Hemophagocytic Lymphohistiocytosis and CMV: Case Management

Cohen Ramon *

Internal Department B, Kaplan Medical Center, Hebrew University Medical School, Rehovot, Israel.

Jammal Saher

Internal Department B, Kaplan Medical Center, Hebrew University Medical School, Rehovot, Israel.

Attali Malka

Internal Department B, Kaplan Medical Center, Hebrew University Medical School, Rehovot, Israel.

*Author to whom correspondence should be addressed.


Abstract

Aim: Hemophagocytic Lymphohistiocytosis is often confused with a septic shock. Hemophagocytic Lymphohistiocytosis is a life-threatening condition in which the immune system overreacts. It causes the release of high levels of inflammatory cytokines, leading to fever, cytopenias, hyperferritinemia, and hypertriglyceridemia. HLH can be primary or secondary to an underlying condition such as infection, malignancy, or autoimmune disease.

Presentation: The article describes a case report of a 65-year-old woman who was admitted to the hospital with bloody diarrhea and a fever. The patient had a medical history of diffuse large B-cell lymphoma, coronary artery disease, heart failure, and diabetes mellitus. On admission, the patient had low hemoglobin, a low platelet count, and leukopenia. The laboratory investigations showed elevated AST and CRP levels, positive CMV PCR, and high levels of ferritin, fibrinogen, and soluble CD25. The patient was treated with dexamethasone and ganciclovir IV. A subsequent lymph node biopsy showed CMV-related lymphadenitis. The article also explain the pathophysiology of the disease and the possibilities of treatment.

Conclusion: Hemophagocytic Lymphohistiocytosis, which has the same symptoms of a septic shock, should be suspected if the patient does not respond quickly to treatment. Also, if Hemophagocytic Lymphohistiocytosis flares again, it seems not enough to give the same immunosuppressive treatment, but a stronger one is needed.

Keywords: Hemophagocytic lymphohistiocytosis, Septic shock, CMV, diffuse large B-cell lymphoma, HLH


How to Cite

Ramon , Cohen, Jammal Saher, and Attali Malka. 2023. “Hemophagocytic Lymphohistiocytosis and CMV: Case Management”. Asian Journal of Case Reports in Medicine and Health 6 (1):94-99. https://journalajcrmh.com/index.php/AJCRMH/article/view/162.

Downloads

Download data is not yet available.

References

Soy M, Atagündüz P, Atagündüz I, Sucak GT. Hemophagocytic lymphohistiocytosis: a review inspired by the COVID-19 pandemic. Rheumatol Int. 2021;41(1):7-18.

Crayne CB, Albeituni S, Nichols KE, Cron RQ. The immunology of macrophage activation syndrome. Front Immunol. 2019;10:119.

DOI: 10.3389/fimmu.2019.00119. PMID: 30774631; PMCID: PMC6367262.

Canna SW, Marsh RA. Pediatric hemophagocytic lymphohistiocytosis. Blood. 2020;135(16):1332-1343. DOI: 10.1182/blood.2019000936. PMID: 32107531; PMCID: PMC8212354.

Koumadoraki E, Madouros N, Sharif S, Saleem A, Jarvis S, Khan S. Hemophagocytic lymphohistiocytosis and infection: A literature review. Cureus. 2022;14(2):e22411.

Ramos-Casals M, Brito-Zerón P, López-Guillermo A, Khamashta MA, Bosch X. Adult haemophagocytic syndrome. Lancet. 2014;383(9927):1503-1516.

Fardet L, Galicier L, Lambotte O, Marzac C, Aumont C, Chahwan D, Coppo P, Hejblum G. Development and validation of the HScore, a score for the diagnosis of reactive hemophagocytic syndrome. Arthritis Rheumatol. 2014;66(9):2613-20

Urbanski G, Terriou L, Fouquet G, Hamel JF, Lavigne C, Orvain C, Larroche C, Hermine O, Bloch-Queyrat C, Lacout C. Factors associated with mortality at D30 in lymphohistiocytic hemophagocytosis in adults: construction of a mortality score from a multicenter cohort of 170 patients and validation on a cohort of 118 patients. The Journal of Internal Medicine. 2020;41:A69-A70.

Rolsdorph LÅ, Mosevoll KA, Helgeland L, Reikvam H. Concomitant hemophagocytic lymphohistiocytosis and cytomegalovirus disease: a case based systemic review. Front Med (Lausanne). 2022;9:819465. DOI: 10.3389/fmed.2022.819465. PMID: 35514747; PMCID: PMC9063453.

Chevalier K, Schmidt J, Coppo P, Galicier L, Noël N, Lambotte O. Hemophagocytic lymphohistiocytosis associated with cytomegalovirus infection: 5 cases and a systematic review of the literature. Clin Infect Dis. 2023;76(2):351-358. DOI: 10.1093/cid/ciac649. PMID: 35974465