scholarly journals A case report: paroxysmal nocturnal hemoglobinuria and systemic lupus erythematosus association

2020 ◽  
pp. IJH30
Author(s):  
Istemi Serin ◽  
Aslıhan Bayir ◽  
Hasan Goze ◽  
Osman Yokus
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Bone Marrow ◽  
Lupus Erythematosus ◽  
Paroxysmal Nocturnal Hemoglobinuria ◽  
Complete Blood Count ◽  
Bone Marrow Failure ◽  
Immunosuppressive Treatment ◽  
Specific Treatment ◽  
Systemic Lupus ◽  
Pnh Clone

Paroxysmal nocturnal hemoglobinuria (PNH) is defined by acquired intravascular hemolytic anemia, thrombosis and bone marrow failure with pancytopenia. Systemic lupus erythematosus (SLE) also appears as an autoimmune disease. The coexistence of both is rarely reported. Here we report the case of a 30-year-old female presenting with pancytopenia and diagnosed as SLE, who also had a PNH clone. Bone marrow biopsy did not support hypoplastic anemia. The patient was then followed up with the consideration of the existence of a PNH clone with SLE. She was treated by the rheumatology department and complete blood count improved under immunosuppressive treatment. The coexistence of CD59–CD55 deficiency with autoimmune diseases has been reported. It is an important example in terms of receiving clinical response with SLE-specific treatment.

scholarly journals Sustained high expression of multiple APOBEC3 cytidine deaminases in systemic lupus erythematosus

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Danielle Perez-Bercoff ◽  
Hélène Laude ◽  
Morgane Lemaire ◽  
Oliver Hunewald ◽  
Valérie Thiers ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Cell Death ◽  
Lupus Erythematosus ◽  
Immunosuppressive Treatment ◽  
Elevated Serum ◽  
Serum Levels ◽  
Chronic Inflammatory Disease ◽  
Mrna Levels ◽  
Systemic Lupus ◽  
Inflammatory Conditions

AbstractAPOBEC3 (A3) enzymes are best known for their role as antiviral restriction factors and as mutagens in cancer. Although four of them, A3A, A3B, A3F and A3G, are induced by type-1-interferon (IFN-I), their role in inflammatory conditions is unknown. We thus investigated the expression of A3, and particularly A3A and A3B because of their ability to edit cellular DNA, in Systemic Lupus Erythematosus (SLE), a chronic inflammatory disease characterized by high IFN-α serum levels. In a cohort of 57 SLE patients, A3A and A3B, but also A3C and A3G, were upregulated ~ 10 to 15-fold (> 1000-fold for A3B) compared to healthy controls, particularly in patients with flares and elevated serum IFN-α levels. Hydroxychloroquine, corticosteroids and immunosuppressive treatment did not reverse A3 levels. The A3AΔ3B polymorphism, which potentiates A3A, was detected in 14.9% of patients and in 10% of controls, and was associated with higher A3A mRNA expression. A3A and A3B mRNA levels, but not A3C or A3G, were correlated positively with dsDNA breaks and negatively with lymphopenia. Exposure of SLE PBMCs to IFN-α in culture induced massive and sustained A3A levels by 4 h and led to massive cell death. Furthermore, the rs2853669 A > G polymorphism in the telomerase reverse transcriptase (TERT) promoter, which disrupts an Ets-TCF-binding site and influences certain cancers, was highly prevalent in SLE patients, possibly contributing to lymphopenia. Taken together, these findings suggest that high baseline A3A and A3B levels may contribute to cell frailty, lymphopenia and to the generation of neoantigens in SLE patients. Targeting A3 expression could be a strategy to reverse cell death and the generation of neoantigens.

Longterm Outcomes and Damage Accrual in Patients with Childhood Systemic Lupus Erythematosus with Psychosis and Severe Cognitive Dysfunction

2013 ◽  
Vol 40 (4) ◽  
pp. 513-519 ◽  
Author(s):  
Lily Siok Hoon Lim ◽  
Arlette Lefebvre ◽  
Susanne Benseler ◽  
Earl D. Silverman
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Cognitive Dysfunction ◽  
Lupus Erythematosus ◽  
Clinical Course ◽  
Immunosuppressive Treatment ◽  
Damage Index ◽  
Response To Treatment ◽  
Systemic Lupus ◽  
Damage Accrual

Objective.(1) To describe the clinical course and response to treatment; and (2) to evaluate and compare damage accrual of distinct phenotypic subgroups of patients with clinically important psychiatric illness of pediatric systemic lupus erythematosus (pSLE).Methods.A single-center cohort study of patients with pSLE followed at a pediatric lupus clinic from 1985 to July 2009. Clinical course and response to treatment were studied. Remission was defined by absence of psychiatric/cognitive symptoms while receiving minimal doses of prednisone. Disease activity and damage were measured using SLE Disease Activity Index and SLE Damage Index.Results.Fifty-three children were included: 40 with psychosis and cognitive dysfunction (PSYC group) and 13 with isolated cognitive dysfunction (COG group). All received immunosuppressive treatment. Eighteen of 32 treated with azathioprine required a change to cyclophosphamide for poor response but none on cyclophosphamide required a change. The median times to remission were 72 weeks (PSYC) and 70 weeks (COG). Eight patients (7 PSYC, 1 COG) experienced flare following response/remission. New damage was noted in 50% of children at a median of 11 months: 57% of PSYC group, 31% of COG group. Persistent cognitive dysfunction was seen in 16% of PSYC patients and 15% of COG patients.Conclusion.Most patients responded to immunosuppressive treatment, although median time to remission was > 1 year. Roughly half the patients acquired a new damage item, most of which did not interfere with functional abilities. Fewer than 20% of patients developed neuropsychiatric damage. Both phenotypes of psychiatric pSLE responded equally well to current treatment.

scholarly journals Cell-mediated amegakaryocytic thrombocytopenia associated with systemic lupus erythematosus

Blood ◽  
1986 ◽  
Vol 67 (2) ◽  
pp. 479-483
Author(s):  
T Nagasawa ◽  
T Sakurai ◽  
H Kashiwagi ◽  
T Abe
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Bone Marrow ◽  
T Cells ◽  
Lupus Erythematosus ◽  
Culture System ◽  
Bone Marrow Cells ◽  
Rare Complication ◽  
Systemic Lupus ◽  
Marrow Cells

We studied a patient with a rare complication of amegakaryocytic thrombocytopenia (AMT) associated with systemic lupus erythematosus (SLE). To investigate the underlying pathogenesis of AMT, the effects of peripheral blood T cells and serum on human megakaryocyte progenitor cells were studied using in vitro coculture techniques. Mononuclear bone marrow cells (2 X 10(5) from normal donors produced 33.6 +/- 8.8 (n = 10) colony-forming unit-megakaryocytes (CFU-M) in our plasma clot system. When 2 X 10(5) of the patient's T cells were added to the culture system, the number of CFU-M decreased to only 3.5 +/- 0.6/2 X 10(5) bone marrow cells. No evidence of inhibitory effects was found by the addition of the patient's serum and complement to the culture system. The T cells stored at -80 degrees C on admission were also capable of suppressing autologous CFU-M after recovery from AMT. These results indicate that in vitro suppression of CFU-M from allogenic and autologous bone marrow cells by this patient's T cells provides an explanation for the pathogenesis of AMT associated with SLE.

scholarly journals 142 Approaching cytopenias in systemic lupus erythematosus: what does the bone marrow reveal?

2019 ◽  
Author(s):  
Samuel Govea-Pelaez ◽  
Ana Barrera-Vargas ◽  
Javier Merayo-Chalico ◽  
Jonathan Campos-Guzmán ◽  
Roberta Demichelis-Gómez ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Bone Marrow ◽  
Lupus Erythematosus ◽  
Systemic Lupus

scholarly journals Relationship between CBC and ESR and duration of disease in patients with systemic lupus erythematosus (SLE)

2018 ◽  
Vol 13 (2) ◽  
pp. 41-46
Author(s):  
Mahmuda Abira ◽  
Qazi Shamima Akhter
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Complete Blood Count ◽  
Hematological Parameters ◽  
Significant Negative Correlation ◽  
Control Group ◽  
Systemic Lupus ◽  
Initial Manifestation ◽  
Cross Sectional ◽  
Duration Of Disease

Background: Systemic lupus erythematosus (SLE) is an autoimmune disease and is associated with considerable morbidity. Hematological abnormalities may be the initial manifestation. Duration may have impact on their hematological parameters. Objective: To observe the relationship of Complete Blood Count (CBC) and Erythrocyte sedimentation Rate(ESR) with duration of disease in patients with SLE. Methods: This cross sectional study was conducted in the Department of Physiology, Dhaka Medical College, Dhaka from July 2016 to June 2017. Total 60 subjects including both sexes were selected with age ranging from 18 to 55 years. Among them, 30 subjects with SLE were study group and 30 age matched healthy subjects were control group. Total count of RBC, WBC, Platelet and ESR were estimated by automated hematology analyzer. Statistical analysis was done by unpaired Students æt” test, Chi Square test and Pearson’s correlation coefficient (r) test. Results: In this study, ESR was significantly (p<0.001) higher and RBC,WBC and platelet counts were significantly (p<0.001) lower in SLE patients in comparison to that of control. On correlation analysis, RBC and WBC showed significant negative correlation with duration of disease in patients with SLE. Conclusion: This study concludes that CBC and ESR were altered in SLE patients and it was related to duration of disease. J Bangladesh Soc Physiol. 2018, December; 13(2): 41-46

scholarly journals Renal transplantation for lupus nephritis: non-adherence and graft survival

Lupus ◽  
2019 ◽  
Vol 28 (5) ◽  
pp. 651-657 ◽  
Author(s):  
E Ntatsaki ◽  
V S Vassiliou ◽  
A Velo-Garcia ◽  
A D Salama ◽  
D A Isenberg
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Renal Transplantation ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Graft Rejection ◽  
Graft Failure ◽  
Immunosuppressive Treatment ◽  
Poor Adherence ◽  
Systemic Lupus ◽  
Group 2

Objectives Poor adherence to immunosuppressive treatment is common in patients with systemic lupus erythematosus and may identify those with lupus nephritis (LN) who have a poorer prognosis. Non-adherence has also been reported to be a potential adverse outcome predictor in renal transplantation (rTp). We investigated whether non-adherence is associated with increased rTp graft rejection and/or failure in patients with LN. Methods Patients with LN undergoing rTp in two major London hospitals were retrospectively included. Medical and electronic records were reviewed for documented concerns of non-adherence as well as laboratory biochemical drug levels. The role of non-adherence and other potential predictors of graft rejection/failure including demographics, comorbidities, age at systemic lupus erythematosus and LN diagnosis, type of LN, time on dialysis prior to rTp and medication use were investigated using logistic regression. Results Out of 361 patients with LN, 40 had rTp. During a median follow-up of 8.7 years, 17/40 (42.5%) of these patients had evidence of non-adherence. A total of 12 (30.0%) patients experienced graft rejection or failure or both. In the adherent group 2/23 (8.7%) had graft rejection, whilst in the non-adherent this rose to 5/17 (29.4%, p = 0.11). Graft failure was seen in 5/23 (21.7%) patients from the adherent group and 4/17 (23.5%) in the non-adherent group ( p = 0.89). Non-adherent patients had a trend towards increased graft rejection, hazard ratio 4.38, 95% confidence interval = 0.73–26.12, p = 0.11. Patients who spent more time on dialysis prior to rTp were more likely to be adherent to medication, p = 0.01. Conclusion Poor adherence to immunosuppressive therapy is common and has been shown to associate with a trend towards increased graft failure in patients with LN requiring rTp. This is the first paper to report that shorter periods on dialysis prior to transplantation might lead to increased non-adherence in lupus patients.

scholarly journals Autoimmune myelofibrosis: a rare haematological involvement in systemic lupus erythematosus

2019 ◽  
Vol 12 (1) ◽  
pp. bcr-2018-227520 ◽  
Author(s):  
Nabil Belfeki ◽  
Gopinath Shankarasivam ◽  
Damienne Declerck ◽  
Sylvain Diamantis
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Bone Marrow ◽  
Clinical Features ◽  
Bone Marrow Biopsy ◽  
Vitamin K ◽  
Lupus Erythematosus ◽  
Antiphospholipid Antibodies ◽  
Vitamin K Antagonists ◽  
Autoimmune Disorders ◽  
Systemic Lupus

Autoimmune myelofibrosis is a distinct clinicopathological entity that occurs with autoimmune disorders. We report the case of a 44-year-old woman admitted with pancytopenia and clinical features of systemic lupus erythematosus, Sjogren’s syndrome and antiphospholipid antibodies syndrome. Bone marrow biopsy showed decreased global cells and an increase of reticulin fibres on argentic coloration, consistent with myelofibrosis. The JAK2 V617, Myeloproliferative leukemia (MPL) and calreticulin mutations were negative. The patient’s condition improved after treatment with hydroxychloroquine, vitamin K antagonists and prednisone.

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