaly is almost always secondary to other disorders. Causes of splenomegaly 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

In the tropics, the most common causes are

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

Splenomegaly can lead to cytopenias, a disorder called 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 such as a hypernephroma.

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 lymphoproliferative, infectious, or autoimmune disorder.

Common Causes of Splenomegaly

Type

Examples

Congestive

External compression or thrombosis of portal or splenic veins

Certain malformations of the portal venous vasculature

Infectious and inflammatory

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

Secondary amyloidosis

Connective tissue disorder (eg, SLE, Felty syndrome)

Myeloproliferative and lymphoproliferative

Myelofibrosis with myeloid metaplasia

Lymphomas, especially hairy cell leukemia

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

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

Nonlipoid (eg, Letterer-Siwe disease)

Structural

Splenic cysts, usually caused by resolution of previous intrasplenic hematoma

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. 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 and immunochemical assays such as light chain measurements of peripheral blood and/or bone marrow sections is indicated if lymphoma is suspected.

Specific peripheral blood findings may suggest underlying disorders (eg, small-cell lymphocytosis in chronic lymphocytic leukemia, large granular lymphocytosis in T-cell granular lymphocyte [TGL] hyperplasia or TGL leukemia, atypical lymphocytes in hairy cell leukemia, and 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 suggests 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.

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