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

Abstract

Background Hemophagocytic lymphohistiocytosis (HLH) is a rare heterogenous genetic or acquired hyperinflammatory syndrome associated with a high degree of morbidity and mortality. HLH has clinical manifestations related to abnormal prolonged activation of T lymphocytes and macrophages with an excess of proinflammatory cytokines. The main causes of secondary HLH are malignancies and infectious diseases. Case presentation The patient was a 54-year-old man, originally from Eastern Africa, who had lived in Northern Europe for 30 years. Here we describe the clinical features, laboratory parameters, diagnostic workup, management and outcome data of a previously healthy 54-year-old man diagnosed with HLH secondary to tuberculosis. The patient was initially treated for a community-acquired pneumonia. He developed multiorgan failure with acute respiratory distress syndrome, hypertransaminasemia, and kidney and bone marrow dysfunction. The clinical course together with a simultaneous increase in serum ferritin raised the suspicion of HLH. The patient fulfilled seven out of eight diagnostic criteria for HLH. A thorough diagnostic workup with respect to HLH and a potential underlying disease was initiated. Cultivation of bronchoalveolar lavage fluid, stool and urine, and polymerase chain reaction of epithelioid cell granulomas in the bone marrow were all positive for Mycobacterium tuberculosis. He was treated for both HLH and tuberculosis, and he survived without any sequelae. Conclusions We present one of few published cases of a patient who survived HLH triggered by miliary tuberculosis. The current case illustrates the need for awareness of these two diagnoses, and the timely initiation of specific and supportive treatment to reduce mortality.

Tuberculosis-Associated Hemophagocytic Lymphohistiocytosis: A Review of Current Literature

“…Even though cytopenias often were present at admission, several cases presented with normal blood counts, and developed cytopenias during disease. This course is exemplified in the case presented by Trovik et al [ 17 ], in which the patient had normal blood counts at admission, which developed into pancytopenia; other cases also exhibited this, developing either bicytopenia or pancytopenia [ 34 , 37 , 47 , 63 , 86 ]. The HLH-04 thresholds for anemia and thrombocytopenia were more commonly achieved in this patient population than neutropenia.…”

“…The number of criteria fulfilled by patients ranged from two to eight, with five being the most common. Table S3 provides an overview of each of the criteria for every patient in this study [ 3 , 4 , 5 , 6 , 7 , 10 , 13 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 , 49 , 50 , 51 , 52 , 53 , 54 , 55 , 56 , 57 , 58 , 59 , 60 , 61 , 62 , 63 , 64 , 65 ...…”

Disseminated tuberculosis complicated by hemophagocytic lymphohistiocytosis in an immunocompetent adult with favorable outcomes: A case report

“…Although MTB is present throughout the lungs in disseminated(or miliary) TB, acid-fast bacilli(AFB) could not be detected on sputum microscopy in several cases, as in this case. In our case, MTB was initially detected from PCR of peritoneal fluid, and then MTB was isolated from the cultures of different specimens (took up to 6 weeks) [11] .…”

Pediatric isoniazid-resistant tuberculosis of the bone marrow manifesting as hemophagocytic syndrome: A case report

“…Most of them were in critical situation at the time of HPS onset. Some patients required mechanical ventilation and extracorporeal membrane oxygenation (ECMO) support and prolonged hospital stay for survival (7)(8)(9)(10).…”