Background. Hemophagocytic lymphohistiocytosis (HLH) may precede malignancy, in particular lymphomas and leukemias. However, the causative factors, appropriate treatment and the prognosis of this association is not established. Case. Herein, we present two patients, one with nodular sclerosing Hodgkin lymphoma (HL) and concomitant Epstein-Barr virus (EBV) infection, and the other with anaplastic large cell lymphoma (ALCL), presented as malignancy associated HLH. Conclusion. In our patients, malignancy directed therapy was sufficient to treat HLH symptoms both at presentation and at recurrence in the second patient.
___
1. Shimada A, Kato M, Tamura K, et al. Hemophagocytic lymphohistiocytosis associated with uncontrolled inflammatory cytokinemia and chemokinemia was caused by systemic anaplastic large cell lymphoma: a case report and review of the literature. J Pediatr Hematol Oncol 2008; 30: 785-787.
2. Henter JI, Horne A, Arico M, et al. HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007; 48: 124-131.
3. Jordan MB, Allen CE, Weitzman S, Filipovich AH, Mcclain LK . How I treat hemophagocytic lymphohistiocytosis. Blood 2011; 118: 4041-4052.
4. Lehmberg K, Sprekels B, Nichols KE, et al. Malignancy-associated haemophagocytic lymphohistiocytosis in children and adolescents. Br J Haematol 2015; 170: 539-549.
5. Daver N, McClain K, Allen CE, et al. A consensus review on malignancy-associated hemophagocytic lymphohistiocytosis in adults. Cancer 2017; 123: 3229-3240.
6. Zhang K, Jordan MB, Marsh RA, et al. Hypomorphic mutations in PRF1 , MUNC13-4, and STXBP2 are associated with adult-onset familial HLH. Blood 2011; 118: 5794-5798.
7. Ciambotti B, Mussolin L, d'Amore ESG, et al. Monoallelic mutations of the perforin gene may represent a predisposing factor to childhood anaplastic large cell lymphoma. J Pediatr Hematol Oncol 2014; 36: e359-e365.
8. Strenger V, Merth G, Lackner H, et al. Malignancy and chemotherapy induced haemophagocytic lymphohistiocytosis in children and adolescents-a single centre experience of 20 years. Ann Hematol 2018; 97: 989-998.
9. Hagihara M, Inoue M, Hua J, Iwaki Y. Lymphocytedepleted Hodgkin lymphoma complicating hemophagocytic lymphohistiocytosis as an initial manifestation: a case report and review of the literature. Intern Med 2012; 51: 3067-3072.
10. Abaza H, Ben Chaabene A, Sallami S, Boussen H, Rahal K, Guemira F. Hemophagocytic syndrome associated to a Hodgkin lymphoma. Tunis Med 2015; 93: 399-400.
11. Chan K, Behling E, Strayer DS, Kocher WS, Dessain SK. Prolonged hemophagocytic lymphohistiocytosis syndrome as an initial presentation of Hodgkin lymphoma: a case report. J Med Case Rep 2008; 2: 367.
12. Dominguez-Munoz MA, Morales-Camacho RM, Prats-Martin C, et al. Unusual co-occurrence of Hodgkin lymphoma and hemophagocytic lymphohistiocytosis in a bone marrow aspirate. Ann Hematol 2016; 95: 1019-1021.
13. Ichikawa S, Takahashi T, Katsushima H, Fukuhara N, Ichinohasama R, Harigae H. Advanced lymphocyterich classical Hodgkin lymphoma complicated with fatal hemophagocytic syndrome. Intern Med 2016; 55: 191-196.
14. Menard F, Besson C, Rince P, et al. Hodgkin lymphoma-associated hemophagocytic syndrome: a disorder strongly correlated with Epstein-Barr virus. Clin Infect Dis 2008; 47: 531-534.
15. Morita Y, Kenzaka T, Yoshimoto H, Ohno N. Hodgkin's lymphoma preceded by haemophagocytic lymphohistiocytosis. BMJ Case Rep 2013; 2013.
16. Roe C, Bennett J, Zhang L, et al. Hemophagocytic lymphohistiocytosis in malignant hematology: uncommon but should not be forgotten? Clin Lymphoma Myeloma Leuk 2015; 15: S147-S150.
17. Tatara R, Sato M, Fujiwara SI, et al. Hemoperfusion for Hodgkin lymphoma-associated hemophagocytic lymphohistiocytosis. Intern Med 2014; 53: 2365-2368.
18. Celkan T, Berrak S, Kazanci E, et al. Malignancyassociated hemophagocytic lymphohistiocytosis in pediatric cases: a multicenter study from Turkey. Turk J Pediatr 2009; 51: 207-213.
19. Swerdlow SH, Campo E, Pileri SA, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood 2016; 127: 2375-2390.
20. Li W, Repnikova E. ALK-positive anaplastic largecell lymphoma with marked leukemoid reaction and hemophagocytic lymphohistiocytosis. Blood 2016; 127: 2041.
21. Al-Hashmi I, Decoteau J, Gruss HJ, et al. Establishment of a cytokine-producing anaplastic large-cell lymphoma cell line containing the t(2;5) translocation: potential role of cytokines in clinical manifestations. Leuk Lymphoma 2001; 40: 599-611.