Tuberculosis-associated hemophagocytic lymphohistiocytosis with initial presentation of fever of unknown origin in a general hospital

Abstract

The study aimed to investigate the clinical features and prognoses of patients with tuberculosis (TB) who had secondary hemophagocytic lymphohistiocytosis (HLH).Patients first presenting with fever of unknown origin, who were ultimately diagnosed with TB-associated secondary HLH, were assessed retrospectively. We summarized and analyzed clinical manifestations, laboratory examinations, diagnoses, treatments, and prognoses of patients using clinical data, outpatient follow-up, and telephone follow-up in combination with literature review.Among patients admitted to the hospital with fever of unknown origin in the past 10 years, 371 patients were diagnosed with TB. Among them, 8 cases were diagnosed as tuberculosis-associated HLH (TB-HLH). The proportion of females among TB-HLH patients was higher than the proportion of females among TB patients. Within the same time period, 227 cases met the diagnostic criteria for HLH, among which TB-HLH patients accounted for 3.52% of the cases. None of the 8 TB-HLH patients had underlying diseases, and a majority of them had short symptom durations, rapid progression, along with multisystem and multiorgan dysfunctions. Their clinical manifestations were inconsistent with the typical clinical manifestations and imaging results characteristic of TB. Compared with patients with TB in our hospital during the same period, the 8 TB-HLH patients had a higher proportion of blood-disseminated TB and tuberculous meningitis. Apart from this, the hematological damage in these patients was higher than the common clinical manifestations of TB, and they also had a high proportion of respiratory failure. All 8 TB-HLH patients received antitubercular therapy, and 6 of them were also treated for HLH. However, their morbidity and mortality were significantly higher than that for reported cases of TB-HLH cases, both domestically and abroad, which may be attributed to the fever of unknown origin.Patients with TB-HLH had poor prognoses and no specific clinical manifestations. Therefore, cases of atypical TB and severe TB should be carefully monitored to achieve early diagnosis and early intervention.

Hemophagocytic lymphohistiocytosis secondary to infectious diseases

“…It was a rare disease, but a potentially fatal disorder, unless diagnosis and appropriate therapy were promptly instituted. It affects more frequently children than adults (8), which was concordant with results noted in our study. In Japan, a national survey reported that pediatric and adult annual incidence was 1 per 800000 (9).…”

A Case Report of Hemophagocytic Lymphohistiocytosis Secondary to Disseminated Tuberculosis

“…5 On the contrary, the combination of antitubercular therapy (ATT) with immunotherapy has been found to significantly reduce mortality. 1 Our patient was initiated on a modified regimen of ATT as soon as the diagnosis of disseminated TB was made, but she still passed several days later. This case highlights the need for ongoing investigation for early detection and management of secondary HLH in the adult setting.…”

“…Although HLH secondary to TB (TB-HLH) has previously been reported in the medical literature, it remains a rare entity. 1 Most patients with TB-HLH had short symptom duration and rapid progression leading to multi-organ dysfunction and ultimately, death.…”

Hemophagocytic lymphohistiocytosis and miliary tuberculosis in a previously healthy individual: a case report

“…Phagocytosis of M. tuberculosis by macrophages, and hence Th1-mediated cytotoxicity, followed by release of a large quantity of cytokines and chemokines are probably involved [ 11 ]. HLH due to TB has a high mortality rate, and most reports describe fatal outcome, particularly if other complicating factors are present [ 11 , 26 , 27 ]. The treatment of HLH is challenging, and simultaneous immunosuppressive treatment for HLH combined with specific treatment of the underlying condition is necessary [ 16 ].…”