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2.
Transfusion ; 62(12): 2503-2514, 2022 12.
Artículo en Inglés | MEDLINE | ID: mdl-36194042

RESUMEN

BACKGROUND: Transfusion-associated hyperkalemia (TAH) is a potentially life-threatening complication of red blood cell (RBC) transfusion. Previously, we reported features of RBC transfusions from 35 pediatric patients (TAH group) who had hyperkalemia with RBC transfusion in one-year period at four facilities. In this study, we used multivariate analyses and artificial intelligence to compare the TAH group to newly collected control group (non-TAH group) to identify factors associated with TAH occurrence. STUDY DESIGN: A review of RBC transfusion with TAH was compared to non-TAH group who did not develop TAH with RBC transfusion at each facility during the same one-year period. The non-TAH group included 12 patients each in 5 age groups. Wilcoxon rank-sum tests recursive feature elimination, least absolute shrinkage, and selection operator (LASSO), and other artificial intelligence techniques were employed to identify the most salient features associated with predicting specific clinical outcomes for TAH occurrence. RESULTS/FINDINGS: Pre-transfusion creatinine, comorbidities of kidney and/or liver dysfunctions, and total transfused volume within 12 h (tV-12) per kg and per estimated total blood volume (eTBV) showed statistically significant differences between TAH and non-TAH groups. Multivariate analysis revealed the biggest factor in TAH occurrence was tV-12/kg followed by age of RBC units. The thresholds of risks were tV-12/kg of 30 ml/kg, tV-12/eTBV of 30%, and RBC unit age of 7.95 days. CONCLUSIONS: The study findings suggest that the biggest factor on TAH occurrence is tV-12/kg. More importantly, 30% of eTBV transfusion could cause TAH in patients with multiple comorbidities.


Asunto(s)
Inteligencia Artificial , Niño , Humanos , Recién Nacido , Factores de Riesgo
3.
J Clin Apher ; 36(6): 878-881, 2021 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-34510542

RESUMEN

Since vaccination for SARS-CoV-2 coronavirus started, the trajectory of patient numbers infected with the virus has improved once; however, variants of SARS-CoV-2 have emerged and more people have been infected; therefore, pandemic status is still far from resolution. Government and social efforts to prevent coronavirus infection continue in most states in the US and globally even after the Centers for Disease Control and Prevention declared some restriction relief for fully vaccinated people in March 2021. Healthcare institutions and various professional organizations have developed guidelines or policies to prevent the spread of these coronaviruses in the setting of apheresis. In this report, the issues that apheresis services may encounter under the current COVID-19 (SARS-CoV-2 coronavirus disease) pandemic will be discussed with potential strategies that can be adapted for efficient and optimum use of apheresis resources.


Asunto(s)
Eliminación de Componentes Sanguíneos , COVID-19/epidemiología , Pandemias , Eliminación de Componentes Sanguíneos/métodos , Eliminación de Componentes Sanguíneos/estadística & datos numéricos , Humanos , Guías de Práctica Clínica como Asunto , SARS-CoV-2 , Sociedades Médicas , Estados Unidos/epidemiología
4.
Transfusion ; 61(4): 1093-1101, 2021 04.
Artículo en Inglés | MEDLINE | ID: mdl-33565635

RESUMEN

BACKGROUND: Hyperkalemia is a rare life-threatening complication of red blood cell (RBC) transfusion. Stored RBCs leak intracellular potassium (K+) into the supernatant; irradiation potentiates the K+ leak. As the characteristics of patients and implicated RBCs have not been studied systematically, a multicenter study of transfusion-associated hyperkalemia (TAH) in the pediatric population was conducted through the AABB Pediatric Transfusion Medicine Subsection. STUDY DESIGN: The medical records of patients <18 years old were retrospectively queried for hyperkalemia occurrence during or ≤12 h after the completion of RBC transfusion in a 1-year period. Collected data included patient demographics, diagnosis, medical history, timing of hyperkalemia and transfusion, mortality, and RBC unit characteristics. RESULTS/FINDINGS: A total of 3777 patients received 19,649 RBC units during the study period in four facilities. TAH was found in 35 patients (0.93%) in 37 occurrences. The patient median age and weight were 1.28 years and 9.80 kg, respectively. All patients had multiple serious comorbidities. There were 79 RBC units transfused in the TAH events; 62% were irradiated, and the median age of the units was 10 days. The median total RBC volume transfused ≤12 h before TAH was 24% of patient estimated total blood volume, and the median infusion rate (IR) was19.6 ml/kg/h. Mortality rate within 1 day after the TAH event was 20%. CONCLUSIONS: The prevalence of TAH in children was low; however, the 1-day mortality rate was 20%. Patients with multiple comorbidities may be at higher risk for TAH. The IR was higher for patients who had TAH than the IR threshold for safe transfusion.


Asunto(s)
Transfusión de Eritrocitos/efectos adversos , Hiperpotasemia/etiología , Infusiones Intravenosas/efectos adversos , Potasio/efectos de la radiación , Adolescente , Estudios de Casos y Controles , Niño , Preescolar , Comorbilidad , Femenino , Humanos , Hiperpotasemia/diagnóstico , Hiperpotasemia/epidemiología , Hiperpotasemia/mortalidad , Lactante , Infusiones Intravenosas/estadística & datos numéricos , Masculino , Mortalidad/tendencias , Potasio/sangre , Prevalencia , Estudios Retrospectivos , Factores de Riesgo , Medicina Transfusional/estadística & datos numéricos
5.
J Clin Apher ; 35(1): 25-32, 2020 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-31705563

RESUMEN

IMPORTANCE: Neuromyelitis optica/neuromyelitis optica spectrum disorder patients' response to therapeutic plasma exchange (TPE) is currently incompletely characterized. OBJECTIVE: Our study aims to understand the clinical status improvement of neuromyelitis optica/neuromyelitis optica spectrum disorder patients treated with TPE. DESIGN, SETTING, AND PARTICIPANTS: This is a multicenter retrospective study conducted between 1 January 2003 and 31 July 2017 at 13 US hospitals performing apheresis procedures. Subjects studied were diagnosed with neuromyelitis optica/neuromyelitis optica spectrum disorder who received TPE during presentation with acute disease. MAIN OUTCOMES AND MEASURES: The primary outcome was clinical status improvement in patients treated with TPE. Secondary measures were procedural and patient characteristics associated with response to treatment. RESULTS: We evaluated 114 patients from 13 institutions. There was a female predilection. The largest ethnic group affected was non-Hispanic Caucasian. The average age of diagnosis was 43.1 years. The average time to diagnosis was 3.1 years. On average, five procedures were performed during each treatment series. The most commonly performed plasma volume exchange was 1.0 to 1.25 using 5% albumin as replacement fluid. Most patients (52%) did not require an additional course of TPE and noted "mild" to "moderate" clinical status improvement. Maximal symptom improvement appeared by the fourth or fifth TPE treatment. CONCLUSION AND RELEVANCE: TPE improved the clinical status of patients. Adults responded more favorably than children. Procedural characteristics, including number of TPEs, plasma volume exchanged, and replacement fluid used, were similar between institutions. TPE was well-tolerated and had a low severe adverse event profile.


Asunto(s)
Neuromielitis Óptica/terapia , Intercambio Plasmático/métodos , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Autoanticuerpos , Eliminación de Componentes Sanguíneos , Niño , Preescolar , Humanos , Lactante , Recién Nacido , Persona de Mediana Edad , Plasmaféresis , Sistema de Registros , Estudios Retrospectivos , Estados Unidos , Adulto Joven
7.
J Clin Apher ; 34(1): 26-32, 2019 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-30375048

RESUMEN

INTRODUCTION: Performing therapeutic plasma exchange (TPE) with albumin replacement decreases coagulation factor and platelet levels. No defined guidelines exist regarding laboratory testing to assess hemostasis in patients undergoing TPE. MATERIALS AND METHODS: A survey to evaluate hemostasis testing with TPE was distributed using online survey software. One response per institution was analyzed based on a hierarchical algorithm, excluding membrane filtration users, resulting in a maximum of 120 respondents per question. Descriptive analysis was performed with results reported as the number and/or frequency (%) of respondents to each question. RESULTS: The practices represented vary by institution type, number of apheresis procedures per year, and performance of TPE on children. Prior to TPE planned with albumin replacement, many respondents obtain laboratory studies for almost all patients (54.9% outpatients and 68.7% inpatients); however, some do not routinely obtain laboratory studies (9.7% outpatients and 4.4% inpatients). Hemoglobin/hematocrit, platelet count, fibrinogen, partial thromboplastin time (aPTT), and international normalized ratio (INR) are obtained prior to all TPE by 62.5%, 53.4%, 31.0%, 18.1%, and 17.7% of respondents, respectively; however, 1.0%, 8.7%, 29.0%, 38.3%, and 35.4%, respectively, do not routinely obtain these studies. Variation was observed in laboratory threshold values for action; the most common reported were hemoglobin/hematocrit <7 g/dL or 21% (31.0%), platelet count <50 × 109 /L (24.1%), fibrinogen <100 mg/dL (65.3%), aPTT >reference range and >1.5 times reference range (tied, 28.1%), and INR >1.5 (20.7%). CONCLUSIONS: Practice variation exists in hemostasis laboratory testing and threshold values for action with TPE. Further studies are needed to determine optimal hemostasis testing strategies with TPE.


Asunto(s)
Hemostasis , Intercambio Plasmático/métodos , Algoritmos , Factores de Coagulación Sanguínea/análisis , Técnicas de Laboratorio Clínico , Humanos , Intercambio Plasmático/efectos adversos , Recuento de Plaquetas , Pautas de la Práctica en Medicina , Encuestas y Cuestionarios
8.
Immunohematology ; 35(4): 139-144, 2019 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-31935329

RESUMEN

CONCLUSIONS: Antibodies (Abs) against antigens on platelets (PLTs), including glycoprotein IV (CD36), can cause PLT refractoriness. Transfusing PLTs to patients with anti-CD36 is challenging because of the rarity of CD36-negative (CD36-) donors and the possibility of additional HLA Abs. We report a case of PLT refractoriness due to anti-CD36 and HLA Abs. A 21-year-old man (group O, D+) with assumed drug-induced aplastic anemia received multiple PLT transfusions and developed severe PLT refractoriness. He was found to have anti-CD36 as well as HLA class I Abs, with a CD36- phenotype on both PLTs and monocytes. He was diagnosed with type 1 CD36 deficiency and received intravenous immunoglobulin (IVIG) and rituximab to decrease future Ab production. The PLT corrected count increment (CCI) improved significantly with subsequent transfusions of flow crossmatchcompatible as well as uncrossmatched PLTs. He eventually received a bone marrow transplant and has been doing well since. The mean CCI before and after IVIG/rituximab treatment was 0.2 and 6.2, respectively. Soon after IVIG started, the patient's CCI after receiving CD36-, group AB, D+, and HLA untested PLTs was 0.8, but his CCI after receiving flow crossmatch-compatible PLTs was 12.6. Two months after IVIG was started, the mean CCIs for uncrossmatched apheresis PLTs and crossmatch-compatible PLTs were comparable (6.1 versus 6.0, respectively). Desensitization treatment with IVIG and rituximab lowered anti-CD36 and HLA Ab levels, and the CCI of PLT transfusion improved significantly. This case demonstrates that immune suppression is effective for successful PLT transfusion of patients with anti-CD36.


Asunto(s)
Trombocitopenia , Plaquetas , Antígenos HLA , Humanos , Masculino , Transfusión de Plaquetas , Trombocitopenia/terapia , Resultado del Tratamiento , Adulto Joven
9.
J Clin Apher ; 33(6): 673-677, 2018 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-30321467

RESUMEN

BACKGROUND: Angiotensin II type-1 receptor antibody (AT1RAb) has been reported to cause antibody mediated rejection (AMR) in kidney transplant recipients possibly by contraction of renal arteries. We here report 2 kidney transplant recipients with elevated AT1RAbs and negative HLA donor specific antibodies (DSA) and anti-major histocompatibility complex class I chain-related gene A (MICA) Abs who received therapeutic plasma exchange (TPE) treatment followed by IVIG. CASE 1: Thirty-eight-year-old patient received second kidney transplant for end stage renal disease (ESRD) with chronic rejection. Three years post-transplant, she developed AMR with AT1RAb level >40 U/mL. She received 5 TPE and AT1RAb decreased by 20%, and biopsy showed improvement of AMR. She received another 3 TPE and AT1RAb decreased by 60%. Her creatinine (Cr) was stabilized at around 1.4 mg/dL. CASE 2: Twenty-four-year-old patient received kidney transplant for ESRD with unclear etiology. Two weeks post-transplant, her Cr rose with AT1RAb level at 18 U/mL and biopsy showed possible AMR. She received 6 TPE treatments and AT1RAb decreased by 55% and biopsy showed improvement of AMR. She received weekly TPE for subsequently rising AT1RAb but TPE was discontinued because of unsuccessful decrease of AT1RAb. Her Cr was stabilized at around 1.7 mL/dL. CONCLUSION: We reported 2 patients who received TPE treatments to decrease AT1RAbs. A course of TPE treatment successfully decreased AT1RAb. Histological improvement was observed quickly and Cr was also stabilized following the TPE treatment. Further study is necessary to determine the optimal use of TPE in renal transplant recipients with AT1RAbs.


Asunto(s)
Anticuerpos/sangre , Trasplante de Riñón/efectos adversos , Intercambio Plasmático/métodos , Receptor de Angiotensina Tipo 1/inmunología , Adulto , Creatinina/sangre , Femenino , Rechazo de Injerto/prevención & control , Humanos , Fallo Renal Crónico/complicaciones , Fallo Renal Crónico/terapia , Adulto Joven
10.
J Clin Apher ; 33(5): 604-610, 2018 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-30207610

RESUMEN

BACKGROUND: Patients undergoing therapeutic plasma exchange (TPE) may present with risks for hemorrhage or thrombosis. Use of replacement fluids devoid of coagulation factors will decrease factor levels and platelet levels. There are no established guidelines for hemostasis management in these situations. MATERIALS AND METHODS: A survey to evaluate current hemostasis management practice during TPE was conducted using online survey software. One response per institution was analyzed based on a hierarchical algorithm, excluding membrane filtration users, resulting in a maximum of 107 respondents. Descriptive analysis was performed with results reported as the number and frequency (%) of respondents to each question. RESULTS: Apheresis Medicine physicians, alone (59.4%) or jointly with the requesting provider (29.2%), choose the replacement fluid. Based on a theoretical patient case receiving five TPEs approximately every other day, the percent of respondents who would use albumin with or without normal saline was 94.7% with no history of a bleeding or clotting disorder, 1.1% with active bleeding, and 8.8% with hypofibrinogenemia (<100 mg/dL) due to recent TPE. More respondents would use albumin with or without normal saline for replacement fluid when a minor invasive procedure (49.5%) vs a major surgery (8.9%) was performed 1 day before TPE. Replacement fluid selection varied among respondents for several other clinical conditions. The most frequent use for cryoprecipitate by respondents (14.3%) was hypofibrinogenemia. CONCLUSIONS: These survey results demonstrate wide interinstitutional variation in replacement fluid selection to manage hemostasis in patients undergoing TPE. Further studies are needed to guide optimal hemostasis management with TPE.


Asunto(s)
Hemostasis , Intercambio Plasmático/efectos adversos , Pautas de la Práctica en Medicina/estadística & datos numéricos , Afibrinogenemia/terapia , Factor VIII/uso terapéutico , Femenino , Fibrinógeno/uso terapéutico , Hemorragia/etiología , Humanos , Masculino , Plasmaféresis/métodos , Albúmina Sérica/uso terapéutico , Encuestas y Cuestionarios , Trombosis/etiología
11.
J Clin Apher ; 32(6): 501-516, 2017 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-28485045

RESUMEN

INTRODUCTION: Autologous peripheral blood hematopoietic progenitor cell collection (A-HPCC) in children typically requires placement of a central venous catheter (CVC) for venous access. There is scant published data regarding the performance and safety of femoral CVCs in pediatric A-HPCC. METHODS: Seven-year, retrospective study of A-HPCC in pediatric patients collected between 2009 and January 2017. Inclusion criteria were an age ≤ 21 years and A-HPCC using a femoral CVC for venous access. Femoral CVC performance was examined by CD34 collection rate, inlet rate, collection efficiency (MNC-FE, CD34-FE), bleeding, flow-related adverse events (AE), CVC removal, and product sterility testing. Statistical analysis and graphing were performed with commercial software. RESULTS: A total of 75/119 (63%) pediatric patients (median age 3 years) met study criteria. Only 16% of children required a CVC for ≥ 3 days. The CD34 collect rate and CD34-FE was stable over time whereas MNC-FE decreased after day 4 in 80% of patients. CD34-FE and MNC-FE showed inter- and intra-patient variability over time and appeared sensitive to plerixafor administration. Femoral CVC showed fewer flow-related AE compared to thoracic CVC, especially in pediatric patients (6.7% vs. 37%, P = 0.0005; OR = 0.12 (95%CI: 0.03-0.45). CVC removal was uneventful in 73/75 (97%) patients with hemostasis achieved after 20-30 min of pressure. In a 10-year period, there were no instances of product contamination associated with femoral CVC colonization. CONCLUSION: Femoral CVC are safe and effective for A-HPCC in young pediatric patients. Femoral CVC performance was maintained over several days with few flow-related alarms when compared to thoracic CVCs.


Asunto(s)
Catéteres Venosos Centrales/normas , Vena Femoral , Células Madre de Sangre Periférica/citología , Adolescente , Antígenos CD34/análisis , Catéteres Venosos Centrales/efectos adversos , Niño , Preescolar , Hemorragia/etiología , Humanos , Lactante , Estudios Retrospectivos , Trasplante Autólogo
12.
Epilepsy Res ; 131: 28-36, 2017 03.
Artículo en Inglés | MEDLINE | ID: mdl-28236736

RESUMEN

PURPOSE: As part of our efforts to develop a non-invasive facial nerve stimulator as an emergency treatment for ischemic stroke, we considered possible safety consequences if the technology was misapplied to stroke mimics, e.g., seizure. We hypothesized that magnetic facial nerve stimulation would worsen epileptiform activity in two animal models of active seizures. The rat intraperitoneal kainate model and pig intracortical penicillin model were employed. Magnetic facial nerve stimulation was delivered unilaterally at a variety of stimulation parameters, and the effect on ictal epileptiform activity measured by electroencephalography was determined according to an established categorical scale. PRINCIPAL RESULTS: In 6 rats and 3 pigs evaluated with 83 stimulation trials, only a single stimulation trial was associated with worsening epileptiform activity according to a standard categorization scheme. Surprisingly, a reduction in the severity of the epileptiform activity was observed in 20 of 50 stimulation trials using patterned stimulation (3 pulses at 30Hz repeated at 0.5-10Hz) versus 2 of 33 stimulation trials using simple monotonic patterns (P<0.005, chi-squared test). The reduction of epileptiform activity after stimulation lasted a few minutes and was reproducible. Major Conclusions Epileptiform activity measured by electroencephalography was not reliably worsened by repetitive facial nerve stimulation with pulsed magnetic energy, even when significant brain exposure to the magnetic field occurred as in the rat model. To the contrary, a temporary reduction in epileptiform activity was often, but not invariably, observed with certain stimulation parameters.


Asunto(s)
Modelos Animales de Enfermedad , Terapia por Estimulación Eléctrica/métodos , Nervio Facial/fisiología , Convulsiones/fisiopatología , Convulsiones/terapia , Estimulación Magnética Transcraneal/métodos , Animales , Electroencefalografía/métodos , Femenino , Masculino , Ratas , Ratas Sprague-Dawley , Porcinos
13.
J Clin Apher ; 32(1): 5-11, 2017 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-26946363

RESUMEN

BACKGROUND: Anti-muscle specific kinase antibody positive (MuSK Ab) myasthenia gravis (MG) patients are known to have different clinical course compared to anti-acetylcholine receptor Ab positive MG patients. Therapeutic plasma exchange (TPE) has been reported to be effective; however, little is known of the response and of TPE procedural information. An ASFA Apheresis Registry was developed to analyze those data. METHODS: The study collected detailed de-identified patient data, TPE procedures, and treatment outcome/complications. Collected data was described in aggregate. RESULTS: A total of 15 MuSK Ab MG patients with exacerbation of MG symptoms, 13 females/2 males, median age 44, were investigated. Thirty TPE courses (median 5 procedures/course, total 145 procedures) were evaluated. All TPE procedures were performed with citrate anticoagulation, 1 - 1.25 plasma volume exchange in 100% fluid balance, and 90% of courses used only albumin as replacement. Calcium was added to albumin or given orally as needed. TPE was performed every other day in 55% of courses. Adverse events occurred in 3.4% of procedures. Ten patients (67%) experienced relapses within a median of 7 weeks. Objective symptoms were resolved in more than 75% of courses. Overall subjective improvement rates were 94.1%/93.3% after 3/4 TPE procedures, respectively. Thirty-one percent of patients responded poorly with minimal recovery. CONCLUSION: Overall subjective improvement was seen up to 94% of patients after one course of TPE. Some patients were poor-responders. Five TPE may be adequate for initial course with additional TPE as needed. Based upon this preliminary data, we will modify our future data collection. J. Clin. Apheresis 32:5-11, 2017. © 2016 Wiley Periodicals, Inc.


Asunto(s)
Miastenia Gravis/terapia , Intercambio Plasmático/métodos , Sistema de Registros , Adulto , Autoanticuerpos , Femenino , Humanos , Masculino , Miastenia Gravis/inmunología , Intercambio Plasmático/efectos adversos , Proteínas Tirosina Quinasas Receptoras/inmunología , Receptores Colinérgicos/inmunología , Estudios Retrospectivos , Resultado del Tratamiento
14.
J Clin Apher ; 32(1): 35-48, 2017 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-27092461

RESUMEN

INTRODUCTION: Autologous peripheral blood hematopoietic progenitor cell collection (A-HPCC) in pediatric patients is considered relatively safe although technically challenging. Very little is known regarding the incidence, risk factors and impact of procedure-related adverse events (AE) on pediatric A-HPCC outcomes. METHODS: Prospective 4.5-year review of AE associated with pediatric A-HPCC. AE were graded by severity and type. Potential demographic and procedural risk factors, and the impact on product quality, were compared by t-test, chi-square, and linear regression. RESULTS: Sixty-two children underwent 110 A-HPCC, including 36 (58%) under 20 kg. Fifty-five AE were documented in 25.4% A-HPCCs and 39% of children (citrate 25%, access 19%, technical 11%, cardiovascular 0%, allergic 1.8%). No AE were noted in children < 10 kg anticoagulated with heparin. Access and technical AE accounted for 73% of severe AE, with line-related problems underlying most technical AE (87.5%, P = 0.006). AE were more likely in older (P = 0.012), heavier patients (P = 0.02), who frequently required more than one A-HPCC (P = 0.012). In contrast, young children were more likely to experience citrate AE with gastrointestinal symptoms (median age, 6 years; P = 0.076). AE had no impact on CD34 collection rates; however, mean CD34 yields (4.2 vs. 20.4 million/kg; P = 0.0035) were decreased in patients with technical AE due to lower peripheral CD34 counts and a high number of aborted procedures (37%). CONCLUSION: Venous access and flow-related issues are a major factor associated with moderate and severe AE, effecting ∼10% of patients. AE are more frequent with increasing patient age, weight, and number of procedures. J. Clin. Apheresis 32:35-48, 2017. © 2016 Wiley Periodicals, Inc.


Asunto(s)
Separación Celular/normas , Trasplante de Células Madre de Sangre Periférica/efectos adversos , Células Madre de Sangre Periférica/citología , Adolescente , Factores de Edad , Antígenos CD34/análisis , Antígenos CD34/sangre , Peso Corporal , Niño , Preescolar , Humanos , Trasplante de Células Madre de Sangre Periférica/métodos , Trasplante Autólogo
15.
Transfusion ; 56(12): 3073-3080, 2016 12.
Artículo en Inglés | MEDLINE | ID: mdl-27601087

RESUMEN

BACKGROUND: Therapeutic plasma exchange (TPE) is increasingly used for treatment of antibody-mediated rejection (AMR) after solid organ transplants. There is concern that TPE may increase risk of bleeding, although data are limited. After TPE, clot-based coagulation tests may not accurately represent the levels of coagulation factors due to the effect of citrate. We investigated protein levels of fibrinogen using antigen detection method (FibAg) and correlated results with a clot-based fibrinogen activity test (Fib). STUDY DESIGN AND METHODS: Nine kidney transplant recipients who received TPE for AMR were investigated. Fib, FibAg, prothrombin time/international normalized ratio (PT/INR), partial thromboplastin time (PTT), coagulation factor X chromogenic activity (CFX), and ionized calcium (iCa) were measured at pre- and post-TPE and 1, 3, 6, 9, 24, and 48 hours after the first TPE. RESULTS: Mean Fib/FibAg ratio before TPE was 1.08; therefore, all Fib values were normalized (n) by dividing by 1.08. Overall, the mean normalized Fib (nFib)/FibAg ratio at post-TPE was 0.89 and returned to close to 1.0 at 6 hours after the first TPE. Decreases in nFib, FibAg, and CFX and increases in PT/INR and PTT post-TPE were observed. The lowest Fib, FibAg, CFX, platelet, and iCa levels were still at levels that would be considered sufficient for hemostasis at all time points. CONCLUSION: The mean nFib/FibAg ratio after TPE was 0.89 and normalized in 6 hours, which demonstrates a persistent effect of citrate for up to 6 hours. Therefore, similar data observed in clot-based tests of PT/INR and PTT may be falsely elevated up to 6 hours after TPE due to the citrate effect.


Asunto(s)
Coagulación Sanguínea/efectos de los fármacos , Ácido Cítrico/farmacología , Trasplante de Riñón/efectos adversos , Intercambio Plasmático/efectos adversos , Pruebas de Coagulación Sanguínea/normas , Fibrinógeno/análisis , Hemostasis/efectos de los fármacos , Humanos , Factores de Tiempo
18.
Am J Kidney Dis ; 67(5): 787-91, 2016 May.
Artículo en Inglés | MEDLINE | ID: mdl-26775022

RESUMEN

Cryocrystalglobulinemia is a rare variant of cryoglobulinemia in which monoclonal immunoglobulins self-assemble into crystalline arrays. We report a case of a 53-year-old man who presented with systemic thrombotic microangiopathy causing multiorgan failure, including decreased kidney, lung, and gastrointestinal function; skin necrosis; and mental status changes. Skin and kidney biopsy specimens showed intravascular thrombi, along with intravascular, intratubular, and periglomerular crystalline deposits. Typical morphologic features of cryoglobulinemia, such as a leukocytoclastic vasculitis and pseudothrombi, were absent. Spindled crystals precipitated in the cryoglobulin assay, and immunofixation showed them to be composed of monoclonal immunoglobulin G κ light chains. Ultrastructural analysis demonstrated deposits to have an array-like substructure. The patient was successfully treated with a combination of plasmapheresis, steroids, and bortezomib, but experienced a relapse and died 12 months after his initial diagnosis. Cryocrystalglobulinemia causes significant morbidity and mortality and should be classified as a monoclonal gammopathy of renal significance when it occurs in patients not meeting diagnostic criteria for multiple myeloma.


Asunto(s)
Crioglobulinemia/patología , Crioglobulinas , Glomerulonefritis/patología , Cadenas kappa de Inmunoglobulina , Túbulos Renales/patología , Gammopatía Monoclonal de Relevancia Indeterminada/patología , Microangiopatías Trombóticas/patología , Corticoesteroides/uso terapéutico , Antineoplásicos/uso terapéutico , Bortezomib/uso terapéutico , Crioglobulinemia/complicaciones , Crioglobulinemia/terapia , Cristalización , Resultado Fatal , Glomerulonefritis/complicaciones , Glomerulonefritis/terapia , Humanos , Masculino , Microscopía Electrónica , Persona de Mediana Edad , Gammopatía Monoclonal de Relevancia Indeterminada/complicaciones , Gammopatía Monoclonal de Relevancia Indeterminada/terapia , Plasmaféresis , Microangiopatías Trombóticas/complicaciones , Microangiopatías Trombóticas/terapia
19.
J Clin Apher ; 31(1): 11-5, 2016 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-26275240

RESUMEN

PURPOSE: Wilson's disease is a rare autosomal recessive genetic disorder that results in accumulation of copper in the liver, brain, cornea and kidney. Therapeutic plasma exchange (TPE) has been used to remove copper and provide a bridge to liver transplantation. We report here the collective experiences through the ASFA apheresis registry on Wilson's disease. METHODS: The ASFA apheresis registry is a multi-center registry study. Both prospective and retrospective data, with the latter involving data collection back to January 2000 are entered in the registry. The registry includes patient demographics, apheresis procedural information, treatment schedules, and treatment outcomes and complications. RESULTS: A total of 10 patients (3 males and 7 females) with Wilson's disease treated between 2005 and 2013 were included. Median age of first diagnosis and first TPE were 16 and 17 years, respectively. Via central venous access, these patients underwent a total of 43 TPEs; the median number of TPE procedures per patient was 3.5. All of the TPEs used ACD-A as anticoagulation, 42/43 TPEs targeted 1-1.25 plasma volumes, and 41/43 TPEs were performed with 100% fluid balance. Post TPE procedures, 9 patients underwent liver transplantation; all 10 patients had at least a 6-month survival. CONCLUSIONS: All 10 patients with Wilson's disease who underwent TPE had a positive outcome in terms of 6-month survival. In this first report of the ASFA apheresis registry study, we have demonstrated the value of using this registry to collect apheresis-related patient outcomes from multiple centers.


Asunto(s)
Eliminación de Componentes Sanguíneos , Degeneración Hepatolenticular/terapia , Adolescente , Adulto , Niño , Femenino , Degeneración Hepatolenticular/complicaciones , Humanos , Fallo Hepático Agudo/etiología , Fallo Hepático Agudo/terapia , Trasplante de Hígado , Masculino , Persona de Mediana Edad , Intercambio Plasmático , Estudios Prospectivos , Sistema de Registros , Estudios Retrospectivos , Resultado del Tratamiento , Adulto Joven
20.
J Clin Apher ; 31(5): 481-9, 2016 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-26463664

RESUMEN

BACKGROUND: Hypereosinophilic syndrome (HEOS) is rare, and the efficacy of leukocytapheresis in this context is unclear. We here report the successful treatment of a patient with idiopathic HEOS with four leukocytapheresis procedures using two protocols. CASE: A 4-year-old female presented with cardiac and respiratory dysfunction, and WBC of 225 K/µL with 96% eosinophils. Leukocytapheresis was started after initiation of methylprednisolone and hydroxyurea. She received two leukocytapheresis with polymorphonuclear cell (PMN) protocol, followed by initiation of imatinib therapy, then two leukocytapheresis with mononuclear cell (MNC) protocol. After the fourth leukocytapheresis, her WBC decreased to 69 K/µL with 82% eosinophils. She was discharged on hospital day 21 under stable condition with WBC of 22 K/µL with 86% eosinophils. WBC count and eosinophil percentage continued to decrease, and were 6.4 K/µL and 52% by 2 weeks and 3.9 K/µL and 4.9% by 3 months after discharge, respectively. FINDINGS: WBC and absolute eosinophil (aEO) counts decreased by an average of 29.0 and 30.4% per leukocytapheresis, respectively. Normalized to estimated blood volume, procedures with PMN and MNC protocols changed, on average, WBC by -10.7 and -12.1%, aEO by -10.4 and -13.4%, platelet by -8.1 and -19.2%, and fluid balance by -129 and -47 mL, respectively. CONCLUSION: Leukocytapheresis was effective in decreasing WBC and aEO counts in HEOS, with PMN and MNC protocols achieving similar reductions. However, PMN protocol resulted in greater negative fluid balance and MNC protocol resulted in greater platelet loss. J. Clin. Apheresis 31:481-489, 2016. © 2015 Wiley Periodicals, Inc.


Asunto(s)
Síndrome Hipereosinofílico/terapia , Leucaféresis/métodos , Plaquetas/citología , Preescolar , Eosinófilos/citología , Femenino , Humanos , Leucaféresis/normas , Recuento de Leucocitos , Leucocitos Mononucleares/citología , Leucocitos Mononucleares/trasplante , Neutrófilos/citología , Neutrófilos/trasplante , Equilibrio Hidroelectrolítico
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