Prevalence of Post-Transplant Lymphoproliferative Disorder with Monoclonal Gammopathy of Unknown Significance in Patients Undergoing Kidney Transplantation.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 4778-4778
Author(s):  
Harris V.K. Naina ◽  
Robert Kyle ◽  
Thomas M. Habermann ◽  
Samar Harris ◽  
Fernando G. Cosio ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Kidney Transplantation ◽  
T Cell ◽  
Organ Transplantation ◽  
Monoclonal Gammopathy ◽  
Lymphoproliferative Disorder ◽  
M Protein ◽  
Monoclonal Protein ◽  
Post Transplant

Abstract Background: Post-transplant lymphoproliferative disorder (PTLD) represents one of the most serious consequences of immunosuppression in patients with solid organ transplantation. The incidence of PTLD is related to the organ transplanted and is dependent on the duration of follow-up. In various publications, the incidence of PTLD in renal transplantation ranges between 0.8% to 1.2%. In a previous study, the development of monoclonal (M) protein following liver transplantation is associated with the development of PTLD. In this study, we investigate this relationship in the kidney transplant population. Methods: A total of 3518 patients underwent kidney transplantation between 1963 to March 2006. These patients were cross referenced with the Monoclonal Gammopathy of Undetermined Significance (MGUS) database. Results: We identified 97 patients who had a monoclonal gammopathy either before or after transplantation. Patients with amyloidosis, multiple myeloma, heavy and light chain deposition disease and multi-organ transplantation were excluded from the analysis. A total of 69 patients met the inclusion criteria. Ten of the 69 (14.5%) patients developed PTLD. Median follow-up was 14.8 years. Twenty three patients had pretransplant MGUS, 20 patients developed MGUS following the transplant, and the other 26 did not have a monoclonal protein study prior to the transplant. Of the 23 patients who had a positive MGUS prior to the transplant, 4 patients (17.3%) developed PTLD, 1 patient developed EBV positive diffuse large cell lymphoma (DLCL), 1 developed EBV negative DLCL, 1 developed Hodgkin’s lymphoma and 1 developed increased plasma cells in bone marrow (20%) with stable M protein with no evidence of progression to multiple myeloma. None of these patients had a quantifiable M-protein prior to transplantation. The mean duration from diagnosis of MGUS to PTLD was 8.2 years (range 3 to 14 years). Of the 20 patients with a negative pre-transplant study for para proteniemia, 2 (10%) developed PTLD (T cell lymphoproliferative disorder). Two patients developed MGUS after the transplant at 1 and 12 years post transplant. It took an average of 15 years to develop PTLD after the diagnosis of MGUS. Four of the 26 patients who did not have a pretransplant study for MGUS developed PTLD. These included an EBV positive gamma delta type T cell lymphoproliferative disorder, an EBV positive plasmablastic lymphoma, one multiple myeloma and a plasmacytoma. The latter two patients had M-protein > 3g/dL. It took an average of 14 years after their transplant for these patients to develop PTLD. Conclusion: Our study showed that the development of a monoclonal protein in patients undergoing kidney transplantation is a strong risk factor for PTLD. Monoclonal protein study should be performed pretransplant and monitored post transplant as a surveillance of PTLD. Those who are positive or convert should be monitored closely for development of lymphoproliferative disorder.

Pre Transplantation MGUS and Transformation to Multiple Myeloma in Kidney Transplantation: A Single Center Experience.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 4779-4779
Author(s):  
Harris V.K. Naina ◽  
Robert Kyle ◽  
Thomas M. Habermann ◽  
Samar Harris ◽  
Fernando G. Cosio ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Bone Marrow ◽  
Kidney Transplantation ◽  
Renal Transplantation ◽  
Monoclonal Gammopathy ◽  
Plasma Cells ◽  
M Protein ◽  
Biclonal Gammopathy ◽  
Undetermined Significance

Abstract Background: Monoclonal gammopathy of undetermined significance (MGUS) is reported in 3 to 5 percent of population, with the prevalence increasing with advancing age. Patients with MGUS are at increased risk for progression to multiple myeloma or other plasma cell dyscrasias. There is a paucity of information on clinical outcomes of patients with MGUS undergoing renal transplantation. A retrospective study was performed to determine wether MGUS is a contraindication to renal transplantation. Methods: Data was collected from both the kidney transplant and MGUS database. The diagnosis of MGUS was made on the basis of either serum protein electrophoresis (SPEP) or immunofixation after excluding multiple myeloma, amyloidosis and monoclonal immunoglobulin deposition disease. Results: Between 1977 and 2004, 3518 patients underwent kidney transplantation of whom 23 patients had a preexisting monoclonal gammopathy of undetermined significance (MGUS). Fourteen (61%) of these patients were males. The median age at the time of transplant was 59 ±12 years. Ten patients (43.5%) had IgG Kappa (GK), 7 (30.4%) had IgG Lambda (GL), 2 (8.7%) had IgA Lambda (AL), 1 (4.3%) had IgA Kappa (AK), 2 (8.7%) had IgM Lambda (ML). One patient had a biclonal gammopathy GL and ML. Patients were monitored with either SPEP or immunofixation for median duration of 1542 days after transplantation. Thirteen patients had either no change or stable monoclonal protein, 6 had a decrease in their paraprotein level. Two patients had a mild increase in their paraprotein. Two patients with GK developed into biclonal gammopathy (GK and AK). The median follow up of this cohort after the renal transplant was 1783 days. Twelve (52%) patients remained alive at the time of the study. A patient with GK prior to the transplant who underwent kidney transplantation twice developed a biclonal gammopathy and was found to have increased plasma cells (20%) in bone marrow after 14 years. On follow up for 6 years, his M-protein remained stable. Another patient was found to have 17% plasma cells around the time of kidney transplantation. He had a stable M-protein at follow-up, but underwent a stem cell transplant for recurrent immunotactoid glomerulonephritis. Two (9%) patients developed more than 15% plasma cells in their bone marrow with a stable M-protein. None of the patients with a preexisting MGUS evolved into multiple myeloma. Conclusion: In this small study, the presence of MGUS prior to kidney transplantation did not appear to have increased the incidence of multiple myeloma post transplant. Therefore, MGUS by itself should not be considered as an absolute contraindication for renal transplantation.

Five years’ Follow-Up Study For Frequency Of Monoclonal Gammopathy Of Undetermined Significance (MGUS) In a Korean Elderly Urban Cohort

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 5332-5332
Author(s):  
Yun-Gyoo Lee ◽  
Soo-Mee Bang ◽  
Jeong-Ok Lee ◽  
Song Jung Han ◽  
Kim Ki Woong ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Monoclonal Gammopathy ◽  
M Protein ◽  
Risk Of Death ◽  
Follow Up Study ◽  
Significant Difference ◽  
Korean Elderly ◽  
Second Wave ◽  
Undetermined Significance

Abstract Background The prevalence of monoclonal gammopathy of undetermined significance (MGUS) increases with patient age and varies by race. However, reliable data on the epidemiology of MGUS is limited in Korea. We previously reported the prevalence of MGUS among 1000 participants of a Korean Elderly Urban Cohort recruited from 2005 to 2006 (First Wave, Park HK Am J Hematol. 2011;86:752-5); age and gender-adjusted prevalence of MGUS was estimated as 3.3% (95% confidence interval [CI] = 2.0-4.6%). Here, we report the five years’ follow-up study for frequency of MGUS between 2010 and 2011. Methods Korean Longitudinal Study on Health and Aging (KLoSHA) is a population-based, prospective cohort study of health, aging, and common geriatric diseases in a population aged ≥ 65 years in Seongnam-si, a satellite city of Seoul. Of the random sample of 1118 candidates from 61,730 Korean elderly individuals, 698 respondents agreed to participate in baseline KLoSHA study between 2005 and 2006. A total of 680 with available samples were screened for MGUS. We followed them and collected their serum between 2010 and 2011 (Second Wave). The screening of MGUS in the Second Wave was performed using serum protein electrophoresis followed by immunofixation assays; MGUS was defined by the presence of a serum monoclonal protein (M-protein), at a concentration <3 g/dL, and in the absence of end organ damage. Bone marrow study was not performed unless the patient was suspicious of multiple myeloma. To validate complete follow-up data, information regarding vital status was obtained from the National Population Registry of Korea National Statistical Office by using a unique resident registration number. Overall survival was calculated from the date of First wave to death from any cause. Results Of the 680 respondents (21 with MGUS, 659 without MGUS) in the First Wave, 361 (53%) agreed to participate in the Second Wave. Causes of nonattendance were death in 20%, refusal in 19%, move to other area in 6%, and loss to contact in 3%. Of the 361 respondents, 10 were identified to have MGUS. Overall frequency of MGUS is 2.8% (95% CI: 1.3 - 5.0%). Among 21 patients with MGUS in the First Wave, 9 were followed up in the Second Wave. Six of them showed persistent MGUS. One of them showed mild anemia with persistent M-protein of 1.4g/dL suggestive of progression to multiple myeloma, but was not confirmed because of early death just after screening. Interestingly, M-protein was disappeared in remaining 2 patients with MGUS in the First wave. Among 659 respondents without MGUS in the First Wave, 352 were followed up in the Second Wave. Four of them were newly diagnosed with MGUS. In Kaplan-Meier survival analysis, there was no significant difference of survival between respondents with MGUS and without MGUS in the First Wave (P = 0.66 by Log-rank test). Conclusion Five years’ follow-up data showed the natural clinical course of MGUS. The diagnosis of MGUS was not associated with an increased risk of death in Korean elderly population. The interesting finding was that M-protein was disappeared in some patients with MGUS. High rate of non-attendance (47%) in Second Wave is the major limitation. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Pediatric T-cell post-transplant lymphoproliferative disorder after solid organ transplantation

2007 ◽  
Vol 50 (2) ◽  
pp. 415-418 ◽  
Author(s):  
Fan Yang ◽  
Ying Li ◽  
Raul Braylan ◽  
Stephen P. Hunger ◽  
Li-Jun Yang
Keyword(s):  
T Cell ◽  
Organ Transplantation ◽  
Lymphoproliferative Disorder ◽  
Solid Organ Transplantation ◽  
Solid Organ ◽  
Post Transplant

scholarly journals Monoclonal Gammopathies: Electronic Subspecialty Consultation

Blood ◽  
2017 ◽  
Vol 130 (Suppl_1) ◽  
pp. 673-673
Author(s):  
Nicholas Burwick ◽  
Jacob Stein ◽  
David A Garcia ◽  
Virginia C. Broudy ◽  
Robert E. Richard
Keyword(s):  
Risk Stratification ◽  
Light Chain ◽  
Monoclonal Gammopathy ◽  
Free Light Chain ◽  
Low Risk ◽  
M Protein ◽  
Monoclonal Protein ◽  
Face To Face ◽  
Electronic Consultation

Abstract Introduction : Non-visit electronic consultation (e-consult) is an important component of care for veterans in the VA healthcare system who require sub-specialty consultation but not urgent face to face evaluation. Since the majority of patients with monoclonal gammopathy of undetermined significance (MGUS) are low-risk of disease transformation, we reasoned that e-consult would be a safe and effective way to manage MGUS in most cases. Here we sought to characterize our current e-consult practice patterns for the surveillance of MGUS and identify key questions for future investigation. Methods : We performed a retrospective analysis of our electronic consult database from 1/1/2010-12/31/2014 to identify cases of monoclonal gammopathy. Monoclonal gammopathy was confirmed on chart review by an attending hematologist. To be included in the analysis, a patient had to have either 1) a monoclonal protein by serum or urine protein electrophoresis (SPEP/UPEP) or immunofixation or 2) abnormal serum free light chain (FLC) ratio, using a normal reference range of 0.26-1.65, with an increase in the involved light chain. Pertinent clinical and demographic data was abstracted and was used to analyze outcomes among the cohort. Results : We screened 3,217 electronic hematology consults to identify a cohort of 152 MGUS patients triaged for e-consult over a five-year period. E-consult services were provided for veterans from 23 different counties with an average time to completion of 3.4 days. The average size of monoclonal (M) protein was 0.25 g/dL (0-1.5 g/dL). 84% of patients had an M-protein concentration less than 0.5 g/dL. Following completion of risk-stratification studies, 113/121 (93%) of patients with available risk scores were lower risk for disease progression (0-1 risk factors). There were 11 cases with negative SPEP for whom a risk score could not be calculated. An additional 20 cases had a positive SPEP without available free light chain data. A minority of patients (29%) had FLC data available at the time of consult. At 3-months post-consult, 71% had completed FLC testing. One-third of patients had an abnormal hemoglobin (hgb) and 41% had an abnormal creatinine (cr) using the normal reference ranges. However, 96% of MGUS e-consults had a hgb &gt;10 g/dL and 90% had a cr &lt;2 mg/dL. Among those tested (n=91), one patient had skeletal abnormalities concerning for myelomatous bone disease on initial screening. One-third of cases utilized multiple e-consult encounters over time, while 15% of MGUS e-consults ultimately required a face-to-face visit with hematology. With an average follow-up of 47 months (median 44 months), there were 6 documented progression events, representing a mean rate of progression of 1% per year (Figure). Conclusions : We find that electronic consultation is a helpful mechanism for evaluating MGUS longitudinally, decreasing travel burden, and improving timely access to care for veterans. The majority of MGUS cases triaged for e-consult at our center are low-risk by established criteria and have very low amounts of monoclonal protein. Most of these patients can be followed with routine paraprotein surveillance and deferred skeletal imaging. Timely completion of biomarker studies is critical for appropriate risk-stratification and triage. The use of additional system tools (such as task trackers) to assist with follow-up of outstanding tests may help augment services provided electronically. These observations may be generalizable to other VA centers and other health-care systems where e-consult is becoming more widely adopted. Figure 1 Figure 1. Disclosures No relevant conflicts of interest to declare.

scholarly journals Malignant Post-Transplant Lymphoproliferative Disorder of Nasopharynx in Myelodysplastic Disorder

Healthcare ◽  
2021 ◽  
Vol 9 (2) ◽  
pp. 217
Author(s):  
Chih-Wei Luan ◽  
Chih-Cheng Chen ◽  
Kam-Fai Lee ◽  
Ming-Shao Tsai ◽  
Yao-Te Tsai ◽  
...  
Keyword(s):  
Organ Transplantation ◽  
Lymphoproliferative Disorder ◽  
Histopathological Examination ◽  
Solid Organ Transplantation ◽  
Low Grade ◽  
Solid Organ ◽  
Hematopoietic Stem ◽  
Post Transplant ◽  
Low Dose Radiotherapy

(1) Background: Post-transplant lymphoproliferative disorder (PTLD) is a hematological disease and occurs because of immunosuppression after organ transplantation. Only a few studies have reported PTLD in the nasopharynx. In most cases, PTLD developed after solid organ transplantation, and cases of PTLD after bone marrow transplantation, are uncommon. (2) Case presentation: We report the case of a 40-year-old woman with myelodysplastic disorder who underwent hematopoietic stem cell transplantation (HSCT). After 3 months, she developed low-grade fever, progressive nasal obstruction, and bloody rhinorrhea. Endoscopy revealed a mass completely occupying the nasopharynx. A polymorphic PTLD was diagnosed on the basis of histopathological examination results. Reduction in immunosuppression and low-dose radiotherapy were prescribed for treatment. After a 3-year follow-up, no recurrence of PTLD or myelodysplastic disorder was detected. (3) Conclusions: While nasopharyngeal PTLD is rare, a routine examination of the nasopharynx should be considered in the post-transplant follow-up of patients for early detection and treatment of PTLD.

scholarly journals Iceland screens, treats, or prevents multiple myeloma (iStopMM): a population-based screening study for monoclonal gammopathy of undetermined significance and randomized controlled trial of follow-up strategies

2021 ◽  
Vol 11 (5) ◽  
Author(s):  
Sæmundur Rögnvaldsson ◽  
Thorvardur Jon Love ◽  
Sigrun Thorsteinsdottir ◽  
Elín Ruth Reed ◽  
Jón Þórir Óskarsson ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Early Treatment ◽  
Monoclonal Gammopathy ◽  
Controlled Trial ◽  
Participation Rate ◽  
Population Based ◽  
Early Therapy ◽  
Screening Study ◽  
Undetermined Significance

AbstractMonoclonal gammopathy of undetermined significance (MGUS) precedes multiple myeloma (MM). Population-based screening for MGUS could identify candidates for early treatment in MM. Here we describe the Iceland Screens, Treats, or Prevents Multiple Myeloma study (iStopMM), the first population-based screening study for MGUS including a randomized trial of follow-up strategies. Icelandic residents born before 1976 were offered participation. Blood samples are collected alongside blood sampling in the Icelandic healthcare system. Participants with MGUS are randomized to three study arms. Arm 1 is not contacted, arm 2 follows current guidelines, and arm 3 follows a more intensive strategy. Participants who progress are offered early treatment. Samples are collected longitudinally from arms 2 and 3 for the study biobank. All participants repeatedly answer questionnaires on various exposures and outcomes including quality of life and psychiatric health. National registries on health are cross-linked to all participants. Of the 148,704 individuals in the target population, 80 759 (54.3%) provided informed consent for participation. With a very high participation rate, the data from the iStopMM study will answer important questions on MGUS, including potentials harms and benefits of screening. The study can lead to a paradigm shift in MM therapy towards screening and early therapy.

scholarly journals Prevalence of Monoclonal Gammopathy of Undetermined Significance in a Large Population with Annual Physical Examination in Beijing, China

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 5519-5519
Author(s):  
Jinuo Wang ◽  
Jian-Hua Han ◽  
Yue-lun Zhang ◽  
Xin-xin Cao ◽  
Dao-Bin Zhou ◽  
...  
Keyword(s):  
Physical Examination ◽  
Light Chain ◽  
Chinese Population ◽  
Monoclonal Gammopathy ◽  
Conflicts Of Interest ◽  
Large Population ◽  
M Protein ◽  
Monoclonal Protein ◽  
Significant Difference ◽  
Undetermined Significance

Introduction Monoclonal gammopathy of undetermined significance (MGUS) is a clinically asymptomatic premalignant plasma cell disorder. Previous studies in Western countries have described the prevalence of MGUS in Caucasians. However, data is limited in Chinese population. We therefore performed this study to ascertain the prevalence and characteristics of MGUS among Chinese population. Methods A total of 154597 consecutive healthy participants from Beijing who underwent annual physical examination between December 2013 and April 2019 at Peking Union Medical College Hospital were enrolled. Serum M protein was evaluated by capillary electrophoresis. Patients with a positive or suspicious serum M protein were suggested to be referred to the hematological clinic for immunofixation electrophoresis (IFE) and free light chain (FLC) assays. MGUS was defined in accordance with previous definitions. We calculated age-specific and sex-specific prevalence and described laboratory characteristics of patients with MGUS among those participants. Results MGUS were diagnosed in 843 patients (0.55%, 95%CI 0.51% to 0.59%). The median age at presentation was 58 years, with a range of 25-96 years. The overall prevalence of MGUS was 1.14% among participants aged 50 years or older and 2.6% among those aged 70 years or older. In both sexes, the prevalence increased with age: 0.1% (<40 years), 0.36% (40-49 years), 0.78% (50-59 years), 1.28% (60-69 years), 2.19% (70-79 years), and 3.77% (≥80 years) separately (Figure 1). The prevalence among men were higher than that among women (0.67% vs. 0.40%, OR =1.719, 95% CI 1.490 to 1.983, P<0.001) (Figure 1). The median concentration of serum Monoclonal protein was 1.4 g/L (0.1 -27.8 g/L). M protein level was less than 0.5g/L in 220 patients (26.1%), less than 5 g/L in 81.1% and more than 15 g/L in only 1.9% of 843 persons. There was no significant difference in the concentration of the monoclonal protein among the age groups. Of the 519 patients who were tested for IFE, the isotype of the monoclonal immunoglobulin was IgG in 344 (66.3%), IgA 112 (21.6%), IgM in 48 (9.2%), IgD in 2 (0.4%), light-chain in 3 (0.6%) and biclonal in 10 (1.9%). The serum light-chain type was kappa in 260 (50.1%), lambda in 255 (49.1%) patients, while 4 patients (0.8%) with biclonal M protein have both kappa and lambda light-chain. Of the 180 people who were tested for FLC, 42 (23.3%) had an abnormal FLC ratio. IgG isotype, M protein <15 g/L and normal FLC ratio were found in 102 patients (56.7%) and the remaining 78 people (43.4%) had 1(30.6%) or 2(12.8%) abnormal factors. Conclusions MGUS was found in 1.14% of persons 50 years of age or older and 2.6% among those 70 years of age or older among healthy Chinese population. The prevalence of MGUS increases with age. Males have a higher frequency of MGUS than Females. These observations offer the overall situation of MGUS epidemiology in a large Chinese population. Disclosures No relevant conflicts of interest to declare.

scholarly journals Primary cutaneous CD4+ small/medium T-cell lymphoma: a case report

2021 ◽  
Vol 22 (4) ◽  
pp. 199-203
Author(s):  
Jeenam Kim ◽  
Minkyoung Jeong ◽  
Dongkeun Jun ◽  
Myungchul Lee ◽  
Donghyeok Shin ◽  
...  
Keyword(s):  
Case Report ◽  
T Cell ◽  
Lymphoproliferative Disorder ◽  
Cell Lymphoma ◽  
Surgical Excision ◽  
Lymphoid Cells ◽  
The Face ◽  
Single Mass ◽  
Histopathologic Findings

Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder is a rare disease characterized by a single mass on the face or upper part of the trunk. It usually presents an asymptomatic and favorable progression, and its histopathologic findings include small and medium-sized lymphoid cells. The authors report a case of primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder on the forehead. A 51-year-old man presented with a protruding mass on his forehead that the patient had noted 1 month previously. Surgical excision and a permanent biopsy were performed under local anesthesia. Based on the biopsy results, the mass was diagnosed as a primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder. There was no evidence of recurrence at a 15-month follow-up visit.

Cytotoxic T-lymphocyte therapy for post-transplant lymphoproliferative disorder after solid organ transplantation in children

2018 ◽  
Vol 22 (2) ◽  
pp. e13133 ◽  
Author(s):  
Fang Kuan Chiou ◽  
Sue V. Beath ◽  
Gwen M. Wilkie ◽  
Mark A. Vickers ◽  
Bruce Morland ◽  
...  
Keyword(s):  
Organ Transplantation ◽  
T Lymphocyte ◽  
Lymphoproliferative Disorder ◽  
Cytotoxic T Lymphocyte ◽  
Solid Organ Transplantation ◽  
Solid Organ ◽  
Post Transplant
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