Tohoku J. Exp. Med., 2021 February, 253(2)

Hemophagocytic Lymphohistiocytosis Associated with Disseminated Nontuberculous Mycobacterial Infection in a Patient with Mesenteric Panniculitis

Yuya Fujita,¹ Haruki Matsumoto,¹ Tomoyuki Asano,¹ Shuzo Sato,¹ Makiko Yashiro-Furuya,¹ Naoki Matsuoka,¹ Jumpei Temmoku,¹ Setsu Nakatani-Enomoto,² Satoshi Kimura,³ Kenta Hoshi,⁴ Manabu Uematsu,⁵ Hiroshi Watanabe¹ and Kiyoshi Migita¹

¹Department of Rheumatology, Fukushima Medical University School of Medicine, Fukushima, Fukushima, Japan
²Department of Neurology, Fukushima Medical University School of Medicine, Fukushima, Fukushima, Japan
³Department of Hematology, Fukushima Medical University School of Medicine, Fukushima, Fukushima, Japan
⁴Department of Internal Medicine, Takeda General Hospital, Aizuwakamatsu, Fukushima, Japan
⁵Department of Pulmonary Medicine, Fukushima Medical University Aizu Medical Center, Aizuwakamatsu, Fukushima, Japan

Mesenteric panniculitis is a chronic inflammatory disease characterized by non-specific inflammation of the adipose tissue in the mesentery. Hemophagocytic lymphohistiocytosis is a life-threating disease associated with aberrant macrophage overactivation, in which infections can be a leading cause in immunocompromised hosts. Here, we report a rare case of mesenteric panniculitis and hemophagocytic lymphohistiocytosis complicated by disseminated Mycobacterium intracellulare. A 71-year-old male with mesenteric panniculitis was admitted to our hospital for fever and pancytopenia. He was treated with oral prednisolone (15 mg/day) and cyclosporin A (150 mg/day) at presentation. Physical and laboratory examinations revealed disseminated infection with nontuberculous mycobacteria; Mycobacterium intracellulare was detected in cultures of cerebrospinal fluid, blood, sputum, and gastric fluid. Patient signs and symptoms fulfilled the five criteria for a diagnosis of hemophagocytic lymphohistiocytosis, including fever, cytopenia, hemophagocytosis, hyperferritinemia, and high soluble interleukin-2 receptor levels. Therefore, the diagnosis of nontuberculous mycobacteria-associated hemophagocytic lymphohistiocytosis was established. An anti-mycobacterial chemotherapy including chloramphenicol (800 mg/day), rifampin (450 mg/day) and ethambutol (750 mg/day) together with streptomycin (750 mg twice per week) was initiated at 30 days after admission; maintenance doses of prednisolone were increased to 60 mg/day. Fever and pancytopenia improved in response to anti-mycobacterial chemotherapy. The present case suggests that mesenteric panniculitis could be complicated with hemophagocytic lymphohistiocytosis caused by immunosuppressive therapy-associated infections as well as underlying disease activity. In conclusion, the possibility of disseminated nontuberculous mycobacteria infection with hemophagocytic lymphohistiocytosis should be considered if unexplained fever or hematological dyscrasia were presented in patients of mesenteric panniculitis.

Keywords —— disseminated NTM infection; fever of unknown origin; hemophagocytic lymphohistiocytosis; immunocompromised host; mesenteric panniculitis

© 2021 Tohoku University Medical Press

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Tohoku J. Exp. Med 2021 253, 253(2), 151-157.

Correspondence: Shuzo Sato, M.D., Ph.D., Department of Rheumatology, Fukushima Medical University School of Medicine, 1 Hikarigaoka, Fukushima, Fukushima 960-1295, Japan.

e-mail: shuzo@fmu.ac.jp