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Doklady. Biochemistry and Biophysics Jun 2024Rituximab (RTX) has been used for the treatment of systemic sclerosis (SSс) for a long time and has shown good efficacy for skin fibrosis and interstitial lung disease...
Rituximab (RTX) has been used for the treatment of systemic sclerosis (SSс) for a long time and has shown good efficacy for skin fibrosis and interstitial lung disease (ILD). However, data on tolerability and long-term adverse events (AEs) during RTX therapy in SSc are insufficient. The objective of this study was to assess the tolerability and safety of RTX in patients with SSс in a long-term prospective follow-up. Our open-label prospective study included 151 SSс patients who received at least one RTX infusion. The mean age of the patients was 47.9 ± 13.4 years; the majority of them were women (83%). The mean disease duration was 6.4 ± 5.8 years. The mean follow-up period after the first RTX infusion was 5.6 ± 2.6 years (845.6 patient-years (PY)). All patients received RTX in addition to ongoing therapy with prednisone and/or immunosuppressants. AEs were assessed and recorded by a doctor in the hospital immediately after RTX infusion and then by patient's reported outcome during the observation period. All causes of death were considered, regardless of treatment. A total of 85 AEs (56%) were registered, the overall incidence of AEs was 10/100 PY (95% confidence interval (CI) 8-12). The highest frequency of all AEs was observed in the first 2-6 months after the first course of RTX, however, these were mainly mild and moderate AEs (71%). The most frequent AEs were infections, they were observed in 40% of cases, with no serious opportunistic infections reported. The overall incidence of all infections was 7.1/100 PY (95% CI 5.5-9), serious infections-1.5/100 PY (95% CI 0.9-2.6). Infusion reactions occurred in 8% of patients. Other AEs were noted in 3% (0.6/100 PY, 95% CI 0.3-1.4). The overall incidence of serious AEs was 18%-3.2/100 PY (95% CI 2.2-4.6). There was a significant decrease of the immunoglobulin G (Ig G) during follow-up; however, its average values remained within normal limits. There were 17 deaths (11%) (2/100 PY, 95% CI 1.3-3.2). In most cases, patients died from the progression of the major organ failure, which arose before RTX treatment. In our study, the safety profile of RTX in SSс was assessed as favorable. It was similar to the AE profile in other autoimmune diseases treated with RTX. With an increase in the cumulative dose of RTX, no increase in AEs was observed. The mortality is comparable to the other severe autoimmune diseases in observational studies. Monitoring of IgG may be useful for patients with SSс on RTX therapy for early detection of the risk of developing infectious complications. RTX could be considered as a relatively safe drug for the complex therapy of SSс when standard therapy is ineffective or impossible.
PubMed: 38861145
DOI: 10.1134/S1607672924700856 -
Cell Transplantation 2024Posttransplant lymphoproliferative disorder (PTLD) is a rare lymphoid and/or plasmocytic proliferation that occurs after allogeneic hematopoietic stem cell...
Posttransplant lymphoproliferative disorder (PTLD) is a rare lymphoid and/or plasmocytic proliferation that occurs after allogeneic hematopoietic stem cell transplantation (allo-HSCT). We aimed to identify the pathologic features and clinical outcomes of T-cell PTLD, an extremely rare subtype of PTLD, after allo-HSCT. In this study, six allo-HSCT recipients with T-cell PTLD from five transplant centers in China were enrolled. All the T-cell PTLD were donor-derived, and three patients were with monomorphic and three with polymorphic types, respectively. All patients received cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP)-based chemotherapy. Five patients achieved complete response (CR), and one experienced progressive disease (PD). The median time from HSCT to onset was 4 (range: 0.6-72) months, analyzed in combination with the other 16 patients with T-cell PTLD identified from previous reports. About 56.3% of the T-cell samples (9/16) were positive for in situ hybridization with an Epstein-Barr virus (EBV)-encoded small nuclear early region (EBER ISH). CHOP-based chemotherapy might be the optimal strategy for patients who showed no response to empiric therapy with a CR rate of 87.5%. In conclusion, our study observed that T-cell PTLD has distinct clinical manifestations and morphological features, which characterized by less relation to EBV, later occurrence, and poorer prognosis when compared with B-cell PTLD.
Topics: Humans; Hematopoietic Stem Cell Transplantation; Lymphoproliferative Disorders; Male; Female; Adult; T-Lymphocytes; Transplantation, Homologous; Adolescent; Child; Middle Aged; Young Adult; Cyclophosphamide
PubMed: 38856035
DOI: 10.1177/09636897241259722 -
Journal of Medical Cases Jun 2024Acquired hemophilia A (AHA) is a clotting disorder characterized by the presence of neutralizing antibodies that inhibit factor VIII, resulting in increased bleeding...
Acquired hemophilia A (AHA) is a clotting disorder characterized by the presence of neutralizing antibodies that inhibit factor VIII, resulting in increased bleeding risk. Known etiologies include malignancy, autoimmune conditions, graft-vs-host disease, and more recently coronavirus disease 2019 (COVID-19) infection. In this case report, we describe an 86-year-old female who was found to have AHA incidentally during preoperative workup for meningioma resection. She was subsequently found to have COVID-19 infection which was the likely cause of her development of AHA. She was treated with factor eight inhibitor bypassing agent (FEIBA) and recombinant factor VII (rVII) for a small hematoma on her right arm along with prednisone and cyclophosphamide. She then developed disseminated intravascular coagulation (DIC) initially secondary to FEIBA and subsequently rFVII. DIC resolved after these factor concentrates were withheld. The aim of this case report was to emphasize the importance of monitoring partial thromboplastin time (PTT) in patients with COVID-19 and proceeding with AHA workup if indicated. It is also imperative to know and understand the potentially life-threatening, albeit rare, adverse effects of DIC associated with the administration of factor concentrates, especially in the elderly population and withholding these factor concentrates once DIC is suspected.
PubMed: 38855293
DOI: 10.14740/jmc4199 -
International Medical Case Reports... 2024Recurrent oral erythema multiforme (ROEM) is an uncommon subtype of erythema multiforme. Immunoglobulin E (IgE) is essential in acute allergy reactions and chronic...
BACKGROUND
Recurrent oral erythema multiforme (ROEM) is an uncommon subtype of erythema multiforme. Immunoglobulin E (IgE) is essential in acute allergy reactions and chronic allergic inflammatory disorders.
PURPOSE
This report aims to describe the advantages of total IgE screening for detecting mouthwash allergic reactions associated with ROEM.
CASE PRESENTATION
A 29-year-old woman came to the Oral Medicine clinic complaining of canker sores all over her mouth and swollen lips accompanied by crusts that had been bleeding easily two months prior. Complaints worsened after the patient used alcohol-containing mouthwash without a history of fever or other symptoms. Extra-oral examination showed upper and lower lip edema with hemorrhagic crusts that bleed easily. No lesions were found in other parts of the body. Intra-oral examination showed ulcers, multiple, irregular in almost the entire oral mucosa. Laboratory examination revealed non-reactive anti-HSV-1 IgG and a total IgE serum level of 612.00 IU/mL. The diagnosis based on the examination results is recurrent oral erythema multiforme.
CASE MANAGEMENT
The patient was instructed to stop using alcohol-containing mouthwash, maintain oral hygiene, a healthy lifestyle, adequate hydration, and a balanced diet. Prednisone, benzydamine HCL mouthwash, 0.025% hyaluronic acid mouthwash, multivitamins, and hydrocortisone cream were given as pharmacological therapy. The oral lesions improved in 12 days and the total IgE serum level examination showed a decrease (385 IU/mL).
CONCLUSION
The total IgE examination can be a screening tool for mouthwash allergy-related reactions to disease and represents the response of ROEM therapy as evidenced by clinical improvement.
PubMed: 38854841
DOI: 10.2147/IMCRJ.S468876 -
Cureus May 2024Non-Hodgkin's lymphoma (NHL) involving skeletal muscle is generally found to be a secondary metastasis and extremely rarely as a primary site of malignancy. Furthermore,...
Non-Hodgkin's lymphoma (NHL) involving skeletal muscle is generally found to be a secondary metastasis and extremely rarely as a primary site of malignancy. Furthermore, in HIV patients, an increased incidence of lymphomas may be identified within the first six months of highly active antiretroviral therapy (HAART) initiation unmasked by immune reconstitution inflammatory syndrome (IRIS). We illustrate an extremely rare instance of NHL of the skeletal muscle in a young immunocompromised male with HIV/AIDS presenting as necrotizing myofasciitis complicated by compartment syndrome and hemodialysis-refractory type B lactic acidosis. A young Hispanic male with AIDS was admitted for acute left thigh pain and was soon found to have abscess formation with compartment syndrome requiring thigh fasciotomy. During the course of the ICU stay, the patient's clinical status acutely worsened with sepsis-induced multiorgan failure, including acute renal and acute liver failure requiring N-acetylcysteine and severe refractory metabolic acidosis requiring renal replacement therapy. Repeat imaging demonstrated diffuse myonecrosis. Left thigh muscle biopsy confirmed aggressive NHL of skeletal muscle. Despite months of arduous medical management in ICU, doxorubicin, vincristine, cyclophosphamide chemotherapy with concurrent high-dose prednisone for the vented patient, and intermittent curves of improvement, our patient succumbed to the nature of the disease and subsequently died from severe sepsis from the immunocompromised state. Interestingly, our patient's initial CD4 count was 1, which improved to 96 after five months of HAART, raising concerns for IRIS lymphoma. Given such rapid improvement with chemotherapy, the possibility of IRIS-related lymphoma, and the surprising dearth of data for chemotherapy use in critically ill patients on mechanical ventilation, more research is needed in these topics to better approach such complicated patients.
PubMed: 38854279
DOI: 10.7759/cureus.59888 -
Clinical Lymphoma, Myeloma & Leukemia May 2024Diffuse large B-cell lymphoma (DLBCL) is the most common type of non-Hodgkin lymphoma, accounting for around 30-60% of all cases. The management of DLBCL in Asia has... (Review)
Review
Diffuse large B-cell lymphoma (DLBCL) is the most common type of non-Hodgkin lymphoma, accounting for around 30-60% of all cases. The management of DLBCL in Asia has several unmet needs due to the diversity of the population, the heterogeneity of local clinical guidelines for DLBCL and the wide disparity in resources and healthcare systems across different regions. Rituximab combined with cyclophosphamide, doxorubicin, vincristine, and prednisone (RCHOP) is widely recognized as the standard first-line treatment for DLBCL; however, alternative regimens are required to improve patient outcomes in challenging subtypes, such as patients with high International Prognostic Index scores, old/frail patients, and patients with double-hit and double-expressor DLBCL or concurrent central nervous system disease. This review article draws from the expertise of practicing hematologists/oncologists in the region, with the aim of integrating data from current scientific evidence to address the unmet needs and unique socioeconomic challenges faced by challenging high risk patient groups in the Asia-Pacific region.
PubMed: 38853026
DOI: 10.1016/j.clml.2024.05.013 -
BMC Cancer Jun 2024Poly (ADP- ribose) polymerase inhibitors (PARPi) has been increasingly adopted for metastatic castration-resistance prostate cancer (mCRPC) patients with homologous... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Poly (ADP- ribose) polymerase inhibitors (PARPi) has been increasingly adopted for metastatic castration-resistance prostate cancer (mCRPC) patients with homologous recombination repair deficiency (HRD). However, it is unclear which PARPi is optimal in mCRPC patients with HRD in 2nd -line setting.
METHOD
We conducted a systematic review of trials regarding PARPi- based therapies on mCRPC in 2nd -line setting and performed a Bayesian network meta-analysis (NMA). Radiographic progression-free survival (rPFS) was assessed as primary outcome. PSA response and adverse events (AEs) were evaluated as secondary outcomes. Subgroup analyses were performed according to specific genetic mutation.
RESULTS
Four RCTs comprised of 1024 patients (763 harbored homologous recombination repair (HRR) mutations) were identified for quantitative analysis. Regarding rPFS, olaparib monotherapy, rucaparib and cediranib plus olaparib showed significant improvement compared with ARAT. Olaparib plus cediranib had the highest surface under cumulative ranking curve (SUCRA) scores (87.5%) for rPFS, followed by rucaparib, olaparib and olaparib plus abiraterone acetate prednisone. For patients with BRCA 1/2 mutations, olaparib associated with the highest probability (98.1%) of improved rPFS. For patients with BRCA-2 mutations, olaparib and olaparib plus cediranib had similar efficacy. However, neither olaparib nor rucaparib showed significant superior effectiveness to androgen receptor-axis-targeted therapy (ARAT) in patients with ATM mutations. For safety, olaparib showed significantly lower ≥ 3 AE rate compared with cediranib plus olaparib (RR: 0.72, 95% CI: 0.51, 0.97), while olaparib plus cediranib was associated with the highest risk of all-grade AE.
CONCLUSION
PARPi-based therapy showed considerable efficacy for mCRPC patients with HRD in 2nd -line setting. However, patients should be treated accordingly based on their genetic background as well as the efficacy and safety of the selected regimen.
TRIAL REGISTRATION
CRD42023454079.
Topics: Humans; Poly(ADP-ribose) Polymerase Inhibitors; Bayes Theorem; Prostatic Neoplasms, Castration-Resistant; Mutation; Male; Phthalazines; Network Meta-Analysis; Piperazines; BRCA2 Protein; Recombinational DNA Repair; Antineoplastic Combined Chemotherapy Protocols; Randomized Controlled Trials as Topic; Progression-Free Survival; Indoles; BRCA1 Protein; Treatment Outcome; Quinazolines
PubMed: 38851712
DOI: 10.1186/s12885-024-12388-2 -
Medicine Jun 2024Intestinal T-cell lymphomas are exceedingly rare diseases. Intestinal T-cell lymphoma NOS, as a "wastebasket" category, is difficult to diagnosis. Endoscopy can identify...
RATIONALE
Intestinal T-cell lymphomas are exceedingly rare diseases. Intestinal T-cell lymphoma NOS, as a "wastebasket" category, is difficult to diagnosis. Endoscopy can identify abnormal mucosa in most patients at a reasonably early stage. Therefore, it is crucial to increase the understanding of endoscopists in terms of the endoscopic characteristics of ITCL.
PATIENT CONCERNS
A 74-year-old male alone with wasting as the major complaint, had multiple polypoid lesions in the large intestine. The patient then had endoscopic care.
DIAGNOSES
Only 1 polypoid lesion on white-light endoscopy in the sigmoid colon was pathologically diagnosed as intestinal T-cell lymphomas, not otherwise specified (ITCL-NOS).
INTERVENTIONS
The patient underwent intensity-reduced CHOP therapy.
OUTCOMES
The patient is still with controlled disease but developed chemotherapy-related side effects.
LESSONS
In the individual with unexplained anemia and waste, endoscopy should not be delayed. For each of polypoid lesion on white-light endoscopy, the endoscopist need to remain cautious, because every lesion in the same patient can exhibit the independence of histopathological features. Meanwhile, we suggest that endoscopists should routinely observe the terminal ileum, even take biopsy samples if necessary.
Topics: Humans; Aged; Male; Antineoplastic Combined Chemotherapy Protocols; Lymphoma, T-Cell; Doxorubicin; Vincristine; Intestinal Neoplasms; Cyclophosphamide; Prednisone; Colonoscopy
PubMed: 38847694
DOI: 10.1097/MD.0000000000038465 -
Eplasty 2024Postsurgical pyoderma gangrenosum (PSPG) is a highly uncommon and unpredictable wound healing complication. Rapid progression of ulcers at incisions can cause unfettered...
BACKGROUND
Postsurgical pyoderma gangrenosum (PSPG) is a highly uncommon and unpredictable wound healing complication. Rapid progression of ulcers at incisions can cause unfettered dehiscence. Most commonly, PSPG involves breast procedures; however, in this work, we detail a case of a patient who developed PSPG 10 days postoperatively after penile inversion vaginoplasty.
METHODS
The patient in this case underwent a penile inversion vaginoplasty with orchiectomy in the standard fashion. She had no risk factors for PSPG. Following an uncomplicated hospital stay, the patient developed difficulty with pain control and increasing serous drainage on the 10th postoperative day. On readmission, the patient was found to have developed large, mildly purulent ulcers throughout the perineal wound edges. On exam under anesthesia, the neovaginal canal was found to be patent and intact. The dehisced portions of the incisions were left open and redressed with occlusive bismuth-petrolatum dressing. Dermatology was promptly consulted with suspicion for PSPG. The patient was started on an 18-day prednisone taper with cyclosporine, along with doxycycline and ciprofloxacin.
RESULTS
After 5 days of immunosuppressive treatment, the ulcers visibly converted to healthy granulation tissue and were no longer actively purulent. Following another washout, the dehisced wound edges were reapproximated. At follow-up, the patient had no evidence of PSPG recurrence and continued dilating on schedule. Our patient recovered from PSPG without further complications and a satisfactory aesthetic result.
CONCLUSIONS
This unique case highlights the importance of prompt dermatological consultation, immunosuppression, and avoidance of further pathergy in the setting of suspicion for PSPG.
PubMed: 38846503
DOI: No ID Found -
[Incidence, characteristics and survival of patients with pneumocystis pneumonia in solid oncology].Bulletin Du Cancer Jun 2024Pulmonary pneumocystis causes interstitial lung disease, particularly in patients with solid cancers. The aim of this study is to clarify its incidence, which remains...
INTRODUCTION
Pulmonary pneumocystis causes interstitial lung disease, particularly in patients with solid cancers. The aim of this study is to clarify its incidence, which remains poorly understood, and to identify patients at risk and prognostic factors.
METHODS
Data on patients with solid tumors and pulmonary pneumocystis were retrospectively collected from January 1, 2014 to December 31, 2019 in two hospitals in Rennes. Incidence was estimated via the Poisson model. Survival data were estimated using Kaplan-Meier method and Log-rank test. A multivariate Cox model was performed to identify risk factors for death.
RESULTS
The incidences of pulmonary pneumocystis in metastatic cancer patients receiving parenteral systemic therapy are 198 and 349 cases per 100,000 patients per year in these two centers, respectively. Most patients were being treated with corticosteroids and chemotherapy at the time of pulmonary pneumocystis. The mortality rate for patients with pulmonary pneumocystis is 38%. Median overall survival was 2,7 months. Risk factors for death are corticotherapy greater than 20mg, prednisone equivalent, daily and chemotherapy.
DISCUSSION
Pulmonary pneumocystis pneumonia is rare but not exceptional and has a poor prognosis in solid oncology. It frequently occurs in patients treated with long-term corticosteroids. Oncologists need to be better informed to discuss prophylaxis whenever corticosteroids are prescribed for several weeks.
PubMed: 38845334
DOI: 10.1016/j.bulcan.2024.04.011