Scrub typhus associated with hemophagocytic lymphohistiocytosis: A report of six pediatric patients

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening immune disorder that may be inherited or secondary to infection, malignancy or rheumatological disease. The aim of the present study was to highlight the clinical features of scrub typhus-associated HLH in children. A retrospective study was performed on 6 pediatric patients with scrub typhus-associated HLH. For each patient, medical records were reviewed and analyzed, and demographic, clinical and laboratory data and outcomes were collected. The duration of fever prior to admission ranged between 4 and 12 days. All patients exhibited persistent or intermittent fever, eschar, hepatosplenomegaly and lymphadenopathy at the time of diagnosis. Five patients experienced acute respiratory distress syndrome and disseminated intravascular coagulation (DIC) during hospitalization. Thrombocytopenia was detected in all patients with cytopenia involving two or three cell types, simultaneously. Coagulopathy with prolonged prothrombin time and/or activated partial thromboplastin time were noted in all patients. Markedly elevated serum ferritin levels (>1,500 µg/ml) were identified in all patients. Elevated lactate dehydrogenase (>1,000 U/l) was detected in 4 (66.7%) patients and elevated alanine aminotransferase was exhibited by 5 (83/3%) patients. Lung infiltrates and consolidation were the most common imaging findings. Only 1 patient succumbed, with DIC and multi-organ failure. Of the survivors, 1 patient was lost to follow-up, and the remaining patients are in remission with excellent general health, to date. In conclusion, HLH should be considered in severe pediatric cases of scrub typhus. Upon the early recognition of this syndrome, prompt and supportive treatment in the pediatric intensive care unit are vital.

Clinical, immunological and genetic findings in patients with UNC13D deficiency (FHL3): A systematic review

“…Generally, viral infections may trigger primary and secondary forms of HLH; however, a viral infection cannot be used as the only criterion for distinguishing a viral‐associated HLH from familial HLH 31 . The most common bacterial and fungal pathogens associated with HLH are Rickettsia , 32 Mycobacterium , 33 and endemic dimorphic fungus Histoplasma , 34 respectively. In addition, the most common parasites implicated were Plasmodium and Leishmania 35,36 .…”

Predictors of Severity of Scrub Typhus in Children Requiring Pediatric Intensive Care Admission

“…23 Indian literature has reported quite a high rate of central nervous system involvement, 24,25 AKI, requirement of renal replacement therapy, 26 thrombocytopenia, disseminated intravascular coagulation, 15,27 and HLH. 28 We have found a significant association of altered sensorium and reduced urine output with the development of MODS during the course of illness. Infant age group was noted as significant risk factor for severe disease.…”

Hemophagocytic Lymphohistiocytosis Associated with Scrub Typhus: Systematic Review and Comparison between Pediatric and Adult Cases

“…A total of 18 articles describing 30 cases of HLH associated with scrub typhus were identified (age range: 2 months to 75 years; median age: 21.5 years), belonging to diverse nationalities, including India (10 cases, 33.3%), China (9 cases, 30%), Japan (6 cases, 20%), South Korea (2 cases, 6.7%), Taiwan (2 cases, 6.7%), and Sri Lanka (1 case, 3.3%) [ 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 ]. Timely diagnosis includes the search for an eschar, which was detected in 20 of the 30 cases (66.7%).…”