Splenomegaly is an enlargement of the
spleen.[1] The spleen usually lies in the left upper quadrant (LUQ) of the
human abdomen. Splenomegaly is one of the four cardinal signs of hypersplenism which include: some reduction in number of circulating blood cells affecting
granulocytes,
erythrocytes or
platelets in any combination; a compensatory proliferative response in the bone marrow; and the potential for correction of these abnormalities by
splenectomy. Splenomegaly is usually associated with increased workload (such as in
hemolytic anemias), which suggests that it is a response to hyperfunction. It is therefore not surprising that splenomegaly is associated with any
disease process that involves abnormal
red blood cells being destroyed in the spleen. Other common causes include congestion due to
portal hypertension and infiltration by
leukemias and
lymphomas. Thus, the finding of an enlarged spleen, along with
caput medusae, is an important sign of portal hypertension.[2]
Definition
The standard system for classifying splenomegaly on
radiography is:[3][4]
Normal (not splenomegaly): the largest dimension is less than 11 cm
Moderate splenomegaly: the largest dimension is between 11 and 20 cm
Severe splenomegaly: the largest dimension is greater than 20 cm
Also, a
cutoff of a craniocaudal height of 13 cm is also used to define splenomegaly.[5] In addition, individual intervals have been established:
For children, the cutoffs for splenomegaly are given in this table, when measuring the greatest length of the spleen between its dome and its tip, in the
coronal plane through its hilum while breathing quietly.[7]
At
autopsy, splenomegaly can be defined as a spleen weight above the upper limit of the standard
reference range of 230 g (8.1 oz).[8][9]
Splenomegaly refers strictly to spleen enlargement, and is distinct from hypersplenism, which connotes overactive function by a spleen of any size. Splenomegaly and hypersplenism should not be confused. Each may be found separately, or they may coexist. Clinically, if a spleen is palpable (felt via
external examination), it means it is enlarged as it has to undergo at least twofold enlargement to become palpable. However, the tip of the spleen may be palpable in a newborn baby up to three months of age.[10]
Signs and symptoms
Symptoms may include
abdominal pain, chest pain, chest pain similar to
pleuritic pain when stomach, bladder or bowels are full, back pain, early satiety due to splenic encroachment, or the symptoms of
anemia due to accompanying
cytopenia.
Signs of splenomegaly may include a palpable left upper quadrant
abdominal mass or splenic rub. It can be detected on
physical examination by using
Castell's sign,
Traube's space percussion or
Nixon's sign, but an
ultrasound can be used to confirm diagnosis. In patients where the likelihood of splenomegaly is high, the physical exam is not sufficiently sensitive to detect it; abdominal imaging is indicated in such patients.[11]
In cases of
infectious mononucleosis splenomegaly is a common symptom and health care providers may consider using
abdominal ultrasonography to get insight into a person's condition.[12] However, because spleen size varies greatly, ultrasonography is not a valid technique for assessing spleen enlargement and should not be used in typical circumstances or to make routine decisions about fitness for playing sports.[12]
Abdominal CT is the most accurate. The spleen needs to be 2–3 times larger than normal to be palpable below the costal margin in physical examination.
Treatment
If the splenomegaly underlies hypersplenism, a
splenectomy is indicated and will correct the hypersplenism. However, the underlying cause of the hypersplenism will most likely remain; consequently, a thorough diagnostic workup is still indicated, as, leukemia, lymphoma and other serious disorders can cause hypersplenism and splenomegaly. After splenectomy, however, patients have an increased risk for infectious diseases.
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