229.1 🎓 醫孞生版

229.1.0.1 📌 䞀頁重點

229.1.0.1.1 Pathogen + Vector
  • Genus: Leishmania
  • Many species — different clinical forms by species
  • Vector: Sandfly — Phlebotomus (Old World — Asia, Africa, Mediterranean) + Lutzomyia (New World — Americas)
  • Reservoirs: dogs, rodents, foxes, humans (anthroponotic in some areas)
229.1.0.1.2 4 Major Clinical Forms
229.1.0.1.2.1 1. Cutaneous Leishmaniasis (CL)
  • Old World: L. tropica, L. major, L. aethiopica — Mediterranean, Mid East, Africa, Asia
  • New World: L. mexicana, L. braziliensis — Latin America
  • “Oriental sore” — papule → ulcer at sandfly bite site
  • Self-limited months-years but heals with scar
  • Mucocutaneous progression in some New World species (especially L. braziliensis)
229.1.0.1.2.2 2. Mucocutaneous Leishmaniasis (MCL)
  • “Espundia”
  • Caused by L. braziliensis complex (Latin America)
  • Cutaneous primary lesion → years later → mucosal destruction of nose, palate, larynx
  • Progressive disfigurement
  • Latin America “leprosy of the rainforest”
229.1.0.1.2.3 3. Visceral Leishmaniasis (VL / Kala-Azar)
  • Causative species: L. donovani (India, E Africa, Sudan), L. infantum / L. chagasi (Mediterranean, Latin America, China)
  • Fatal if untreated (95%+ mortality)
  • Systemic disease:
    • Prolonged fever (months)
    • Massive splenomegaly + hepatomegaly
    • Lymphadenopathy
    • Pancytopenia (anemia + thrombocytopenia + neutropenia)
    • Hypergammaglobulinemia (polyclonal IgG)
    • Hypoalbuminemia + cachexia
    • Hyperpigmentation (“Kala-azar” = “black sickness”)
    • Death from intercurrent bacterial infection
229.1.0.1.2.4 4. Post-Kala-Azar Dermal Leishmaniasis (PKDL)
  • After VL treatment in some patients (especially L. donovani in India + Sudan)
  • Hypopigmented + nodular skin lesions
  • Recurrent / chronic
  • Reservoir for transmission
229.1.0.1.3 Epidemiology
  • 700,000 - 1 million new cases/yr globally (WHO)
  • 26,000 - 65,000 deaths/yr (mostly VL)
  • VL hot spots: India, Bangladesh, Nepal, Sudan, Ethiopia, Brazil
  • CL: Brazil, Iran, Peru, Saudi Arabia, Syria, Afghanistan
  • MCL: South America (Bolivia, Brazil, Peru)
229.1.0.1.4 Diagnosis
  • Direct visualization:
    • Bone marrow aspirate (most sensitive for VL)
    • Splenic aspirate (highest sensitivity, but bleeding risk)
    • Liver biopsy
    • Skin biopsy (CL)
  • Amastigote forms (intracellular, no flagella) on Giemsa stain
  • Promastigote forms (extracellular, flagellated) in culture media (NNN — Novy-MacNeal-Nicolle)
  • PCR — sensitive, species-specific
  • Serology:
    • VL: rK39 rapid test (highly sensitive + specific for VL caused by L. donovani)
    • rK28 for L. infantum
    • ELISA, IFA, DAT (direct agglutination test)
  • Leishmanin skin test (Montenegro test) — for past exposure, not active
229.1.0.1.5 Treatment
229.1.0.1.5.1 Visceral Leishmaniasis
  • Liposomal amphotericin B (AmBisome) — preferred (high efficacy, less toxicity)
    • Single dose (10 mg/kg) for VL in India (used in mass campaigns)
    • 3-5 mg/kg/day × 10-21 days for other endemic
  • Miltefosine (oral) — alternative
    • 50 mg PO bid × 28 days
    • Teratogenic — pregnancy contraindicated
    • Lactation contraindicated
  • Pentavalent antimony (sodium stibogluconate, meglumine antimoniate)
    • IV/IM × 28+ days
    • Cardiac toxicity (QTc)
    • Pancreatitis, hepatic, hematologic
    • Increasing resistance in India
  • Paromomycin + amphotericin combinations
  • Pentamidine — historical
229.1.0.1.5.2 HIV + VL Co-Infection
  • More severe + relapsing
  • Liposomal AmB × longer course
  • Maintenance / suppression often needed
  • ART concurrent
229.1.0.1.5.3 Cutaneous Leishmaniasis
  • Many self-limited; observation acceptable for simple ulcers
  • Pentavalent antimony intralesional or systemic
  • Miltefosine oral
  • Liposomal AmB
  • Cryotherapy + paromomycin topical for simple Old World CL
  • Treatment more aggressive if:
    • New World CL (mucocutaneous risk)
    • Multiple lesions
    • Facial / mucosal proximity
    • Immunocompromise
229.1.0.1.5.4 Mucocutaneous Leishmaniasis
  • Pentavalent antimony × 28+ days systemic
  • Liposomal AmB alternative
  • Miltefosine oral
  • Surgical reconstruction often needed
229.1.0.1.5.5 PKDL
  • L. donovani (Sudan, India)
  • Miltefosine or liposomal AmB
  • Long course
229.1.0.1.6 Prevention
  • Sandfly avoidance (dusk + dawn protective measures)
  • DEET + permethrin clothing
  • Bed nets (fine mesh — sandflies smaller than mosquitoes)
  • Insecticide-treated nets
  • Vector control programs
  • Treat human reservoir (PKDL)
  • No human vaccine licensed; canine vaccines exist for L. infantum control

229.1.0.2 1⃣ Leishmania Microbiology + Life Cycle

229.1.0.2.1 Morphology
  • Amastigote: intracellular form (no visible flagellum), in macrophages
  • Promastigote: extracellular form (free flagellum), in sandfly + culture
229.1.0.2.2 Life Cycle
  1. Sandfly bites infected mammal → promastigotes ingested
  2. Multiply in sandfly midgut
  3. Sandfly bites human → promastigotes injected
  4. Promastigotes enter macrophages (via complement)
  5. Inside macrophage → transform to amastigotes
  6. Multiply in macrophage phagolysosome
  7. Macrophage lyses → release amastigotes → infect new macrophages
  8. Tissue tropism varies by species:
    • Skin macrophages: CL
    • Mucosal: MCL
    • Reticuloendothelial system (spleen, liver, bone marrow): VL
229.1.0.2.3 Cell-Mediated Immunity Key
  • Th1 (IFN-γ, IL-12) clears infection
  • Th2 (IL-4, IL-10) promotes susceptibility
  • HIV / immunosuppression worsens clinical course

229.1.0.3 2⃣ Cutaneous Leishmaniasis (CL)

229.1.0.3.1 Etiology + Geography
229.1.0.3.1.1 Old World
  • L. major: arid + semi-arid (Mid East, N Africa, Central Asia)
  • L. tropica: urban, anthroponotic
  • L. aethiopica: highland Ethiopia
  • Russia, Uzbekistan, Turkey, Iran, Saudi Arabia, Syria, Israel
229.1.0.3.1.2 New World
  • L. mexicana: Central America
  • L. braziliensis: Brazil + Andes (risk of mucocutaneous progression)
  • L. amazonensis: Amazon Basin
  • L. panamensis, L. peruviana, others
229.1.0.3.2 Clinical
  • Sandfly bite site: papule → nodule → ulcer
  • “Oriental sore”
  • Painless typically
  • Heals 6-18 months with scar
  • 1-multiple lesions
  • Some species cause anergic / diffuse CL in immunosuppression
  • Mucocutaneous progression in 1-15% of New World L. braziliensis cases (years later)
229.1.0.3.3 Diagnosis
  • Skin biopsy + Giemsa stain (amastigotes in macrophages)
  • PCR of biopsy
  • Serology less useful for CL
229.1.0.3.4 Treatment
  • Simple Old World L. major / L. tropica:
    • Often observation (self-limited)
    • Cryotherapy + paromomycin topical
    • Intralesional pentavalent antimony
  • More aggressive treatment if:
    • New World species (mucocutaneous risk)
    • L. braziliensis complex (always treat systemically)
    • Multiple lesions
    • Facial / immune compromise
  • Options:
    • Pentavalent antimony systemic
    • Liposomal AmB
    • Miltefosine
    • Fluconazole, ketoconazole (some Old World species)
    • Pentamidine, paromomycin alternatives

229.1.0.4 3⃣ Mucocutaneous Leishmaniasis (MCL / Espundia)

229.1.0.4.1 Etiology
  • L. braziliensis complex (especially L. braziliensis, L. panamensis)
  • New World Latin America
229.1.0.4.2 Pathogenesis
  • CL primary lesion years prior
  • Hematogenous spread to mucosal surfaces
  • Inflammatory destruction
229.1.0.4.3 Clinical
  • Nasal involvement first: nodules, ulceration
  • Progressive destruction of nasal septum, palate, pharynx, larynx
  • Disfigurement
  • Airway compromise (severe)
  • Self-limited unlikely
229.1.0.4.4 Diagnosis
  • Skin / mucosal biopsy + Giemsa
  • PCR
229.1.0.4.5 Treatment
  • Pentavalent antimony × 28+ days systemic
  • Liposomal AmB alternative
  • Miltefosine
  • Pentamidine (less effective)
  • Surgical reconstruction for disfigurement (after parasite control)

229.1.0.5 4⃣ Visceral Leishmaniasis (VL / Kala-Azar)

229.1.0.5.1 Etiology
  • L. donovani (Indian subcontinent, Sudan, E Africa)
  • L. infantum (Mediterranean, China, Latin America)
  • L. chagasi (synonym of L. infantum in Latin America)
229.1.0.5.2 Epidemiology
  • 90% of cases in 6 countries: India, Bangladesh, Nepal, Sudan, Brazil, Ethiopia
  • WHO 2030 goal: eliminate VL as public health problem
  • India: nearly eliminated (subnational targets met)
  • E Africa (Sudan, Ethiopia) ongoing
229.1.0.5.3 Reservoirs
  • Anthroponotic in India + Sudan (humans)
  • Zoonotic in Mediterranean + Latin America (dogs primarily)
229.1.0.5.4 Pathogenesis
  • Visceralization of amastigotes to spleen, liver, bone marrow
  • Pancytopenia from marrow suppression + splenic sequestration
  • Hepatosplenic enlargement
  • Hypergammaglobulinemia (polyclonal IgG)
  • Severe immunosuppression
229.1.0.5.5 Clinical
  • Incubation 2-6 months (range 10 d - 2 yr)
  • Prolonged fever (months)
  • Massive splenomegaly + hepatomegaly
  • Pancytopenia (anemia + thrombocytopenia + neutropenia)
  • Lymphadenopathy
  • Cachexia + weight loss
  • Hyperpigmentation (“Kala-azar” = “black sickness”)
  • Hypoalbuminemia + edema
  • Bleeding from thrombocytopenia
  • Death typically from:
    • Bacterial sepsis (immunosuppressed)
    • Severe anemia
    • Cardiac failure
    • Severe bleeding
  • 95%+ mortality untreated
229.1.0.5.6 Diagnosis
  • rK39 rapid test — antibody to L. donovani recombinant antigen
    • Sensitive + specific for VL (Indian + S American strains)
    • Point-of-care
    • Useful in field settings
  • Bone marrow aspirate — high sensitivity (60-85%)
    • Amastigotes on Giemsa
  • Splenic aspirate — highest sensitivity (95%) but bleeding risk
  • Lymph node aspirate (Sudan, where common)
  • Liver biopsy (less common)
  • Buffy coat smear of blood
  • Culture: NNN media; slow
  • PCR of marrow, blood, tissue — sensitive, species-specific
  • Direct Agglutination Test (DAT), ELISA, IFA serology
229.1.0.5.7 Treatment
229.1.0.5.7.1 Liposomal Amphotericin B (Preferred)
  • Single dose 10 mg/kg for Indian VL (used in mass campaigns; WHO endorsement)
  • 3-5 mg/kg/day × 10-21 days for other endemic regions / severe
  • Lower toxicity than antimony
  • Effective + reduced renal toxicity
  • WHO essential medicine
229.1.0.5.7.2 Miltefosine (Oral)
  • 50 mg PO bid × 28 days
  • Effective for VL + CL + MCL
  • Pregnancy contraindicated (teratogenic)
  • Lactation contraindicated (long half-life)
  • Gastrointestinal side effects
  • Cardiac side effects (QTc)
229.1.0.5.7.3 Pentavalent Antimony
  • Sodium stibogluconate (Pentostam) or meglumine antimoniate (Glucantime)
  • IV/IM × 28-40 days
  • Cardiac (QTc, T-wave changes)
  • Pancreatitis (elevated amylase)
  • Hepatic + hematologic
  • Increasing resistance in India (L. donovani)
229.1.0.5.7.4 Paromomycin
  • IM × 21 days
  • Used in combinations
  • Aminoglycoside
229.1.0.5.7.5 Combinations
  • Liposomal AmB + miltefosine
  • AmB + paromomycin
  • Reduce treatment duration + improve cure rate
229.1.0.5.8 VL + HIV Co-Infection
  • More severe + relapsing course
  • Atypical presentations (limited fever)
  • Treatment more aggressive + longer
  • Liposomal AmB × longer (40 mg/kg total instead of 30)
  • Maintenance / suppression often needed
  • ART concurrent
229.1.0.5.9 Pregnancy + VL
  • Liposomal AmB safe
  • Pentavalent antimony — relative contraindication
  • Miltefosine + pentamidine contraindicated
229.1.0.5.10 Pediatric VL
  • L. infantum in Mediterranean (mostly pediatric)
  • Severe disease
  • Liposomal AmB

229.1.0.6 5⃣ Post-Kala-Azar Dermal Leishmaniasis (PKDL)

229.1.0.6.1 Background
  • Develops in 5-15% of VL patients after treatment
  • Mostly L. donovani (Sudan, India)
  • Latency: months to years post-VL
229.1.0.6.2 Clinical
  • Hypopigmented macules + nodules + papules
  • Especially face + extremities
  • Long-standing
  • Cosmetic concern + social stigma
229.1.0.6.3 Significance
  • Reservoir for transmission in anthroponotic regions
  • Public health importance
229.1.0.6.4 Treatment
  • Miltefosine PO
  • Liposomal AmB
  • Long course
  • May resolve spontaneously over years

229.1.0.7 6⃣ Prevention

229.1.0.7.1 Personal
  • DEET + picaridin repellents
  • Long sleeves + pants at dusk-dawn (sandfly activity)
  • Permethrin-treated clothing
  • Fine-mesh bed nets (sandflies are smaller than mosquitoes — regular mosquito nets less effective)
  • Insecticide-treated nets
229.1.0.7.2 Vector Control
  • Spraying with pyrethroid insecticides
  • Indoor residual spraying (IRS)
  • Environmental management (eliminating breeding sites)
229.1.0.7.3 Reservoir Control
  • Dog testing + treatment (Mediterranean, Latin America)
  • Canine vaccine (CaniLeish, LeishVet) — for veterinary use to reduce transmission
229.1.0.7.4 Treatment of Human Reservoir
  • PKDL patients
  • VL active treatment + cure
229.1.0.7.5 No Human Vaccine
  • Multiple candidates in clinical trials (Phase 1-2)
  • Whole-killed, recombinant, attenuated, DNA, mRNA
  • LEISH-F2 + GLA-SE adjuvant (in development)
  • IDRI vaccines
229.1.0.7.6 WHO 2030 Elimination Strategy
  • VL elimination as public health problem (< 1 case per 10,000 in endemic areas)
  • India near elimination
  • E Africa ongoing
  • Mass drug administration in some areas
  • Integrated vector management