What are the clinical manifestations of visceral leishmaniasis?

Updated: Feb 18, 2020
  • Author: Craig G Stark, MD, FACP, FFTM, RCPS(Glasg), FISTM; Chief Editor: Pranatharthi Haran Chandrasekar, MBBS, MD  more...
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Answer

Visceral disease, the most devastating and fatal form of leishmaniasis, is classically known as kala-azar or the Indian name for “black fever/disease,” which is a reference to the characteristic darkening of the skin that is seen in patients with this condition. Other terms used to describe visceral disease include Dumdum fever, Assam fever, and infantile splenomegaly in various parts of the world.

This condition occurs with both New and Old World species and results from systemic infection of the liver, spleen, and bone marrow. The spectrum of illness ranges from asymptomatic infection or self-resolving disease to fulminant, severe, life-threatening infection; many subclinical cases occur and go unrecognized for each clinically recognized case.

The syndrome is characterized by the pentad of fever, weight loss, hepatosplenomegaly, pancytopenia, and hypergammaglobulinemia. The fever is continuous or remittent and becomes intermittent at a later stage. It is also characteristically described as a double rise in 24 hours, in which waves of pyrexia may be followed by a period without fever. Patients may also report night sweats, weakness, diarrhea, malaise, and anorexia. Melanocyte stimulation and xerosis can occur, causing characteristic skin hyperpigmentation.

Onset of visceral disease can be insidious or sudden. The incubation period varies after infection (usually 3-6 mo, but can be months or years) and may depend on the patient's age and immune status as well as the species of Leishmania. Young malnourished children are most susceptible to developing progressive infection; those who present later in the course of the disease may present with edema caused by hypoalbuminemia, hemorrhage caused by thrombocytopenia, or growth failure caused by features of chronic infection.

If visceral disease is left untreated, death frequently occurs within 2 years which may be due to hemorrhage (secondary to infiltration of the hematopoietic system), severe anemia, immunosuppression, and/or secondary infections.

A variant of visceral leishmaniasis has been described in US soldiers who participated in the Gulf War. This is associated with light parasitic burden and mild symptoms including fever, malaise, and nausea.


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