scholarly journals Retrospective Study of High Hemoglobin Levels in 56 Young Adults

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 1262-1262
Author(s):  
Alexandra Desnoyers ◽  
Michel Pavic ◽  
Jean-Francois Castilloux ◽  
Patrice Beauregard ◽  
Line Delisle ◽  
...  
Keyword(s):  
Young Adults ◽  
Complete Blood Count ◽  
Conflicts Of Interest ◽  
University Hospital ◽  
Jak2 Mutation ◽  
Female Ratio ◽  
Myeloproliferative Syndrome ◽  
Age Range ◽  
Made In

Abstract Introduction. Erythrocytosis is a common reason for a hematology consultation. Most of the patients are over 50 years of age. The diagnostic approach is already well established, but determining the etiology of erythrocytosis in the young adult may be a real diagnostic challenge. Hereditary causes are often first suspected, but the real etiologic panorama of erythrocytosis in this population is still poorly understood. Methods. This Canadian single-center retrospective observational study was conducted in a Québec university hospital over a period of 20 years (1995 - 2015). Data were collected by a single investigator in a standardized database. From a computer-generated query over a chosen period of time, all patients between 16 and 35 years of age with high levels of hemoglobin or hematocrit were selected (hemoglobin > 185 g/L and/or hematocrit > 0.52 in men; hemoglobin > 165 g/L and/or hematocrit > 0.48 in women). Files were analyzed after approval from the scientific and ethical committees. Results. 426 patients fulfilled the above criteria (on a total of 113 453 complete blood counts conducted in the same age range during the analysis period), but only 56 benefitted from further investigations. Only those cases were included in the study. The male-to-female ratio was 5 : 3. Among them, 59% were smokers, 11% had a high alcohol intake and 27% were using drugs on a regular basis. Obesity was present in 43% of patients (morbid obesity in nearly one third of cases). More than half of the patients had one visit or less with a specialist for investigation of their erythrocytosis. Among those who had a medical follow-up, the median length is 42 months. Erythropoietin was measured in 23% of cases and the JAK2 mutation was screened in 18% of cases. The diagnosis of an acquired polycythemia was made in 41% of cases: hypoxemia (n= 13), renal disorders (n = 6) and drug-induced (n= 4). No case of myeloproliferative syndrome was identified. Two cases were associated with congenital polycythemia (high oxygen affinity hemoglobinopathy). Diagnosis of relative polycythemia was made in slightly over 10% of cases (n= 7). In 24 patients, no conclusive cause could be identified. One third of those patients (n= 8) were hospitalised for mental health decompensation or self-induced intoxication when high hemoglobin levels were detected. When JAK2 mutation testing was not available, the complete blood count was further analyzed and it did not provide any evidence for myeloproliferative syndrome. One in five patients received treatment for their polycythemia, whatever the cause: aspirin (n= 7) and phlebotomy (n= 5). There has been no reported patient death associated with polycythemia or its treatment. Only two major complications occurred: thrombosis (n =1) and bleeding (n= 1). Conclusion. This is the first study to specifically analyze erythrocytosis in young adults. Despite the retrospective design, it demonstrates patient management often inconsistent with the actual recommendations and a lack of follow-up after initial investigations. Congenital polycythemia deserves to be systematically evoked in this age range, but represents only a small part of cases. Acquired polycythemia dominates the etiologies whereas myeloproliferative syndromes were not depicted in this study. Harmonisation of patient care for young adults with erythrocytosis is therefore highly desirable. Disclosures No relevant conflicts of interest to declare.

CLINICAL FEATURES, INDICATIONS AND RESULT OF TRACHEOTOMY

2017 ◽  
pp. 50-55
Author(s):  
Duc Luu Ngo ◽  
Tu The Nguyen ◽  
Manh Hung Ho ◽  
Thanh Thai Le
Keyword(s):  
Clinical Features ◽  
University Hospital ◽  
Female Ratio ◽  
Tube Obstruction ◽  
Level Ii ◽  
Study Results ◽  
Male Female Ratio ◽  
Hospital Patients ◽  
Level I

Background: This study aims to survey some clinical features, indications and results of tracheotomy at Hue Central Hospital and Hue University Hospital. Patients and method: Studying on 77 patients who underwent tracheotomy at all of departments and designed as an prospective, descriptive and interventional study. Results: Male-female ratio was 4/1. Mean age was 49 years. Career: farmer 44.2%, worker 27.2%, officials 14.3%, student 7.8%, other jobs 6.5%. Respiratory condition before tracheotomy: underwent intubation 62.3%, didn’t undergo intubation 37.7%. Period of stay of endotracheal tube: 1-5 days 29.2%, 6-14 days 52.1%, >14 days 18.7%. Levels of dyspnea before tracheotomy: level I 41.4%, level II 48.3%, level III 0%, 10.3% of cases didn’t have dyspnea. Twenty cases (26%) were performed as an emergency while fifty seven (74%) as elective produces. Classic indications (37.7%) and modern indications (62.3%). On the bases of the site, we divided tracheostomy into three groups: high (0%), mid (25.3%) and low (74.7%). During follow-up, 44 complications occurred in 29 patients (37.7%). Tracheobronchitis 14.3%, tube obstruction 13%, subcutaneous empysema 10.4%, hemorrhage 5%, diffcult decannulation 5.2%, tube displacement 3.9%, canule watery past 2.6%, wound infection 1.3%. The final result after tracheotomy 3 months: there are 33 patients (42.9%) were successfully decannulated. In the 33 patients who were successfully decannulated: the duration of tracheotomy ranged from 1 day to 90 days, beautiful scar (51.5%), medium scar (36.4%), bad scar (12.1%). Conclusions: In tracheotomy male were more than female, adult were more than children. The main indication was morden indication. Tracheobronchitis and tube obstruction were more common than other complications. Key words: Tracheotomy

PS01.030: ROBOTIC LAPAROSCOPIC HELLER-DOR FOR ACHALASIA: SINGLE INSTITUTION’S EXPERIENCE ON 89 FIRST PATIENTS

2018 ◽  
Vol 31 (Supplement_1) ◽  
pp. 58-58
Author(s):  
Jari Rasanen
Keyword(s):  
Operative Time ◽  
Conflicts Of Interest ◽  
Symptom Relief ◽  
Symptom Control ◽  
University Hospital ◽  
Dor Fundoplication ◽  
Robot Assisted ◽  
Proximal Stomach ◽  
Laparoscopic Myotomy

Abstract Background Robotics allows precise dissection during the Heller-Dor. We wanted clarify whether this has produced good results in achalasia treatment of our patients in our institution. Methods We reviewed eighty-nine patients who were treated with robot-assisted laparoscopic Heller-Dor between October 2010 and January 2018 at Helsinki University Hospital. They all underwent laparoscopic myotomy for achalasia extending 8 cm onto esophagus and 3 cm onto proximal stomach with partial Dor fundoplication by two surgeons. Diagnosis of achalasia was confirmed by radiography, endoscopy, and manometry. Success of the myotomy was verified by intraoperative EGD, postoperative contrast radiography, and subjective postoperative symptom recording. Results There were 44 men and 45 women, with a mean age 43 + /- 15 years. Thirty-three percent of patients reported weight loss and 95% of patients experienced dysphagia at least once every week preoperatively. Mean operative time was 124 + /- 34 minutes. There were no conversions. Intraoperatively there were 6 patients with minor tears in mucosa of esophagus and 1 patient with minor tear in the mucosa of stomach. They were all recognized and repaired intraoperatively without any significant consequences to the patients (no deaths or ICU admissions). Median hospitalization was 4 days (2 - 17). Postoperatively 93% of patients reported significant improvement in dysphagia. All 6 patients with intra-operative mucosal tear experienced good or excellent symptom relief. Three of patients experienced significant pain in their esophagus even after surgery. All patients but 3 rated their overall symptom control either excellent or good after median follow-up of 31 months. Conclusion Robotic-assisted laparoscopic Heller-Dor is feasible with good median term results although some patients may experience intraoperative mucosal tears during the learning curve Disclosure All authors have declared no conflicts of interest.

PS01.171: DOUBLE-PEDICLED FREE JEJUNAL TRANSFER FOR RECONSTRUCTION OF PHARYNGOLARYNGOESOPHAGECTOMY

2018 ◽  
Vol 31 (Supplement_1) ◽  
pp. 97-99
Author(s):  
Hiromi Mukaide ◽  
Taku Michiura ◽  
Kentaro Inoue ◽  
Hirokazu Miki ◽  
Keigo Yamamichi ◽  
...  
Keyword(s):  
Anastomotic Leakage ◽  
Conflicts Of Interest ◽  
University Hospital ◽  
Perioperative Outcomes ◽  
Arteriovenous Anastomoses ◽  
Vascular Thrombosis ◽  
Transfer Group ◽  
Free Jejunal Transfer ◽  
Medical University ◽  
Age Range

Abstract Background We recently selected double-pedicled free jejunal transfer for reconstruction of pharyngolaryngoesophagectomy to reduce the vascular thrombosis-induced necrosis in free jejunal transfer. We herein report our experience with this procedure. Methods Single-pedicled free jejunal transfer for reconstruction of pharyngolaryngoesophagectomy was performed from January 2006 to November 2013, and double-pedicled free jejunal transfer (i.e. two pairs of jejunal arteriovenous anastomoses) was performed from December 2013 to December 2016 in Kansai Medical University Hospital. We compared the perioperative outcomes and complications between these two procedures. Results Sixty-two patients (58 men, 4 women; median age, 66 years; age range, 51–83 years) underwent single-pedicled free jejunal transfer. Twenty-eight patients (25 men, 3 women; median age, 70 years; age range, 47–84 years) underwent double-pedicled free jejunal transfer. Twenty-eight patients received preoperative treatments. Table 1 shows the perioperative outcomes and complications. Conclusion No thrombus of the free jejunum occurred, no anastomotic leakage occurred, and all flaps survived in the double-pedicled free jejunal transfer group. We believe that double-pedicled free jejunal transfer for reconstruction of pharyngolaryngoesophagectomy is a reliable and useful procedure. Disclosure All authors have declared no conflicts of interest.

Analysis of the Clinical Features and the Prognosis of Acute Lymphoblastic Leukemia with Complex Chromosomal Aberrations

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 4914-4914
Author(s):  
Xiujin Ye ◽  
Lixia Zhu ◽  
Li Li ◽  
Jie Zhang ◽  
Jingsong He ◽  
...  
Keyword(s):  
Chromosomal Aberrations ◽  
Conflicts Of Interest ◽  
Lymphoblastic Leukemia ◽  
Allogeneic Bone Marrow Transplantation ◽  
Philadelphia Chromosome ◽  
Survival Outcome ◽  
Allogeneic Bone ◽  
Female Ratio ◽  
Laboratory Features

Abstract Abstract 4914 Background: Acute lympoblastic leukemia (ALL) with complex chromosomal aberrations (CCAs) which patients harboring three or more acquired chromosomal aberrations always have a poor outcome. Philadelphia chromosome–positive (Ph+) ALL is the largest genetically defined subtype in adult ALL with the most unfavorable prognosis. It is necessary to investigate the clinical and laboratory features of ALL patients with CCAs, analyze the distribution of Ph+ in these patients and assess the prognosis. Methods: In a retrospective follow-up study from 2005.1∼2011.6, 38 ALL patients with CCAs were investigated. All cases were diagnosed with morphological,immunological,cytogenetic classification and molecular biology. The karyotypes were interpreted according to the International System for Human Cytogenetic Nomenclature (ISCN). Clinical characteristics, laboratory features, treatment response and prognosis of ALL patients with CCAs were analyzed. Results: The study included 17 Ph+ ALL patients with an age range of 12 to 81 years (mean, 34.5 years) and a male to female ratio of 1:1. Ph+ ALL patients with CCAs were older than Ph- cases (P=0.096). The median survival time was 9.5 months (range, 1–44 months). The complete remission (CR) rate after two cycles of systemic chemotherapy was 53.6% (15/28) and the relapse rate was 53.3% (8/15). Ph+ ALL patients with CCAs showed a lower CR rate than Ph- cases. Eleven patients died during the follow-up period, six of them died within 2 months from the initial ALL diagnosis, and nine patients were failure to achieve CR. Three patients underwent allogeneic bone marrow transplantation (BMT): two of them presented Ph+ relapsed after 2 and 10 months, respectively. Using Kaplan-Meier survival analysis, we found the survival outcome of Ph+ ALL patients with CCAs is inferior to Ph- cases. Conclusion: Treatment outcomes of ALL patients with CCAs receiving chemotherapy or BMT are very poor. Ph+ as an unfavorable parameter influences on the CR rate and survival outcome. It is necessary to accumulate more clinical data to find innovative treatments that can improve the prognosis of this refractory leukemia. Disclosures: No relevant conflicts of interest to declare.

Interest of the XN-10® Analyzer to Screen for Myelodysplastic Syndromes on Complete Blood Count (CBC) Especially with Hemoglobin Levels and Neut-WX

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 5518-5518
Author(s):  
Robin Boutault ◽  
Sebastien Tremblais ◽  
Mathilde De Oliveira Lopes ◽  
Pierre Peterlin ◽  
Yannick Le Bris ◽  
...  
Keyword(s):  
Myelodysplastic Syndromes ◽  
Blood Count ◽  
Complete Blood Count ◽  
Conflicts Of Interest ◽  
Normal Karyotype ◽  
University Hospital ◽  
Multilineage Dysplasia ◽  
End Point ◽  
Group 2 ◽  
A Prospective Study

Abstract A prospective study was performed over one year at Nantes University Hospital in France, in order to investigate whether suspected myelodysplastic syndromes (MDS) could be detected on a complete blood count (CBC), the most rapid laboratory investigation. Indeed, the recently developed XN-10® (Sysmex, Kobe, Japan), provides novel CBC parameters witch could be useful to discriminate such patients from normal samples or from cytopenia of other etiology. Seventy-nine patients were enrolled in the study, for whom a diagnosis of MDS was concluded based on CBC, bone marrow smears examination and karyotype. All patients were free of treatment, including transfusions, at inclusion. They were 40 men and 39 women with a median age of 77,9 years (range 36,4-92,4). CBC were performed on a Sysmex analyzer XN-10®, including investigation of reticulocytes and fluorimetric analysis of platelets. For comparison with normal values, results from 776 healthy samples, for which CBC were performed on the same analyzer and generated no flag, were used. All had parameters within the normal range according to age. The classical parameters of hemoglobin level, Mean Corpuscular Volume (MCV), reticulocytes, platelets and neutrophil counts were recorded. In addition, the extra-parameters, immature reticulocytes fraction (IRF%), platelets by fluorescence (PLT-F) and immature platelets fraction (IPF%), were taken into account. The neutrophils median position on the three axes as well as their dispersion (Neut-WX) were also measured by the analyser. The primary end-point was to discriminate between MDS and healthy patients and the secondary end-point was to distinguish MDS with excess blasts, MDS with multilineage dysplasia and MDS with single lineage dysplasia within the MDS group and by comparison with controls. According to the WHO 2016 classification, 27 patients in the cohort had MDS with excess blasts, 26 MDS with multilineage dysplasia (among whom 7 had ring sideroblasts [RS], group 2), 16 MDS-RS and single lineage dysplasia, 7 MDS with single lineage dysplasia and 3 MDS with isolated del(5q). Forty-four patients had a normal karyotype and 28 displayed anomalies classically reported in MDS, including 5 complex karyotypes. Among the latter, 4 were associated with MDS with excess blasts. Both classical and extra parameters indeed showed significant differences between the subgroups tested. Among the whole group of MDS patients, a number of parameters of all lineages were statistically different from the healthy cohort. The median level of hemoglobin was 9,8 g/dL (range 4,7-14,9), (p<0,0001), the median MCV 104,3 fL (range 75,4-123,9; p<0,0001), reticulocyte counts 44,3x109/L (range 8-165,9; p=0,041) and IRF% 16,7% (range 2,4-50,9; p<0,0001). An hemoglobin value below 11,5 g/dL was strongly suggestive of MDS with a sensitivity of 81% and specificity of 100%. The median platelet count was 164x109/L (range 8-505; p<0,0001) and median IPF% 8,8% (1,2-42; p<0,0001). Among leukocyte parameters, the MDS median neutrophil count was significantly lower at 2,15x109/L (range 0,17-13,67; p<0,001) and the Neut-WX value increased above 350. The latter, by itself, allowed to make a diagnosis of MDS with a sensitivity of 73,1% and a specificity of 96,9%. When considering the three MDS subgroups of MDS with excess blasts, multilineage or single lineage dysplasia, although each of them was significantly different from controls for hemoglobin levels, MCV, IRF% and neutrophil counts (p<0,0001), they could not be discriminated by these parameters. In the subgroup of MDS with single lineage dysplasia, platelet counts were similar to those of controls, yet significantly higher than for MDS with excess blast or with multilineage dysplasia (p=0,004 and p=0,029 respectively). Taken together, this study demonstrates that a simple CBC allows to screen for MDS using thresholds of 11,5 g/dL for hemoglobin and of 350 for Neut-WX. Blood smear examination should be performed in this situation even if the XN-10® analyzer does not raise an alarm, especially in unknown older patients. Disclosures No relevant conflicts of interest to declare.

scholarly journals Follow Up of COVID-19 Features in Recovered Adults without Comorbidities– Persistent Symptoms and Lab-Abnormalities

2020 ◽  
Author(s):  
Julian Varghese ◽  
Sarah Sandmann ◽  
Richard Vollenberg ◽  
Kevin Ochs ◽  
Inga Schrempf ◽  
...  
Keyword(s):  
University Hospital ◽  
Persistent Symptoms ◽  
Plasma Donation ◽  
Serum Iga ◽  
Significant Difference ◽  
Follow Up Care ◽  
Follow Up Studies ◽  
Age Range ◽  
Positive Effect

Abstract Background: With increasing numbers of patients recovering from COVID-19, there is increasing evidence for persisting symptoms and the need for follow-up studies. Methods: This retrospective study included patients without comorbidities, who recovered from COVID-19 and attended an outpatient clinic at a university hospital for follow-up care and potential convalescent plasma donation. Network analysis was applied to visualize symptom combinations and persistent symptoms. Comprehensive lab-testing was ascertained at each follow-up to analyze differences regarding patients with vs without persistent symptoms. Results: 116 patients were included, age range was 18-69 years (median: 41) with follow-ups ranging from 22 to 102 days. The three most frequent persisting symptoms were Fatigue (54%), Dyspnea (29%) and Anosmia (25%). Lymphopenia was present in 13 of 112 (12%) cases. Five of 35 cases (14%) had Lymphopenia in the later follow-up range of 80-102 days. Serum IgA concentration was the only lab parameter with significant difference between patients with vs without persistent symptoms with reduced serum IgA concentrations in the patient cohort of persisting symptoms (p=0.0219). Conclusion: Lymphopenia persisted in a noticeable percentage of recovered patients. Moreover, higher serum IgA concentration is associated with a positive effect regarding symptom persistence.

scholarly journals SUN-080 We Mind Your Step: Understanding and Preventing Drop-Out in the Transition from Paediatric to Adult Tertiary Endocrine Healthcare

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Kirsten Davidse ◽  
Anne-Loes Staa ◽  
Karlijn Pellikaan ◽  
Wanda Geilvoet ◽  
Judith Eck ◽  
...  
Keyword(s):  
Young Adults ◽  
Transition Process ◽  
Drop Out ◽  
University Hospital ◽  
Referral Letter ◽  
Endocrine Disorders ◽  
Physical Consequences ◽  
Lost To Follow Up ◽  
Regular Transition

Abstract Introduction Transition from paediatric to adult endocrinology is a challenge for adolescents, their families and their healthcare professionals. Previous studies show that up to 25% of young adults with endocrine disorders are lost to follow-up once they move out of paediatric care. This poses a health risk for young adults, as lack of medical treatment and surveillance can have both psycho-social and physical consequences. Apart from absenteeism from school or work, this can lead to serious and expensive medical complications like Addison crisis. Methods In 2019 we studied electronic medical records of 387 patients who were over 15 years old when they attended the paediatric endocrine outpatient clinic (OPC) of our medical centre in 2013-2014. We collected data from medical charts, the hospital digital agenda and medical correspondence. Results Of 387 adolescents, 161 (42%) did not need adult endocrine follow-up because paediatric endocrine care was only puberty- or growth-related. Forty-six patients did not enter regular transition because they 1) participated in a pilot to improve transition (N=10), 2) had intellectual disability (ID) and transferred to ID care (N=28), or 3) died (N=8, mostly cancer-related). Hundred-and-eighty patients entered regular transition: 49 (27%) to a regional hospital and 131 (73%) within our university hospital. Of these 131 patients, 33 (25%) were lost to follow up; in 24 of them (73%), the invitation for the adult OPC had never been sent. Loss to follow up occurred when three subsequent critical steps failed: 1) the adult endocrinologist had not received or read the paediatrician’s referral letter and/or had not invited the patient; 2) the paediatrician had not checked whether the appointment was really made and received by the patient and 3) the patients and/or caregivers had not alarmed the hospital when no invitation for an appointment was received. Conclusion We found a 25% dropout during transfer from paediatric to adult tertiary endocrine care. Starting the transition process early and in a structured manner, as well as assigning a transition coordinator, can prevent part of the dropouts. However, 73% of all dropouts appeared to be attributable to failure of practical, logistic steps. In order to prevent this part of the dropouts, we provide practical recommendations for all three parties involved: 1) the adult endocrinologist should carefully read paediatricians’ letters and check whether action is required (i.e. check whether an appointment is requested) 2) the paediatrician should ascertain whether the appointment is really made and received by the patient 3) the patients and/or caregivers should be instructed to alarm the hospital when they do not receive the appointment. These actions require relatively little effort and may prevent the part of drop-outs that is caused by logistic failures.

HAART Does Not Improve the Outcome of HIV-Related Multicentric Castleman Disease: Results of a Multicentric Retrospective Study

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 4918-4918 ◽  
Author(s):  
Chiara Cattaneo ◽  
Emanuela Vaccher ◽  
Alessandro Re ◽  
Salvatore Casari ◽  
Erika Borlenghi ◽  
...  
Keyword(s):  
Median Time ◽  
Conflicts Of Interest ◽  
Primary Effusion Lymphoma ◽  
Case Series ◽  
Central Nervous System Lymphoma ◽  
Castleman Disease ◽  
Time Interval ◽  
Female Ratio ◽  
Multicentric Castleman Disease

Abstract Abstract 4918 Background. Multicentric Castleman Disease (MCD) is a rare lymphoproliferative disorder, strictly related to Kaposi Sarcoma (KS) as both associated to HHV-8 infection. Like other HIV-related diseases, such as Non Hodgkin Lymphoma (NHL) and Primary Central Nervous System Lymphoma, MCD prevalence is known to be increased in HIV-pos subjects. However, limited case series among this subset of patients (pts) are reported and data regarding epidemiological variations in the pre and post HAART era are often non conclusive. In order to evaluate possible differences between pre- and post-HAART era, we retrospectively evaluated epidemiological and clinical characteristics of all pts affected by HIV-pos MCD afferent to two Italian Institutions. Patients and Methods. Data concerning biological, clinical and prognostic factors of HIV-pos MCD pts were collected and reported on a database. Pts were grouped according to the date of diagnosis in pre- (before 1997) and post-HAART (after 1997) era. Results. During a 21-year period (1990-2011), 35 HIV-pos MCD pts were observed at our Institutions, nine in the pre- and 26 in the post-HAART era. Male/Female ratio was 30/5; median age 37y (23-65). Histological subtype in 34 evaluable cases was hyaline-vascular in 6, plasmacytic in 20 and mixed cellularity in 8. Median time interval from HIV-pos detection to MCD diagnosis was 24 months (range 0–157) and CD4 count at MCD diagnosis 233/mcL (26-839). All these MCD baseline characteristics were not statistically different between pre- and post-HAART era. A concomitant diagnosis of KS was made in 18/35 (51.4%) cases, all but one in the post-HAART era. NHL was diagnosed concurrently with MCD in 2/35 (5.7%) pts (1 Primary Effusion Lymphoma, PEL, and 1 Plasmablastic Lymphoma, PBL); in 8/35 (22.8%) cases NHL developed after MCD diagnosis (2 PEL, 1 PBL, 2 Diffuse Large Cell, DLC, 1 unspecified, respectively). Median time from MCD to NHL was 19 mo (0-71). Evolution toward NHL was observed in 3 (33%) cases in the pre-HAART era and in 5 (19%) in the post-HAART era (p=0.39, Fisher's exact test). Six pts did not receive any type of treatment, 6 were treated with HAART only and 23 with different therapies, including antivirals, steroids, chemotherapy and rituximab (alone in 1 pt, in combination with chemotherapy in 5). Nineteen/23 pts received HAART together with other therapies. Two pts treated with rituximab developed NHL (1 PEL and 1 DLC). A complete or partial radiological response, together with clinical improvement was observed in 19/25 of evaluable pts (76%). Thirty pts were evaluable for relapse/progression, mainly in the post-HAART era. Overall, 19/30 pts showed MCD progression or transformation to NHL; median PFS was 15 months. Nineteen pts died and 5 were lost to follow-up. Overall survival (OS) of the entire series was 28 months, without significant differences between pre and post-HAART era (18 and 28 months, OR 0.643 [CI 0.2406–1.045], median follow-up 18 and 9 months, respectively). Causes of death were evaluable in 18 cases: NHL (7), MCD (6), opportunistic infections (1), liver cyrrhosis (1), acute myocardial infarction (1), KS (1) and therapy-related toxicity (1). NHL and MCD were the most frequent cause of death in the post-HAART era (4 and 5 of the 10 cases, respectively). Although no differences in OS between MCD pts without or with NHL were seen (19 vs 28 months, OR 0.6786 [CI 0.2763–1.081], median follow-up 13 and 23 months, respectively), eight/10 pts with NHL died, in comparison with 11/21 pts without NHL evaluable for outcome and median time from NHL diagnosis to death was 2 months (0-31). Conclusions. Our data confirm that the prognosis of HIV-related MCD remains poor even after the advent of HAART. Unlike other lymphoproliferative disorders, HAART did not impact on outcome of HIV-related MCD, suggesting that MCD can “escape” immune reconstitution. A concomitant diagnosis of NHL and uncontrolled MCD seem to be the main reason for an unfavourable outcome, particularly in the post-HAART era. New therapeutic approaches, including rituximab, should therefore aim at avoiding NHL transformation and controlling “MCD-related cytokine storm”. Disclosures: No relevant conflicts of interest to declare.

Association Of Stringed Response and Immunoparesis Normalization After Bortezomib-Based Therapy Is Related To Better Outcomes In Multiple Myeloma

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 5354-5354
Author(s):  
Marcio M Andrade Sr. ◽  
Ilda Murillo-Florez ◽  
Anel Montes-Limon ◽  
Beatriz de Rueda ◽  
Jose-Maria Grasa ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Partial Response ◽  
Conflicts Of Interest ◽  
Retrospective Chart Review ◽  
Standard Of Care ◽  
Complete Response ◽  
Response To Therapy ◽  
First Line ◽  
Female Ratio

Abstract Background Proteasoma inhibitors have proven to be one of the major advances on multiple myeloma (MM) therapy. Their principal effect in growth inhibition of MM cells is achieved not only through the inhibition of proteasomes but also by preventing the adhesion of myeloma cells to stromal cells, induction of cytokines by the microenvironment, decrease angiogenic activity and a direct apoptotic effect on MM cells. Actually it is part of the first-line standard of care therapy for patients with MM. On the other hand, multiple strategies have been developed for trying to predict response or improve the assessment of response and follow-up of MM patients. Currently, the International Myeloma Working Group (IMWG) criteria of response include immunophenotype and immunoparesis analysis. The HevyLiteTM and FreeLiteTM assays (The Binding Site Ltd. Birminghan. UK) permit a separate quantification of the amount of kappa- and lambda-bound to a given immunoglobulin (HLC) and the free light chains kappa or lambda amount quantification (FLC), both being excellent tools for immunoparesis assessment. Aims To analyze the usefulness of immunoparesis analysis by HevyLiteTM and FreeliteTM in patients who receive bortezomib-based therapy in our institution. Patients and Methods A retrospective chart review was performed including the patients diagnosed with secretor IgA or IgG MM who received therapy with bortezomib either at relapse or as first-line therapy. General clinical characteristics, therapy schedules, number of cycles, response to therapy according IMWG criteria and relapse were recorded. For the analysis, only patients with at least 4 cycles of bortezomib based regimen and HLC and/or FLC analysis performed between 4-12 weeks after complete therapy were included. Period of study: June 2004 to April 2013. Results At the end of study a total of 67 MM patients had received bortezomib-based therapy, 63 of them completed 4 or more cycles and were included in the analysis. Male/Female ratio: 31/32, mean age: 66.9 years old (46-81), therapeutic schedules were: bortezomib-prednisone: 3 (4.7%), bortezomib-dexametasone: 33 (51.6%), bortezomib-melfalan-prednisone: 18 (28.1%), bortezomib-dexametasone-lenalidomide: 8 (12.5%) and bortezomib-talidomide-dexametasone: 1 (1.6%). 55% of patients received at least 6 cycles of therapy. Immunoglobulin Myeloma subtype: IgAL: 13 (20.4%) patients, IgAK: 10 (15.6%) patients, IgGK: 32 (50.8%) patients and IgGL: 8 (14.1%) patients. A total of 46 (73%) patients showed an abnormal HLC ratio at diagnosis and 48 (76,2%) had immunoparesis before therapy; a total of 47 (74.6%) registered an abnormal FLC ratio at diagnosis. The response to therapy was: 15 (23.8%) of cases achieved a stringent complete response (SR), 3 (4.8%) a very good partial response (VGPR), 36 (57.1%) obtained a partial response (PR) and 9 (14.3%) patients had not-response/progressive-disease. At the time of post-therapy evaluation, 26 (37%) of patients had normalized FLC-ratio, 15 (23.8%) maintain the SR, 1 (1,6%) patient in VGPR and 5 (11.1%) in PR and 1 (1.6%) of non-responder patients. Normalization of HLC-ratio was only observed in patients with SR and VGPR: 13 (20.6%). Regarding the immunoparesis analysis, only 15 (23.8%) of patients with immunoparesis recovered the immune restitution (IR) at the end of therapy, of which 8 (11.7%) were SR patients, 2 VGPR and 5 PR patients. At the end of the study 47(71.4%) patients relapsed, 5 (11.11%) are on maintenance therapy and 11(17.4%) after a median follow-up of 29 months (9-94) without therapy not-relapsed; the association of SR with IR was related to a less tendency to relapse and need of therapy, 7/8 patients who achieved this status are not-relapsed. Conclusion In our cohort, patients who achieved a SR with a normalization of immunoparesis shows a clear tendency to less incidence of relapse; probably reflecting a better response with not only an undetectable monoclonal protein but also the recovery of the immune function. Even in small cohorts, the immunoparesis recovery analysis through HLC quantifications seems to be an useful tool to determine a new level of response. More investigations on this field are warranted. This work has been partially supported by a grant from Fundación para el Estudio de la Hematología y hemoterapia en Aragón (FEHHA) Disclosures: No relevant conflicts of interest to declare.

Outcome of Lymphocyte Predominant Hodgkin Lymphoma

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 5463-5463
Author(s):  
Arifa Khalid ◽  
Huma Zafar ◽  
Rabia Wali ◽  
Shazia Riaz ◽  
Farhana Badar
Keyword(s):  
Hodgkin Lymphoma ◽  
Conflicts Of Interest ◽  
Treatment Modalities ◽  
Western World ◽  
Rare Type ◽  
Frequent Relapses ◽  
Radiotherapy Alone ◽  
Anti Cd20 ◽  
Age Range

Abstract BACKGROUND: Lymphocyte predominant Hodgkin Lymphoma (LPHL) , also known as Hodgkin paragranuloma or lymphocytic and histiocytic predominant Hodgkin disease. It accounts for 5% of the lymphoma in the western world Since LPHL is a rare type of lymphoma different treatment modalities used to treat it including radiotherapy alone, combined chemotherapy and radiotherapy, chemotherapy alone and recently the use of Rituximab anti CD20 monoclonal antibody as LPHL expresses CD20 marker . The results are variable. Patients of LPHL also have frequent relapses beyond 5 years of treatment as compared to cHL and high rates of transformation to DLBCL . OBJECTIVE: To review outcome of LPHL patients treated with various chemotherapy regimens ( ChlVpp/ABVD) and (ABVD/COPDac) alone or in combination with radiotherapy. METHODS AND MATERIALS: Data of total 301 patients reviewed . Out of which 15 were of LP Hodgkin (4.98%). Retrospective review of 15 patients of LP Hodgkin Lymphoma patients enrolled between January 2006 to December 2013 was done. Data regarding age, sex, stage of disease , treatment and outcome was analyzed. RESULTS: Total 15 LP HD patients with age range from 2 to 18 years were included from 2006 to 2013. Mean age of patients was 10 years. Median was 9. Out of these 13 (86.7% ) were males and 2 (13.3%) females, most of them are of stage III (40%). Majority , 9 patients (60 %). were treated with ChlVpp/ABVD protocol. XRT was given to 5 patients (33.3%). Total 12 (80%) are still on follow up and 3 (20%) relapsed. CONCLUSION: Patients of Lymphocyte predominant Hodgkin Lymphoma treated with chemotherapy had a better outcome . Disclosures No relevant conflicts of interest to declare.

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