Visceral leishmaniasis: an unusual cervical presentation

( 1994)32.3X5--327 $5 1094

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Visceral leishmaniasis: an unusual cervical presentation A. M. Pcachcy, Departmslt

G. H. Irvine, H. White

of Oral and MaxilloJirciul

Surgery*, Southrnecld I-lospital, Bristol

SUMMA II Y. Visceral leishmaniasis is an infectious disease with a classical clinical presentation of fever, anaemia and splenomegaly forming the three cardinal signs. A rare presentation of the disease is described where cervical lymph node enlargement -was the only sign and there were no symptoms. It is emphasised that with extensive travel to and from areas of endemic leishmaniasis now established, it is important that clinicians and pathologists become acquaint4 with the characteristics of the disease.

histiocytes (Fig. I ). Subsequent serological tests for lcishmania proved positive. and a definitive diagnosis of leishmanial lymphadenitis was made. From the pathology and the geographic history, it was considered most likely that the infection was L. infantum acquired in the Mediterranean. The patient was referred to a consultant in infectious diseases who decided that active treatment was not indicated. The patient has been kept under close review. and to date, there is no further spread of the discasc.

Leishmaniasis is an infectious disease which is endemic in Asia, South America, Africa and the Mediterranean countries.’ Historically: it presents a significant diagnostic problem for clinicians when afiectcd travellers return home. This is compounded by the variable presentation and potentially very long incubation period: l--2 weeks to scvcral years.’ A case is described whcrc lcishmaniasis was unexpectedly found to be the cause of cervical lymphadcnopathy.

DISCUSSlON Lcishmania are dimorphic protozoa which reside within mammalian mononuclear phagocytes as aflagcllar amstigotes.3 In their arthropod vector. the sandlly, leishmania multiply as flagellated, extraccllular promastigotcs, which are innoculatcd into man when the sandlly attempts to take a blood meal (Fig. 2).

Case report A %-year

by her &encral dental neck swellings. A history revealed that whilst she had been born in Jamaica, she had

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in southern Spain for the past 5 years. She was in good health and had not experienced any symptoms sugg~‘stivc of systemic disease. Examination of the neck revealed enlarged, firm, rubbery, mobile lymph nodes in the submental and submandibular regions and the right posterior triangle. A full blood count reported occasional atypical lymphocytes but was otherwise normal. The provisional diagnosis was lymphoma and therefore several of the enlarged anterior cervical nodes were removed. Histopathological examination was suggcstivc of toxoplasma lymphadenitis. However. serological tests for toxoplasmosis, tuberculosis. mycoplasma and various viral infections all proved negative. Therefore, the histopathology was revicwcd and cat scratch fever was then considered to be the most likely diagnosis. The patient was kept under review and 6 weeks later, presented with an increased number of enlarged cervical lymph nodes. Clusters of enlarged nodes were again obvious in the submental and right submandibular region, and a single enlarged node was visible overlying the right trapezius. Extra-cervical nodes. liver and spleen WCJC once again. not enlarged. Further biopsies of the involved nodes wcrc taken. This time histopathological examination revealed the presence of irregularly distributed Leishman-Donovan bodies in

Fig. I - Leishman-Donovan bodies in histiocytes arrow (H&E Original magnification x 100). 325

indicated by

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of Oral and Maxillofacial +

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Depending on the immune response of the patient, the species of the protozoon and the geographical site, the disease may manifest as either cutaneous, mucocutaneous or visceral disease. Cutaneous lcishmaniasis manifests as ulcers on the skin while mucocutaneous leishmaniasis, found in South America: is associated not only with localized lesions of skin but also with secondary mctastatic ulcers in the mucosa of the upper respiratory tract.’ Visceral leishmaniasis is most commonly seen in children and is classically a chronic infectious disease caused by Leishmania donovani and characterised by irregular fever, enlargement of the spleen and liver, weight loss, anaemia, leukopacnia and hypergammaglobulinaemia.4 The disease is also known as kalaazar (Hindi for black sickness), Dumdum fever, and ponos (Greek for hurt). Although the diagnosis is not usually difficult in children, atypical presentations of visceral leishmaniasis affecting adults often result in a delay in diagnosis. As described in this article, adults with leishmaniasis may present with localised lymph node involvement in the absence of systemic illness. This is a very rare presentation and the literature reports only twelve cases of lymph node involvement where no other clinical manifestation of leishmaniasis was evident.‘.2*5*6 The clinical picture of leishmanial cervical lymphadenopathy is indistinguishable from many other conditions. A definitive diagnosis is reached by histopathological examination of a lymph node biopsy. Although there arc characteristic hacmatological and serological changes in visceral lcishmaniasis, there is no pathognomonic haematological or serological finding for other manifestations of leishmaniasis,

including leishmania lymphadenitis,’ and this may contribute further to a delay in diagnosis. The leishmanin skin test is negative in visceral leishmaniasis but positive in cutaneous and mucocutaneous leishmaniasis.’ Histologically. lcishmanial lymphadenitis closely resembles other granulomatous disease such as toxoplasmosis. histoplasmosis, tuberculosis and sarcoidosis. Diffcrcntiation between leishmania and histoplasma is particularly diEcult: the size and shape of leishmania and its nucleus are identical to the corresponding features of toxoplasma.’ Identification of leishmania is confirmed by the prcscnce of a rodshaped kinetoplast and a round, target-shaped basal body in the cytoplasm. Lymphadenopathy caused by leishmaniasis infection may resolve spontaneously.’ In cases where the disease shows signs of progressing the treatment of choice is pentavalent antimonial compounds such as sodium stibogluconate. Pentamidine and Amphotcricin B have also been used successfully on patients who have failed to respond to antimony compounds. The prognosis of leishmanial lymphadenopathy is very good. All reported cases either resolved spontaneously or responded to a course of antimony. Cryosurgery has been reported to be effective in some cases of cutaneous leishmaniasis.3 The case described is one of few reported cases of isolated cervical lcishmanial lymphadenitis, and we believe, the first of its kind to be reported as prcsenting in the UK. More generalised forms of visceral leishmaniasis have presented in this country, where the source of infection is thought to have been from the Mediterranean littoral.5*8.g~‘0 A dominant feature in the reported cases, is the difficulty and delay in diagnosis before the patient was referred to an appropriate consultant. Such case reports highlight the importance for both clinicians and pathologists to include leishmanial infection in the differential diagnosis in patients who have visited areas of endemic lcishmaniasis. This will avoid unnecessary invcstigations and ensure that the correct treatment is embarked upon as soon as possible. High lcvcls of foreign travel have already been established, with over 8 000000 visits in 1991 to the Mediterranean littoral alone” and the numbers are likely to continue to rise. Therefore, cases of leishmaniasis may present much more commonly as a diagnostic problem to clinicians who are not familiar with the disease. References I. Dancshbod K. Localised lymphadenitis due to leishmania simulaling toxoplasmosis. Am J Clin Pdthol 1978; 69: 462-467. 2. Fcncch FF. Visceral leishmaniasis in the Mediterranean. J Trop Med Hyg 1976; 79: 85-88. 3. Pearson RD: de Sousa A. Leishmaniasis in travellcrs. Tram Med Int 1985: 3: 2 -8. 4. Chulay JD, Mattson-Bahr PEC. Leishmaniasis. In: Strickland GT. (cd.) Hunter’s Tropical Medicine. 6th cd. Philadelphia: WR Saunders Company, 1984: 574-593. 5. Angevine DM. Hamilton TK, Wallace FG. Hazard JH. Lymph nodes in leishmaniasis. Am J Mcd Sci 1945: 2 IO: 33-38.

Visceral 6. Bell DW, Carmichael JAG, Williams RS. Holman RL, Stewart PD. Local&d leishmaniasis of lymph nodes: report of four casts. BMJ 1958: 740 743. 7. Maguirc JH, Gantz NM, Moschella S, Pan SC. Lcishmanial infections: a consideration in travellers returning from abroad. Am J Med Sci 1983; 285: 32-40. 8. Wheatley T. Sacks S. Flemans RJ, Rubcnstein D. Visceral Icishmamasis: a rare imported disease. J Infec 1983: 7: 166-167. 9. Khot AS. Thompson MH. Visceral leishmaniasis contracted in the Mediterranean area. Arch Dis Child 1983; 58: 930 I. 10. Galea P. Gael KM. Visceral leishmaniasis in a Scottish child. Arch Dis Child 1990: 65: 1269 70. 11. Visits abroad by UK residents. Depanmcnt of National Heritage, J.ondon: 1993.

lcishmaniasis:

an unusual

cervical

presentation

The Authors A. M. Peachq

BDS G. H. Irvine FRCS, FDSRCS H. White MRCPath, DMJ Department of Oral and Maxillofacial Southmead Hospital Westbury-on-Trym Bristol BSIO 5KB

Correspondence

Surgery

and request for ofTprints to Mr G. H. Irvine

Paper received I4 June 1993 Accepted 26 November 1993

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