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ENeurologicalSci Sep 2023Langerhans cell histiocytosis (LCH) is a rare disease that usually occurs in children <15 years of age. Adult-onset LCH is extremely rare. Previous published guidelines...
INTRODUCTION
Langerhans cell histiocytosis (LCH) is a rare disease that usually occurs in children <15 years of age. Adult-onset LCH is extremely rare. Previous published guidelines and studies mainly focused on pediatric patients. The rarity and also insufficient knowledge of LCH in adults, especially central neuvous system (CNS) involvement of LCH, often resulted in missed and delayed diagnosis.
CASE PRESENTATION
A 35-year-old woman presented with cognitive impairment, anxietydepression, decreased eyesight, skin rash, hypernatremia, gonadal hormone deficiency and hypothyroidism. She had experienced menstrual disturbance and infertility since 10 years ago. MRI examination showed a mass lesion in the hypothalamic-pituitary region. Sighs of radiologic neurodegeneration were not found on brain MRI scans, however. Biopsy of skin rash confirmed the the diagnosis of multisystem LCH. BRAF V600E mutation was detected in the peripheral blood mononuclear cells. She accepted combination chemotherapy of vindesine and prednisone and accquired partial remission. The patient died of severe pneumonia during the second course of chemotherapy.
CONCLUSION
Given the complicated differential diagnoses of neuroendocrine disorders, it was essential to be aware of CNS involvement of LCH at first, especially in adults. BRAF V600E mutation may participated in disease progression.
PubMed: 37435445
DOI: 10.1016/j.ensci.2023.100471 -
Zhongguo Dang Dai Er Ke Za Zhi =... Oct 2018A boy aged 14 years had abdominal pain as the major manifestation, with elevated serum amylase and lipase. Abdominal ultrasound performed early after onset in another...
A boy aged 14 years had abdominal pain as the major manifestation, with elevated serum amylase and lipase. Abdominal ultrasound performed early after onset in another hospital showed enlargement of the pancreas and a reduction in echo. Magnetic resonance cholangiopancreatography (MRCP) showed pancreatic duct dilation and an unclear image of the head of the pancreas. Acute pancreatitis was considered. However, his symptoms were not relieved after fasting, fluid infusion, anti-acid therapy, and somatostatin therapy. Then, abdominal CT scan and MRCP found multiple low-density lesions of the pancreas and enlargement of the hilar and retroperitoneal lymph nodes. Exploratory laparotomy found pancreatic edema and multiple hilar nodules with unclear boundaries, and pathological biopsy showed anaplastic large-cell lymphoma. Since the liver, the spleen, bone marrow, and the central nervous system were not involved, he was diagnosed with stage III primary pancreatic lymphoma. After vindesine and dexamethasone were used to reduce tumor load, the patient underwent vindesine-pirarubicin-asparaginase-dexamethasone chemotherapy once and vinorelbine-dexamethasone chemotherapy 8 times. Imaging examination still showed multiple low-density lesions of the pancreas and retroperitoneal lymph node enlargement. His parents discontinued treatment. It is concluded that the rare causes of acute pancreatitis with poor response to conventional treatment should be considered, especially for patients with abdominal lymph node enlargement. Extranodal lymphoma should be considered, and lymph node biopsy should be performed as early as possible to confirm diagnosis. The prognosis of pancreatic lymphoma is associated with clinical stage and pathology.
Topics: Adolescent; Cholangiopancreatography, Magnetic Resonance; Humans; Lymph Nodes; Lymphoma; Male; Pancreatic Neoplasms; Pancreatitis
PubMed: 30369361
DOI: 10.7499/j.issn.1008-8830.2018.10.012 -
Cancer Medicine Mar 2024Blinatumomab early-line treatment in B-cell precursor acute lymphoblastic leukemia (B-ALL) might improve clinical outcomes.
BACKGROUND
Blinatumomab early-line treatment in B-cell precursor acute lymphoblastic leukemia (B-ALL) might improve clinical outcomes.
METHODS
We conducted a retrospective real-world cohort analysis in 20 newly diagnosed B-ALL patients who received reduced-dose chemotherapy (idarubicin, vindesine, and dexamethasone) for 1-3 weeks, followed by blinatumomab for 1-4 weeks as an induction therapy.
RESULTS
At the end of the induction therapy, a complete remission rate of 100% was achieved; 17 (85%) patients were minimal residual disease (MRD) negative (<1 × 10 ). Adverse events (AEs) were reported in 12 (60%) patients-43.8% were grade 1-2 and 56.2% were grade 3-4. No incidence of neurotoxicity or grade ≥3 cytokine release syndrome was reported.
CONCLUSIONS
Blinatumomab demonstrated a significant improvement in clinical outcomes in patients with newly diagnosed B-ALL irrespective of their poor-risk factor status and the pretreatment blast burden.
Topics: Humans; Retrospective Studies; Induction Chemotherapy; Antibodies, Bispecific; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma; Burkitt Lymphoma
PubMed: 38491815
DOI: 10.1002/cam4.7062 -
Frontiers in Oncology 2023SSBP2-CSF1R is an important biomarker for clinical diagnosis and prognosis of Philadelphia chromosome-like acute lymphoblastic leukemia (Ph-like ALL). This case report...
SSBP2-CSF1R is an important biomarker for clinical diagnosis and prognosis of Philadelphia chromosome-like acute lymphoblastic leukemia (Ph-like ALL). This case report presents a pediatric Ph-like ALL patient carrying the SSBP2-CSF1R fusion gene. The patient was resistant to most conventional chemotherapy regimens and to dasatinib, an inhibitor that has been reported to have a therapeutic effect on SSBP2-CSF1R fusion Ph-like ALL, as she remained minimal residual disease (MRD) positive (detection by flow cytometry) and SSBP2-CSF1R fusion gene (detection by RT-PCR) positive after five rounds of such regimens. We thus conducted a large-scale screening to assess the sensitivity of the patient's leukemic cells to anti-cancer drugs. Based on the susceptibility results, we chose to combine cytarabine, homoharringtonine, dexamethasone, fludarabine, vindesine, and epirubicin for treatment. Clinical results showed that after a course of treatment, both MRD and SSBP2-CSF1R fusion gene turned negative, and there was no recurrence during an 18-month follow-up. In conclusion, our study suggests that the SSBP2-CSF1R fusion gene may be an important biomarker of primary drug resistance in Ph-like ALL, and indicate that the combination of cytarabine, homoharringtonine, dexamethasone, fludarabine, vindesine, and epirubicin can achieve optimal therapeutic results in this category of patients.
PubMed: 38107066
DOI: 10.3389/fonc.2023.1291570 -
British Journal of Haematology Nov 2019Patients with relapsed/refractory diffuse large B-cell lymphoma (DLBCL) after, or ineligible for, autologous stem cell transplantation (ASCT) have a dismal prognosis.... (Clinical Trial)
Clinical Trial
Rituximab-PECC induction followed by Y-ibritumomab tiuxetan consolidation in relapsed or refractory DLBCL patients who are ineligible for or have failed ASCT: results from a phase II HOVON study.
Patients with relapsed/refractory diffuse large B-cell lymphoma (DLBCL) after, or ineligible for, autologous stem cell transplantation (ASCT) have a dismal prognosis. This phase II study evaluated treatment with R-PECC (rituximab, prednisolone, etoposide, chlorambucil, lomustine), every 28 days for 4 cycles in 62 patients, followed by radio-immunotherapy consolidation with Y-ibritumomab tiuxetan in responsive patients. Primary endpoints were failure-free survival (FFS) and incidence of grade ≥3 adverse events from start of Y-ibritumomab tiuxetan. The overall response rate after R-PECC was 50%. Twenty-nine of 31 responsive patients proceeded to Y-ibritumomab tiuxetan. Five out of 15 partial remission patients converted to complete remission after Y-ibritumomab tiuxetan. One-year FFS and overall survival (OS) from start of Y-ibritumomab tiuxetan was 52% (95% confidence interval [CI], 33-68%) and 62% (95% CI, 42-77%), respectively. One-year FFS and OS from start of R-PECC was 28% (95% CI, 17-39%) and 49% (95% CI, 36-61%), respectively. Toxicities of R-PECC and Y-ibritumomab tiuxetan were mainly haematological. In conclusion, for relapsed DLBCL patients the largely oral R-PECC regimen achieves promising response rates, combined with an acceptable safety profile. Consolidation with Y-ibritumomab tiuxetan resulted in long-term response durations in approximately one third of the patients that received it.
Topics: Aged; Aged, 80 and over; Antibodies, Monoclonal; Antineoplastic Combined Chemotherapy Protocols; Autografts; Carmustine; Cyclophosphamide; Disease-Free Survival; Female; Humans; Lymphoma, Large B-Cell, Diffuse; Male; Middle Aged; Prednisone; Rituximab; Stem Cell Transplantation; Survival Rate; Vindesine
PubMed: 31290569
DOI: 10.1111/bjh.16087 -
Pharmaceuticals (Basel, Switzerland) Jul 2023Multidrug resistance (MDR) is one of the most problematic issues in chemotherapeutic carcinoma therapy. The ABCB1 transporter, a drug efflux pump overexpressed in cancer...
In-Silico Mining of the Toxins Database (T3DB) towards Hunting Prospective Candidates as ABCB1 Inhibitors: Integrated Molecular Docking and Lipid Bilayer-Enhanced Molecular Dynamics Study.
Multidrug resistance (MDR) is one of the most problematic issues in chemotherapeutic carcinoma therapy. The ABCB1 transporter, a drug efflux pump overexpressed in cancer cells, has been thoroughly investigated for its association with MDR. Thus, discovering ABCB1 inhibitors can reverse the MDR in cancer cells. In the current work, a molecular docking technique was utilized for hunting the most prospective ABCB1 inhibitors from the Toxin and Toxin-Target Database (T3DB). Based on the docking computations, the most promising T3DB compounds complexed with the ABCB1 transporter were subjected to molecular dynamics (MD) simulations over 100 ns. Utilizing the MM-GBSA approach, the corresponding binding affinities were computed. Compared to ZQU (calc. -49.8 kcal/mol), Emamectin B1a (T3D1043), Emamectin B1b (T3D1044), Vincristine (T3D4016), Vinblastine (T3D4017), and Vindesine (T3D2479) complexed with ABCB1 transporter demonstrated outstanding binding affinities with Δ values of -93.0, -92.6, -93.8, -92.2, and -90.8 kcal/mol, respectively. The structural and energetic investigations confirmed the constancy of the identified T3DB compounds complexed with the ABCB1 transporter during the 100 ns MD course. To mimic the physiological conditions, MD simulations were conducted for those identified inhibitors complexed with ABCB1 transporter in the presence of a POPC membrane. These findings revealed that Emamectin B1a, Emamectin B1b, Vincristine, Vinblastine, and Vindesine are promising ABCB1 inhibitors that can reverse the MDR. Therefore, subjecting those compounds to further in-vitro and in-vivo investigations is worthwhile.
PubMed: 37513931
DOI: 10.3390/ph16071019 -
Medicine Oct 2023Subcutaneous panniculitis like T-cell lymphoma (SPTCL) is a rare primary cutaneous lymphoma that belongs to peripheral T cell lymphomas, of which the overall prognosis...
RATIONALE
Subcutaneous panniculitis like T-cell lymphoma (SPTCL) is a rare primary cutaneous lymphoma that belongs to peripheral T cell lymphomas, of which the overall prognosis is poor. Chidamide, a deacetylase inhibitor, has been approved for the treatment of peripheral T cell lymphomas. However, due to the rare occurrence of SPTCL, it is currently unknown whether Chidamide is effective for all SPTCL patients and whether there are molecular markers that can predict its therapeutic effect on SPTCL.
PATIENT CONCERNS AND DIAGNOSES
The patient was a sixteen-year-old male and underwent subcutaneous nodule biopsy which showed SPTCL. Next-generation sequencing revealed AT-rich interaction domain 1A (ARID1A) mutation, and positron emission tomography/computed tomography showed scattered subcutaneous fluorodeoxyglucose metabolic lesions throughout the body.
INTERVENTIONS AND OUTCOMES
During the first 3 CHOP (cyclophosphamide, doxorubicin, vindesine, and prednisone) treatment, the patient relapsed again after remission, and the successive addition of methotrexate and cyclosporine did not make the patient relapsing again. Then, after adding Chidamide to the last 3 CHOP treatment, the patient was relieved again. The patient underwent autologous hematopoietic stem cell transplantation (auto-HSCT) after completing a total of 8 cycles of chemotherapy, and continued maintenance therapy with Chidamide after auto-HSCT. Currently, the patient has been in continuous remission for 35 months.
LESSONS SUBSECTIONS
This case is the first report of a refractory/recurrent SPTCL with ARID1A mutation treated with Chidamide. The treatment of Chidamide on the basis of CHOP plus auto-HSCT therapy achieved good results, suggesting that ARID1A may act as a molecular marker to predict the therapeutic effect of Chidamide on SPTCL patients, which helps to improve the precision of SPTCL treatment and the overall prognosis of SPTCL patients.
Topics: Male; Humans; Adolescent; Lymphoma, T-Cell, Peripheral; Neoplasm Recurrence, Local; Lymphoma, T-Cell; Hematopoietic Stem Cell Transplantation; Panniculitis; Mycosis Fungoides; Skin Neoplasms; Antineoplastic Combined Chemotherapy Protocols; DNA-Binding Proteins; Transcription Factors
PubMed: 37800816
DOI: 10.1097/MD.0000000000035413 -
Frontiers in Immunology 2022Cardiac involvement in hematological malignancies is uncommon, with only a few cases reported to date, and it often leads to a poor prognosis. Here, we report a case of...
Cardiac involvement in a patient with B-cell lymphoblastic lymphoma/acute lymphoblastic leukemia and a history of allogeneic hematopoietic stem cell transplantation and CAR T-cell therapy: A case report.
Cardiac involvement in hematological malignancies is uncommon, with only a few cases reported to date, and it often leads to a poor prognosis. Here, we report a case of a 42-year-old woman with a history of allogeneic hematopoietic stem cell transplantation (allo-HSCT) and anti-CD19 chimeric antigen receptor (CAR) T-cell therapy for B-cell lymphoblastic lymphoma/acute lymphoblastic leukemia in whom cardiac mass and myocardial infiltration were detected. Prior to this presentation, massive pericardial effusion had occurred 6 months after CAR T-cell therapy, which was improved ultrasound-guided pericardiocentesis. We observed elevated cytokine levels and increased copy number of CAR DNA in both pericardial effusion and serum. Upon detecting cardiac mass and myocardial infiltration, the patient was administered tocilizumab (a humanized monoclonal antibody against IL-6 receptor), which controlled the serum cytokine levels, and reduced intensity chemotherapy, including vindesine, cyclophosphamide, and prednisolone. However, the patient finally died of multiple organ failure. To the best of our knowledge, this is the first report on the development of a cardiac mass and occurrence of myocardial infiltration after allo-HSCT and CAR T-cell therapy. This report may provide supporting data for the early diagnosis and immediate treatment of patients with cardiac involvement.
Topics: Female; Humans; Adult; Immunotherapy, Adoptive; Pericardial Effusion; T-Lymphocytes; Hematopoietic Stem Cell Transplantation; Precursor Cell Lymphoblastic Leukemia-Lymphoma; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma; Burkitt Lymphoma; Antigens, CD19
PubMed: 36685607
DOI: 10.3389/fimmu.2022.1052336 -
Oncology Letters Jan 2015Solitary plasmacytomas (SPs) represent ≤5% of all plasma cell neoplasms and mostly occur in the spine, pelvis, ribs and pectoral girdle, while rarely occurring in the...
Solitary plasmacytomas (SPs) represent ≤5% of all plasma cell neoplasms and mostly occur in the spine, pelvis, ribs and pectoral girdle, while rarely occurring in the sternum. The tumors typically appear as osteolytic lesions. In rare cases, SPs can manifest as bony spicules on the surface of the bone. The present study reports the case of a 74 year-old female with an osteolytic tumor localized in the sternum. The tumor displayed extensive bony destruction, with a large quantity of thick straight spicules on the surface of the bone, resembling a sunray in appearance. The imaging, laboratory and pathological examinations of the patient met the diagnostic criteria of SP. The patient was initially treated with radiotherapy at a dose of 45 Gy. Six months later, chemotherapy consisting of vindesine, Adriamycin and dexamethasone was administered. Vindesine and Adriamycin were administered at a dose of 2 and 15 mg/day, respectively on days 1-4 in a 20-day cycle. Dexamethasone was administered at a dose of 20 mg/day on days 1-4, 9-12 and 17-20 in the 20-day cycle. In total, the patient underwent 6 cycles of chemotherapy, with a total duration of 7 months. The patient was followed-up for two years after beginning therapy. At present, the patient is well, without any evidence of progressive disease or multiple myeloma. To the best of our knowledge, this is the first case in the English literature of SP in the sternum, with an unusual sunray periosteal reaction on radiological imaging. The sites of bony spiculation in the lesions that have previously been described in the literature are the mandible, orbit, vertebral body and skull vault. To the best of our knowledge, the current study presents the first case of a SP of the sternum with a unusual spiculated periosteal reaction on radiological imaging to be reported in the English literature.
PubMed: 25435957
DOI: 10.3892/ol.2014.2636 -
Translational Cancer Research Sep 2022Mucosa-associated lymphoid tissue (MALT) lymphoma is an indolent B cell lymphoma. Its occurrence in the pleura is rare, with atypical clinical manifestations. MALT of...
BACKGROUND
Mucosa-associated lymphoid tissue (MALT) lymphoma is an indolent B cell lymphoma. Its occurrence in the pleura is rare, with atypical clinical manifestations. MALT of the pleura is easily misdiagnosed. This is the first case report of pleural MALT lymphoma in China.
CASE DESCRIPTION
We report the case of a 54-year-old Chinese man with no notable medical history who complained of cough, sputum, and shortness of breath for 3 months. He had a positive purified protein derivative (PPD) test. An initial misdiagnosis of pleural tuberculosis was corrected, after 3 thoracoscopic biopsies and tests, to primary pleural MALT lymphoma. He received treatments of R-CHOP (rituximab, cyclophosphamide, epirubicin, vindesine and prednisolone) and traditional Chinese medicine. The patient was followed for 3 years until June 2022, with no obvious respiratory symptoms. Pleural MALT lymphoma is extremely rare, with only a few cases reported. This article describes our case, and includes an overview of 15 previously reported cases to summarize the characteristics, treatments, and prognosis of primary pleural MALT lymphoma.
CONCLUSIONS
Pleural MALT lymphoma is rare, and a correct diagnosis depends on tissue biopsy, immunohistochemical staining, and detection of gene rearrangement. Thoracoscopy is important to diagnose this disease. Multiple thoracoscopic biopsies may be necessary.
PubMed: 36237246
DOI: 10.21037/tcr-22-671