scholarly journals A Rare Presentation of Isolated CNS Posttransplantation Lymphoproliferative Disorder

2017 ◽  
Vol 2017 ◽  
pp. 1-5 ◽  
Author(s):  
Jaime Morris ◽  
Casey Smith ◽  
Andrew Streicher ◽  
Allison Magnuson ◽  
Susan Newman ◽  
...  

Posttransplantation lymphoproliferative disorder (PTLD) is a recognized and extremely morbid complication of solid organ transplantation, but central nervous system involvement, particularly in isolation, is rare. There are no standardized treatment strategies for PTLD, though commonly used strategies include reduction of immunosuppression, chemotherapy, rituximab, radiation, and surgery. We present a case of an unusual morphologic variant of primary central nervous system PTLD with successful response to rituximab and cranial radiation. A 69-year-old Asian male, who underwent postrenal transplant nine years earlier, presented with a one-month history of new onset seizure activity. His evaluation revealed multiple brain lesions on magnetic resonance imaging (MRI), as well as serologic and cerebrospinal fluid studies which were positive for Epstein-Barr Virus (EBV) infection. Ultimately, he underwent craniotomy with tissue biopsy with the final pathology report showing posttransplant lymphoproliferative disorder, polymorphic type. The patient was managed with reduction in immunosuppression, rituximab therapy, and cranial radiation treatments. He had demonstrated marked improvement in his neurologic function and was ultimately discharged to inpatient rehabilitation facility.

Neurosurgery ◽  
2013 ◽  
Vol 72 (6) ◽  
pp. 960-970 ◽  
Author(s):  
Wendell Lake ◽  
Julie E. Chang ◽  
Tabassum Kennedy ◽  
Adam Morgan ◽  
Shahriar Salamat ◽  
...  

Abstract BACKGROUND: Primary central nervous system posttransplantation lymphoproliferative disorder (PCNS-PTLD) is a rare complication after solid organ transplantation (SOT). With increasing rates of SOT, PCNS-PTLD incidence is increasing. OBJECTIVE: To describe the characteristics of PCNS-PTLD patients requiring neurosurgical intervention. METHODS: From 2000 to 2011, 10 patients with prior SOT underwent biopsy for evaluation of brain lesions and were diagnosed with PCNS-PTLD. Data collected included imaging characteristics, pathology, treatments administered, and survival outcomes. RESULTS: All patients had kidney transplantation, and 3 had concurrent pancreas transplantation. Median age at diagnosis was 49 years, with a median of 4.5 years from SOT to diagnosis (range, 1.8-11.4 years). Presenting symptoms most often included focal neurological deficits (n = 6), although several patients had nonspecific symptoms of headache and altered mental status. Brain lesions were generally multiple (n = 7), supratentorial (n = 8), and lobar or periventricular in distribution with ring enhancement. Diagnosis was established by stereotactic (n = 4) and open surgical (n = 6) biopsy. Treatments most frequently administered included reduction of immunosuppression (n = 10), dexamethasone (n = 10), rituximab (n = 8), high-dose methotrexate (n = 3), and whole-brain radiotherapy (n = 6). Six patients remain alive without PCNS-PTLD relapse, including 4 patients who have sustained remissions beyond 2 years from diagnosis of PCNS-PTLD. Of 4 observed deaths, 1 was related to progressive PCNS-PTLD. CONCLUSION: PCNS-PTLD must be considered in the differential diagnosis of any patient with prior SOT presenting with an intracranial lesion. Histological diagnosis with brain biopsy is imperative, given the risk for opportunistic infections that may have similar imaging findings and presentation. Prognosis is variable, although long-term survival has been reported.


2020 ◽  
Vol 12 (3) ◽  
pp. 402-409
Author(s):  
Ashraf Omer Elamin Ahmed ◽  
Mona Mohammad Ibraheem Babikir ◽  
Amir Elssoni Mahjoup Khojali ◽  
Suresh Nalaka Menik Arachchige ◽  
Abdirahman Mohamud Abdirahman ◽  
...  

<i>Mycobacteria pneumoniae</i> (MP) commonly causes upper and lower respiratory tract infections. The clinical manifestation is classified as pulmonary and extrapulmonary. These manifestations vary according to the involved system. MP may affect one system or more at a time. Commonly prodromal respiratory symptoms precede systemic involvement. Central nervous system involvement in uncommon. This report is presenting a rare case of central nervous system vasculitis secondary to MP, highlighting the diagnosis and management with a succinct literature review.


Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 4352-4352
Author(s):  
Qifa Liu ◽  
Meiqing Wu ◽  
Jing Sun ◽  
Yu Zhang ◽  
Fen Huang ◽  
...  

Abstract Backgroud: Although the introduction of rituximab as a first-line treatment has improved outcome of post-transplant lymphoproliferative disorder (PTLD), PTLD with central nervous system involvement (CNS-PTLD) still has a dismal prognosis because of low penetrance across the blood-brain barrier. In this prospective study, we reported intrathecal rituximab was efficacy and safety in the patients with CNS-PTLD who had failed to respond to the intravenous rituximab-based treatments. Methods: From June 2009 to November 2013, 32 cases of EBV-associated PTLD were recorded in the Southern Medical University Institute of Hematology. Fourteen patients diagnosed with CNS-PTLD were enrolled in this prospective study. For the patients who failed to response to the initial intravenous rituximab-based treatments, sequential dose-escalation schedule of intrathecal rituximab (initial dose of 20mg, increased by 10mg each week and maximum dose of 50 mg) was administrated weekly. Results: Three patients were responsive and 11 were unresponsive to the initial treatments within one week after the treatments. For the 11 patients who failed to respond to the initial treatments, 9 patients received intrathecal rituximab within 7-11 days and 2 patients refused the treatment. After two cycles of rituximab-based treatments, 10 patients achieved complete response (CR), 2 patients were partial response and 2 patients were non-response. With a median follow up of 664 (range 18 to 1545) days after the diagnosis of CNS-PTLD, 7 patients survived and 7 died. The causes of death included PTLD progressing (n=3), PTLD relapse (n=1), GVHD (n=1), CMV pneumonia (n=1) and pseudomonas aeruginosa sepsis (n=1). The 3-year probability of OS was 45.7% ±14.7% in CNS-PTLD, which was no significant difference as compared to PTLD without CNS involvement(55.6% ±11.7%, P=0.706). Conclusion: Intrathecal rituximab might be an effective and safe method for CNS-PTLD in the allo-HSCT recipients who were unresponsive to the intravenous rituximab-based treatments. Disclosures No relevant conflicts of interest to declare.


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