Miliary tuberculosis-associated hemophagocytic lymphohistiocytosis with a high level of soluble interleukin-2 receptor successfully treated with concomitant recombinant thrombomodulin: A case report |
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| Affiliation: | 1. Department of Hematology, Kawaguchi Municipal Medical Center, Saitama, Japan;2. Department of Nephrology, Kawaguchi Municipal Medical Center, Saitama, Japan;3. Department of Respiratory Medicine, Kawaguchi Municipal Medical Center, Saitama, Japan;4. Department of Pathology, Kawaguchi Municipal Medical Center, Saitama, Japan;1. Department of Infectious Diseases, Kobe City Medical Center General Hospital, 2-1-1, Minamimachi, Minatojima, Chuoku, Kobe, Hyogo, 650-0047, Japan;2. Division of Infectious Diseases, Kobe University Hospital, 7-5-2 Kusunokicho, Chuoku, Kobe, Hyogo, 650-0017, Japan;3. Department of Infectious Diseases, National Hospital Organization Kobe Medical Center, 3-1-1, Nishi-Ochiai, Sumaku, Kobe, Hyogo, 654-0155, Japan;4. Department of General Internal Medicine, Kobe City Medical Center West Hospital, 2-4 Ichiban-cho, Nagataku, Kobe, Hyogo, 653-0013, Japan;5. Department of Pediatrics, Kobe City Nishi-Kobe Medical Center, 5-7-1, Kojidai, Nishiku, Kobe, Hyogo, 651-2273, Japan;6. Emergency Department, Kobe City Medical Center General Hospital, 2-1-1, Minamimachi, Minatojima, Chuoku, Kobe, Hyogo, 650-0047, Japan;7. Kobe City Public Health Management Center, 6-5-1, Kanocho, Chuoku, Kobe, Hyogo, 650-8570, Japan;8. Kobe Institute of Health, 6-5, Nakamachi, Minatojima, Chuoku, Kobe, Hyogo, 650-0046, Japan;9. Department of Respiratory Medicine, Kobe City Medical Center General Hospital, 2-1-1, Minamimachi, Minatojima, Chuoku, Kobe, Hyogo, 650-0047, Japan;10. Kobe City Medical Center General Hospital, 2-1-1, Minamimachi, Minatojima, Chuoku, Kobe, Hyogo, 650-0047, Japan;1. Department of Surgery, Graduate School of Biomedical & Health Sciences, Hiroshima University, 1-2-3 Kasumi, Minami-Ku, Hiroshima City, Hiroshima Prefecture, 734-8551, Japan;2. Department of Infectious Diseases, Hiroshima University, 1-2-3 Kasumi, Minami-Ku, Hiroshima City, Hiroshima Prefecture, 734-8551, Japan;3. Department of Clinical Pharmacotherapy, Hiroshima University, 1-2-3 Kasumi, Minami-Ku, Hiroshima City, Hiroshima Prefecture, 734-8551, Japan;1. Department of Cardiology, National Center for Global Health and Medicine, Tokyo, Japan;2. AMR Clinical Reference Center, National Center for Global Health and Medicine, Tokyo, Japan;3. Disease Control and Prevention Center, National Center for Global Health and Medicine, Tokyo, Japan;4. Center for Clinical Sciences, National Center for Global Health and Medicine, Tokyo, Japan;5. Department of Infection Control and Prevention, Graduate School of Medicine, Faculty of Medicine, Osaka University, Osaka, Japan;1. Service de Microbiologie, Hôpital Européen Georges Pompidou, APHP-Centre, Université Paris Cité, Paris, France;2. Centre National de Référence des Rickettsia, Coxiella et Bartonella, IHU Mediterranée-Infection, Marseille, France |
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| Abstract: | Hemophagocytic lymphohistiocytosis (HLH) is a fatal disease characterized by a highly inflammatory state due to the abnormal activation of T lymphocytes and macrophages. Miliary tuberculosis (MTB) is a rare cause of HLH and its clinical appearances occasionally resembles that of intravascular lymphoma (IVL). A 76-year-old woman presented with persistent fever and fatigue. Abnormal laboratory findings showing thrombocytopenia (13,000/μL), hypofibrinogenemia (101 mg/dL), hyperferritinemia (2,312 ng/mL), and markedly elevated soluble interleukin-2 receptor (sIL-2R) level (32,200 U/mL), in addition, hemophagocytosis in the bone marrow (BM) smear, were suggestive of IVL-associated HLH. The pathology of the BM biopsy specimen showed granuloma with non-caseous necrosis, and culture tests using sputum, gastric fluid, urine, and peripheral and bone marrow blood revealed the presence of Mycobacterium tuberculosis, leading to the final diagnosis of MTB-associated HLH. Anti-TB medications and corticosteroids were administered, but thrombocytopenia, hypofibrinogenemia, and hyperferritinemia persisted. Concomitant use of recombinant thrombomodulin (rTM) enabled regression of clinical status. In this case, BM biopsy served as the diagnosis of MTB-associated HLH, although IVL-associated HLH is initially suspected by an extremely high level of sIL-2R. Furthermore, this case report informs that using rTM could improve the outcomes of MTB-associated HLH. |
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| Keywords: | Hemophagocytic lymphohistiocytosis Miliary tuberculosis Intravascular lymphoma Soluble interleukin-2 receptor Recombinant thrombomodulin |
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