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.
Highlights
Iatrogenic, immunodeficiency-associated lymphoproliferative disorders (IA-LPDs) are defined as lymphoid proliferations or lymphomas, arising in patients after long-term treatment with immunosuppressive drugs for an underlying autoimmune disease or other disease in the non-transplant setting [1]
We present a case of iatrogenic, immunodeficiency-associated, EBV-positive polymorphic LPD with morphology mimicking Hodgkin lymphoma in the CNS after long-term treatment with both methotrexate and infliximab (TNF inhibitor)
We describe an unusual case of immunodeficiencyassociated polymorphic B-cell LPD with cells mimicking Hodgkin cells which presented as a solitary lesion in the brain without evidence of systemic disease
Summary
Iatrogenic, immunodeficiency-associated lymphoproliferative disorders (IA-LPDs) are defined as lymphoid proliferations or lymphomas, arising in patients after long-term treatment with immunosuppressive drugs for an underlying autoimmune disease or other (non-hematological) disease in the non-transplant setting [1]. We present a case of iatrogenic, immunodeficiency-associated, EBV-positive polymorphic LPD with morphology mimicking Hodgkin lymphoma in the CNS after long-term treatment with both methotrexate and infliximab (TNF inhibitor). A 75-year-old woman presented to the emergency department with acute onset of hemiparesis, impaired consciousness, and facial droop Her past medical history included a diagnosis of neurosarcoidosis and long-term immunosuppressive treatment with methotrexate and infliximab (TNF inhibitor) for at least 6 years. Histological examination revealed a sharply demarcated, EBV-positive polymorphic infiltrate with Hodgkinlike cells, consistent with a diagnosis of iatrogenic, immunodeficiency associated lymphoproliferative disorder (IA-LPD). An extensive staging work-up, including ultrasound and CT scan of the chest and abdomen, revealed no lymphadenopathy or other sign of systemic disease
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