LEISHMANIASIS / MedUrgent


LEISHMANIASIS


Leishmaniasis is caused by leishmania species which are obligate intracellular flagellated protozoa parasites. The disease is transmitted to man by the black female sand fly phlebotomus which breeds in cool, shady places, on top of houses and in hills. During a blood feed, the promastigotes are inoculated intradermal where they transform into amastigotes and either proliferate locally to form a nodule that ulcerate later (cutaneous Leishmaniasis) or disseminate into the midline facial structures e.g. nose and mouth (espundia) or spread through macrophages into the spleen, liver, lymphatics and bone marrow (visceral Leishmaniasis). In humans the disease presents as granuloma with lymphocytes and macrophages engulfing organisms. Amastigotes (Leishman Donovan Bodies - LD bodies) are the only stage found in man. Clinically the disease is classified into three types:

1. Visceral Leishmaniasis : Leishmania Donovan

2.  Cutaneous Leishmaniasis : L.tropica. 

A) Old world Leishmaniasis: Leishmania T. major and leishmania T. minor, l.aethiopica 

B) New world Leishmaniasis : Leishmania Braziliansis and Leishmania Mexicans 3. Mucocutaneous Leishmaniasis (Espundia) - leishmania braziliansis It is caused by infection of the reticuloendothelial cells throughout the body by Leishmania donovani; a parasite that divides into three major biological forms. These species are morphologically similar and can only be differentiated clinically or by biochemical differences. 

VISCERAL LEISHMANIASIS (kala-azar) 

This is a chronic disease caused by one of the sub-species of L.donovani that are widely distributed throughout the tropics (L. Donovani found in India; L. Donovani infantum mainly in the Mediterranean and Middle Eastern countries, Africa and China; L. Donovani Chagasi in South America). The disease is usually transmitted by sand fly; however, blood transfusion, sexual contact; nasal discharge and needle pricks are all implicated.

Clinical manifestations

The incubation period varies from 9 to 10 months, but it could be short or as long as some years (an average of 3-6 months). Onset of the disease may be acute (high grade fever not associated with rigors) or gradual (prolonged intermittent fever). The fever is characteristically double peaked during the day. There is marked splenomegaly due to hyperplasia of the reticuloendothelial cells that are filled with parasites. Kupffer cells in the liver and the lymph nodes are similarly affected and are filled with the amasigotes. In the nasopharyngeal mucosa, granuloma and ulcerations may appear and extend down to the duodenum and jejunum. Bone marrow, skin, heart muscles, kidneys, adrenals and parotids may all be parasitized.

The patient does not look ill but anemic, anxious and wasted. The appetite is good with protuberant abdomen and dragging pain due to the presence of significant hepatosplenomegaly although liver enlargement is of a lesser degree. Lymphadenopathy, hypopigmented hair, black discoloration of the face (black sickness) and epistaxis secondary to hypersplenism and associated thrombocytopenia. Jaundice is considered as a bad prognostic sign. Anemia is due to iron deficiency, hyperspelism and autoimmune process.

Complications

include lobar pneumonia or bronchopneumonia, secondary bacterial infection, dysentery (bacterial or amoebic) which are considered serious complications, pulmonary T.B., Cancrum oris, liver cirrhosis Uveitis DIC, glomerulonephritis and amyloidosis affecting mainly the kidneys and nephrotic syndrome. Occular complications including retinal hemorrhage and papiloedema. Some of these complications could be the initial pretention symptoms especially chest infections. Relapses may occur between 6 months to two years. Causes of death are exhaustion, bacillary or amoebic dysentery pneumonia or coma.

If untreated, some patients may undergo spontaneous cure while others may die due to intercurrent infection. The mortality rate is high and death usually follows within 2 years of onset.

 




Post-kala-azar Dermal Leishmaniasis


The skin lesions appear within few months or longer following treatment of the African visceral Leishmaniasis. The macules and papules affect the face, extremities and sometimes involve the trunk. They start as hypopigmented macules, then change into papules that look like lepromatous leprosy and parasites are usually found in these lesions. Hepatosplenomegaly and marked ill health are not recognized features of this condition. The diagnosis depends time of appearance of the lesions and recovery of LD bodies from skin lesions. For Post-Kala-azar Dermal Leishmaniasis it usually does not respond to treatment with diamidine and is more refractory to antimonials.


Diagnosis

Visceral leishmaniasis with splenomegaly should be differentiated from other conditions such as typhoid, brucellosis, tuberculosis, chronic malaria, myelofibrosis, lymphomas and viral diseases

Laboratory Investigations

A number of specific tests can be done, but confirmation is by isolation of the organism.

• CBC: anemia, leucopenia with high eosinophil count, and thrombocytopenia

• Splenic aspirate (not advisable in children <5 yrs old or those with thrombocytopenia) stained for L.D bodies using Leishman or Gemsa stain

• Liver and lymph node biopsy and bone marrow punctures for detection of L.D. bodies

• Monoclonal antibody staining of tissue smears

• Serology: rk39 - ICT, Formol Gel Aldehyde, Direct Agglutination Test, Polymerase Chain Reaction,

• Complement Fixation Test, Indirect Fluorescent Antibody Titer and ELISA

• Fluorescent Antibody Technique which is positive in early infection.

• Hamagglutination test.

• Culture in NNN media or inoculation of the tissue aspirate into hamsters

• Leishmanin Skin Test (Montenegro test): It measures delayed hypersensitivity to the antigen prepared from promastigotes. It is positive in cutaneous and mucocutaneous leishmaniasis and negative in visceral leismaniasis and diffuse cutaneous leishmaniasis where it indicates present or past infection and depressed cellular immunity.

TREATMENT

1- Admission to hospital with absolute bed rest

2- Management of fever and complications including anemia, pneumonia and other infections.

3- Chemotherapy and drugs available are:

A) Antimony compounds:

 Sodium stibogluconate (Pentostam) is a Sodium Antimony compound and is the drug of choice. It is prepared in an isotonic neutral solution having 40% of the active substance and each ml of solution has 100mg of the active drug. The drug should be initially administered in a dose of 100mg and then increased by 100mg on daily basis until the total full dose of 600mg/day is reached. 30 injections are recommended for the Sudanese type. As for Indian kala-azar 6 injections are enough. In children aged 3-4 years the total dose is 400mg and those less than 2 years are given 200mg. The drug is given in a dose of 10-20 mg/kg/day IV slowly for 30 days and may be repeated twice. Contra-indications include jaundice, liver cirrhosis and nephritis. 

• Ethyl stibamine (Neostam) may be given starting with a small dose of 100mg IV. This is increased daily by 100mg to a maximuin of 300mg/day, which is then continued for 8 days. 

B) Diamidines:

i) Pentamidine isoethionate IV diluted in %5 dextrose in a dose of 2 4mg/ kg/day or every other day IM for a total of 14 doses. The dose may be repeated after 10 days.

ii) Hydroxystilbamidine Isoethionate IV in a dose of 250mg /day mixed in 5% dextrose to be given slowly for a period of 10 days. The course is repeated in three occasions with a 10 day gap between each course, (a total of 30 injections).

C) Others

• Meglumin antimonite: 20mg/kg/day IV for 20 - 28 days

• Amphotericin B: (used in antimony resistant cases of kala- azar) 0.5 -1 mg/kg/day IV diluted in 5% glucose infusion or normal saline given IV over 3 - 6 hours. The treatment is with a dose of 0.25mg / kg that may be gradually increased to 1mg/kg on alternate days until 1-2 am have been given. It is administered on alternate days for 3-8 weeks depending on the severity of the infection. It is extremely nephrotoxic 

• Relapasers can be treated with Pentostam or Amphotercin B

• Splenectomy may be done in resistant cases


Old World Cutaneous leishmaniasis (Baghdad boil. Delhi boil)

This form of the disease is characterized by nodular and ulcerative skin lesions caused by L. tropica major, L. tropica minor and Laethiopica. tropica inoculation in human tissue induces cell-mediated immune response that include lymphocytes, plasma cells and macrophages leading to granuloma formation and ulceration that heal with fibrosis and scar formation. The lesions tend to be multiple, have scales over an ulcerating center and end up with severe disfiguring scars especially on the face and extremities. Local lymphadenopathy may occur. Urban or Dry type is also caused by L tropica major. Its reservoirs are dogs and man and it is transmitted by the sandfly. It is common in the Middle East where 80% of the affected individuals live in the big cities. The lesions occur on the exposed part of the body, and begin as a small itchy nodule, which grow very slowly. Leishmaniasis recidivans presents with large ulcers on the face with active peripheries and healing from the center leaving severe scarring. Leishmanin Skin Test is positive. Diffuse Cutaneous Leishmaniasis is caused by L.tropica aethiopica and start as a small single and non-ulcerating nodule on the face and then multiple nodules appear covering all body. Lesions do not ulcerate but coalesce to form plaques. Leishmanin Skin Test is negative. Diagnosis is based mainly on the identification of the parasites in the smears taken from the edge of the ulcers and not the center.

Treatment

• Sodium stibogluconate

• Meglumin antimoniate (systemic or local infiltration)

• Mepacrine (local infiltration)

• Heat therapy (local application) Petamidine (4mg/kg/week)

New World Cutaneous Leishmaniasis (Espundia)

 This type of leishmaniasis is caused by the species L. Mexicana and Lbraziliensis. It is transmitted by the sand fly lutzomyia and characterized by ulcerative skin lesions and destructive mucocutaneous lesions in the mouth, nose and pharynx called Espundia. Cutaneous lesions start as a small papule at the site of inoculation, gradually enlarge to become an indurated ulcer. It is painless and usually heals spontaneously within 6 - 18 months. Mucosal lesions appear after the cutaneous lesions have healed by months or years, or they may develop as metastatic spread from the cutaneous lesion. There is often progressive mutilating destruction of the nasal septum, palate, lips, pharynx and larynx. Diagnosis is by identification of the parasite in aspirates of tissue juice from biopsy of the lesions and scrapings from the nasal mucosa. It is usually resistant to pentavalent antimonies although at times it could respond to treatment when given in maximum dosage.

Treatment                     

• Meglumine antimoniate: 14 mg/kg/day for 10 – 15 days to be repeated after 15 days if there is no healing. Local infiltration may be added

• Amphotericin B: starting with small dose (0.1mg/kg/2 days) up to a total of 2-3 gm

• Pentamidine

• Nifurtimox 

• Paromomycin: topical and parenteral 

• Cryotherapy 

• Radiofrequency or heat pads may be used locally.


PREVENTION AND CONTROL

• Health education to explain to people the life-cycle of the parasite, mode of infection, means of protection, avoiding regions where the sand fly is known to live and breed, destroying the sand fly by spraying homes with insecticides and removing breeding areas

• Supplying protection in the form of screening, use of safety nets, repellants, and wearing clothes that cover all the body.

• Vaccination which is derived from L. tropica and is only useful for cutaneous leishmaniasis.

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