Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019829 (Hodgkin's disease)
 30,247 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Paraneoplastic pemphigus is a newly described syndrome in which patients have a severe mucocutaneous eruption with clinical features reminiscent of both erythema multiforme major (Stevens-Johnson syndrome) and pemphigus vulgaris, in association with non-Hodgkin's lymphomas and other malignant neoplasms. These patients have autoantibodies that bind to a characteristic set of epidermal proteins: desmoplakin I and desmoplakin II (molecular weight equals 250 kd and 210 kd, respectively), both major cytoskeletal structural proteins associated with desmosome cellular junctions within all epithelia, the bullous pemphigoid antigen, a 230 kd protein associated with hemidesmosomes, and a 190,000-d protein that has not been characterized. In this report, we describe a patient with paraneoplastic pemphigus who had (1) non-Hodgkin's lymphoma in apparent complete remission following autologous bone marrow transplantation, (2) very tense blisters reminiscent of bullous pemphigoid, (3) a unique pattern of immune deposits within the skin, and (4) IgG deposits within the epithelium of the pulmonary bronchi associated with respiratory compromise.
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PMID:Paraneoplastic pemphigus with autoantibody deposition in bronchial epithelium after autologous bone marrow transplantation. 162 84

The rituximab antibody is a genetically engineered chimeric murine/human monoclonal antibody directed against the CD20 antigen found on the surface of normal and malignant B lymphocytes. Rituximab is indicated for the treatment of patients with relapsed or refractory, low-grade or follicular, CD20-positive, B-cell non-Hodgkin lymphoma. Rituximab is also commonly used to treat chronic lymphocytic leukemia, Waldenstrom's macroglobulinemia, and immune or idiopathic thrombocytopenic purpura (ITP). Rituximab is an effective treatment for primary cutaneous B-cell lymphoma and other cutaneous lymphomas. Rituximab is an effective treatment for mixed cryoglobulinemia. Rituximab is a promising treatment for systemic lupus erythematosus, dermatomyositis, pemphigus, vasculitis, and a variety of hematologic diseases. Black-box warnings on rituximab include fatal infusion reactions, tumor lysis syndrome, and severe mucocutaneous reactions. A variety of cardiac, pulmonary, renal, and hematologic side effects can occur. It commonly causes mild cutaneous side effect and rarely has caused paraneoplastic pemphigus, Stevens-Johnson syndrome, lichenoid dermatitis, vesiculobullous dermatitis, and toxic epidermal necrolysis.
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PMID:A review of rituximab in cutaneous medicine. 1663 71

Scant information about the early toxicity of high-dose chemotherapy regimens for the treatment of mature B-cell malignancies (B-non-Hodgkin lymphoma) in developing countries is available, so we performed a retrospective evaluation of children with B-non-Hodgkin lymphoma treated with Berlin-Frankfurt-Muenster-based protocols in Argentina (1993 to 2007). In the second protocol, induction chemotherapy was modified introducing high-dose cytarabine and etoposide (block CC) instead of high-dose methotrexate (block AA). Forty-one patients with stage III and elevated lactate dehydrogenase or stage IV or B-acute lymphoblastic leukemia were included. Five patients (12.1%) had an early death at a median of 23 days after treatment initiation, caused by sepsis in 4 and by a Stevens Johnson syndrome in 1. Children that had an early death were significantly more likely to present with renal failure (P=0.04) and have significantly higher levels of phosphate and creatinine on admission (P=0.02 and 0.008). Eighty percent of children dying early had prior extensive abdominal surgery and positive blood cultures after the first cycle. Induction with AA block was associated with a higher frequency of severe orointestinal toxicity (P=0.04). We conclude that renal failure was associated to increased risk of mortality leading to a higher risk of sepsis, especially in patients that underwent abdominal surgery.
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PMID:Early mortality in children with advanced mature B-cell malignancies in a middle-income country. 2285 72

Hodgkin lymphoma (HL) and systemic anaplastic large cell lymphoma (sALCL), which is a subtype of non-Hodgkin lymphoma, are relatively uncommon lymphoproliferative types of cancer. These malignancies are highly curable with initial treatment. Nonetheless, some patients are refractory to or relapse after first- and second-line therapies, and outcomes for these patients are less promising. Brentuximab vedotin is a CD30-directed antibody-cytotoxic drug conjugate that has demonstrated efficacy in response rates (objective response rates and complete response) when given to patients with refractory or relapsed HL and sALCL. Although not compared directly in clinical trials, the response rates with brentuximab vedotin are higher than those of several current treatments for refractory or relapsed HL and sALCL. Adverse effects associated with brentuximab vedotin are considered manageable. Nonetheless, several serious adverse effects (e.g., neutropenia, peripheral sensory neuropathy, tumor lysis syndrome, Stevens-Johnson syndrome, and progressive multifocal leukoencephalopathy, resulting in death) have been reported with its use. Despite a lack of survival and patient reported outcome data, the United States Food and Drug Administration (FDA) granted accelerated approval to brentuximab vedotin for the treatment of HL after failure of autologous stem cell transplantation or at least two combination chemotherapy regimens, and for sALCL after failure of at least one combination chemotherapy regimen. With this approval, brentuximab vedotin is the first FDA-approved agent for the treatment of HL in over three decades and the first agent specifically indicated to treat sALCL. Results of ongoing prospective trials should determine if brentuximab vedotin has a survival benefit when compared directly with standard treatment and if brentuximab vedotin is safe and efficacious when given earlier in the disease process, or when used with other chemotherapy for the treatment of HL and sALCL or other CD30-positive malignancies.
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PMID:Brentuximab vedotin: a CD30-directed antibody-cytotoxic drug conjugate. 2330 50

Stevens-Johnson syndrome/toxic epidermal necrolysis overlap is an acute hypersensitivity reaction that compromises the integrity of mucous membranes and cutaneous tissue. While the pathophysiology of this syndrome has not been fully elucidated, it is commonly associated with the medication use and carries a significant mortality risk of approximately 30%. No commonalities among causative medications have been identified, and determining the offending agent can be challenging. This case report describes fatal Stevens-Johnson syndrome/toxic epidermal necrolysis overlap in a patient after receiving his first cycle of allopurinol, rituximab, and bendamustine treatment for non-Hodgkin's B-cell lymphoma. An analysis of FDA Medwatch adverse reaction case reports involving allopurinol, rituximab, and bendamustine is also presented.
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PMID:Fatal Stevens-Johnson syndrome/toxic epidermal necrolysis induced by allopurinol-rituximab-bendamustine therapy. 2500 69

Methotrexate is an antineoplastic drug used commonly in leukemia treatment. Because of becoming resistant to standard doses after 1970s, it is used intermediate or high doses. The complications of high doses are mucositis, vomiting, dermatitis exfoliativa, B-cell dysfunction, hepatotoxicity, nephrotoxicity and bone marrow depression. There were only two studies in literature about Stevens-Johnson syndrome occuring in two patients with acute lymphocytic leukemia and non-Hodgkin lymphoma after receiving high doses methotrexate and leukoverin. We have reported a two-year-old boy patient suffering from acute lymphocytic leukemia (ALL) developed a severe skin reaction two days after administration of high dose methotrexate. The skin lesions simulated Stevens-Johnson syndrome with ulceration of the oral mucosa and erythema multiforme-like target lesions.
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PMID:Stevens-Johnson Syndrome-Like Exanthema Secondary to Methotrexate. 2726 81

The introduction of immunotherapy into the treatment of cancer patients has revolutionised the oncological approach and significantly improved patient survival. The key drugs are immune checkpoint inhibitors (CPIs), whose mechanism of action is to elicit immune response against cancer cell antigens. Three types of CPIs are currently used and approved: an anti-CTLA-4 antibody, ipilimumab; anti-PD-1 antibodies, nivolumab and pembrolizumab; and anti-PD-L1 antibodies: atezolizumab, avelumab and durvalumab. CPIs have been widely used in metastatic and adjuvant melanoma settings, metastatic lung cancer, Hodgkin's lymphoma, renal cancer, bladder cancer, head and neck tumours, and Merkel cell carcinoma. However, side effects of CPIs differ from toxicities of other oncological drugs. According to literature data, in 10-30% of patients CPIs are responsible for immune-related adverse events (irAE) associated with excessive activation of the immune system. Systemic irAEs include enterocolitis, pneumonitis, hepatitis, nephritis, hypophysitis, and autoimmune thyroid disease. However, the most common irAEs of checkpoint inhibitors are dermatologic toxicities ranging from pruritus and mild dermatoses to severe reactions including Stevens-Johnson syndrome and toxic epidermal necrolysis. Each irAE can become serious if not early diagnosed and appropriately treated. In the article we present different types of skin irAEs related to CPIs together with the recommended therapies.
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PMID:Principles of prophylactic and therapeutic management of skin toxicity during treatment with checkpoint inhibitors. 3161 10

The aim of this study is to evaluate the clinical and laboratory findings of pediatric patients with non-Hodgkin lymphoma (NHL) who developed Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). Between 2006 and 2018, the medical records of child patients with NHL who developed SJS and TEN were reviewed retrospectively. SJS/TEN developed in 7 of 70 patients with NHL (10%). The pathologic subgroups of the patients with SJS/TEN were ALK-negative anaplastic large cell lymphoma (n: 3), Burkitt lymphoma (n: 2), lymphoblastic lymphoma (n: 1), and primary mediastinal B-cell lymphoma (n: 1). Five patients had TEN, 1 patient had SJS/TEN, and 1 patient developed only SJS. In 5 patients, both steroids and intravenous immunoglobulin were administered for treatment, and clinical improvement was achieved in 3 of these patients. Only steroid treatment was used for 1 patient, whereas for the other patient, intravenous immunoglobin was preferred. In addition, N-acetylcysteine treatment was administered for these 2 patients. Four patients with acute renal failure died, and it was found that SJS/TEN is observed more frequently in patients with NHL in which intensive treatment protocols with high-dose methotrexate are used more than with other childhood malignant diseases. Early diagnosis and administration of appropriate and supportive treatment approaches may improve the prognosis.
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PMID:Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in Children With Non-Hodgkin Lymphoma. 3257 84