Varied spectrum of clinical presentation and mortality in a prospective registry of visceral leishmaniasis in a low endemicity area of Northern Italy

Abstract

Background: Visceral Leishmaniasis (VL) is endemic in 88 countries, in areas of relatively low incidence with a relevant proportion of immune suppressed patients clinical presentation, diagnosis and management may present difficulties and pitfalls.

Methods: Demographic data, clinical, laboratory features and therapeutic findings were recorded in patients identified by a regional VL disease registry from January 2007 to December 2010.

Results: A total of 55 patients (36 adults mean age 48.7 years, 19 children median age 37.5 months) were observed presenting with 65 episodes. All childen were immunocompetent, whereas adults affected by VL included both immunocompetent (n°17) and immunesuppressed (n°19) patients. The clinical presentation was homogeneous in children with predominance of fever and hepato-splenomegaly. A wider spectrum of clinical presentations was observed in immunocompromised adults. Bone marrow detection of intracellular parasites (Giemsa staining) and serology (IFAT) were the most frequently used diagnostic tools. In addition, detection of urinary antigen was used in adult patients with good specificity (90%). Liposomal amphotericin B was the most frequently prescribed first line drug (98.2% of cases) with 100% clinical cure. VL relapses (n°10) represented a crucial finding: they occurred only in adult patients, mainly in immunocompromised patients (40% of HIV, 22% of non-HIV immunocompromised patients, 5,9% of immunocompetent patients). Furthermore, three deaths with VL were reported, all occurring in relapsing immunocompromised patients accounting for a still high overall mortality in this group (15.8%).

Conclusions: The wide spectrum of clinical presentation in immunesuppresed patients and high recurrence rates still represent a clinical challenge accounting for high mortality. Early clinical identification and satisfactory treatment performance with liposomal amphotericin B are confirmed in areas with low-level endemicity and good clinical standards. VL needs continuing attention in endemic areas where increasing numbers of immunocompromised patients at risk are dwelling.

Figure 1

Map showing area of residency of patients in Liguria region and location within Italy and Europe. Signs show area of residency and hospital Infectious Diseases Unit where seen as inpatients. Star: children seen at Gaslini Hospital, Cross: patients seen at Sanremo Unit, Triangles: patients seen at Pietra Ligure Unit, Circles: patients seen at the Savona Unit, Greyed Squares: Patients seen at the Galliera Hospital Unit, Open Square: patients seen at the IRCCS S. Martino Hospital Unit.

Figure 2

Kaplan-Meyer analysis of Relapse-free time after the first Visceral Leishmaniasis event among observed patients. Panel A. Time to relapse (in days) in Immunocompetent(ICC)vs. Immunocompromised (ISS) pts. Log-rank test for equality of survivor functions (chi2 = 0.0002); Wilcoxon (Breslow) test for equality of survivor functions (Pr > chi2 =0.0004). Panel B. Time to relapse (in days) in Immunesuppresed pts.: HIV + vs. other causes (Others). Log-rank test for equality of survivor functions (chi2 = n.s.); Wilcoxon (Breslow) test for equality of survivor functions (Pr > chi2 = n.s.).