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Leishmaniasis

Chapter 233 | Harrison's 22e · Part 5 – Infectious Diseases: Parasitic · Chapter 233


Key Clinical Points

  1. Caused by ~20 species of the genus Leishmania in the Kinetoplastida family.
  2. Three clinical syndromes: visceral (VL), cutaneous (CL), and mucosal (ML).
  3. Transmission via sandfly bites (Phlebotomus in Old World; Lutzomyia in New World).
  4. Global burden: 30,000 VL cases/year; 1 million CL cases/year.
  5. HIV co-infection significantly increases mortality risk (e.g., 30% in Ethiopia).
  6. VL is characterized by hepatosplenomegaly (pathognomonic), pancytopenia, and hyperglobulinemia.
  7. PKDL occurs in 5–20% of VL survivors and serves as a parasite reservoir.
  8. Diagnosis involves leishmanin skin tests, Giemsa-stained microscopy (amastigotes with kinetoplasts), and PCR.
  9. Treatment for VL: Liposomal AmB or Miltefosine; Treatment for CL: Sodium stibogluconate.
  10. No vaccine available; prevention focuses on vector control and personal protection.

1. DEFINITION & OVERVIEW

Leishmaniasis is a complex group of diseases caused by unicellular eukaryotic protozoa of the genus Leishmania, primarily affecting the reticuloendothelial system.

Pathogen Diversity: ◦ Caused by ~20 species in the Kinetoplastida family.

Clinical Syndromes: ◦ Visceral leishmaniasis (VL) → often fatal if untreated; caused by L. donovani complex. ◦ Cutaneous leishmaniasis (CL) → characterized by self-healing ulcers; caused by Viannia subspecies and L. major. ◦ Mucosal leishmaniasis (ML) → limited to South America; causes destruction of nasal mucosa and palate; caused by L. braziliensis.

1.1 Etiology & Life Cycle

Leishmania species have complex life cycles involving sandfly vectors and mammalian hosts:

Vector Transmission: ◦ Old World: Phlebotomus species ◦ New World: Lutzomyia species

Life Cycle Stages: ◦ Promastigotes: Flagellated form in sandfly midgut → multiply by binary fission. ◦ Transmission: Occurs via sandfly proboscis during blood meal. ◦ Amastigotes: Non-flagellated intracellular form → infect macrophages. ◦ Replication: Macrophage rupture releases amastigotes → infect new cells. ◦ Cycle Completion: Sandflies acquire amastigotes from hosts → transform into promastigotes.


2. EPIDEMIOLOGY

Leishmaniasis occurs in 99 countries across tropical and temperate regions.

Global Burden: ◦ 30,000 VL cases/year; 1 million CL cases/year.

Regional Distribution: ◦ VL: Highest incidence in East Africa, Brazil, and the Indian subcontinent. ◦ CL: Prevalent in South America, Africa, and Asia. ◦ ML: Restricted to South America.

Transmission Dynamics: ◦ Zoonotic: Common in Middle East, Pakistan, China (often involving dogs/foxes). ◦ Anthroponotic: Dominates in Sudan/South Sudan.

Impact of Co-infection: ◦ HIV co-infection → increased mortality risk (30% in Ethiopia).

Public Health Success: ◦ 98.7% decline in Indian VL incidence following elimination programs.

2.1 Geographic Distribution (Table 233-1)

Leishmania donovani Complex: ◦ Regions: South Asia, Africa, Mediterranean, Middle East, Central Asia, China. ◦ Syndromes: VL, PKDL; also some cases of CL. ◦ Vectors: Phlebotomus species.

Viannia Subspecies (e.g., L. braziliensis, L. guyanensis): ◦ Regions: Central and South America. ◦ Syndromes: CL, ML. ◦ Vectors: Lutzomyia species.

L. major: ◦ Regions: Western/Central Asia, North/Sub-Saharan Africa; Kazakhstan, Turkmenistan, Uzbekistan. ◦ Syndrome: CL.

L. tropica: ◦ Syndromes: CL, leishmaniasis recidivans.

L. aethiopica: ◦ Syndromes: CL, DCL.

2.2 Post-Kala-Azar Dermal Leishmaniasis (PKDL) (Table 233-2)

East Africa (Sudan/South Sudan): ◦ Incidence: ~50% of patients with VL. ◦ Timing: During VL to 6 months. ◦ Treatment: Sodium stibogluconate for 2–3 months → spontaneous cure in majority.

Indian Subcontinent (Bangladesh): ◦ Timing: 6 months to 3 years after VL. ◦ Treatment: Miltefosine for 12 weeks.


3. CLINICAL MANIFESTATIONS

Clinical presentations vary by Leishmania species and host immune response:

Visceral leishmaniasis (Kala-azar): ◦ Systemic Symptoms: Prolonged fever, weight loss. ◦ Organ Involvement: Hepatosplenomegaly (pathognomonic). ◦ Laboratory Findings: Hypoglycemia, pancytopenia, hyperglobulinemia.

Cutaneous leishmaniasis: ◦ Presentation: Ulcerated plaques on exposed skin (face, hands). ◦ Progression: May progress to post-kala-azar dermal leishmaniasis (PKDL).

Mucosal leishmaniasis: ◦ Site: Destruction of nasal mucosa and palate. ◦ Pathogen: L. braziliensis.

3.1 Complications

PKDL: ◦ Prevalence: 5–20% of VL survivors. ◦ Presentation: Hypopigmented nodules on face and extremities. ◦ Impact: Social stigma and disfigurement.

HIV Co-infection: ◦ Risk: Increased risk of disseminated disease and treatment failure.


4. DIAGNOSIS

Diagnostic approach depends on clinical suspicion and available resources:

Leishmanin skin test (Montenegro test): ◦ Mechanism: Delayed-type hypersensitivity to leishmanial antigens. ◦ Sensitivity: Positive in 80% of exposed individuals.

Microscopy: ◦ Method: Giemsa-stained smears (e.g., splenic smear for VL). ◦ Findings: Amastigotes (2–4 μm) with nucleus and kinetoplast. ◦ Sensitivity: <50% in VL, >90% in CL.

Molecular methods (PCR): ◦ Use: Rapid and specific for species identification from blood or lesion samples.

Differential Diagnosis: ◦ Cutaneous lesions may mimic: ◦ Tuberculosis ◦ Fungal infections (e.g., sporotrichosis) ◦ Leprosy ◦ Sarcoidosis ◦ Malignant ulcers.


5. TREATMENT

Therapeutic options vary by disease form and geographic region:

Visceral leishmaniasis (VL): ◦ First-line: Liposomal amphotericin B (AmB) 3–5 mg/kg/day for 10–20 days. ◦ Alternative: Miltefosine 100 mg/day for 28 days. ◦ Note: HIV co-infected patients require extended treatment duration.

Cutaneous leishmaniasis (CL): ◦ Local therapy: Cryotherapy or intralesional antimonials. ◦ Systemic: Sodium stibogluconate 20 mg/kg/day for 20–30 days.

Mucosal leishmaniasis (ML): ◦ Combination: Systemic antimonials and surgical debridement.


6. PREVENTION & CONTROL

Preventive measures focus on vector control and public health initiatives:

Personal Protection: ◦ Clothing: Permethrin-treated. ◦ Repellents: DEET-based. ◦ Behavior: Avoid outdoor activities during sandfly season (May–October).

Public Health Strategies: ◦ Vector Control: Indoor residual spraying in endemic areas. ◦ Reservoir Management: Dog population management for zoonotic VL control. ◦ Community Action: Mass drug administration in high-risk communities.

Vaccination: ◦ Status: No vaccine available; research ongoing.


Reference Tables

TABLE 233-1 Geographic Distribution and Characteristic Epidemiology of Leishmaniases

Harrison's 22e, p.1782

ORGANISM, ENDEMIC REGION CLINICAL
SYNDROME
SPECIES VECTOR RESERVOIR TRANSMISSION SETTING
Leishmania donovani Complex
South Asia VL, PKDL L. donovani Phlebotomus
argentipes
Humans Anthroponotic Rural, domestic
Sudan, South Sudan, Somalia,
Ethiopia, Kenya, Uganda
VL, PKDL L. donovani P. orientalis,
P. martini
Humans, rodents in
Sudan, canines
Anthroponotic,
occasionally zoonotic
Majority peridomestic,
occasionally sylvatic
Mediterranean basin, Middle
East, Central Asia, China
VL, CL L. infantum P. perniciosus,
P. ariasi
Dogs, foxes, jackals Zoonotic Domestic, peridomestic
Middle East, Saudi Arabia,
Yemen
VL L. donovani P. perniciosus,
P. ariasi
Dogs, foxes, jackals Zoonotic Domestic, peridomestic
Central and South America VL, CL L. infantuma Lutzomyia
longipalpis
Foxes, dogs,
opossums
Zoonotic Domestic, peridomestic,
periurban
Azerbaijan, Armenia, Georgia,
Kazakhstan, Kyrgyzstan,
Tajikistan, Turkmenistan,
Uzbekistan
VL L. infantum P. turanicus Humans, dogs, foxes Anthroponotic,
zoonotic
Domestic
L. tropica
CL,
leishmaniasis
recidivans
L. tropica P. sergenti Humans Anthroponotic
L. major
Western and Central Asia,
North and sub-Saharan Africa
CL L. major P. papatasi,
P. duboscqi
Nile rats, rodents Zoonotic Sylvatic, peridomestic
Kazakhstan, Turkmenistan,
Uzbekistan
CL L. major P. papatasi,
P. duboscqi
Gerbils Zoonotic Rural
L. aethiopica
CL, DCL L. aethiopica P. longipes,
P. pedifer
Hyraxes Zoonotic
Subspecies Viannia
Peru, Ecuador CL, ML L. (V.) peruviana Lutzomyia
verrucarum,
L. peruensis
Wild rodents Zoonotic Andean Valleys
Guyana, Surinam, French
Guyana, Ecuador, Brazil,
Colombia, Bolivia
CL, ML L. (V.) guyanensis L. umbratilis Sloths, arboreal
anteaters, opossums
Zoonotic Tropical forest
Central America, Ecuador,
Colombia
CL, ML L. (V.) panamensis L. trapidoi Sloths Zoonotic Tropical forest and
deforested areas
South and Central America CL, ML L. (V.) braziliensis Lutzomyia spp.,
L. umbratilis,
Psychodopygus
wellcomei
Forest rodents,
peridomestic animals
Zoonotic Tropical forest and
deforested areas
L. mexicana Complex
CL, ML, DCL L. amazonensis L. flaviscutellata Forest rodents Zoonotic
CL, ML, DCL L. mexicana L. olmeca Variety of forest
rodents and
marsupials
Zoonotic
CL, DCL L. pifanoi L. olmeca Variety of forest
rodents and
marsupials
Zoonotic

TABLE 233-2 Clinical, Epidemiologic, and Therapeutic Features of Post–Kala-Azar Dermal Leishmaniasis: East Africa and…

Harrison's 22e, p.1785

FEATURE EAST AFRICA INDIAN SUBCONTINENT
Most affected country Sudan and South Sudan Bangladesh
~50%
Interval between VL and
PKDL
During VL to 6 months 6 months to 3 years
Mainly children
History of prior VL Yes Not necessarily
Yes
Treatment Sodium stibogluconate
for 2–3 months
Miltefosine for 12 weeks
Spontaneous cure in
majority of patients