Case Presentation: A 71-year-old male from Vietnam with HTN and TIIDM presented to an outside hospital with several weeks of fatigue, lower extremity bruising, and an unintentional weight loss of 20lbs. On admission to Stanford, he also endorsed right lower extremity numbness and weakness with right foot dorsiflexion. His exam was notable for scattered ecchymosis to his extremities and 2/5 right foot dorsiflexion strength. His labs were notable for hemoglobin 8.1, platelets 3.1, ferritin 15.8K, triglycerides 190, fibrinogen 344, and elevated liver associated enzymes in a hepatocellular pattern. A CT chest/abdomen/pelvis demonstrated mediastinal lymphadenopathy (LAD) and splenomegaly. A bone marrow biopsy (BMbx) revealed hemophagocytosis, H score was 210 and hematology was consulted to initiate empiric hemophagocytic lymphohistiocytosis (HLH) treatment with dexamethasone and anakinra. He underwent an extensive work-up for an underlying driver of HLH. A PET/CT showed enlarged intensely hypermetabolic LAD, hepatosplenomegaly with moderate uptake, diffuse bone marrow uptake, and a 1 cm ground glass nodule in left upper lobe. His nutritional work-up was unremarkable. MRI right knee identified common peroneal nerve neuropathy which was attributed to trauma from a prior fall. The LP was negative for infection or malignancy. BMbx was negative for malignancy but did show numerous non-necrotizing granulomas. QuantiFERON gold later returned positive with subsequently positive serum MTB PCR and sputum AFB despite no reports of cough or shortness of breath. Given these findings, he was diagnosed with disseminated tuberculosis (TB) and TB-associated HLH and started on RIPE therapy in addition to continuing anakinra.
Discussion: This case illustrates the importance of maintaining a broad differential diagnosis for drivers of HLH that includes TB, especially in patients from endemic areas. TB-HLH is associated with high mortality and an early diagnosis can improve outcomes [1]. Therefore, BMbx should be considered in HLH work-up including microbiological testing for Mycobacterium tuberculosis. Lastly, clinicians should maintain a high level of suspicion for pulmonary TB when lung findings are present even in patients without respiratory symptoms.
Conclusions: Hospitalists should recognize extrapulmonary manifestations of TB and consider it as a driver of HLH in certain populations.