Diagnostic Considerations
Traditionally, splenomegaly was classified as mild, moderate, or massive. This distinction can help with etiologic diagnosis.
Conditions to consider in the differential diagnosis of massive splenomegaly include the following:
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Leishmaniasis
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Malaria
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Myeloproliferative disease
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Portal vein obstruction/portal hypertension
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Schistosomiasis
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Niemann-Pick disease
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Mucopolysaccharidosis
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Lymphomas
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Gaucher disease [13]
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Hereditary spherocytosis
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Thalassemias major
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Histiocytosis X
Conditions to consider in the differential diagnosis of mild to moderate splenomegaly include all of the above, as well as the following:
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Bacterial sepsis
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Infective endocarditis
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Sickle cell disease
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Splenic abscess
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Acute infectious illnesses (eg, typhoid, malaria, other tropical diseases)
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Acute viral infections (eg, infectious mononucleosis)
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Systemic lupus erythematosus
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Tuberculosis
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Angioimmunoblastic lymphadenopathy
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Banti disease
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Congestive heart failure
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Drug reactions with serum sickness syndromes
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Hyperlipidemias
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Idiopathic splenomegaly
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Immune hemolytic anemias
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Immune thrombocytopenic disorders
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Leukocyte disorders
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Ovalocytosis
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Splenic vein obstruction
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Symptomatic human immunodeficiency virus (HIV) infection
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Trypanosomiasis
While certain causes of splenomegaly are usually obvious due to the concurrent illness (eg, endocarditis, malaria, infections), etiologic diagnosis of splenomegaly in the outpatient setting involves extensive history taking, with inquiries about the range of possible causes, including any history of liver disease, hereditary anemias, or infiltrative disorders. The presence of B symptoms and constitutional symptoms may indicate a primary bone marrow malignancy or myeloproliferative disorders.
On laboratory testing, if the erythrocyte sedimentation rate is high, the differential diagnosis includes the following:
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Infection
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Acute leukemias
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Sarcoidosis
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Other inflammatory disorders
If the sedimentation rate is low, the differential diagnosis includes the following:
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Chronic myeloproliferative disorders
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Hereditary hemolytic anemias
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Infiltrative disorders
Anemia with peripheral smear findings showing spherocytosis, sickle cells, hemoglobin SC, or elliptocytosis would indicate splenomegaly due to those disorders. Anemia with a high lactate dehydrogenase (LDH) or low haptoglobulin level and a high bilirubin level may indicate a hemolytic disorder or liver disease.
An abnormal coagulation profile with a high prothrombin time (PT), international normalized ratio (INR), and activated partial thromboplastin time (aPTT) usually indicates an associated liver disorder, with cirrhosis and portal hypertension as the etiology of splenomegaly. It may also indicate an underlying acute bone marrow malignancy or disseminated intravascular coagulation (DIC).
Splenomegaly with fevers may indicate one of the following:
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Epstein-Barr virus (EBV) infection
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Acute bacterial or fungal infection
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Acute leukemia or lymphoma
Differential Diagnoses
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This patient has a splenic abscess due to pneumococcal bacteremia. Note that the massively enlarged spleen is readily visible, with minimal retraction in the left upper quadrant.
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Resected specimen from the patient in the previous image. Note the discrete abscesses adjacent to normal parenchyma.
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The margins of this massive spleen were palpated easily preoperatively. Medially, the 3.18 kg (7 lb) spleen crosses the midline. Inferiorly, it extends into the pelvis.
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Massive splenomegaly does not preclude splenectomy through a minimally invasive approach. This photograph depicts a fragmented 3.2 kg (7.05 lb) spleen after removal via a hand-assisted laparoscopic technique.
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A portion of a massive spleen is extracted via hand-assisted laparoscopy.
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Intraoperative photograph of a laparoscopic splenectomy being taken down using the hanging-pedicle technique. The tip of the spleen is visualized in the background, whereas the stapler is detailed in the foreground across a segment of the pedicle.
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A massive spleen that was removed from an elderly woman with lymphoma.
