Japanese Spotted Fever with Hemophagocytic Lymphohistiocytosis

Abstract

Japanese spotted fever (JSF) is an uncommon but potentially fatal infection transmitted by tick bites. We herein report a fulminant case of JSF infection that occurred in an immunocompetent adult that was complicated by disseminated intravascular coagulation and hemophagocytic lymphohistiocytosis (HLH). We discuss the difficulty in making the diagnosis and identifying the complication of HLH in our patient. HLH is a rare complication of rickettsiosis, and this is the first reported case in English of JSF complicated by HLH in an immunocompetent adult. Secondary HLH caused by rickettsiosis requires a different treatment from primary HLH. Rickettsiosis must therefore be considered in patients with HLH.

Secondary hemophagocytic lymphohistiocytosis associated with Rocky Mountain spotted fever in a toddler: A case report

“…steroids and chemotherapy (etoposide, and/or cyclosporine), and treatment was unknown in three cases. Twenty-three cases survived and four cases died [15]. HLH secondary to infection has a favorable outcome provided the infection is appropriately treated in a timely fashion.…”

“…Twelve of these cases were treated with rickettsia specific antibiotics, two cases received antibiotics with immunoglobulin (IVIG), eight were given antibiotics with systemic steroids with/without IVIG, two were given antibiotics with systemic steroids and chemotherapy (etoposide, and/or cyclosporine), and treatment was unknown in three cases. Twenty‐three cases survived and four cases died [ 15 ]. HLH secondary to infection has a favorable outcome provided the infection is appropriately treated in a timely fashion.…”

A Case of Hemophagocytic Lymphohistiocytosis Associated With Mediterranean Spotted Fever in a Healthy 29-Year-Old Female

“…Other pathogens from the Rickettsia family have also been associated with HLH ( Table 2 ) [ 25 ]. In fact, HLH has been primarily reported with scrub typhus and ehrlichiosis and to a lesser extent with Rickettsia conorii and Rickettsia Japonica ( Table 2 ) [ 26 , 27 ]. A recent comprehensive review on scrub typhus–associated HLH identified 30 cases, all in the Far East [ 28 ].…”

“…Cohen et al reported a case of ISF complicated by DIC and purpura fulminans where the patient eventually died [ 16 ]. Similarly, a case of JSF complicated by HLH and DIC was fatal despite maximal treatment [ 26 ]. In contrast, a good outcome was achieved here.…”

Fiebre de las montañas rocosas con linfohistiocitosis hemofagocítica en paciente de terapia intensiva pediátrica: reporte de caso

“…El menor llega con fiebre de seis días de evolución y cuatro días de erupción maculopapular generalizada (figura 1); a su ingreso integra choque séptico, acidosis metabólica e hiperlactatemia, hepatomegalia, hipoalbuminemia, transaminacemia, trombocitopenia, bandemia, hiperferritinemia, hipertrigliceridemia e hiponatremia. Por todo lo anterior, el infante inicia tratamiento con doxiciclina (considerado el tratamiento de elección) 5 y soporte hemodinámico con aminas vasoactivas e inotrópicas. Al octavo día de inicio de cuadro clínico el menor presenta deterioro neurológico, por lo que es intubado como medida de protección de vía aérea.…”