1. Leishmaniasis
Thomas Fourie MD M. Taher Shabani-Rad MD CLS, Division of Hematopathology

2. Leishmaniasis
HLH
Transfusion
Chagas Disease

3. What is Leishmaniasis?
Leishmaniasis is caused by infection with a protozoan parasite that is spread by the bite of infected sandflies
Protozoans are historically defined as a single-celled organisms with animal-like behaviour, such as motility and predation (attacking)

4. Two forms of Leishmaniasis
Amastigote of Leishmania

5. The life cycle of Leishmania

6. Sand fly
At least 93 sandfly species are proven or probable vectors worldwide.

7. World distribution of Visceral Leishmaniasis
Canada is not highlighted on leishmana map and No leishmaniasis had been previously reported in Alberta

8. What is HLH? Hemophagocytic LymphoHistiocytosis Immunologic disorder
Over production and –activation of T cells and histiocytes
Phagocytosis of normal hematopoietic cells by histiocytes

10. Primary/inherited HLH
Rare – 1 case per million children
Primary HLH is caused by an inherited problem with the immune system
5 genes have been identified, including the perforin gene
Onset of the disease occurs in first year of life in 70% of cases

11. Secondary/reactive HLH
No family history
No genetic abnormalities
Triggers:
Infection - Viral, Bacterial, Protozoan, Fungi
Malignancy – T cell lymphoma
Auto-immune - SLE

12. Visceral Leishmaniasis HLH
Enlarged liver or spleen
Fever
Anemia
HLH
Enlarged liver or spleen
Fever
Cytopenias

13. Visceral Leishmaniasis (kala-azar) HLH
Mortality rate of more than 90% within 2 years if left untreated
Antibiotics including Amphotericin B
HLH
Mortality rate of 100% over the course of 2 months if left untreated
Cytotoxic chemotherapy to supress the immune system

14. Case
2 year old child Diagnosed with HLH Bone marrow: Routine pre-BMT Assessment for Cord Stem Cell Transplant for HLH

16. It is difficult to differentiate leishmania bodies from platelet and cellular debris (Double dot nucleus)

18. Presence of leishman bodies in BM H&E sections are very diagnostic of BM leishmaniosis
(Platelets are not seen in BM biopsy sections)

19. BM performed 6 months earlier

20. Canadian born child
No sandfly of the type that transmit Leishmaniasis in North America
Leishmaniasis does not exist in Alberta
History of blood transfusions
Biting midge, Sand fly or Punkies

21. Could this have been transmitted by a blood transfusion?
Cryptic infection in blood donors from endemic areas
Cases of transfusion transmitted Leishmaniasis have been reported in non-endemic areas
Most of these cases involved infants or children
Yes

22. CBS screening for Leishmaniasis
Ask donors if they ever had Leishmaniasis
If yes – deferred indefinitely
Lab test?
Not really

23. Chagas disease (Trypanosomiasis)
Also transmitted by an insect
Mexico, Central- and South America

24. Chagas disease Donors from endemic areas are tested
Abbott Chagas antibody test
Some cross reactivity with Leishmaniasis
Not reliable for exclusion of Leishmaniasis

25. What was the source of infection?
Travel history
Family visited Albania after the child's birth
Albania is endemic for Leishmaniasis
Mediterranean Visceral Leishmaniasis affects primarily children or immunocompromised individuals
It is known to occur since 1938, typically as a childhood disease in Albania
Developing country with suboptimal disease specific surveillance
Newborns can be infected, even with asymptomatic mother

26. Conclusion (1) HLH can occur in cases with Leishmaniasias
Leishmaniasis can be difficult to diagnose if you are not looking for it
Leishmania parasites can be mistaken for phagocytosed platelets or cellular debris

27. Conclusion (2)
Leishmaniasis can be transmitted by transfused blood of asymptomatic, infected individuals
Very rare - only 11 reports of transfusion transmitted Leishmaniasis worldwide
All reports were from Asia and Europe
9 of the 11 cases occurred in children less than 6 years of age

28. Conclusion (3)
Chagas antibody test can be “false” positive in a patient with Leishmaniasis
PCR technology can be used for mass screening of donor blood samples for Leishmaniasis
In Canada the cost outweigh the benefit
Currently donors are deferred only if they where diagnosed with Leishmaniasis
Transmission of Leishmaniasis by an asymptomatic individual is a possibility, but very unlikely