TY - JOUR
T1 - Herpes simplex infection simulating Richter transformation
T2 - a series of four cases and review of the literature
AU - Salem, Alireza
AU - Loghavi, Sanam
AU - Khoury, Joseph D.
AU - Agbay, Rose Lou Marie C.
AU - Jorgensen, Jeffrey L.
AU - Medeiros, L. Jeffrey
N1 - Publisher Copyright:
© 2016 John Wiley & Sons Ltd
PY - 2017/4/1
Y1 - 2017/4/1
N2 - Aims: Richter transformation (RT) occurs in 5–10% of patients with chronic lymphocytic leukaemia/small lymphocytic lymphoma (CLL/SLL), and is associated with a poor outcome. The histological manifestations of RT are variable, and include an increase in the proportion of prolymphocytes, immunoblasts, and other pleomorphic cells, along with an increased proliferation rate and often necrosis. Rarely, superimposed herpes simplex virus (HSV) infection involving lymph nodes in patients with CLL/SLL may mimic RT clinically and histologically. In this study, we describe four cases of CLL/SLL with superimposed HSV lymphadenitis, and we review the literature. Methods and results: We describe the clinicopathological and immunophenotypic findings of four cases of CLL/SLL with superimposed HSV infection. The patients presented with B-type symptoms, and laboratory and imaging findings suggested progression of lymphoma. Histologically, there were geographical areas of necrosis and increased numbers of prolymphocytes and immunoblasts, in some areas forming sheets, with increased mitotic figures and a high proliferation (Ki67) rate. However, the necrotic regions were associated with acute inflammatory cells and larger cells, mostly necrotic, showing viral cytopathic effects. Therapy with antiviral agents resulted in improvement of clinical symptoms and laboratory and imaging findings. Additionally, we identified 11 cases of CLL/SLL with superimposed HSV infection described in the English-language literature, and integrated the cases that we report here into this wider context. Conclusions: Herpes simplex virus infection of lymph nodes in patients with CLL/SLL results in clinicopathological and radiological findings that may mimic RT. It is essential to refrain from misclassifying these cases as RT, as patients can respond remarkably well to antiviral therapy.
AB - Aims: Richter transformation (RT) occurs in 5–10% of patients with chronic lymphocytic leukaemia/small lymphocytic lymphoma (CLL/SLL), and is associated with a poor outcome. The histological manifestations of RT are variable, and include an increase in the proportion of prolymphocytes, immunoblasts, and other pleomorphic cells, along with an increased proliferation rate and often necrosis. Rarely, superimposed herpes simplex virus (HSV) infection involving lymph nodes in patients with CLL/SLL may mimic RT clinically and histologically. In this study, we describe four cases of CLL/SLL with superimposed HSV lymphadenitis, and we review the literature. Methods and results: We describe the clinicopathological and immunophenotypic findings of four cases of CLL/SLL with superimposed HSV infection. The patients presented with B-type symptoms, and laboratory and imaging findings suggested progression of lymphoma. Histologically, there were geographical areas of necrosis and increased numbers of prolymphocytes and immunoblasts, in some areas forming sheets, with increased mitotic figures and a high proliferation (Ki67) rate. However, the necrotic regions were associated with acute inflammatory cells and larger cells, mostly necrotic, showing viral cytopathic effects. Therapy with antiviral agents resulted in improvement of clinical symptoms and laboratory and imaging findings. Additionally, we identified 11 cases of CLL/SLL with superimposed HSV infection described in the English-language literature, and integrated the cases that we report here into this wider context. Conclusions: Herpes simplex virus infection of lymph nodes in patients with CLL/SLL results in clinicopathological and radiological findings that may mimic RT. It is essential to refrain from misclassifying these cases as RT, as patients can respond remarkably well to antiviral therapy.
KW - CLL/SLL
KW - herpes
KW - Richter
KW - Transformation
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U2 - 10.1111/his.13137
DO - 10.1111/his.13137
M3 - Article
C2 - 27905140
AN - SCOPUS:85012107923
SN - 0309-0167
VL - 70
SP - 821
EP - 831
JO - Histopathology
JF - Histopathology
IS - 5
ER -