Sequential therapy of targeting CD19 and CD22 chimeric antigen receptor T-cell following autologous stem cell transplantation for renal diffuse large B-cell lymphoma with central nervous system recurrence: report of 1 case and review of literature / 白血病·淋巴瘤

Objective:To investigate the efficacy and safety of sequential therapy of targeting CD19 and CD22 chimeric antigen receptor T-cell (CAR-T) following autologous stem cell transplantation (ASCT) in treatment of renal diffuse large B-cell lymphoma (DLBCL) with central nervous system (CNS) recurrence.Methods:The clinical data of 1 renal DLBCL patient with CNS recurrence admitted to Tongji Hospital, Tongji Medical College of Huazhong University of Science and Technology in May 2019 were retrospectively analyzed. The patient received sequential therapy of targeting CD19 and CD22 CAR-T following ASCT. The relative indicators of the primary disease remission at 1, 3, 6, 12, 18 and 24 months after therapy were analyzed, and relevant literature was reviewed.Results:The male patient aged 23 years had CNS recurrence after 8 courses of R-CHOP chemotherapy and then he received sequential therapy of targeting CD19 and CD22 CAR-T following ASCT. During the process of treatment, this patient developed grade 1 cytokine release syndrome and his condition was well controlled after active treatment. The white blood cell and platetes were successfully implanted. CNS symptoms along with immature cells in cerebrospinal fluid disappeared completely. Liquid biopsy was used to dynamically monitor the residue disease of the patient and the duration of remission period lasted 26 months. This patient developed tuberculosis one year after treatment and recovered from anti-tuberculosis agents.Conclusions:Sequential therapy of targeting CD19 and CD22 CAR-T following ASCT provides a novel therapeutic approach for renal DLBCL with CNS recurrence. Especially for patients who are neither sensitive to conventional chemotherapy nor CAR-T therapy alone, this regimen may improve remission rate and survival.

Toxic Optic Neuropathy Caused by Chlorfenapyr Poisoning

PURPOSE: To report a case of toxic optic neuropathy caused by chlorfenapyr ingestion accompanied by central nervous system involvement. CASE SUMMARY: A 44-year-old female visited our clinic complaining of reduced visual acuity in both eyes for 7 days. She had ingested a mouthful of chlorfenapyr for a suicide attempt 2 weeks prior to the visit. Gastric lavage was performed immediately after ingestion at the other hospital. Her best-corrected visual acuity was finger count 30 cm in the right eye and hand motion in the left eye. Both pupils were dilated by 5.0 mm and the response to light was sluggish in both eyes. A relative afferent pupillary defect was detected in her left eye. Funduscopy revealed optic disc swelling in both eyes. Magnetic resonance imaging of the brain showed a symmetric hyper-intense signal in the white matter tract including the internal capsule, corpus callosum, middle cerebellar peduncle, and brainstem. The patient was diagnosed with toxic optic neuropathy induced by chlorfenapyr ingestion, and underwent high-dose intravenous corticosteroid pulse therapy. Three days later, the best-corrected visual acuity was no light perception in both eyes. Three months later, optic atrophy was observed in both eyes. Optical coherence tomography revealed a reduction in the thicknesses of the retinal nerve fiber layer and ganglion cell and inner plexiform layer in the macular area. CONCLUSIONS: Ingestion of even a small amount of chlorfenapyr can cause severe optic nerve damage through the latent period, despite prompt lavage and high-dose steroid treatment.

A Case of Wegener's Granulomatosis with Central Nervous System Involvement Mimicking Lung Cancer with Brain Metastasis

Wegener's granulomatosis (WG) classically consists of necrotizing granulomatous inflammation of the upper and/or lower respiratory tract, necrotizing glomerulonephritis, and an autoimmune necrotizing systemic vasculitis affecting predominantly small vessels. We report a case of WG with central nervous system (CNS) involvement. WG is being diagnosed through pulmonary nodule biopsy. A small nodular lesion in the left posterior basal ganglia of brain being highly suspicious for granulomatosis was detected by MRI. After IV pulse cyclophosphamide and oral corticosteroid treatment for over 4 months, clinical manifestations and CNS lesions in brain MRI is improved. WG might have multiple granulomatous lesions which could be misdiagnosed due to malignancy. CNS involvement in WG is rare but careful evaluation is necessary when there are suspicious symptoms or lesions in CNS.

Central nervous system (CNS) involvement is a critical prognostic factor for hemophagocytic lymphohistiocytosis / 대한혈액학회지

BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare multisystem disorder that frequently involves the central nervous system (CNS). We compared the clinical characteristics, treatment, and prognosis of patients with HLH according to the degree of CNS involvement. METHODS: The clinical manifestations, initial laboratory data, treatment, and outcomes for 50 patients diagnosed with HLH and treated at Asan Medical Center between January 1995 and August 2011 were retrospectively reviewed and analyzed. CNS involvement was defined as the presence of neurological symptoms or an elevated white blood cell (WBC) count in the cerebrospinal fluid (CSF). RESULTS: Among these 50 patients, 23 (46%) developed CNS disease. Among patients with CNS disease, 19 had neurological symptoms, including seizures, altered consciousness, facial palsy, dysarthria, and dysphagia. Four patients had elevated CSF WBC counts without neurological symptoms. Twelve patients had abnormal brain imaging results, including high signal intensity lesions on T2-weighted magnetic resonance imaging (MRI) findings, ventriculomegaly, hemorrhage, atrophy, and leptomeningeal enhancement. Patients with CNS disease had lower ferritin, aspartate aminotransferase (AST), and alanine aminotransferase (ALT) levels as well as reduced 5-year survival as compared to those without CNS disease. CONCLUSION: CNS involvement is common among patients with HLH. Overall, patients with CNS disease achieve poorer outcomes than patients without CNS involvement. To improve outcomes, physicians must carefully monitor the neurological manifestations in patients with HLH and administer the appropriate course of intensified chemotherapy to patients with CNS disease.

Acute Promyelocytic Leukemia Presenting with Central Nervous System Involvement: A Report of 2 Cases / 대한진단검사의학회지

Central nervous system (CNS) involvement in acute promyelocytic leukemia (APL) is rare, and the presence of CNS symptoms at the time of diagnosis of APL is even rarer. We report 2 cases of APL presenting with CNS involvement. A 43-yr-old woman presented with easy bruising and stuporous mentality. Her complete blood count (CBC) revealed leukocytosis with increased blasts. Bone marrow (BM) analysis was carried out, and the diagnosis of APL was confirmed. This was done by cytogenetic analysis and demonstration of PML-RARalpha rearrangement by reverse transcriptase PCR in the BM cells. A lumbar puncture was performed to investigate the cause of her stuporous mentality, and her cerebrospinal fluid (CSF) analysis revealed 97% leukemic promyelocytes. Despite systemic and CNS therapy, she died due to septic shock by infection and rapid disease progression only 3 days after her admission. Another patient, a 3-yr-old girl, presented with easy bruising and epistaxis, and her CBC showed pancytopenia with increased blasts. BM studies confirmed APL. Quantitative PCR for PML-RARalpha in the BM cells revealed a PML-RARalpha/ABL ratio of 0.33 and CSF analysis revealed 9.5% leukemic promyelocytes (2 of 21 cells). She received induction chemotherapy and intrathecal therapy and achieved complete remission (CR) in the BM and CNS. She has been maintained in the CR status for the past 31 months. Thus, patients with APL must be evaluated for CNS involvement if any neurological symptoms are present at the time of diagnosis.

Characterization of rickettsia rickettsii in a case of Fatal Brazilian spotted fever in the city of Rio de Janeiro, Brazil

A lethal case of Brazilian spotted fever (BSF) is presented. Clinical features were initially of gastrointestinal involvement and evolved with progression to septic shock, meningoencephalitis and death on the 6th day of illness. Indirect immunofluorescence assay (IFA) for spotted fever group rickettsia (SFGR) was non-reactive. Diagnosis was confirmed by the polymerase chain reaction (PCR) and the nucleotide sequencing of a fragment of the ompA gene showed 100 percent homology to Rickettsia rickettsii. BSF has not been reported in the city of Rio de Janeiro in the last three decades, and the present description should alert the clinicians to its presence in urban Rio de Janeiro, and to the differential diagnosis with dengue fever, gastroenteritis, leptospirosis and bacterial septic shock, among others.

Central Nervous System Involvement in Eosinophilia-Myalgia Syndrome Associated with L-tryptophan Ingestion: A case report

We report a case of Eosinophilia- Myalgia syndrome with central nervous system (CNS) involvement following ingestion of the essential amino acid, tryptophan. The Eosinophilia- Myalgia syndrome can be diagnosed when there is a severe myalgia and peripheral eosinophilia in the absence of other diseases causing eosinophilia. This condition has been associated with the ingestion of the tryptophan in many cases, but the amount and duration of ingestion have been yet to be elucidated. Although many cases have been reported, few cases involved the CNS. We present a 28 year- old male who complained of a severe myalgia and disorientation after taking the taken tryptophan for 2 weeks. Thorough examination revealed the peripheral eosinophilia, vasculitis on brain MRI, abnormal VEP, and muscle atrophy on muscle biopsy. Symptoms improved with the steroid pulse after discontinuation of the tryptophan.