Lymphoma-associated hemophagocytic syndrome: clinical features and treatment outcome

Abstract

The clinical features and prognostic factor of lymphoma-associated hemophagocytic syndrome (LAHS), diagnosed according to World Health Organization classification, were investigated by reviewing the clinical records of 29 patients between September 1994 and September 2006. Compared with patients with T or natural killer (NK)/T cell LAHS, patients with B cell LAHS were older (p = 0.022), were less likely to exhibit disseminated intravascular coagulation (DIC; p = 0.011), and had less direct involvement of bone marrow (p = 0.03). Clinical response was achieved in 15 (65.2%) and complete remission (CR) was achieved in 4 (17%) of 23 patients who received chemotherapy. Four patients received high-dose chemotherapy and autologous stem cell transplantation (A-SCT), and three of these four patients showed CR. The median survival was 36 days (95%CI, 20.2-51.8). Univariate analysis showed that poor performance status (p = 0.028), T or NK/T cell lymphoma (p = 0.016), presence of jaundice (p = 0.063), the presence of DIC (p = 0.002), and poor clinical response to treatment (p < 0.001) predicted poor overall survival. These data suggest that the clinical features differ significantly between B cell LAHS and T or NK/T cell LAHS. Intensive treatment including high-dose chemotherapy and A-SCT should be investigated.

Hemophagocyte Lymphohistiocytosis Secondary to Bilateral Epididymal Diffuse Large B-Cell Lymphoma

“…Machaczka [15] examined eight patients and found that only one patient with HLH-04 who was treated with an allogeneic stem cell transplant survived. In another article by Tong [2], long-term survival was achieved in four out of 29 patients, three of which received autologous stem cell transplants. The present HLH patient relapsed during the fourth week of treatment after decreasing the etoposide and immunosuppressive regimen, which suggested that regimen like HLH-04 alone is not sufficient to control lymphoma.…”

“…The prognosis of HLH secondary to lymphoma is unfavorable, and the median survival time is less than 40 days [2,10]. Importantly, survival has been shown to be dependent on the type of lymphoma.…”

“…The acquired forms of HLH have been associated with infections, inflammatory diseases and malignancies. Lymphoma is the primary malignancy-related cause of HLH [2,3]; however, HLH secondary to lymphoma in a sanctuary site, such as the central nervous system, testis or epididymides, has not been reported. The present study reports a rare case of HLH secondary to bilateral epididymal lymphoma.…”

Elements of confusion

“…[2][3][4][5] The central pathogenesis involves dysregulated Th1 cytokine secretion. This results in an uncontrolled accumulation of activated T-lymphocytes and histiocytes in various organs including the liver, spleen and bone marrow.…”

“…6 Viruses are the most common infectious triggers of HLH, particularly EBV, and lymphoma is the most common associated malignancy. [2][3][4][5] It is hypothesized that EBV can interfere with normal lymphocyte signaling pathways leading to the aforementioned over-expression of Th1 cytokines, which can then trigger HLH. 7 The diagnosis of HLH is based on a combination of clinical and laboratory parameters as outlined in Table 1.…”