Secondary Hemophagocytic Lymphohistocytosis in COVID-19

  • Stephanie Lilia Pushpam Jetty Department of Pathology Father Muller Medical College Kankanady, Mangalore, Karnataka, India, 575002
  • Dotton Denis Noronha Department of Critical care & Internal medicine A.J. Hospital & Research Centre Kuntikana, Mangalore, Karnataka, India, 575004
  • Akshatha Nayak Department of Hematology A.J. Hospital & Research Centre Kuntikana, Mangalore, Karnataka, India, 575004
  • Altin Dsouza Department of Respiratory Therapy A.J. Hospital & Research Centre Kuntikana, Mangalore, Karnataka, India, 575004
Keywords: Hemophagocytic lymphohistiocytosis, COVID-19, Severe Acute Respiratory Infection

Abstract

 We present case of a 53-year-old lady who is a known case of rheumatoid arthritis and hypertension in Karnataka, South India, who presented with Severe Acute Respiratory Infection (SARI) like symptoms  (fever, cough and breathlessness) due to COVID-19. During the course of treatment further investigations revealed evidence of Hemophagocytic Lymphohistocytosis (HLH). This case emphasizes the need for a thorough workup to identify the etiology of HLH as it may unmask a treatable entity in a COVID-19 patient.

References

Atteritano, M., David, A., Bagnato, G., Beninati, C., Frisina, A., Iaria, C. & Cascio, A. (2012) Haemophagocytic syndrome in rheumatic patients. A systematic review. European Review for Medical and Pharmacological Sciences, 16, 1414– 24.

Janka, G.E. & Lehmberg, K. (2014) Hemophagocytic syndromes ‐ An update. Blood Reviews, 28, 135– 142.

Ramos‐Casals, M., Brito‐Zerón, P., López‐Guillermo, A., Khamashta, M.A. & Bosch, X. (2014) Adult haemophagocytic syndrome. Lancet, 383, 1503– 16.

Kasahara, Y., Yachie, A., Takei, K., Kanegane, C., Okada, K., Ohta, K., Seki, H., Igarashi, N., Maruhashi, K., Katayama, K., Katoh, E., Terao, G., Sakiyama, Y. & Koizumi, S. (2001) Differential cellular targets of Epstein‐Barr virus (EBV) infection between acute EBV‐associated hemophagocytic lymphohistiocytosis and chronic active EBV infection. Blood, 98, 1882– 1888.

Rouphael, N.G., Talati, N.J., Vaughan, C., Cunningham, K., Moreira, R. & Gould, C. (2007) Infections associated with haemophagocytic syndrome. The Lancet. Infectious diseases, 7, 814– 822.

George, M.R. (2014) Hemophagocytic lymphohistiocytosis: review of etiologies and management. Journal of Blood Medicine, 5, 69– 86.

Atteritano, M., David, A., Bagnato, G., Beninati, C., Frisina, A., Iaria, C. & Cascio, A. (2012) Haemophagocytic syndrome in rheumatic patients. A systematic review. European Review for Medical and Pharmacological Sciences, 16, 1414– 24.

Zhang, M., Behrens, E.M., Atkinson, T.P., Shakoory, B., Grom, A.A. & Cron, R.Q. (2014b) Genetic defects in cytolysis in macrophage activation syndrome. Current Rheumatology Reports, 16, 439– 446.

Grom, A., Villanueva, J., Lee, S., Goldmuntz, E., Passo, M. & Filipovich, A.H. (2003) Natural killer cell dysfunction in patients with systemic‐onset rheumatoid arthritis and macrophage activation syndrome. Journal of Pediatrics, 29, 2– 296.

Ramanan, A.V. & Schneider, R. (2003) Macrophage activation syndrome–what's in a name!. The Journal of Rheumatology, 30, 2513– 6.

Grom, A.A. (2004) Natural killer cell dysfunction: a common pathway in systemic‐onset juvenile rheumatoid arthritis, macrophage activation syndrome, and hemophagocytic lymphohistiocytosis? Arthritis and Rheumatism, 50, 689– 98.

Bryceson, Y.T., Pende, D., Maul‐Pavicic, A., Gilmour, K.C., Ufheil, H., Vraetz, T., Chiang, S.C., Marcenaro, S., Meazza, R., Bondzio, I., Walshe, D., Janka, G., Lehmberg, K., Beutel, K., zur Stadt, U., Binder, N., Arico, M., Moretta, L., Henter, J.I. & Ehl, S. (2012) A prospective evaluation of degranulation assays in the rapid diagnosis of familial hemophagocytic syndromes. Blood, 119, 2754– 2763.

Avau, A., Put, K., Wouters, C.H. & Matthys, P. (2015) Cytokine balance and cytokine‐driven natural killer cell dysfunction in systemic juvenile idiopathic arthritis. Cytokine & Growth Factor Reviews, 26, 35– 45.

Canna, S.W. & Goldbach‐Mansky, R. (2015) New monogenetic autoinflammatory diseases—a clinical overview. Seminars in Immunopathology, 37, 387– 394.

Published
2021-02-28
Section
Case Report