Lymphocyte-depleted Hodgkin Lymphoma Complicating Hemophagocytic Lymphohistiocytosis as an Initial Manifestation: A Case Report and Review of the Literature

Abstract

Hemophagocytic lymphohistocytosis (HLH) is commonly associated with infectious diseases or T/NK celllymphoma; however that with Hodgkin lymphoma (HL) was rarely reported. Herein, we describe a young male diagnosed with lymphocyte-depleted HL (LD-HL) complicated by HLH as an initial manifestation. He was given high-dose steroid therapy plus recombinant thrombomodulin, and subsequent ABVd (doxorubicin, bleomycin, vinblastine, dacarbazine) treatment. In spite of the achievement of a partial remission treated with one cycle of ABVd, he relapsed after 3 cycles. To our knowledge, the present case is very rare, and more intensive treatment might be needed for the long-term control of HLH-complicated HL.

Hemoperfusion for Hodgkin Lymphoma-associated Hemophagocytic Lymphohistiocytosis

“…Those reports revealed that the mixed cellularity type and lymphocytedepleted type are two major types of Hodgkin lymphoma that accompany HLH. In addition, most cases showed Epstein-Barr virus to be present in the Hodgkin cells or Reed-Sternberg cells (4,5,10). Consistent with these findings, the pathological diagnosis of our patient was the mixed cellularity type in which Reed-Sternberg cells were positive for EBER1 staining.…”

“…Hodgkin lymphoma-associated HLH is rare with only a limited number of reported cases (3)(4)(5). Those reports revealed that the mixed cellularity type and lymphocytedepleted type are two major types of Hodgkin lymphoma that accompany HLH.…”

“…However, if such treatments show little or no effect on HLH, immediate mitigation of the cytokine storm is vital to save patients. In fact, patients with Hodgkin lymphoma and HLH have often shown poor prognosis due to the rapid progression of HLH even after chemotherapy (4,5).…”

Diffuse large b-cell lymphoma with hemophagocytic syndrome in an HIV+ patient treated with CCR5- bone marrow transplant

“…Although T cells lack CD21 receptor for the EBV entrance into the cells, EBV genome was present in T cell lymphomas and T lymphocytes from patients with HLH [12,13]. EBV driven T cell and NK-cell lymphomas are among the most common lymphoid neoplasms associated with the development of HLH [14]. On the other hand, B cell lymphomas are rarely associated with HLH development.…”

Successful treatment of two cases of classical Hodgkin lymphoma-associated hemophagocytic lymphohistiocyosis with R-CEPP

“…Furthermore, the use of ABVD for cHL-associated HLH is associated with poor outcomes – in four reported cases, one recovered, one relapsed, and two died. [8–10,12] Therefore, the optimal treatment approach for cHL-associated HLH is not known. Notably, CEPP has been shown to be a well-tolerated and effective therapy in non-Hodgkin lymphoma and cHL, even in the setting of marked liver and renal dysfunction.…”