DOI: 10.12890/2024_004847
Haemophagocytic lymphohistiocytosis triggered by cytomegalovirus reactivation in an immunosuppressed patient with pauci-immune glomerulonephritis
Authors
l_docquier@hotmail.com
Keywords
Haemophagocytic lymphohistiocytosis, cytomegalovirus, immunosuppressionAbstract
We report on a 67-year-old male patient admitted to the Internal Medicine department for fever, joint pain and exertional dyspnoea. Two months before his admission, the patient had been diagnosed with pauci-immune necrotising glomerulonephritis, for which he had been treated with rituximab and corticosteroids.
Upon admission the patient was stable, but within a few hours he became unstable as liver failure and acute cytopaenia occurred. Blood investigations revealed cytopaenia, altered coagulation tests, high ferritin, triglycerides, lactate dehydrogenase and C-reactive protein levels, and severe cytocholestasis. A liver echography was normal. The patient had been transferred to the intensive care unit to receive supportive support when the cytomegalovirus polymerase chain reaction (CMV-PCR) test came back positive. The diagnosis of haemophagocytic lymphohistiocytosis associated with a CMV infection and/or reactivation in an immunosuppressed patient was made. Specific treatment was administrated, along with symptomatic treatment. The patient clinically improved during hospitalisation with complete resolution of symptoms.
References
- Jordan MB, Allen CE, Greenberg J, Henry M, Hermiston ML, Kumar A, et al. Challenges in the diagnosis of hemophagocytic lymphohistiocytosis: Recommendations from the North American Consortium for Histiocytosis (NACHO). Pediatr Blood Cancer 2019;66:e27929.
- Ponnatt TS, Lilley CM, Mirza KM. Hemophagocytic lymphohistiocytosis. Arch Pathol Lab Med 2022;146:507–519.
- Rolsdorph LÅ, Mosevoll KA, Reikvam H. Cytomegalovirus induced hemophagocytic lymphohistiocytosis: diagnostic and treatment challenges for the future. Expert Rev Hematol 2022;15:667–670.
- Shakoory B, Geerlinks A, Wilejto M, Kernan K, Hines M, Romano M, et al. The 2022 EULAR/ACR points to consider at the early stages of diagnosis and management of suspected haemophagocytic lymphohistiocytosis/macrophage activation syndrome (HLH/MAS). Ann Rheum Dis 2023;82:1271–1285.
- Usmani GN, Woda BA, Newburger PE. Advances in understanding the pathogenesis of HLH. Br J Haematol 2013;161:609–622.
- Machaczka M, Vaktnäs J, Klimkowska M, Hägglund H. Malignancy-associated hemophagocytic lymphohistiocytosis in adults: a retrospective population-based analysis from a single center. Leuk Lymphoma 2011;52:613–619.
- Imashuku S, Hibi S, Sako M, Ishida Y, Mugishima H, Chen J, et al. Soluble interleukin-2 receptor: a useful prognostic factor for patients with hemophagocytic lymphohistiocytosis. Blood 1995;86:4706–4707.
