Intracerebral manifestation of iatrogenic, immunodeficiency-associated polymorphic B-LPD with morphology mimicking Hodgkin lymphoma: a case report and literature review

Abstract

Iatrogenic immunodeficiency-associated lymphoproliferative disorders (IA-LPD) may arise in patients treated with immunosuppressive drugs for autoimmune disease or other conditions. Polymorphic EBV-positive B-lymphoproliferations often have features mimicking Hodgkin lymphoma and typically a self-limited, indolent course. We present an unusual case with isolated, intracerebral manifestation of polymorphic B-LPD with features of classic Hodgkin-lymphoma in an immunosuppressed patient treated with methotrexate and infliximab, including clinical-radiological features and a detailed description of morphological findings, together with a literature review on reported cases of primary CNS manifestation of cHL and IA-LPD with Hodgkin-like morphology. The patient achieved complete remission following neurosurgery with gross total tumor resection and drug withdrawal without any additional treatment. Post-operative staging revealed no evidence for focal relapse or systemic disease during the 18 months follow-up period. Among the previously reported 24 cases of primary, isolated Hodgkin lymphoma in the central nervous system, three similar cases of iatrogenic, IA-LPDs were identified and are discussed here. Polymorphic B-LPD are destructive lesions with a range of morphologic features and disease manifestations. It is clinically important to recognize the spectrum of proliferations with features of classic Hodgkin lymphoma in immunodeficiency, iatrogenic settings, because they are likely to impact the choice of treatment strategies.

Supplementary information: The online version contains supplementary material available at 10.1007/s12308-021-00478-0.

Keywords: CNS; EBV; Hodgkin-like cells; Iatrogenic; Lymphoproliferative disorder; Polymorphic B-LPD.

Figure 1.

T1-weighted magnetic resonance image shows a left-sided, well-circumscribed contrast-enhancing lesion measuring 2 cm in diameter (a). T2-weighted FLAIR image (fluid attenuated inversion recovery) reveals marked surrounding edema (b)

Figure 2.

Histological examination demonstrates a well-demarcated, intracerebral polymorphic infiltrate with small lymphocytes, numerous plasma cells, eosinophilic granulocytes, and scattered large cells with Hodgkin/Reed-Sternberg morphology (a,b). The atypical cells stain positive for CD30 (c), negative for CD15 (d) and CD20 (e), CD79a weakly positive (f), and positive for both LMP-1 (g) and EBER/EBV (h)