scholarly journals Management of HAM/TSP

2020 ◽  
pp. 10.1212/CPJ.0000000000000832 ◽  
Author(s):  
Abelardo Araujo ◽  
Charles R.M. Bangham ◽  
Jorge Casseb ◽  
Eduardo Gotuzzo ◽  
Steve Jacobson ◽  
...  
Keyword(s):  
Progressive Disease ◽  
Spastic Paraparesis ◽  
Oral Prednisolone ◽  
Interferon Α ◽  
High Dose ◽  
First Line Therapy ◽  
Methyl Prednisolone ◽  
Disease Modifying Therapy ◽  
History Of

Purpose of reviewTo provide an evidence-based approach to the use of therapies that are prescribed to improve the natural history of HTLV-1–associated myelopathy/tropical spastic paraparesis (HAM/TSP)—a rare disease.Recent findingsAll 41 articles on the clinical outcome of disease-modifying therapy for HAM/TSP were included in a systematic review by members of the International Retrovirology Association; we report here the consensus assessment and recommendations. The quality of available evidence is low, based for the most part on observational studies, with only 1 double-masked placebo-controlled randomized trial.SummaryThere is evidence to support the use of both high-dose pulsed methyl prednisolone for induction and low-dose (5 mg) oral prednisolone as maintenance therapy for progressive disease. There is no evidence to support the use of antiretroviral therapy. There is insufficient evidence to support the use of interferon-α as a first-line therapy.

Avascular necrosis of the jaw in multiple myeloma (MM) patients (pts) treated with bisphosphonates (BP)

2007 ◽  
Vol 25 (18_suppl) ◽  
pp. 18538-18538
Author(s):  
L. Curreli ◽  
A. D. Palmas ◽  
G. Latte ◽  
A. Murgia ◽  
A. Gabbas
Keyword(s):  
Oral Cavity ◽  
Progressive Disease ◽  
Alveolar Bone ◽  
High Dose ◽  
Allogenic Bone ◽  
History Of Disease ◽  
Aggressive Surgical Approach ◽  
History Of ◽  
The Right

18538 Background: Oral cavity avascular bone necrosis (ABN) has been recently reported as an emerging serious complication in pts receiving BP for the treatment of hypercalcemia related to MM or metastatic solid tumors. Methods: We report the cases of 6 pts with MM treated initially with pamidronate and later with zoledronic acid (ZA). Results: Pts characteristics : M/F 3/3; mean age 58.4 (46–78); 4 IgG κ,1 γ and 1 κ MM; 5 St IIIA and 1 IIIB; mean history of disease 61.3 mo. (23–103); 5 pts had a relapsing MM refractory to several lines of therapy but 1 pt had received only high dose dexametazone (D); 2 pts had received autologous stem cell transplantation and 1 pt allogenic bone marrow transplantation; mean n.° of BP doses was 41.3 (17–81). At the time of ABN onset all pts were receiving ZA along with, respectively: D (2 pts); cyclophosphamide plus D (1 pt), bortezomib plus D (2 pts) and oral melphalan (1 pt). ABN was localized in 2 pts at alveolar bone of the right maxilla and presented as an inflammation of the gum, followed by a painful bone exposure. In the other 4 pts ABN was localized at mandible and presented as dental abscesses followed in 2/4 pts by cutaneous fistulization. Treatment has included in all pts discontinuation of ZA, antibiotics, chlorhexidine mouthwashes, pain control, minor regional débridement, and bone trimming. In 1 pt a more aggressive surgical approach was attempted at an other Institution and postoperative course was complicated by massive haemorrhage and complete loss of chewing. Four pts dead with progressive disease with a mean overall survival after ABN presentation of 6 mo.; 2 pts are alive after 3 and 4 mo. after ABN presentation; however in all pts ABN significantly worsened quality of life. Conclusions: Oral cavity ABN is a severe complication in refractory MM pts receiving BP. Mechanisms of action of BP that determine a reduction in osteoclastic activity and an accumulation of nonvital osteocytes with microfractures of old mineral matrix appear to play an important role. However other causes may be involved as a long history of disease; an uncontrolled progressive disease; type and doses of previous and present therapies, primarily steroids; status of oral cavity and teeth of pts and possibly the n.° of doses of BP. No significant financial relationships to disclose.

Pseudo-Parkinson Disease Secondary to Ritonavir–Buspirone Interaction

2003 ◽  
Vol 37 (2) ◽  
pp. 202-205 ◽  
Author(s):  
Patrick G Clay ◽  
Molly M Adams
Keyword(s):  
Drug Interaction ◽  
Inhibitory Effect ◽  
Hiv Positive ◽  
High Dose ◽  
Resting Tremor ◽  
Case Summary ◽  
History Of ◽  
Drug Drug Interaction ◽  
To Receive

OBJECTIVE: To report a case of Parkinson-like symptoms appearing in a patient after introduction of ritonavir to buspirone therapy. CASE SUMMARY: A 54-year-old HIV-positive white man presented to the clinic with a 2-week history of ataxia, shuffling gait, cogwheel rigidity, resting tremor, and sad affect with masked features. This patient had been receiving high-dose buspirone (40 mg every morning and 30 mg every evening) for 2 years prior to the introduction of ritonavir/indinavir combination therapy (400 mg/400 mg twice daily) 6 weeks prior to initiation of the above symptoms. Buspirone was decreased to 15 mg 3 times daily, ritonavir/indinavir was discontinued, and amprenavir 1200 mg twice daily was added. The patient's symptoms began to subside after 1 week, with complete resolution after about 2 weeks. The patient continued to receive buspirone for an additional 12 months without recurrence of symptoms. DISCUSSION: This is the first reported interaction of buspirone and antiretrovirals. Buspirone, extensively metabolized by CYP3A4, was likely at supratherapeutic levels due to the inhibitory effect of ritonavir and, secondarily, indinavir. The Parkinson-like symptoms developed rapidly and severely, impacted this patient's quality of life, and necessitated significant clinic expenditures to identify this drug–drug interaction. CONCLUSIONS: This case demonstrates a severe drug–drug interaction between buspirone and ritonavir and further demonstrates the need for awareness of the metabolic profile for all agents an HIV-infected patient is receiving.

Alka Ptonuria Disorder

2021 ◽  
pp. 364-366
Author(s):  
Bhawan B. Bhende
Keyword(s):  
Autosomal Recessive ◽  
Autosomal Recessive Inheritance ◽  
Homogentisic Acid ◽  
Dark Urine ◽  
Disease Modifying Therapy ◽  
Collagenous Tissue ◽  
Reduce Life Expectancy ◽  
Recent Developments ◽  
History Of

Alkaptonuria (AKU) is a rare disorder of autosomal recessive inheritance. It is caused by a mutation in a gene that results in the accumulation of homogentisic acid (HGA). Characteristically, the excess HGA means sufferers pass dark urine, which upon standing turns black. This is a feature present from birth. Over time patients develop other manifestations of AKU, due to deposition of HGA in collagenous tissue namely ochronosis and ochronotic osteoarthropathy. Although this condition does not reduce life expectancy, it significantly affects quality of life. The natural history of this condition is becoming better understood, despite gaps in knowledge. Clinical assessment of the condition has also improved along with the development of a potentially disease-modifying therapy. Furthermore, recent developments in AKU research have led to new understanding of the disease, and further study of the AKU arthropathy has the potential to influence therapy in the management of osteoarthritis.

scholarly journals REPORTE DE CASO: ARTERITIS DE TAKAYASU EN ECUADOR

2021 ◽  
Vol 32 (1) ◽  
pp. s23-s24
Author(s):  
Rocío Álvarez ◽  
Diana Ramirez ◽  
Patricia Villacís ◽  
Gabriel Acosta
Keyword(s):  
Takayasu Arteritis ◽  
Combined Therapy ◽  
Immunosuppressive Agents ◽  
High Dose ◽  
Acute Phase Reactants ◽  
First Line Therapy ◽  
American College Of Rheumatology ◽  
Low Incidence ◽  
Large Vessels

Introduction Takayasu's arteritis is a panarteritis, characterized by chronic inflammation, that affects large vessels, especially the Aorta and its main branches. This pathology is extremely rare, has a higher incidence in Japan, presenting low incidence in Latin America. Case description We present the case of a young adult, with no significant medical history, who presents a 3 months of evolution intense headache initially classified as trigeminal neuralgia and cluster headache. Asymmetric pulses and variation in blood pressure between both upper extremities where observed which allowed several presumptive diagnoses to be considered. In the complementary examinations, the patient showed sustained elevation of acute phase reactants. Our patient met four diagnostic criteria of the American College of Rheumatology for Takayasu arteritis and the diagnosis was confirmed by magnetic Angio resonance of the neck, chest and abdomen, where involvement of the entire route of the aorta was observed. In context to treatment, the first-line therapy is high-dose steroids. In patients in whom the suspension of steroids is difficult, combined therapy with immunosuppressive agents is used. In the case of our patient, combined therapy with prednisone and methotrexate was chosen, which presented a favorable evolution. Conclusion Takayasu arteritis is an infrequent vasculitis, however, it is important to make an early diagnosis and treatment in order to improve the prognosis, stop the progression of the disease and improve the quality of life of patients suffering from this pathology.

scholarly journals A Sudden Onset of Severe Thrombocytopenia While Using Evolocumab

2020 ◽  
Vol 2020 ◽  
pp. 1-4
Author(s):  
Ikuo Inoue ◽  
Yasuhiro Takenaka ◽  
Yoshitora Kin ◽  
Satoshi Yamazaki ◽  
Yuichi Ikegami ◽  
...  
Keyword(s):  
Platelet Count ◽  
Immunoglobulin Therapy ◽  
Sudden Onset ◽  
High Dose ◽  
Severe Thrombocytopenia ◽  
First Line Therapy ◽  
Thrombopoietin Receptor ◽  
Thrombopoietin Receptor Agonist ◽  
History Of ◽  
Coa Reductase

A 72-year-old man with a 10-year history of coronary heart disease started evolocumab treatment once a month after developing excess myalgia due to therapy with a 3-hydroxy-methylglutaryl CoA reductase inhibitor. No side effects such as myalgia symptoms had been reported during the first 14 months of evolocumab treatment; however, he suddenly presented with acute severe thrombocytopenia following the 14th treatment. His platelet count continued to decrease to a nadir of 1,000/μL. His platelet-associated immunoglobulin G level had elevated to 790 ng/107 cells. He started receiving a combination of steroid therapy, high-dose immunoglobulin therapy, and platelet transfusions, but the first-line therapy was ineffective. He was subsequently treated with a thrombopoietin receptor agonist, and his platelet count recovered to 250,000/μL.

Strychnine poisoning causing generalized tetanic spasm

2020 ◽  
pp. 10.1212/CPJ.0000000000000943
Author(s):  
Simon Winzer ◽  
Kristian Barlinn
Keyword(s):  
Brain Activity ◽  
Selective Inhibition ◽  
Muscle Contractions ◽  
Whole Body ◽  
High Dose ◽  
Repeated Testing ◽  
Limited Effect ◽  
First Line Therapy ◽  
History Of

A 55-year old male presented with spontaneous and stimulus-triggered tetanic-like activity of the whole body without losing consciousness or orientation (video, http://links.lww.com/CPJ/A196). There was no history of trauma, and clinical examination did not reveal any tetanus-prone wound. Electroencephalography showed no significant alteration in brain activity. Serial intravenous benzodiazepines showed limited effect. Frequency and duration of muscle contractions increased over time and led to severe hypoxemia requiring intubation and sedation with propofol for almost 24 hours followed by continuous infusion of midazolam. Daily sedation pauses revealed ongoing generalized muscle spasm triggered by tactile and auditory stimuli and severe vegetative dysregulation. Tetanus antibody level suggested long-term protection. On day 6, toxicology revealed toxic levels of strychnine in serum (180 ng/ml [toxicity > 75 ng/ml]) and urine (514 ng/ml [no toxicity level defined]). Repeated testing showed markedly declined strychnine levels in serum (2.8 ng/ml) and urine (1.1 ng/ml) on day 11. Sedation was ultimately stopped on day 10. Subsequently, the patient recovered completely and was discharged on day 25. Strychnine ingestion mode remained unclear. Generalized tetanic spasm with sustained alertness, presumably caused by selective inhibition of post-synaptic glycine receptors in the spinal cord, should trigger testing for strychnine poisoning.1,2 High-dose intravenous benzodiazepines are considered first-line therapy for controlling muscle spasms; however, in patients whose muscle contractions are refractory to benzodiazepines, sedation with propofol or barbiturates is deemed necessary.1

scholarly journals Checkpoint inhibitors and radiotherapy in refractory malignant melanocytic schwannoma with Carney complex: first evidence of efficacy

2021 ◽  
Vol 14 (5) ◽  
pp. e240296
Author(s):  
Jyoti Bajpai ◽  
Akhil Kapoor ◽  
Rakesh Jalali ◽  
Mrinal M Gounder
Keyword(s):  
Progressive Disease ◽  
Checkpoint Inhibitors ◽  
External Beam Radiotherapy ◽  
Carney Complex ◽  
Spinal Nerve ◽  
The Novel ◽  
Nerve Roots ◽  
Novel Approach ◽  
History Of

Melanocytic schwannoma (MS) is a rare nerve sheath tumour characterised by melanin-producing neoplastic schwann cells that typically affects the posterior spinal nerve roots. We report an ultrarare case of recurrent/metastatic MS associated with Carney complex in a young woman with family history of breast cancer. This highlights the novel approach of combined checkpoint inhibitors (CPI) and radiotherapy. The patient was initially treated with Nivolumab along with concurrent external beam radiotherapy. There was sustained clinical benefit achieved for over 15 months with preserved quality of life. Addition of Ipilimumab, which she tolerated reasonably well, helped to control the progressive disease again for another 12 months. She harboured a rare PRKAR1A R228 mutation (Carney complex) and received appropriate targeted therapy. She survived for 51 and 35 months from her initial diagnosis and start of CPI, respectively, which to the best of our knowledge is the longest documented survival in this rare entity.

scholarly journals Hodgkin lymphoma. ASCO, EHA and ASH 2020 news

2021 ◽  
Vol 23 (1) ◽  
pp. 185-191
Author(s):  
Elena A. Demina
Keyword(s):  
Hodgkin Lymphoma ◽  
High Dose Chemotherapy ◽  
Brentuximab Vedotin ◽  
High Dose ◽  
The Past ◽  
First Line Therapy ◽  
Positron Emission ◽  
American Society ◽  
Interesting Direction

The review shows the most interesting and promising studies presented at major international conferences of the American Society of Clinical Oncology, the European Hematology Association, and the American Society of Hematology in 2020. The main aim of the therapy for Hodgkin lymphoma (HL), which have been formulated over the past 20 years, is the highly effective therapy and high quality of life, and the main possibility to solve this task is positron emission tomography (PET). The studies presented at conferences this year have shown that PET-adapted therapy has become the basis of modern HL therapy. The second equally interesting direction presented at the conferences this year is the widespread use of targeted (brentuximab vedotin) therapy and immunotherapy (nivolumab, pembrolizumab) in combination with standard chemotherapy not only in patients with relapsed/refractory HL, but also as first-line therapy, including the patients of an elderly section. The combination of brentuximab vedotin and immunotherapy during all lines of HL treatment, as well as brentuximab vedotin and immunotherapy consolidation after high-dose chemotherapy and autologous stem cell transplantation are another direction to improve the efficacy of therapy in patients with HL.

Irinotecan Plus Temozolomide for Relapsed or Refractory Neuroblastoma

2006 ◽  
Vol 24 (33) ◽  
pp. 5271-5276 ◽  
Author(s):  
Brian H. Kushner ◽  
Kim Kramer ◽  
Shakeel Modak ◽  
Nai-Kong V. Cheung
Keyword(s):  
Quality Of Life ◽  
Bone Marrow ◽  
Platelet Count ◽  
Progressive Disease ◽  
High Dose ◽  
Granulocyte Colony Stimulating Factor ◽  
Colony Stimulating Factor ◽  
Lymphocyte Responses ◽  
Stimulating Factor

Purpose To report on an irinotecan and temozolomide regimen for neuroblastoma (NB). Quality of life and minimizing toxicity were major considerations. Patients and Methods The plan stipulated 5-day courses of irinotecan 50 mg/m2 (1-hour infusion) and temozolomide 150 mg/m2 (oral) every 3 to 4 weeks, with a pretreatment platelet count more than 30,000/μL. Granulocyte colony-stimulating factor was used when the absolute neutrophil count was less than 1,000/μL. Results Forty-nine NB patients received 1 to 15 courses (median, 5). Gastrointestinal and myelosuppressive toxicities were readily managed. Lymphocyte responses to phytohemagglutinin after 2 to 10 courses (median, 3.5) were normal in 10 of 10 patients treated after nonimmunosuppressive therapy, and normalized in five of seven patients first treated less than 2 months after high-dose alkylators. Of 19 patients treated for refractory NB and assessable for response, nine showed evidence of disease regression, including two complete responses and seven objective responses. Of 17 patients treated for progressive disease, three showed evidence of disease regression, including one partial response and two objective responses. Multiple courses entailed no cumulative toxicity and controlled disease for prolonged periods in many patients, including some who were unable to complete prior treatments because of hematologic, infectious, cardiac, or renal problems. Conclusion This regimen has anti-NB activity, spares vital organs, is feasible with poor bone marrow reserve, causes limited immunosuppression, and allows good quality of life.

scholarly journals Disseminated Mycobacterium Sepsis with Bone Marrow, Liver, and Lung Involvement Following Intravesical Bacillus Calmette-Guerin (BCG) Therapy

2019 ◽  
Vol 14 (2) ◽  
pp. 34-37
Author(s):  
Drew Hager ◽  
Zeenib Kohja ◽  
Terry Wuerz ◽  
Arjuna Ponnampalam
Keyword(s):  
Bone Marrow ◽  
High Dose ◽  
Case Presentation ◽  
First Line Therapy ◽  
Elevated Liver Enzymes ◽  
Bcg Therapy ◽  
Intravesical Bcg ◽  
History Of ◽  
Muscle Invasive ◽  
High Index Of Suspicion

Introduction: BCG therapy is first line therapy for high grade non-muscle invasive bladder cancer (NMIBC).Case Presentation: A 54-year-old male presented with fevers, rigors and hematuria one week following intravesical BCG administration for treatment of NMIBC. He developed fever, pancytopenia, elevated liver enzymes and pulmonary infiltrates with progression of symptoms despite broad spectrum antimicrobial therapy. A bone marrow biopsy showed granulomatous infiltration; cultures of urine demonstrated growth of Mycobacterium bovis. A diagnosis of disseminated BCG infection secondary to intravesical administration was made; rifampin, isoniazid, ethambutol, and high dose prednisone were initiated.Conclusion: Adverse events associated with BCG administration have been attributed to both the primary mycobacterium infection and to hypersensitivity reactions. Timely collection of histopathology can lead to early treatment of disseminated BCG with good outcomes. Internists should have a high index of suspicion for patients presenting with organ dysfunction with an immediate or remote history of intravesical BCG administration.

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