Haemophagocytic lymphohistiocytosis as a consequence of untreated B-cell chronic lymphocytic leukaemia

Abstract

Haemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening syndrome characterised by defective cytotoxic function and hypercytokinaemia leading to macrophage expansion and haemophagocytosis. Patients often present with unexplained fevers, hepatosplenomegaly and pancytopenia, with elevation in serum ferritin and triglyceride. Acquired forms are triggered by infection, malignancy or rheumatological disorders. HLH in the setting of chronic lymphocytic leukaemia is rarely reported, however, and is usually associated with infection or as a consequence of chemotherapy. We present a case of HLH in a 64-year-old Caucasian woman with the only identified trigger being her hitherto untreated CLL.

A rare presentation of hemophagocytic lymphohistiocytosis in a patient with untreated chronic lymphocytic leukemia: A case report and review of the literature

“…In the case of untreated CLL where an infectious cause was ruled out, the progression of CLL itself should be implicated as the trigger for HLH. To our knowledge, there has been only four cases reported to date of HLH secondary to CLL progression in addition to our case [5,13,16,21].…”

“…However, the association between HLH and B-cell leukemia has been rarely associated ( Table 1). It is important to note that in the majority of these cases, an infectious trigger was detected [10][11][12]14,17,18,[20][21][22]. It can be therefore postulated that in the setting of a dysfunctional immune system secondary to malignancy, infections are more likely to become overwhelming and act as a trigger for HLH.…”

Ibrutinib‐associated hemophagocytic lymphohistiocytosis: A case series from Johns Hopkins

“…9,10 There are only two cases describing HLH arising out of CLL without another trigger. 11,12 In our own HLH series, 13 there were only two other cases of CLL-associated HLH, one occurring after bone marrow transplantation, and another in the setting of transformation. In the three cases described in this manuscript, extensive testing for autoimmune and infectious etiologies was negative.…”

Clinical Warning of Hemophagocytic Syndrome Caused by Epstein-barr Virus

“…During EBV infection, monocytes, macrophages, and endothelial cells not only produce in ammatory factors, but also express tissue factor and initiate exogenous coagulation reactions, thereby consuming a large amount of coagulation factors and leading to brinolytic hyperactivity and decreased coagulation factor synthesis. The levels of Scr, BUN, UA, TG, and EBV DNA load in children with EBV-HLH were positively correlated, indicating that in ammatory factors affected liver synthesis and secretion as well as kidney function and directly inhibited the level of lipoprotein lipase, causing an increase of triglyceride [16] . In ammatory factors can invade all organs of the body.…”