Hemophagocytic lymphohistiocytosis in imported pediatric visceral leishmaniasis in a nonendemic area
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Hemophagocytic lymphohistiocytosis in imported pediatric visceral leishmaniasis in a nonendemic area. / Bode, Sebastian F N; Bogdan, Christian; Beutel, Karin; Behnisch, Wolfgang; Greiner, Jeanette; Henning, Stephan; Jorch, Norbert; Jankofsky, Martin; Jakob, Marcus; Schmid, Irene; Veelken, Norbert; Vraetz, Thomas; Janka, Gritta; Ehl, Stephan; Lehmberg, Kai.
In: J PEDIATR-US, Vol. 165, No. 1, 01.07.2014, p. 147-153.e1.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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TY - JOUR
T1 - Hemophagocytic lymphohistiocytosis in imported pediatric visceral leishmaniasis in a nonendemic area
AU - Bode, Sebastian F N
AU - Bogdan, Christian
AU - Beutel, Karin
AU - Behnisch, Wolfgang
AU - Greiner, Jeanette
AU - Henning, Stephan
AU - Jorch, Norbert
AU - Jankofsky, Martin
AU - Jakob, Marcus
AU - Schmid, Irene
AU - Veelken, Norbert
AU - Vraetz, Thomas
AU - Janka, Gritta
AU - Ehl, Stephan
AU - Lehmberg, Kai
N1 - Copyright © 2014 Elsevier Inc. All rights reserved.
PY - 2014/7/1
Y1 - 2014/7/1
N2 - OBJECTIVES: To describe characteristics of visceral leishmaniasis-associated hemophagocytic lymphohistiocytosis (HLH) with focus on diagnostic clues and pitfalls, including the frequency of central nervous system (CNS) involvement, and to determine the efficacy of liposomal amphotericin B (L-AmB).STUDY DESIGN: We retrospectively analyzed clinical and laboratory features, diagnostic procedures, and treatment of 13 patients with HLH with imported visceral leishmaniasis, reported to the German HLH reference center (1999-2012).RESULTS: The spectrum of presentations was indistinguishable from patients with hereditary HLH or with acquired HLH because of infections with other pathogens. In 8 patients, disease onset occurred before the age of 2 years, coinciding with the typical age of manifestation of primary HLH. Two patients had mild nonspecific CNS findings. Misleading antiviral IgM (n = 6) and autoantibodies (n = 2) led to inaccurate interpretation of the etiology of HLH, sometimes with inappropriate therapeutic consequences. False negative results for Leishmania were obtained by initial bone marrow microscopy in 6/13, serology in 1/12, bone marrow culture in 2/5, and polymerase chain reaction of peripheral blood in 1/3 patients, and all bone marrow samples tested were Leishmania-positive by polymerase chain reaction (n = 7). L-AmB was administered to 12 patients, 5 of whom had no prior HLH-directed immunosuppressive therapy; sodium stibogluconate was administered to 1 patient. Persistent remission was achieved in 11 cases. Two patients required repeated or prolonged L-AmB therapy.CONCLUSIONS: Awareness of diagnostic pitfalls may save patients from unnecessary toxic treatment. CNS involvement is rare. L-AmB shows efficacy in visceral leishmaniasis-associated HLH.
AB - OBJECTIVES: To describe characteristics of visceral leishmaniasis-associated hemophagocytic lymphohistiocytosis (HLH) with focus on diagnostic clues and pitfalls, including the frequency of central nervous system (CNS) involvement, and to determine the efficacy of liposomal amphotericin B (L-AmB).STUDY DESIGN: We retrospectively analyzed clinical and laboratory features, diagnostic procedures, and treatment of 13 patients with HLH with imported visceral leishmaniasis, reported to the German HLH reference center (1999-2012).RESULTS: The spectrum of presentations was indistinguishable from patients with hereditary HLH or with acquired HLH because of infections with other pathogens. In 8 patients, disease onset occurred before the age of 2 years, coinciding with the typical age of manifestation of primary HLH. Two patients had mild nonspecific CNS findings. Misleading antiviral IgM (n = 6) and autoantibodies (n = 2) led to inaccurate interpretation of the etiology of HLH, sometimes with inappropriate therapeutic consequences. False negative results for Leishmania were obtained by initial bone marrow microscopy in 6/13, serology in 1/12, bone marrow culture in 2/5, and polymerase chain reaction of peripheral blood in 1/3 patients, and all bone marrow samples tested were Leishmania-positive by polymerase chain reaction (n = 7). L-AmB was administered to 12 patients, 5 of whom had no prior HLH-directed immunosuppressive therapy; sodium stibogluconate was administered to 1 patient. Persistent remission was achieved in 11 cases. Two patients required repeated or prolonged L-AmB therapy.CONCLUSIONS: Awareness of diagnostic pitfalls may save patients from unnecessary toxic treatment. CNS involvement is rare. L-AmB shows efficacy in visceral leishmaniasis-associated HLH.
KW - Amphotericin B
KW - Antibodies, Protozoan
KW - Antiprotozoal Agents
KW - Autoantibodies
KW - Bone Marrow
KW - Child
KW - Child, Preschool
KW - DNA, Protozoan
KW - Female
KW - Humans
KW - Infant
KW - Leishmania donovani
KW - Leishmaniasis, Visceral
KW - Lymphohistiocytosis, Hemophagocytic
KW - Male
KW - Polymerase Chain Reaction
KW - Retrospective Studies
KW - Treatment Outcome
U2 - 10.1016/j.jpeds.2014.03.047
DO - 10.1016/j.jpeds.2014.03.047
M3 - SCORING: Journal article
C2 - 24797953
VL - 165
SP - 147-153.e1
JO - J PEDIATR-US
JF - J PEDIATR-US
SN - 0022-3476
IS - 1
ER -
