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Visceral Leishmaniasis

Visceral leishmaniasis is one of the forms of infection caused by the protozoan parasite Leishmania. The majority of cases occur in the South American, African, and Asian continents. Blood-sucking sand flies are the vectors responsible for transmission of this microorganism to the human hosts. Infection is characterized by pancytopenia (anemia, thrombocytopenia, neutropenia), hepatosplenomegaly, fever, and severe immunosuppression that can lead to fatal opportunistic diseases without early recognition. The diagnosis is made through clinical findings, laboratory studies, and either direct or indirect microbiological methods.


Presentation

The clinical presentation of visceral leishmaniasis possesses a long incubation period (ranging from a few weeks to even years), a nonspecific symptomatology, and a variable clinical course, and may pose a diagnostic challenge [1] [2] [3] [4]. The two main signs of visceral leishmaniasis are fever and pancytopenia, both being almost universally present in all patients who suffer from this type of leishmaniasis [1] [2]. Fever can range anywhere between 37-40°C and often has an irregular pattern of occurrence [5]. On the other hand, pancytopenia (including thrombocytopenia, anemia, and leukopenia) causes weakness and severe immunosuppression, thus promoting various superinfections of bacterial, viral, or parasitic origin [1] [2] [5]. Opportunistic infections are, in fact, the leading cause of death from visceral leishmaniasis, with mortality rates being as high as 75%-95% in the absence of an early diagnosis and appropriate therapy [1]. The term "kala-azar" (meaning black death) is used to describe visceral leishmaniasis that is restricted to the Old World and caused by Leishmania donovani [1]. Wasting and poor general condition induced by pancytopenia are seen in advanced stages of the disease [6]. In addition to fever and bone marrow suppression, hepatosplenomegaly is another important sign in visceral leishmaniasis, seen in up to 85-100% of patients in some studies [5]. Lymphadenitis is a less frequent manifestation [2] [5].

Splenomegaly
  • Infantile VL may begin suddenly, with high fever and vomiting, or insidiously, with irregular daily fever, anorexia, and marked splenomegaly.[ncbi.nlm.nih.gov]
  • It can be difficult to diagnose this disease in endemic settings as several causes of febrile splenomegaly exist, notably malaria.[doi.org]
  • This is the report of a 2-year-old boy who presented with fever, cytopenia, and splenomegaly. The patient was diagnosed with hemophagocytic lymphohistiocytosis (HLH) and treated with HLH-2004 protocol.[ncbi.nlm.nih.gov]
  • Here we describe a case of visceral leishmaniasis infection in a 65-year old Greek male with psoriatic arthritis treated with methotrexate, who presented with high grade fever, chills, splenomegaly, pancytopenia and polyclonal hypergammaglobulinaemia.[ncbi.nlm.nih.gov]
Massive Splenomegaly
  • He had massive splenomegaly. His cardiovascular, respiratory and neurological examination were normal.[ncbi.nlm.nih.gov]
  • ., infection with Leishmania sp. associated with high fever, weight loss, massive splenomegaly and markedly altered laboratory parameters, is generally fatal if untreated.[ncbi.nlm.nih.gov]
  • splenomegaly Laboratory tests may show pancytopenia and hypergammaglobulinaemia Complications include gastrointestinal bleeding, peripheral oedema, acute renal failure and secondary bacterial infections Generalised hyperpigmentation is a late feature[dermnetnz.org]
Generalized Lymphadenopathy
  • Clinical manifestations of VL include recurrent fever, hepatosplenomegaly, generalized lymphadenopathy, pancytopenia, and anemia. Death occurs in the absence of appropriate chemotherapy ( 4 ).[jimmunol.org]
Fever
  • Herein we report an 11 month-old male infant with diagnosis of kala-azar who presented with pallor, hepatosplenomegaly, failure to gain weight, and no history of fever.[ncbi.nlm.nih.gov]
  • Infantile VL may begin suddenly, with high fever and vomiting, or insidiously, with irregular daily fever, anorexia, and marked splenomegaly.[ncbi.nlm.nih.gov]
  • A type of leishmaniasis occurring chiefly in India, caused by the protozoan parasite Leishmania donovani and characterized by fever, enlargement of the spleen and liver, progressive anemia and leukopenia, and weight loss.[medical-dictionary.thefreedictionary.com]
  • […] ka·la-azar \ ˌkä-lə-ə-ˈzär, ˌka- \ : a severe parasitic disease chiefly of tropical areas that is marked by fever, progressive anemia, leukopenia, and enlargement of the spleen and liver and caused by a leishmania ( Leishmania donovani ) transmitted by[merriam-webster.com]
  • Fever can range anywhere between 37-40 C and often has an irregular pattern of occurrence.[symptoma.com]
Anemia
  • A type of leishmaniasis occurring chiefly in India, caused by the protozoan parasite Leishmania donovani and characterized by fever, enlargement of the spleen and liver, progressive anemia and leukopenia, and weight loss.[medical-dictionary.thefreedictionary.com]
  • […] ka·la-azar \ ˌkä-lə-ə-ˈzär, ˌka- \ : a severe parasitic disease chiefly of tropical areas that is marked by fever, progressive anemia, leukopenia, and enlargement of the spleen and liver and caused by a leishmania ( Leishmania donovani ) transmitted by[merriam-webster.com]
  • Adverse events in the liposomal-therapy group were infusion-related fever or rigors (in 40%) and increased anemia or thrombocytopenia (in 2%); such events in the conventional-therapy group were fever or rigors (in 64%), increased anemia (in 19%), and[ncbi.nlm.nih.gov]
  • The disease presented with fatigue, anemia, and hepatosplenomegaly in a 61-year-old man with human immunodeficiency virus (HIV) infection who was receiving antiretroviral therapy. Diagnosis was made by bone marrow biopsy.[ncbi.nlm.nih.gov]
  • Infection is characterized by pancytopenia (anemia, thrombocytopenia, neutropenia), hepatosplenomegaly, fever, and severe immunosuppression that can lead to fatal opportunistic diseases without early recognition.[symptoma.com]
Chills
  • We report the case of a 73-year-old American traveler who presented with 3 weeks of fatigue, fevers, chills, and pancytopenia.[ncbi.nlm.nih.gov]
  • Here we describe a case of visceral leishmaniasis infection in a 65-year old Greek male with psoriatic arthritis treated with methotrexate, who presented with high grade fever, chills, splenomegaly, pancytopenia and polyclonal hypergammaglobulinaemia.[ncbi.nlm.nih.gov]
  • Although infusion-related fever and chills occurred in 56%-68% of patients in the 3 different dose groups, 401 of 405 patients completed the treatment. All 135 patients in group A completed treatment, and the final cure rate for this group was 97%.[ncbi.nlm.nih.gov]
  • Chills and rigors were the most commonly occurring adverse events (AEs). All the AEs were mild in intensity, and none of the patients experienced any serious AEs. No patients developed nephrotoxicity.[ncbi.nlm.nih.gov]
  • It is commonly characterized by fever, chills, vomiting, anemia, hepatosplenomegaly, leukopenia, hypergammaglobulinemia, emaciation, and an earth-gray color of the skin.[finto.fi]
Malaise
  • […] systemic disease characterized by darkening of the skin as well as the pentad of fever, weight loss, hepatosplenomegaly, pancytopenia, and hypergammaglobulinemia Viscerotropic leishmaniasis: Nonspecific abdominal tenderness; fever, rigors, fatigue, malaise[medscape.com]
  • Common symptoms are fever, malaise, weight loss and anaemia, with swelling of the spleen, liver and lymph nodes in visceral human leishmaniasis.[ecdc.europa.eu]
  • Antibiotics resulted in resolution of the fever and malaise, but the splenomegaly persisted. Her splenomegaly progressed causing abdominal distention, pain, and evidence of hypersplenism (anemia and thrombocytopenia).[acgcasereports.gi.org]
Persistent Cough
  • We present a case of a 12-year-old girl with a history of recurrent fever, anorexia, cachexia, chronic fatigue, weight loss, left palpebral unilateral edema, persistent cough and pancytopenia.[ncbi.nlm.nih.gov]
Abdominal Distension
  • On general physical examination, the patient was found to be thin built, pale, and afebrile with abdominal distension. Left submandibular and left axillary lymph nodes were enlarged and measured 1.5 cm in diameter.[cytojournal.com]
  • Patients presenting with abdominal distension and nausea showed higher IC-induced production of IL-6 ( p 0.0400 and 0.0371, respectively; data not shown), whereas IC from patients jaundiced at the time of blood sampling induced higher levels of GM-CSF[jimmunol.org]
  • The main clinical manifestations of VL are fever of insidious onset, weakness, loss of appetite, weight loss, abdominal distension due to enlargement of the spleen or the liver or both, lymph node enlargement, and a low blood cell count (pancytopenia)[doi.org]
Hepatosplenomegaly
  • VL should be considered in the differential diagnosis in returning travellers presenting with prolonged fever, hepatosplenomegaly and/or pancytopenia.[ncbi.nlm.nih.gov]
  • The case involves a 19-month-old male infant presenting with a high persistent fever with a fluctuating pattern, pancytopaenia, hepatosplenomegaly, and a high triglyceride level. Initial investigations showed an EBV infection.[ncbi.nlm.nih.gov]
  • The disease presented with fatigue, anemia, and hepatosplenomegaly in a 61-year-old man with human immunodeficiency virus (HIV) infection who was receiving antiretroviral therapy. Diagnosis was made by bone marrow biopsy.[ncbi.nlm.nih.gov]
  • It clinically manifests as fever, weight loss, swelling, hepatosplenomegaly, paleness, and edema.[ncbi.nlm.nih.gov]
  • We present the case of a woman with 24 gestational weeks presenting fever, hepatosplenomegaly, pancytopenia, and inversion of albumin/globulin ratio. Anti-rK39 was positive and amastigotes were visualized on myelogram.[ncbi.nlm.nih.gov]
Hepatomegaly
  • The features of VL may be different in immunosuppressed patients, with more fever and less hepatomegaly and leukopenia than in HIV-infected patients.[ncbi.nlm.nih.gov]
  • CASE PRESENTATION: A 9 year-old boy was admitted to Imam Reza teaching hospital in Mashhad, Iran in 2010 with fever, sweating, abdominal distention, massive hematemesis, weight loss, huge splenomegaly and hepatomegaly.[ncbi.nlm.nih.gov]
  • […] titer positivity of immunofluorescence antibodies, Leishmania amastigotes in Giemsa-stained slides of bone marrow, as well as molecular identification confirmed that in all three the infecting pathogen was Leishmania infantum Fever, splenomegaly, and hepatomegaly[ncbi.nlm.nih.gov]
  • METHODS: Patients suspected of having visceral leishmaniasis (n 120) fulfilling at least two of six criteria (fever 2 weeks, weight loss, tiredness affecting daily functions, splenomegaly, hepatomegaly and anemia) were studied using clinic-epidemiological[ncbi.nlm.nih.gov]
  • HIV-infected patients with visceral leishmaniasis presented with fever (76%), hepatomegaly (77%), splenomegaly (78%), and varying degrees of cytopenias. Leishmania was detected in atypical sites in 22 (14%) patients.[ncbi.nlm.nih.gov]
Progressive Visual Loss
  • An immunocompetent, 42 year-old male, presented with progressive visual loss and metamorphopsia in his right eye. The fundus examination showed a foveal round yellow lesion and intraretinal hemorrhages in the macula.[ncbi.nlm.nih.gov]
Retinal Hemorrhage
  • Bilateral retinal hemorrhages in an endemic country could suggest the diagnosis of visceral leishmaniasis.[ncbi.nlm.nih.gov]
Hyperactivity
  • The patient subsequently developed haemophagocytic lymphohistiocytosis, a life-threatening immune hyperactivity state that very rarely complicates leishmaniasis infection.[ncbi.nlm.nih.gov]
  • Immune complexes from SLE sera induce IL10 production from normal peripheral blood mononuclear cells by an FcγRII dependent mechanism: implications for a possible vicious cycle maintaining B cell hyperactivity in SLE. Ann. Rheum. Dis. 62 : 37 -42.[jimmunol.org]

Workup

A detailed patient history and a thorough physical examination are the first and perhaps crucial steps for making an initial diagnosis of visceral leishmaniasis. The vast majority of patients (> 90%) who suffer from visceral leishmaniasis come from six countries of the world: India, Bangladesh, Sudan, South Sudan, Ethiopia, and Brazil [6] [7] [8]. For this reason, the physician should conduct a detailed patient history that will cover recent travel to endemic countries, as well as possible exposure to sand flies in the last few years. After assessing the course of symptoms and their progression during the interview, a meticulous physical examination, particularly abdominal palpation, will reveal either hepatomegaly, splenomegaly, or both. In order to solidify the diagnosis, laboratory and microbiological tests need to be conducted. A basic complete blood count (CBC) is sufficient to detect pancytopenia, while directed diagnostic testing for Leishmania is broadly divided into indirect and direct methods. Serological testing (specifically enzyme-linked immunosorbent assay, or ELISA, and fluorescence antibody testing) is widely used as the main indirect test that has good sensitivity/specificity [1] [2] [3] [4]. Detection of antigen rk39 through ELISA is a very reliable procedure and is used by many laboratories over the world [3] Conversely, identification of the parasite by light microscopy and Giemsa staining from blood samples is a relatively easy and effective procedure, while lymph node, liver, spleen, or even bone marrow samples might be used for evaluation, but the risks of bleeding (particularly in the case of spleen and bone marrow) often outweigh the benefit of confirming the diagnosis histologically [1] [2] [3] [4].

Visceromegaly
  • The most common manifestations in patients infected with VL were intermittent fever, pancytopenia, visceromegaly, and increased serum level of acute phase reactants.[ncbi.nlm.nih.gov]

Treatment

  • The estimated cost of treatment with amphotericin B deoxycholate was USD 1,522.70.[ncbi.nlm.nih.gov]
  • BACKGROUND: There is insufficient evidence to support visceral leishmaniasis (VL) treatment recommendations in Brazil and an urgent need to improve current treatments. Drug combinations may be an option.[ncbi.nlm.nih.gov]
  • Although the cost of ABCD is prohibitive, the high level of efficacy associated with short-term treatment with low-dose ABCD provides another alternative for the treatment of VL, especially in regions where VL is antimony refractory.[ncbi.nlm.nih.gov]
  • […] had treatment failures.[doi.org]
  • We report here a case of a 41-year-old parasitologically confirmed male case of VL, who developed acute pancreatitis during treatment with miltefosine. On the 13 th day of treatment, he presented with abdominal pain and vomiting.[ncbi.nlm.nih.gov]

Prognosis

  • Visceral leishmaniasis, associated with HIV/AIDS coinfection, is becoming a more aggressive disease, complicating an accurate prognosis.[ncbi.nlm.nih.gov]
  • The presentation and prognosis will vary depending on the species involved.[dermnetnz.org]
  • […] megutamineantimoniate Non drug therapy-local heat therapy, cryo 30. 3.MUCUCUTANEOUS LEISHMANIASIS * Pentavalent antimony IVIM 20mgkg qd for 28 days * Amphotericine B(deoxycholate) IV 1mgkg qd (total 20-40mg) * Pentamidine IVIM 2-4mgkg thricewkly for 15 doses 31. prognosis[slideshare.net]
  • Enzyme-linked immunosorbent assay for recombinant K39 antigen in diagnosis and prognosis of Indian visceral leishmaniasis. Clin. Diagn. Lab. Immunol. 8 : 1220 -1224. Maurya, R., R. K. Singh, B. Kumar, P. Salotra, M. Rai, and S. Sundar. 2005.[doi.org]
  • Enzyme-linked immunosorbent assay for recombinant K39 antigen in diagnosis and prognosis of Indian visceral leishmaniasis. Clin. Diagn. Lab. Immunol. 8 : 1220 -1224. Maurya, R., R. K. Singh, B. Kumar, P. Salotra, M. Rai, and S. Sundar. 2005 .[jcm.asm.org]

Etiology

  • CONCLUSIONS: VL in HLH patients does not exclude a genetic etiology and requires structured clinical management.[ncbi.nlm.nih.gov]
  • However, a strong clinical suspicion compelled further evaluation by polymerase chain reaction (PCR), which validated the etiology. This short report highlights the usefulness of PCR in diagnosing cases of suspected smear-negative VL recurrence.[ncbi.nlm.nih.gov]
  • […] immune reconstitution syndrome; immune response; visceral leishmaniasis Publication type, MeSH terms, Substances Publication type Case Reports MeSH terms Adolescent Adult Aged Aged, 80 and over Arthritis, Infectious/diagnosis* Arthritis, Infectious/etiology[ncbi.nlm.nih.gov]
  • Abstract Leishmania amazonensis is a major etiological agent of human cutaneous leishmaniasis in the Americas; nevertheless there are some reports of this species causing visceral disease in dogs and men.[ncbi.nlm.nih.gov]
  • This case illustrates the importance of recognizing other infectious etiologies of HLH, especially in patients from areas of endemicity, and initiating appropriate therapy to prevent life-threatening complications.[bloodjournal.org]

Epidemiology

  • epidemiology* DNA, Intergenic/genetics DNA, Protozoan/blood DNA, Protozoan/genetics Enzyme-Linked Immunosorbent Assay Female HIV Infections/complications HIV Infections/epidemiology* Humans Iran/epidemiology Leishmania infantum/immunology Leishmania[ncbi.nlm.nih.gov]
  • Ref: WHO/CTD/LEISH/98.23 Leishmania/HIV co-infection - Epidemiological analysis of 692 retrospective cases.[who.int]
  • This study aimed to describe the clinical and epidemiological profile of VL/HIV coinfected patients and compare this profile to non-coinfected VL patients.[ncbi.nlm.nih.gov]
  • BACKGROUND: Zoonotic visceral leishmaniasis (ZVL) caused by Leishmania infantum is endemic with an epidemiological profile of a paediatric disease in Tunisia.[ncbi.nlm.nih.gov]
  • In the current study, we evaluate multilevel entomological, epidemiological and ecological factors associated with infection and disease through fine-scale eco-epidemiological analyses in three villages.[ncbi.nlm.nih.gov]
Sex distribution
Age distribution

Pathophysiology

  • Useful For Suggests clinical disorders or settings where the test may be helpful Diagnosis of active visceral leishmaniasis Clinical Information Discusses physiology, pathophysiology, and general clinical aspects, as they relate to a laboratory test Visceral[mayomedicallaboratories.com]
  • Pathophysiology Modes of transmission In leishmaniasis, the obligatory intracellular protozoa are transmitted to mammals via the bite of the tiny 2- to 3-mm female sandfly of the genus Phlebotomus in the Old World (Eastern Hemisphere) and Lutzomyia in[emedicine.com]
  • ., Barral, A. (1997) Recent advances in the pathophysiology and treatment of human cutaneous Leishmaniasis.[scindeks.ceon.rs]

Prevention

  • An early diagnosis based on a high suspicion can prevent a fatal outcome.[ncbi.nlm.nih.gov]
  • Therefore, clinicians especially in endemic areas are highly recommended to include kala-azar among differential diagnosis of unexplained anemia without fever to prevent misdiagnosis of this potentially fatal, but treatable condition.[ncbi.nlm.nih.gov]
  • Though immunocompetent patients may not develop the disease, in transplant recipients the use of corticoids and intensified immunosuppressants to prevent graft rejection may accelerate the disease, causing severe damage to the liver, spleen, and hematopoietic[ncbi.nlm.nih.gov]
  • It is important that travelers be counseled on preventive measures. Patients should be monitored after treatment for relapse.[ncbi.nlm.nih.gov]
  • Unfortunately, the difficult route of drug administration, toxicity issues, and cost prevent amphotericin B from reaching the infected population, and mortality continues to rise.[ncbi.nlm.nih.gov]

References

Article

  1. Ready PD. Epidemiology of visceral leishmaniasis. Clin Epidemiol. 2014;6:147-154.
  2. Barrett MP, Croft SL. Management of trypanosomiasis and leishmaniasis. Br Med Bull. 2012;104:175–196.
  3. Srivastava P, Dayama A, Mehrotra S, Sundar S. Diagnosis of visceral leishmaniasis. Trans R Soc Trop Med Hyg. 2011;105(1):1-6.
  4. Sassi A, Louzir H, Ben Salah A, Mokni M, Ben Osman A, Dellagi K. Leishmanin skin test lymphoproliferative responses and cytokine production after symptomatic or asymptomatic Leishmania major infection in Tunisia. Clin Exp Immunol. 1999;116:127–132.
  5. Harizanov RN, Kaftandjiev IT, Jordanova DP, Marinova IB, Tsvetkova ND. Clinical features, diagnostic tools, and treatment regimens for visceral leishmaniasis in Bulgaria. Pathog Glob Health. 2013;107(5):260-266.
  6. Singh OP, Sundar S. Developments in Diagnosis of Visceral Leishmaniasis in the Elimination Era. J Parasitol Res. 2015;2015:239469.
  7. Gawade S, Nanaware M, Gokhale R, Adhav P. Visceral leishmaniasis: A case report. Australas Med J. 2012;5(2):130-134.
  8. Alvar J, Vélez ID, Bern C, et al. Leishmaniasis worldwide and global estimates of its incidence. PLoS ONE. 2012;7(5)

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Last updated: 2019-07-11 20:50