Rasmussen’s syndrome is a neuroimmune disease characterized by progressive unilateral cerebral dysfunction, for which hemispherectomy remains the definitive treatment. We report a 9-year-old right-handed Filipino girl with drug-resistant focal seizures, epilepsia partialis continua, and progressive right hemiparesis due to left hemispheric involvement. Epilepsy surgery was not immediately feasible because of parental concerns and logistical constraints in this resource-limited setting. Despite treatment with multiple antiseizure medications, seizures remained uncontrolled, prompting initiation of immunotherapy with intravenous immunoglobulin followed by rituximab. Rituximab was associated with marked seizure reduction and functional improvement, highlighting its potential role in the management of Rasmussen’s syndrome when surgery is not feasible.
Human ; Female ; Child: 6-12 Yrs Old ; Epilepsia Partialis Continua ; Immunoglobulins, Intravenous ; Rituximab

OBJECTIVES: To investigate the clinical efficacy and safety of low-dose rituximab (RTX) (<375 mg/m²) maintenance therapy in children with primary nephrotic syndrome (PNS). METHODS: A retrospective analysis was conducted on the clinical data of PNS children who received low-dose RTX therapy at the Department of Renal Immunology, Children's Hospital of Soochow University from July 2016 to March 2024. Remission rate, recurrence frequency, corticosteroid and tacrolimus usage, and adverse reactions before and after RTX treatment were analyzed. RESULTS: Compared with before treatment, low-dose RTX maintained remission in PNS, reduced the relapse frequency, and decreased the dosage of corticosteroids and tacrolimus (P<0.05). IgG levels did not significantly decrease, and no additional preventive anti-infective treatment was required. CONCLUSIONS Low-dose RTX therapy is effective and safe for treating PNS in children.
Humans ; Nephrotic Syndrome/drug therapy* ; Rituximab/adverse effects* ; Male ; Female ; Child ; Retrospective Studies ; Child, Preschool ; Adolescent ; Infant

OBJECTIVE: To study the clinical effects of ibrutinib in the treatment of relapsed/refractory diffuse large B-cell lymphoma (RRDLBCL). METHODS: A total of 101 patients with RRDLBCL in Daqing People's Hospital from September 2019 to September 2022 were selected. 45 patients were received ibrutinib monotherapy, 36 patients were received a combination therapy of ibrutinib, rituximab, and lenalidomide, and 20 patients were received a combination therapy of ibrutinib and lenalidomide. The clinical effects were observed. RESULTS: The median duration of treatment for all patients was 4 (2-9) months. The disease control rates(DCR) and objective response rates(ORR) in the ibrutinib monotherapy group were 46.67% and 26.67%, respectively. In the combination therapy group of ibrutinib, rituximab, and lenalidomide, the DCR and ORR were 69.44% and 44.44%, respectively. In the combination therapy group of ibrutinib and lenalidomide, the DCR and ORR were 60.00% and 35.00%, respectively. The DCR and ORR in the combination therapy group of ibrutinib, rituximab, and lenalidomide were significantly higher than those in the ibrutinib monotherapy group (P < 0.05). There were no significant differences in DCR and ORR between the combination therapy group of ibrutinib and lenalidomide and the ibrutinib monotherapy group (P >0.05). The median follow-up time of all patients was 15 (5-35) months, with a median overall survival(OS) of 21.0 (15.8-26.2) months and a median progression-free survival(PFS) of 14.0 (12.1-15.9) months. In the ibrutinib monotherapy group, the median OS and PFS were 15.0 (12.1-17.9) months and 12.0 (11.0-13.0) months, respectively. In the combination therapy group of ibrutinib and lenalidomide, the median OS and PFS were 22.0 (13.3-30.7) months and 16.0 (14.1-19.7) months, respectively. In the combination therapy group of ibrutinib, rituximab, and lenalidomide, the median OS and PFS were 23.0 (19.7-26.3) months and 17.0 (14.8-19.1) months, respectively. The median OS and PFS in the combination therapy group of ibrutinib, rituximab, and lenalidomide were significantly higher than those in the ibrutinib monotherapy group (P < 0.05). There were no significant differences in median OS and PFS between the combination therapy group of ibrutinib and lenalidomide and the combination therapy group of ibrutinib, rituximab, and lenalidomide (P >0.05). Hematological adverse reactions included neutropenia in 14 cases (13.86%), thrombocytopenia in 16 cases (15.84%), and leukopenia in 13 cases (12.87%). Non-hematological adverse reactions mainly included nausea and vomiting in 33 cases (32.67%) and fatigue in 44 cases (43.56%). CONCLUSION Ibrutinib has certain clinical effects and good safety in the treatment of RRDLBCL.
Humans ; Piperidines/therapeutic use* ; Lymphoma, Large B-Cell, Diffuse/drug therapy* ; Adenine/therapeutic use* ; Rituximab/therapeutic use* ; Lenalidomide/therapeutic use* ; Male ; Female ; Middle Aged ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use* ; Adult ; Aged ; Pyrimidines/therapeutic use* ; Pyrazoles/therapeutic use* ; Treatment Outcome

Herpes zoster is a clinical syndrome associated with reactivation of varicella zoster virus (VZV), often occurring years after VZV infection, and characterized typically by painful grouped vesicles in a dermatomal distribution. Bullous herpes zoster, an atypical presentation of herpes zoster, is a relatively rare phenomenon; to the authors’ knowledge, there have only been eight reports in worldwide literature. We present a case of a 59-year-old female with lupus nephritis who presented with multiple grouped vesicles evolving into large tender bullae filled with serosanguinous fluid on the lateral aspect of the right leg, and dorsal and medial aspects of the right foot, four days after the first dose of 1g of rituximab therapy. The diagnosis of bullous herpes zoster along L4-L5 dermatomes was made based on the clinical presentation and the presence of multinucleated giant cells on Tzanck smear. The giant bullae were drained and dressed, and the patient was treated with valacyclovir at the renally adjusted dose of 1g once a day for seven days and pregabalin 150 mg once daily. After seven days of antiviral treatment, there were no new bullae or vesicles, and the pain improved. Recognizing this atypical presentation of a common disease, especially in patients with an immunocompromised state, highlights the importance of prompt recognition and treatment.
Human ; herpes zoster ; lupus nephritis ; rituximab ; diagnosis, differential

Pemphigus is a rare, chronic, life-threatening autoimmune blistering disease characterized by blisters and erosions of the skin and mucous membranes. Rituximab has been approved as a first-line treatment for moderate to severe pemphigus vulgaris. Despite of its efficacy in achieving remission, Rituximab can also lead to serious complications such as Hepatitis B reactivation.

We present a case of a 42-year-old Filipino male with severe pemphigus vulgaris on chronic immunosuppressive therapy. He had a 10-month history of multiple bullae and crusted erosions associated with pruritus and burning pain on the mouth resulting to odynophagia and dysphagia. He is a known case of chronic Hepatitis B with unrecalled vaccination status. On physical examination, Nikolsky and Asboe-Hansen signs were positive. Histopathology show intraepidermal split and row of suprabasal keratinocytes pattern. ELISA showed very high levels (>200 RU/ml) for both anti-desmoglein 1 & 3. DIF was positive for IgG & C3. Prior to Rituximab administration, Tenofovir 300 mg/tab/day was started as pre-emptive therapy. To lessen the dependence on systemic corticosteroids, two infusions of Rituximab 1g 2 weeks apart were then given. Notable improvement was seen, evidenced by absence of new bullae, reduction of affected BSA, from 19% to 5.3% and decreased PDAI (78 to 1) and ABSIS (46.5 to 2.75) four months after treatment. Maintained remission and undetectable Hepatitis B viral load 4 months following the last dose of Rituximab were noted, indicating a positive treatment response to both Rituximab and Tenofovir.

Rituximab represents a viable treatment option even for patients with chronic Hepatitis B. Pre-emptive therapy may be done prior to infusion to prevent hepatitis reactivation. Clinical evidence supports the efficacy and safety of Rituximab in this case where preventive measures are taken.

Humans ; Hemolysis ; Lymphoma, Large B-Cell, Diffuse/complications* ; Rituximab ; Pancreatic Neoplasms/complications*

Objective: To analyze the detection rate, clinical significance, and prognosis of Epstein-Barr virus (EBV) in the cerebrospinal fluid (CSF) of patients following allogeneic hematopoietic stem cell transplantation. Methods: A retrospective analysis was performed on 1100 patients who underwent the CSF virus test after allogeneic hematopoietic stem cell transplantation in Peking University People's Hospital between January 2017 and June 2022. Among them, 19 patients were screened positive for EBV in their CSF, and their clinical characteristics, treatment, and prognosis were analyzed. Results: Among 19 patients with EBV-positive cerebrospinal fluid, 12 were male and 7 were female, with 5 patients aged <18 years and 12 aged ≥18 years, with a median age of 27 (5-58) years old. There were 7 cases of acute myeloid leukemia, 8 of acute lymphocytic leukemia, 2 of aplastic anemia, 1 of Hodgkin's lymphoma, and 1 of hemophagocytic syndrome. All 19 patients underwent haploid hematopoietic stem cell transplantation, including 1 secondary transplant. Nineteen patients had neurological symptoms (headache, dizziness, convulsions, or seizures), of which 13 had fever. Ten cases showed no abnormalities in cranial imaging examination. Among the 19 patients, 6 were diagnosed with EB virus-related central nervous system diseases, with a median diagnosis time of 50 (22-363) days after transplantation. In 9 (47.3%) patients, EBV was detected in their peripheral blood, and they were treated with intravenous infusion of rituximab (including two patients who underwent lumbar puncture and intrathecal injection of rituximab). After treatment, EBV was not detected in seven patients. Among the 19 patients, 2 died from EBV infection and 2 from other causes. Conclusion: In patients who exhibited central nervous system symptoms after allogeneic hematopoietic stem cell transplantation, EBV should be screened as a potential pathogen. EBV detected in the CSF may indicate an infection; however, it does not confirm the diagnosis.
Humans ; Male ; Female ; Adolescent ; Adult ; Middle Aged ; Herpesvirus 4, Human ; Epstein-Barr Virus Infections/complications* ; Rituximab/therapeutic use* ; Retrospective Studies ; Clinical Relevance ; Hematopoietic Stem Cell Transplantation/adverse effects* ; Lymphoproliferative Disorders/drug therapy*

OBJECTIVES: To study the efficacy and safety of repeated application of rituximab (RTX) at a low dose (200 mg/m²) versus the recommended dose (375 mg/m²) for remission maintenance in frequently relapsing nephrotic syndrome (FRNS) or steroid-dependent nephrotic syndrome (SDNS). METHODS: A randomized controlled trial was conducted for 29 children with FRNS/SDNS who received systemic treatment in the Department of Nephrology, Anhui Provincial Children's Hospital, from September 2020 to December 2021. These children were divided into a recommended dose group (n=14) and a low dose group (n=15) using a random number table. The two groups were compared in terms of general characteristics, changes in CD19 expression after RTX treatment, number of relapses, glucocorticoid dose, adverse reactions of RTX, and hospital costs. RESULTS: After RTX treatment, both the low dose group and the recommended dose group achieved B-lymphocyte depletion and had significant reductions in the number of relapses and glucocorticoid dose (P<0.05). The low dose group had a comparable clinical effect to the recommended dose group after RTX treatment (P>0.05), and the low dose group had a significant reduction in hospital costs for the second, third, and fourth times of hospitalization (P<0.05). There were no serious adverse reactions in either group during RTX treatment and late follow-up, and there was no significant difference in adverse reactions between the two groups (P>0.05). CONCLUSIONS Repeated RTX treatment at a low dose has comparable clinical efficacy and safety to that at the recommended dose and can significantly reduce the number of FRNS/SDNS relapses and the amount of glucocorticoids used, with little adverse effect throughout the treatment cycle. Therefore, it holds promise for clinical application.
Humans ; Child ; Nephrotic Syndrome/drug therapy* ; Rituximab/adverse effects* ; Glucocorticoids/adverse effects* ; Prospective Studies ; Adaptor Proteins, Signal Transducing

OBJECTIVE: To analyze the clinical characteristics of the patients with B-cell chronic lymphoproliferative disease（B-CLPD） in the new drug era and the effect of new drug treatment on efficacy and survival. METHODS: The clinical and laboratory data of 200 cases B-CLPD patients diagnosed between April 2015 and August 2021 were analyzed retrospectively. The clinical efficacy and survival of the patients under different treatments including Bruton tyrosine kinase（BTK） inhibitors， rituximab， and chemotherapy alone were analyzed. The prognostic factors affecting the survival of patients were analyzed by univarite analysis and multivariate analysis. RESULTS: There were 119 male（59.5％） and 81 female（40.5%） in 200 cases B-CLPD patients， the sex ratio(male/female) was 1.5∶1 with median age of 61（30- 91） years old. The distribution of subtypes were as fallows: 51 cases (25.5%) of chronic lymphocytic leukemia/small lymphocytic lymphoma（CLL/SLL）， 64（32.0%） cases of follicular lymphoma（FL）， 40（20.0%） cases mantle cell lymphoma（MCL）， 30（15.0%） cases of marginal zone lymphoma（MZL）， 10（5%） cases of lymphoplasmacytic lymphoma/waldenstrom macroglobulinemia（LPL/WM）， 5（2.5%） cases of B cell chronic lymphoproliferative disorders unclassified（B-CLPD-U） . The main clinical manifestation of 102 patients was lymph node enlargement, 32 cases were complicated with B symptoms. Among CLL/SLL patients， there were 12（23.5%） cases in Binet A and 39（76.5%） cases in Binet B/C. There were 29 patients（20.9%） in Ann Arbor or Lugano stage I-II and 110 cases（79.1%） in stage III-IV of other subtypes. The complete remission（CR） rate was 43.1%（25/58）， 40.2%（39/97）， 7.1%（1/14）， and overaIl response rate（ORR） was 87.9%（51/58）， 62.9%（61/97）， 28.6%（4/14） in the groups of BTK inhibitors， rituximab-based therapy， and chemotherapy alone. The 3-year OS rate and PFS rate in all patients was 79.2% and 72.4% respectively. The 3-year OS rate of patient with MZL, CLL/SLL, FL,WM was 94.7%, 87.7%, 86.8% and 83.3% respectively, while the 3-year OS rate of MCL was only 40.6%, which was significantly lower than other subtypes. The median OS of patients treated with BTK inhibitors and rituximab-based therapy was 20.5 and 18.5 months respectively， and the 3-year OS rate was 97.4% and 90.7%. However， the median PFS of patients receiving chemotherapy alone was 4 months， and the 1-year OS rate was 52.7%， which was statistically significant compared with the other two groups（P<0.05）. Univarite analysis showed that anemia， elevated lactate dehydrogenase， elevated β2-microglobulin， and splenomegaly were the poor prognostic factors for OS（P<0.05）， elevated lactate dehydrogenase was also poor prognostic factors for PFS（P<0.05）. Multifactor analysis showed that anemia and elevated lactate dehydrogenase were the independent poor prognostic factors for survival（P<0.05）. CONCLUSION The clinical features of B-CLPD was various， anemia and elevated lactate dehydrogenase are the prognostic factors for poor survival. BTK inhibitors and new immunotherapy can improve the survival and prognosis of patients in the new drug era.
Humans ; Adult ; Female ; Male ; Middle Aged ; Aged ; Aged, 80 and over ; Rituximab/therapeutic use* ; Leukemia, Lymphocytic, Chronic, B-Cell/drug therapy* ; Retrospective Studies ; Lymphoma, Mantle-Cell ; Prognosis ; Lymphoma, B-Cell, Marginal Zone ; Lactate Dehydrogenases

OBJECTIVE: To retrospectively analyze clinical characteristics and survival time of patients with diffuse large B-cell lymphoma (DLBCL), detect prognosis-related markers, and establish a nomogram prognostic model of clinical factors combined with biomarkers. METHODS: One hundred and thirty-seven patients with DLBCL were included in this study from January 2014 to March 2019 in the First Affiliated Hospital of Nanchang University. The expression of GCET1, LMO2, BCL-6, BCL-2 and MYC protein were detected by immunohistochemistry (IHC), then the influences of these proteins on the survival and prognosis of the patients were analyzed. Univariate and multivariate Cox regression analysis were used to gradually screen the prognostic factors in nomogram model. Finally, nomogram model was established according to the result of multivariate analysis. RESULTS: The positive expression of GCET1 protein was more common in patients with Ann Arbor staging I/II (P =0.011). Compared with negative patients, patients with positive expression of LMO2 protein did not often show B symptoms (P =0.042), and could achieve better short-term curative effect (P =0.005). The overall survival (OS) time of patients with positive expression of LMO2 protein was significantly longer than those with negative expression of LMO2 protein (P =0.018), though the expression of LMO2 protein did not correlate with progression-free survival (PFS) (P >0.05). However, the expression of GCET1 protein had no significant correlation with OS and PFS. Multivariate Cox regression analysis showed that nomogram model consisted of 5 prognostic factors, including international prognostic index (IPI), LMO2 protein, BCL-2 protein, MYC protein and rituximab. The C-index applied to the nomogram model for predicting 4-year OS rate was 0.847. Moreover, the calibrated curve of 4-year OS showed that nomogram prediction had good agreement with actual prognosis. CONCLUSION The nomogram model incorporating clinical characteristics and IHC biomarkers has good discrimination and calibration, which provides a useful tool for the risk stratification of DLBCL.
Humans ; Prognosis ; Nomograms ; Immunohistochemistry ; Retrospective Studies ; Clinical Relevance ; Lymphoma, Large B-Cell, Diffuse/drug therapy* ; Rituximab/therapeutic use* ; Proto-Oncogene Proteins c-bcl-2 ; Transcription Factors ; Antineoplastic Combined Chemotherapy Protocols