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. 2022 Feb 17;37(6):1542–1546. doi: 10.1007/s11606-022-07395-7

Table 2.

Triggers for HLH in CLL Patients

Predisposing trigger Case report/series N of CLL patients The exact trigger Conclusions
Disease transformation (Picque et al., 2014)19 1 Transformation to PTCL under CLL treatment Immune dysregulation may explain HLH and composite lymphomas and a high level of transformation suspicion should be kept in these cases
(Ambinder et al., 2019)27 1 A spontaneous transformation to HSTCL
Ibrutinib treatment (Ambinder et al., 2019)27 4 In four of the cases, there is a clear temporal relationship between the initiation of ibrutinib and the onset of HLH. Ibrutinib may rarely contribute to the development of HLH
(Cavallari et al., 2017)31 1 Ibrutinib and EBV reactivation Ibrutinib-induced impairment of NK degranulation, associated with EBV reactivation and CLL-related immunodeficiency may contribute to HLH development
Untreated CLL (Bailey et al., 2017)28 1 No identified trigger Untreated CLL may be a trigger for HLH
(Kilari et al., 2013)29 1
(Meki et al., 2011)32 1
EBV (Lim et al., 2014)22 1 Cellulitis, EBV reactivation EBV reactivation may trigger HLH in stable, low-risk, and untreated CLL
CMV (Bergmann et al., 2018)23 1 CMV that developed under chemotherapy CMV can trigger HLH in treated and untreated patients
(Broe et al., 2014)21 1 CMV
Histoplasma (van Koeveringe et al., 2010)24 1 Histoplasma Histoplasma reactivation can trigger HLH in a non-endemic area in an immunocompromised host
(Rao et al., 2002)25 1 Histoplasma
H1N1 influenza (Lai et al., 2012)26 1 H1N1 causing CLL reactivation H1N1 influenza A-associated hemophagocytic lymphohistiocytosis is often rapidly fatal

CLL chronic lymphocytic leukemia; EBV Epstein Barr virus; CMV cytomegalovirus; PTCL peripheral T cell lymphoma; HSTCL hepatosplenic T cell lymphoma