P0327SUCCESSFUL ACHIEVMENT OF LONG-TERM COMPLETE REMISSION OF LUPUS NEPHRITIS IN A PATIENT WITH SEVERE SYSTEMIC LUPUS ERYTHEMATOSUS AND SECONDARY INFECTIOUS COMPLICATOINS

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Anna Bespalova ◽  
Tatiana Bondarenko ◽  
Natalya Kozlovskaya

Abstract Background and Aims A significant contribution to the structure of mortality in SLE is made by infectious complications, which complicate the course of SLE and complicate the management of patients due to the impossibility of prescribing immunosuppressive therapy in full. Method A clinical case of successful achievement of LN remission in a patient with severe SLE and secondary APS is described, despite the development of secondary infectious complications and the inability to fully immunosuppressive therapy. Results A 42-year-old woman has been observed at the clinic from autumn 2013 to the present. Since the end of Aug.2013 she got a severe dyspnea, generalized edema, ulcers of the legs, blood pressure persistently increased to 180/110 mm Hg, diuresis decreased. Laboratory examination at admission in Oct.2013: (table 1), polyserositis, non-inclusive thrombosis of the posterior tibial vein on the left. An immunological examination: anti-dsDNA 101.6 U/L, ANA 5.2, C3 complement fragment 0.29 g/L, antibodies to beta-2-glycoprotein-1 55.7 U/ml, positive lupus anticoagulant. Intravenous methylprednisolone at a dosе of 1000 mg/day for 3 days plus high dose CYC at a dosе of 800 mg/day for 1 days was started, followed by prednisone 60 mg/day. This intravenous pulsewas repeated after 3 weeks. Renal biopsy in Nov.2013: diffuse proliferative glomerulonephritis with 7% cellular crescents, ISN/RPS class IV LN. Positive dynamics in Dec.2013 (table 1). By mid-Dec.2013, the patient had complaints of pain and limited range of motion in the right shoulder joint, the appearance of edema in this region. According to the puncture of the joint cavity, MRI, purulent arthritis of the right shoulder joint was diagnosed with the formation of intermuscular phlegmon of this area. Sepsis was diagnosed. The patient underwent emergency surgical drainage of phlegmon, followed by repeated revisions of this area. Prescribed meropenem at a dose of 3 g/day. Implementation of the complete immunosuppressive therapy protocol was impossible; a gradual decrease in the dose of prednisone per os was started. Due to the continued activity of SLE, in January 2014, a repeated intravenous pulse methylprednisolone without СYС was performed. Since Dec.2013, the patient has not received cystostatics. But despite it positive dynamics in Feb.2014 (table 1) . In Mar.2014 current treatment was hydroxychloroquine 400 mg/day, prednisone 15 mg/day, azathioprine 100 mg/twid. After 2 years of complete remission of LN in April 2016: prednisone 10 mg/day, hydroxychloroquine 400 mg/day, azathioprine 50 mg/twid. After another 2 years of complete remission of LN in 2018: prednisone 5 mg/day, hydroxychloroquine 400 mg/day. During the observation, a complete clinical and laboratory remission of SLE was noted. Laboratory examination at admission in Oct.2019: complete remission of SLE (table 1). The patient takes prednisone 5 mg/day, hydroxychloroquine 400 mg/day. The patient has limited mobility of the right shoulder joint, but complete loss of function did not occur. According to the control MRI there are the formation of aseptic necrosis of the head of the right humerus. Conclusion Infectious complications remain one of the leading causes of mortality in patients with SLE. The uniqueness of this clinical case in the successful achievement of long-term complete remission of SLE, despite the secondary infectious complications. The success of achieving long-term remission of SLE is associated with the onset of immunosuppressive therapy as soon as possible from the debut of the disease, with the conduct in the maximum allowable volumes. The morphological picture with a small % cellular crescents was also a favorable factor.

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 2154-2154
Author(s):  
Emma Hernlund ◽  
Josefine Redig ◽  
Åsa Rangert Derolf ◽  
Bjorn Paulsson ◽  
Martin Höglund ◽  
...  

Introduction: AML affects all ages with an incidence rate of 5 per 100,000, but is much more frequent in older population. The overall lifetime risk of AML is estimated to be 0.5-1%. Long-term overall survival in younger (age < 60 years) is about 50%, but much worse among older population. Although AML therapy is one of the most resource-intensive cancer treatments, there are few estimates of the resource use and economic burden by treatment phase. Methods: This study was a retrospective database study performed on Swedish national data. Adult patients (age ≥18 years) diagnosed with AML in Sweden between 2007 and 2015 were identified in the Swedish Cancer Registry, along with vital status. Data on resource use were collected from national registers for inpatient- and outpatient specialized care and prescribed drugs. Information on diagnostics and treatment was accessed from the Swedish national AML Registry (SwAMLR). Data on sick leave (SL) and early retirement (ER) came from the Swedish Social Insurance Agency (absent days costed with the mean salary in Sweden). Hospital care resource use was costed using diagnosis-related group (DRG) remunerations, and include cost of inpatient drugs. The mean cost from the defined start of the treatment phase until the end of the treatment phase was divided by the mean number of days for the corresponding treatment phase to estimate the mean cost per day. The defined treatment phases were restricted to a maximum of 5 years. All costs are represented in US$. Results: Of the 2,954 patients identified in the Swedish Cancer Registry, 1,772 patients with a median age of 64 years were identified in the SwAMLR as fit for receiving high-dose chemotherapy . Of these, 1,243 were recorded with both curative intent of treatment and dates for achieving complete remission. Mean costs from the first AML-related hospital admission until the date of complete remission amount to $27,244. The mean number of days for the corresponding period were 45.16, resulting in a mean cost per day of $603 from first admission to first complete remission. The corresponding cost per day for patients recorded with curative intent but no complete remission (n=428) are $494. Time was counted from first AML-related admission until 90 days after first admission, or SCT or death, whichever occurred first. Costs after complete remission to either relapse, SCT, death or re-induction (n=1,237) amount to $50,793 for a mean of 438.63 days ($116/day). This treatment phase includes long-term survivors, whereas the costs from SCT, relapse or re-induction are not included. From relapse to death, the total cost is almost twofold for patients with re-induction (n=350) compared to palliative treatment (n=254). Cost per day amount to $179 for patients with palliative treatment and $256 for patients with re-induction treatment, respectively. The cost per day from date of SCT to death (n=511) is estimated to $192, incurred over a long period of time (mean number of days 844.02). The age of transplant recipients ranged between 18-71 years, with a median of 52 years. Conclusions: Costs of AML up to remission are feasible to estimate through DRG-costing methods, and studies have shown these costs are intense. Indeed this study shows that the highest cost per day is observed from first admission to complete remission. In addition results from our study show that there are high costs incurred also in the long-term, i.e. after remission. Of the included treatment phases the total cost from date of SCT to death is the largest, amounting to over $160,000. Approximately 20% are due to SL/ER, which is the second largest cost component after inpatient costs accounting for 60% of the total costs. Table. Disclosures Hernlund: ICON: Employment. Redig:ICON: Employment. Paulsson:Novartis: Employment. Vertuani:Novartis: Employment.


2021 ◽  
Vol 7 (2) ◽  
pp. 12-16
Author(s):  
A. Fleyshman ◽  
Elena Belyaeva

A case of severe systemic osteoporosis complicated by multiple fractures of the ribs and vertebrae in a young patient with late diagnosis of Still's disease as a result of independent long-term uncontrolled use of corticosteroids is presented. Lack of medical supervision, taking a high dose (35 mg / day) of prednisolone without recommendation and control of treatment led to untimely recognition and treatment of the underlying disease and the development of irreversible disabling transformations of the skeleton.


1997 ◽  
Vol 15 (2) ◽  
pp. 528-534 ◽  
Author(s):  
V Bonfante ◽  
A Santoro ◽  
S Viviani ◽  
L Devizzi ◽  
M Balzarotti ◽  
...  

PURPOSE This study analyzed long-term results in patients with Hodgkin's disease who were resistant to or relapsed after first-line treatment with MOPP and ABVD. Response to salvage treatments and prognostic factors were also evaluated. PATIENTS AND METHODS The study population included 115 refractory or relapsed patients among a total of 415 patients treated with alternating or hybrid MOPP-ABVD followed by radiotherapy (25 to 30 Gy) to initial bulky sites. The median follow-up duration of the present series was 91 months. Thirty-nine of 115 patients (34%) showed disease progression while on primary treatment (induction failures); 48 relapsed after complete remissions that lasted < or = 12 months and 28 after complete remission that lasted more than 12 months from the end of all treatments. RESULTS At 8 years, the overall survival rate was 27%, being 54% and 28% in patients whose initial complete remission was longer or shorter than 12 months, respectively, and 8% in induction failures (P < .001). Response to first-line chemotherapy and disease extent at first progression significantly influenced long-term results, as well as the incidence and duration of complete remission. CONCLUSION The present data confirm previous observations that showed the main prognostic factors to influence outcome after salvage treatment are response duration to first-line therapy and disease extent at relapse. The results indicate that patients who relapse after the alternating MOPP/ABVD regimen have a prognosis similar to that of patients who relapse after a four-drug regimen (MOPP or ABVD alone). Re-treatment with initial chemotherapy seems the treatment of choice for patients who relapse after an initial complete remission that lasts greater than 12 months, while the real impact of high-dose chemotherapy or new regimens should be assessed in resistant patients.


Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 132-132
Author(s):  
Justin M Watts ◽  
Lynette Zickl ◽  
Mark R Litzow ◽  
Selina M Luger ◽  
Hillard M Lazarus ◽  
...  

Abstract Abstract 132 Late relapse in acute myeloid leukemia (AML) has been infrequently studied and variably defined in the literature. Two series have shown that late relapse of AML ≥5 years after first complete remission (CR1) is uncommon, with rates of 1.19–3% (Medeiros et al, Leuk Lymphoma 2007; Verma et al, Leuk Lymphoma 2010). We searched the long-term data available on 784 adults (<60 years-old) who were treated on 1 of 4 ECOG clinical trials (E3483, PC486, E3489, or E1900) and achieved CR1 for reports of late relapse (defined as recurrence of AML ≥3 years after CR1). Median follow-up for the 553 patients last known alive was 11.1 years. The longest median follow-up was 17.2 years on trial PC486. Outcomes We found that 11 patients (1.4%) relapsed late; of these, 2 were treated on E3483, 1 on PC486, 5 on E3489, and 3 on E1900. Seven patients with late relapse died from their disease and 4 were living at last known follow-up. Only 1 patient (0.13%) had recurrence of AML ≥5 years after achieving CR1. It is possible that more late relapses will occur on E1900 (a more recent study with ongoing follow-up). All of these trials except E3483 treated some patients with autologous hematopoietic cell transplantation (autoHCT) as part of post remission therapy. On PC486, no post remission consolidation chemotherapy was administered before autoHCT. Ninety-eight total patients on E3489 and PC486 received autoHCT, and there were no late relapses; on E1900, 2 of the 141 patients treated with autoHCT developed late relapse. No patients who underwent allogeneic (allo) HCT in CR1 experienced late relapse on any of the 4 clinical trials. Nine of the 11 patients with late relapse did not undergo HCT; of these, 5 were consolidated with high-dose cytarabine, 2 received maintenance with low-dose cytarabine and 6-thioguanine, and 2 received unknown post remission therapy. Of the 3 patients with late relapse on E1900, 2 received standard-dose and 1 high-dose daunorubicin with induction. Conclusions Across all 4 trials, only 2 of the 239 patients (0.8%) treated with post remission autoHCT experienced late relapse of AML (≥3 years after CR1), which reinforces previously published data that late relapse after autoHCT is uncommon (Cassileth et al, J Clin Oncol 1993). Furthermore, of the 35 patients treated with autoHCT on PC486, 11 relapsed early and no patients relapsed late, suggesting that post remission chemotherapy may not be necessary before autoHCT. Based on this large AML cohort of nearly 800 patients with long-term follow-up, patients who remain in CCR for at least 3 years have a very low risk of relapse and can be considered cured of their disease. Moreover, given that recurrent AML was extremely rare after 5 years or more of CCR (<0.2%), the risk of therapy-related AML from contemporary induction and post remission strategies including HCT appears to be minimal. Disclosures: No relevant conflicts of interest to declare.


2021 ◽  
Vol 2 (3) ◽  
pp. 42-47
Author(s):  
O. Yu. Kutsevalova ◽  
Yu. Yu. Kozel ◽  
N. E. Nifantiev ◽  
A. V. Antonets ◽  
V. B. Krylov

The regimens of anticancer therapy have been intensified and methods of high-dose chemotherapy (HDCT) have been introduced for recent years which made it possible to achieve significant progress in the results of tumor treatments. Intensification of chemotherapy regimens in cancer patients leads to the emergence of risk factors of invasive candidiasis (IC) development: agranulocytosis, disruption of the integrity of the mucous membranes, prolonged use of CVC, repeated antibiotic therapy, long-term parenteral nutrition. Thus, intensification of anticancer therapy may be accompanied by an increase in infection-mediated mortality.IC is the most common invasive mycosis in Russia. More than 11 thousand cases of IC occur in our country every year. The frequency IC in Russia is 8.29 per 100 thousand of the population, which corresponds to the results of the LIFE study in European countries where this indicator varies from 2.2 to 11 per 100 thousand of the population. There are no clinical signs or symptoms specific for IC. It develops in patients with concomitant diseases, which significantly complicates the diagnosis. In this regard, an urgent issue is to improve the diagnosis of candidal infectious complications in cancer patients in order to optimize treatment by studying serological markers that have the greatest value in the diagnosis of infectious complications in cancer patients.


2021 ◽  
Vol 2 (1) ◽  
pp. 109-114
Author(s):  
E. V. Solovyoval ◽  
N. A. Popova ◽  
T. V. Vlasoval ◽  
M. L. Gorbunova ◽  
L. N. Antsygina

Arhythmogenic right ventricular dysplasia (ADP) refers to hereditary myocardial diseases, in which there are structural and functional disorders in the right ventricular myocardium, causing rhythm and conduction disorders, including fatal ventricular arrhythmias. ADP is considered one of the most common causes of sudden cardiac death in young people and people who are engaged in sports. However, in practice, there are cases of this disease in people of an older age category. Diagnosis of ADP is still difficult due to the possible long-term asymptomatic course of the disease. The article describes a clinical case of ADP in a 48-year-old man.


2021 ◽  
pp. 79-82
Author(s):  
O.M. Okhotnikova ◽  
◽  
T.M. Tkachоva ◽  
A.S. Andriyko ◽  
I.O. Kurian ◽  
...  

The article provides a review of literature on the long-term consequences of Lyell's syndrome (one of the forms of toxic epidermal necrolysis). Among them: chronic eczema with xerosis and itching, skin depigmentation, hypertrophic and keloid scars, nail lesions, eyes, including dry eye syndrome and chronic conjunctivitis with/without obliteration of the nasolacrimal canal, kidney disease, lung disease, Sjogren's syndrome, Hashimoto's thyroiditis and others. Clinical case. The authors present their clinical case of an 11-year-old girl who suffered from Lyell's syndrome at the age of 8 and has been observed for the past three years, share their clinical and laboratory-instrumental observations and treatment strategies. In particular, they report about the revision of the clinical diagnosis with which the girl was admitted to the OHMADYT hospital, from BA to chronic secondary obstructive bronchitis, the detection of chronic conjunctivitis with obliteration of the nasolacrimal canals, chronic sinusitis, secondary immune deficiency, keloid skin scars. Conclusions. Despite constant rehabilitation therapy and a significant improvement in the general condition of the girl, it was not possible to fully restore the function of mucociliary transport, as a result ventilation of small bronchi gradually deteriorates, bronchiectasis is observed in the basal segments of the right lung, a polyserial strain S. aureus is formed, which is constantly sown from the child's sputum; it was not possible to fully restore the patency of the nasolacrimal canals and achieve a stable remission of chronic sinusitis. The research was carried out in accordance with the principles of the Helsinki Declaration. The informed consent of the patient was obtained for conducting the studies. No conflict of interest was declared by the authors. Key words: Lyell's syndrome, children, Long-term consequences, chronic obstructive bronchitis, obesity, treatment.


2021 ◽  
Vol 6 (5) ◽  
pp. 178-183
Author(s):  
D. V. Menshova ◽  
N. S. Ponomarenko ◽  
I. A. Kuklin

The frequency of rotator cuff injuries in people over 45 years of age is 25.6–50 %, and  40  % of these injuries are massive. Shoulder rotator cuff injury causes disorders in biomechanics of the shoulder joint such as anterior-superior dislocation of the humeral head. Injury of the deltoid muscle combined with a massive rupture of the rotator cuff causes proximal dislocation of the humeral head during any active movement. In  the  treatment of these cases, surgical methods of treatment are used, such as transposition of the latissimus dorsi muscle, proximal capsule plasty, and reverse shoulder arthroplasty. We present a successful clinical case of treatment of a patient with chronic massive injury of the right shoulder joint rotator cuff tendons in combination with the injury of shoulder joint proximal capsule, dislocation of the right humerus head, and hypotrophy of the anterior portion of the deltoid muscle. We performed transposition of the latissimus dorsi tendon in combination with shoulder joint proximal capsule plasty with an autograft of the peroneal longus tendon. The check-up X-rays show that the dislocation of the right shoulder joint was eliminated. In the early postoperative period, the patient started physiotherapy exercises of the operated limb using abduction pillow. By the 7th day after surgery, the abduction of the operated limb reached 70°. The described surgical technique allows to restore congruence in the shoulder joint and the function of the injured limb in severe multiple injuries of the shoulder joint structures. 


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