scholarly journals Pure Red Cell Aplasia and Other Haematological Diseases Associated With Thymoma: A Case Series and Systematic Review

2021 ◽  
Vol 8 ◽  
Author(s):  
Chih-Chieh Yen ◽  
Wei-Li Huang ◽  
Sin-Syue Li ◽  
Ya-Ping Chen ◽  
Yau-Lin Tseng ◽  
...  
Keyword(s):  
Systematic Review ◽  
Autoimmune Diseases ◽  
Large Scale ◽  
Remission Rate ◽  
Case Reports ◽  
Case Series ◽  
Pure Red Cell Aplasia ◽  
Red Cell ◽  
Good’S Syndrome ◽  
Red Cell Aplasia

Background: Thymoma-associated haematological diseases (HDs), such as pure red cell aplasia (PRCA) and Good's syndrome, are extremely rare, and due to the paucity of large-scale studies, the characteristics, remission after thymectomy, and long-term evaluation remain undetermined.Methods: We retrospectively assessed patients with thymoma and associated HDs from Jan 2005 to Dec 2020. All patients received thymectomy and/or additional treatments for HDs. A comparison with thymoma-associated myasthenic gravis (MG), and a systematic review from PubMed/MEDLINE and Embase were conducted.Results: In the median follow-up of 56 months, 130 patients were enrolled. Patients with thymoma-associated MG (n = 46) and HDs [n = 8; PRCA (n = 5), PRCA and Good's syndrome (n = 2) and autoimmune haemolytic anaemia (n = 1)] were evaluated. Patients with MG had a significantly higher remission rate after thymectomy (50 vs. 17%; p = 0.0378) as compared to those with other autoimmune diseases. Two of seven patients with PRCA experienced remission with thymectomy alone, and an additional two patients achieved remission with thymectomy plus immunosuppressive therapy (IST). In the systematic review, 60 studies (case reports, n = 46; case series including the present study, n = 14) were evaluated. Forty-four percent of patients were diagnosed with PRCA after thymoma, and 61% achieved remission with thymectomy plus IST; however, Good's syndrome was unaffected.Conclusions: Our study indicates that patients with thymoma-associated autoimmune diseases other than MG have a lower remission rate than those with MG. Remission of thymoma-associated PRCA can be achieved by thymectomy and IST. This study provides insight into extremely rare but puzzling autoimmune manifestations.

scholarly journals Thymoma with Concomitant Pure Red Cell Aplasia, Good’s Syndrome and Myasthenia Gravis Responding to Rituximab

2014 ◽  
Vol 32 (S1) ◽  
pp. 219-222 ◽  
Author(s):  
Ahmad I. Antar ◽  
Zaher K. Otrock ◽  
Mohamed A. Kharfan-Dabaja ◽  
Rami A. Mahfouz ◽  
Raafat S. Alameddine ◽  
...  
Keyword(s):  
Myasthenia Gravis ◽  
Pure Red Cell Aplasia ◽  
Red Cell ◽  
Good’S Syndrome ◽  
Red Cell Aplasia

Immunological and Hematological Findings Associated with Thymic Epithelial Neoplasms

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 1742-1742
Author(s):  
Muhammad Rizwan Khawaja ◽  
Nicholas J Miller ◽  
Patrick J Loehrer ◽  
Robert P. Nelson
Keyword(s):  
T Cell ◽  
Oral Candidiasis ◽  
Pure Red Cell Aplasia ◽  
Red Cell ◽  
Good’S Syndrome ◽  
Red Cell Aplasia ◽  
Morvan’S Syndrome ◽  
Number Of Patients ◽  
Autoimmune Conditions ◽  
Epithelial Neoplasms

Abstract Abstract 1742 Introduction: Although it is well known that thymic epithelial neoplasms are associated with autoimmune and manifestations of immunodeficiency, the extent to which abnormatilies in clinical hematological or immunological function occur in this population has not been completely documented. This retrospective study aimed to characterize the clinical hematological and immunological features of patients with thymic epithelial neoplasms. Methods: From a cohort of 512 patients with thymic epithelial neoplasms, 79 patients (33 males, 46 females) with diagnosed autoimmune/immunodeficiency conditions or signs and/or symptoms suggesting an autoimmune or immunodeficiency state were evaluated by standard immunogical and hematological testing. “Autoimmune conditions” included myasthenia gravis, pure red cell aplasia (PRCA), thyroiditis/hypothyroidism, glomerulonephritis, Sjõgren's syndrome, systemic lupus erythematosus, transverse myelitis, Issac's syndrome, Raynaud's disease, granuloma annulare, vitiligo, alopecia, dermatomyositis, Morvan's syndrome and chronic urticaria. Conditions designated as “immunodeficiencies” included Good's syndrome, onychomycosis, recurrent oral candidiasis, herpes zoster and opportunistic infections designated as AIDS-defining illnesses. Results were collected and tabulated from data in the electronic medical record CareWeb™, the web browser version of the Regenstrief Medical Records System of the Indiana University and Melvin and Bren Simon Cancer Center. Results: Pure red cell aplasia (PRCA) was present in 6 (7.6%) and Good's syndrome in 7 (8.9%) patients. Cytopenias were diagnosed in 40 (50.6%); anemia in 25 (31.6%); leucopenia in 12 (15.2%) and thrombocytopenia in 7 (8.9%) patients. IgG, IgA and IgM levels were low in 12 (15.2%), 9 (11.4%) and 15 (18.9%) patients respectively. Quantification of peripheral blood lymphocyte immunophenotypes revealed increases in CD2+ (n=44; 57.1%), CD3+ (n=33; 41.8%) and CD8+ (32; 40.5%) cell percentages and decreases in CD4+ (25; 31.6%) and CD19+ (36; 46.2%) cell percentages. Inverted CD4/CD8 ratios were present in 18 (23.7%) patients. The presence of “immunodeficiency condition(s)” was associated with a high CD8+ percentage (p=0.040), low CD19+ percentage (p=0.025) and an inverted CD4/CD8 ratio (p=0.034). The presence of an “autoimmune condition(s)” was associated with a high/normal CD4+ percentage (p=0.038). A high CD2+ percentage was associated with lower mean IgG and IgA levels (p=0.030 and p=0.017, respectively). Patients with high CD3+ and CD8+ percentages had lower mean IgA levels (p=0.046 and p=0.013, respectively). A low CD19+ percentage was strongly associated with lower mean IgG and IgA levels (p=0.004 and p=0.001, respectively). No associations were observed between lymphocyte subpopulation percentages and mean serum IgM levels. Conclusion: Although we observed a number of patients with PRCA and Good's syndrome, this selected cohort of patients with thymic epithelial neoplasms had a broad spectrum of cytopenias and concomitant autoimmune/immunodeficiency conditions. We observed high T cell percentages and low B cell percentages compared to adult normal values. High T cell profiles were commonly attributed to increased CD8+ percentages. Patients with autoimmune conditions are more likely to have normal or high CD4+ percentages; patients with clinical immunodeficiencies are more likely to have high CD8+, low CD19+ percentages and inverted CD8/CD4 ratios. These observations suggest a role for the assessment of immunoglobulin levels and lymphocyte immunophenotypes in patients with thymic epithelial neoplasms and associated immune mediated conditions. Disclosures: No relevant conflicts of interest to declare.

P1726C3 GLOMERULOPATHY RECURRENCE AFTER KIDNEY TRANSPLANT: A SYSTEMATIC REVIEW

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Oumayma Taroua ◽  
Yassir Selouani
Keyword(s):  
Systematic Review ◽  
Kidney Transplant ◽  
Large Scale ◽  
Case Reports ◽  
Meta Analysis ◽  
Case Series ◽  
Qualitative Synthesis ◽  
Post Transplantation ◽  
Post Transplant ◽  
Disease Free

Abstract Background and Aims C3 glomerulopathy (C3G) is a recently defined entity, characterized by the dysregulation of the complement pathway, leading to deposition of C3 complement in the glomeruli, with no immunoglobulins, leading to glomerular inflammation. C3G encompasses 2 disorders: C3 glomerulonephritis (C3GN) and Dense Deposit Disease (DDD), distinguished by their morphological pattern. Although multiple pharmacological treatments exist to control the progression of the disease, for patients reaching End-Stage Kidney Disease, kidney transplantation remains the last resort. While it is known that membranoproliferative glomerulopathies carry a risk of recurrence after transplant, no large-scale meta-analysis was done after 2015 to assess if the precise recurrence risk and remission duration for C3 glomerulopathies. The goal of this work is to determine if there is currently enough literature specific for C3G to conduct such a meta-analysis. Method Our research protocol was guided by the Preferred Reporting Items for Systematic Review and Meta-Analysis Protocols (PRISMA-P), and the Joanna Briggs Institute Critical Appraisal Tools. A search was conducted in PubMed, Web of Science and Scopus databases, using a specific search string, at the conclusion of which, 185 papers were found. The identified papers were subsequently screened by the 2 authors independently using precise inclusion and exclusion criteria. The screening resulted in the final inclusion of 7 papers, on which a qualitative synthesis was performed. The information extracted was organized on basis of demographics, time of transplantation, disease recurrence and disease-free period post-transplantation. Results Among the 7 papers selected, 2 were case series and 5 were case reports. In total, 23 patients were reported as having recurrence of C3G. For the patients whose age at diagnosis was known, it ranged between 9 and 51 years of age. Among the 23 patients, 16 of them were male, while 7 of them were females. The C3G subtype was determined for 21 patients, with 7 being classified as having DDD, and 14 having C3GN. The age of transplant was reported for 20 patients, ranging from 11 to 70 years old. The disease-free period between the kidney transplant and the recurrence of the disease ranged from 14 days to 101 months, with 1 case series paper only reported the median time to recurrence in months (14[0-80] for C3GN patients and 15[2-32] for DDD patients). The same paper reported the post-transplant recurrence rate among transplanted patients, determined as 10 to 12 for C3GN patients and 6 to 7 for DDD patients. Conclusion While C3G, with its 2 subtypes, have been well defined entities for 5 years, our review reveals that very little research about the post-transplant evolution and recurrence of these disease has been done. While extensive research can be found on the recurrence risks of Membranoproliferative Glomerulonephritis, we believe that with the new classification, more data on the new subtypes is necessary to guide the decision-making of clinicians for these patients.

scholarly journals Good's Syndrome-Associated Pure Red Cell Aplasia with Myelodysplastic Syndrome

2011 ◽  
Vol 50 (18) ◽  
pp. 2011-2014 ◽  
Author(s):  
Hideaki Nitta ◽  
Yuka Harada ◽  
Yoshiko Okikawa ◽  
Masayoshi Fujii ◽  
Koji Arihiro ◽  
...  
Keyword(s):  
Myelodysplastic Syndrome ◽  
Pure Red Cell Aplasia ◽  
Red Cell ◽  
Good’S Syndrome ◽  
Red Cell Aplasia

scholarly journals Pure Red Cell Aplasia Associated with Good's Syndrome Accompanied by Decreased Stem Cell Factor Production in the Bone Marrow

2010 ◽  
Vol 49 (5) ◽  
pp. 377-382 ◽  
Author(s):  
Kageaki Kuribayashi ◽  
Akihito Fujimi ◽  
Masayoshi Kobune ◽  
Rishu Takimoto ◽  
Shohei Kikuchi ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Stem Cell ◽  
Stem Cell Factor ◽  
Pure Red Cell Aplasia ◽  
Red Cell ◽  
Good’S Syndrome ◽  
Red Cell Aplasia ◽  
Factor Production

Coexistence of Pure Red Cell Aplasia and Autoimmune Haemolytic Anaemia Associated with Thymoma

2020 ◽  
Vol 143 (5) ◽  
pp. 491-495
Author(s):  
Wei Wang ◽  
Li-Yan Chen ◽  
Weiwei Zhao ◽  
Yuyue Ren ◽  
Lianjie Wang ◽  
...  
Keyword(s):  
Epithelial Cells ◽  
Autoimmune Diseases ◽  
Haemolytic Anaemia ◽  
Pure Red Cell Aplasia ◽  
Immune Dysregulation ◽  
Autoimmune Haemolytic Anaemia ◽  
Red Cell ◽  
Red Cell Aplasia ◽  
Disease Entities

Thymoma is an uncommon neoplasia derived from the epithelial cells of the thymus, which leads to immune dysregulation and is associated with a series of autoimmune diseases. However, the concurrence of these disease entities is rare, and the exact mechanisms of these diseases are still unclear. We have admitted several cases who were diagnosed with thymoma, autoimmune haemolytic anaemia, and pure red cell aplasia. These cases were the first to report the concurrence of these three disorders. After thymectomy, anaemia improved, haemolytic cells decreased, and haemoglobin was normalized.

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