Urgent considerations
See Differentials for more details
A crucial diagnostic question is to determine why a spleen is large. The most urgent situations are sudden pain associated with the splenomegaly, splenic sequestration crisis of sickle cell anaemia, and splenic vein or portal vein thrombosis.
Pain in the left upper quadrant may occur, either secondary to gradual splenic enlargement by any underlying aetiological process or as the result of a superimposed splenic infarct in an already enlarged spleen. Pain may occur in myeloproliferative disorders or leukaemias. Embolic infarcts (as seen in cholesterol embolisation or endocarditis) can give rise to painful splenomegaly. Relief of pain usually involves opioid analgesics. Testing, such as imaging (computed tomography [CT] scans) and blood cultures in suspected endocarditis, is important to establish the cause of pain.
Hepatic
Patients who present with either acute alcohol poisoning or chronic alcohol misuse and cirrhosis may have splenomegaly. The overriding feature in these individuals is hepatic disease with jaundice, transaminase elevation, encephalopathy, and coma. The patients may have severe bleeding due to underproduction of clotting factors by the deranged liver, and concomitant thrombocytopenia due to hypersplenism.
Most urgent considerations in patients with liver disease and concomitant splenomegaly revolve around bleeding from oesophageal varices and coagulopathy, especially reduced production of hepatic clotting factors and ongoing disseminated intravascular coagulation. The infusion of clotting factors (fresh frozen plasma and cryoprecipitate, as a source of fibrinogen) is necessary.
Neoplastic or myeloproliferative
Splenomegaly can represent any of a number of malignant conditions, and prompt recognition can be life-saving. Most of the time, especially in the acute and chronic leukaemias, a diagnosis can be made based on a review of the full blood count with differential and peripheral blood smear. Further investigations to make a diagnosis may require urgent referral to the appropriate specialty, depending on the presentation of the individual patient.
Patients who have myeloproliferative disorders, especially polycythaemia vera or essential thrombocytosis, may present with splenomegaly with or without pain. Young women with JAK2-positive polycythaemia vera may present with portal vein thrombosis and may have an enlarged spleen both from the myeloproliferation and portal vein thrombosis or even from Budd-Chiari syndrome. This type of presentation occurs less frequently in myelofibrosis. Review of the peripheral smear usually demonstrates teardrop red cells, left-shifted granulopoiesis, and nucleated red blood cells.
Initial treatment will depend on the specific diagnosis.
Vascular occlusion
Splenic vein thrombosis can lead to a sudden back-up of blood flow draining out of the spleen. If no collateral flow is established, the spleen can become swollen and rapidly painful. The diagnosis should be suspected in a patient presenting with rapid-onset tender splenomegaly and a history of acute pancreatitis. Anticoagulation can help to prevent further thromboses and re-establish more normal venous drainage out of the spleen. This should be initiated following confirmation of splenic vein occlusion on abdominal imaging.
Immunological/inflammatory
These conditions may present with life-threatening urgency, though the splenomegaly aspect of the presentation is not likely to be the overriding problem.
Patients with autoimmune haemolysis will have anaemia, jaundice, and weakness.
Haemophagocytic lymphohistiocytosis is a life-threatening condition.[29] Patients will often have an unexplained fever, cytopenias, and an elevated ferritin level. Patients may also have multi-organ failure. Splenomegaly and possibly hepatomegaly may be seen.
Infectious
Plasmodium falciparum malaria is potentially fatal if not treated promptly. Life-threatening complications can develop quickly in patients who initially appear well, and even short delays increase morbidity and mortality.
Diagnosis rests on a high index of suspicion in a patient with suggestive symptoms and an appropriate travel history. Reviewing the peripheral smear is usually sufficient to make this diagnosis. Splenomegaly may be absent in non-immune falciparum malaria, but is a marker of endemicity in transmission areas.[46]
Infectious endocarditis is usually diagnosed in an individual with fevers, rigors, splinter haemorrhages of the nail beds, Janeway lesions, Roth spots, positive blood cultures, or echocardiographic findings of vegetations. The splenomegaly is usually not an important part of the clinical presentation but can be identified on trans-oesophageal echocardiography.[47]
Approximately 1 in 10 people with severe dengue have secondary haemophagocytic lymphohistiocytosis (HLH), which has a high mortality rate.[41] Patients with dengue-associated HLH have fever, thrombocytopenia, splenomegaly, anaemia, and hepatomegaly, and raised serum ferritin.[42]
Initial treatment will depend on the specific diagnosis.
Inherent red blood cell abnormalities
Patients with homozygous sickle cell disease can have a splenic sequestration crisis as children, with sudden pooling of a large volume of blood into the spleen. This can cause hypotension and cardiac arrest. Sequestration crisis is a life-threatening emergency and needs to be treated with volume support and transfusion.
Traumatic
If a subcapsular haematoma is the cause of splenomegaly, there is risk of rupture. Frequent monitoring, potentially with periodic radiographical tests, may be important to demonstrate stability. Emergency splenectomy or repair of the splenic capsule may be necessary in case of rupture.
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