Tropical Splenomegaly Syndrome Clinical Presentation

Updated: Sep 11, 2019
  • Author: Mundeep K Kainth, DO, MPH; Chief Editor: Vikramjit S Kanwar, MBBS, MBA, MRCP(UK), FAAP  more...
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Presentation

History

The most common presenting symptoms of hyperreactive malarial syndrome (HMS) are chronic abdominal swelling (64%) and pain (52%). [3] Abdominal swelling may wax and wane, and pressure on the abdominal contents may lead to hernias and leg swelling; abdominal pain may be acute.

A history of chronic splenic enlargement differentiates HMS from simple malarial splenomegaly. Extensive travel history should be taken, including travel in the distant past to endemic areas, in patients who present with massive splenomegaly. HMS may develop years after returning home in expatriates and visitors.

Almost all patients report weight loss.

Many patients do not have any symptoms and are capable of normal daily activity. Patients adapt physiologically to chronic anemia and are symptomatic only when it is severe. Weakness, loss of energy, and headache may signify severe anemia.

Rarely, patients have intermittent fever. Persistent, severe fever should raise the possibility of an alternative diagnosis. [25]

Bleeding complications such as epistaxis are uncommon because thrombocytopenia is usually not severe.

Susceptibility to skin and respiratory infections is slightly increased.

Pregnant women are susceptible to episodes of massive Coombs-negative hemolysis, which are usually preceded by febrile episodes. Coombs-positive hemolysis is associated rarely with acute attacks of Plasmodium vivax malaria. [26]

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Physical

Patients with hyperreactive malarial syndrome (HMS) are usually afebrile at presentation. Pallor is common, and the patient may be malnourished and jaundiced.

In general, tachycardia is absent. If tachycardia is present, it indicates a concurrent complication.

The hallmark of HMS is moderate-to-massive splenomegaly. Few spleens (around one third) are tender, and almost all have a smooth surface, soft consistency, and sharp border. [24] The enlarged spleen may be seen to protrude against the abdominal wall, and a splenic bruit may be audible. Despite the size of the spleen, splenic rupture is rare.

Extensive travel history should be taken, including travel in the distant past to endemic areas, in patients who present with massive splenomegaly. HMS may develop years after returning home in expatriates and visitors.

Hepatomegaly is common; in a study of 69 Nigerian patients, 93% had accompanying hepatomegaly. [3]  Ascites is uncommon.

Dilatation of the veins, cardiomegaly, low blood pressure, and flow murmurs reflect hypervolemia.

Lymphadenopathy is absent, but bilateral parotid swelling has been described.

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Causes

The most important predisposing factor for hyperreactive malarial syndrome (HMS) is residence in or visitation to an area where malaria is endemic. Other risk factors include malnutrition and an as-yet-undefined genetic predisposition.

A study by Gai et al found that in the southern Indian city of Mangaluru (Mangalore), the rate of splenomegaly in patients with malaria caused by Plasmodium falciparum (29.6%) was higher than that in patients with Plasmodium vivax malaria (16.6%). [27]

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