A 21-Year-Old Woman with Thrombotic Thrombocytopenic Purpura as Initial Symptom of Systemic Lupus Erythematosus and a Literature Review.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 3963-3963
Author(s):  
Rongfu Zhou ◽  
Jian OuYang ◽  
Ming Zhou ◽  
Qiguo Zhang ◽  
Yong Xu
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Lupus Erythematosus ◽  
Treatment Time ◽  
Clinical Manifestations ◽  
Initial Symptom ◽  
Coombs Test ◽  
Systemic Lupus ◽  
Thrombocytopenic Purpura ◽  
Direct Coombs Test

Abstract We describe a 21-year-old woman with systemic lupus erythematosus (SLE) who first presented with an thrombotic thrombocytopenic purpura (TTP), and compare the clinical manifestations and prognosis between SLE patients with thrombotic thrombocytopenic purpura in the reported literature. At the time of admission, she was suffering from petechia, purpura and had neurological symptoms. Laboratory findings demonstrated haemolytic anaemia, thrombocytopaenia and high levels of fragmentocytes. Serological test results were highly positive for antinuclear antibodies (ANA) with a particle fluorescent pattern, and also detected antibodies against Smith antibody, RNP antibody and SSA antibody but was negative for dsDNA. Direct Coombs-test was positive and D-dimer was negtive. The activity of ADAMTS13 was significantly reduced even after 4 times plasmapheresis. TTP as presenting sign in the patient with SLE was diagnosed. After 5 times plasmapheresis treatments and immunosuppressive therapy she recovered and abnormal laboratory tests were gradually returned to normal. With a follow-up of 20 months, she had a normal life. Compared with other reports, TTP can be differentiated from other thrombotic microangiopathic syndromes by its normal levels of prothrombin time, partially activated thromboplastin time (APTT), fibrinogen and direct Coombs-test. But TTP might also be a complication of SLE and the manifestations of TTP are similar to those in SLE. The detection of the fragmentation of peripheral red blood cells helped the early diagnosis of TTP. Coomb’s test might be positive when patient with TTP had SLE. ADAMTS13 and its inhibitory antibody had an important role in the pathogenesis of TTP. Plasma exchange combined with corticosteroid and cyclophsphamide should be used as early as possible in TTP patient with SLE. The prognosis was related to the treatment time and methods including plasmapheresis.

Thrombotic Thrombocytopenic Purpura Associated with Systemic Lupus Erythematosus and Antiphospholipid Syndrome: A Case Report

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 4685-4685
Author(s):  
Yang He ◽  
Weiwen Yang ◽  
Changgeng Ruan
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Case Report ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Conflicts Of Interest ◽  
Clinical Manifestations ◽  
Systemic Lupus ◽  
Plasma Therapy ◽  
Thrombocytopenic Purpura

Abstract Abstract 4685 Background: The occurrence of thrombotic thrombocytopenic purpura (TTP) in patients with systemic lupus erythematosus (SLE) is rare TTP frequence. In pregnancy women is increased. TTP with simultaneous SLE, and antiphospholipid syndrome (APS) occurring in the same patient is extremely rare. Case report: We described a 41-year-old Chinese female with TTP in her third trimester (33+4 gestational weeks) of pregnancy who suffered from SLE. and APS at the same time. The clinical features and positive laboratory abnormalities included fever, repeated abortion in first trimester, palpitation, dyspnea, orthopnea, hypertension (180/113mmHg), proteinuria, renal dysfunction, thrombocytopenia (platelets count 12×109/L), hemolytic anemia (Hb 67g/L), positive Coombs test, positive lupus test, positive antinuclear antibody, anti-Sm antibody, anti-insulin antibody, anti-ENA antibody and anti-RNP antibody. The level of plasma ADAMTS13 activity was below 5%, and ADAMTS13 inhibitor was present in the patient's plasma. This patient was treated with a combination of corticosteroids and plasma infusion and plasmapheresis. At 33+5 weeks of gestation she delivered a healthy infant via cesarean section. The patient improved quickly with these therapies. Conclusion: This is a rare TTP case with simultaneous SLE, APS and preeclampsia. Her overlaping clinical manifestations and laboratory abnormalities made the diagnosis very difficult. These data indicate that plasma therapy and corticosteroids can improve the prognosis of TTP associated with SLE and APS. In addition, the termination of pregnancy may be also helpful for controlling the development of the disease. Disclosures: No relevant conflicts of interest to declare.

scholarly journals AB0007 STAT4 RS7574865 G/T POLYMORPHISM AS MARKER OF PREDISPOSITION TO SYSTEMIC LUPUS ERYTHEMATOSUS WITH JUVENILE ONSET

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1038.2-1039
Author(s):  
M. Kaleda ◽  
M. Krylov ◽  
I. Nikishina
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Pilot Study ◽  
Lupus Erythematosus ◽  
Renal Involvement ◽  
Control Group ◽  
Coombs Test ◽  
Systemic Lupus ◽  
Direct Coombs Test ◽  
Positive Direct ◽  
Cutaneous Lupus

Background:Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with a significant genetic predisposition. Recent studies have identified STAT4 (signal transducers and transcription activators 4) as a susceptibility gene for SLE.Objectives:To investigate the hypothesis of the association of STAT4 rs7574865 G/T polymorphism with the predisposition to SLE in children and its relationship with some of SLE manifestations.Methods:The case-control pilot study included 143 children (39 with SLE and 103 healthy unrelated volunteers as a control group). Diagnosis of SLE was based on 2012 SLICC criteria. STAT4 rs7574865 G/T polymorphism was investigated using allele-specific real-time polymerase chain reaction (RT-PCR).Results:The group of pts with SLE consisted of 29 girls and 10 boys, with an average age of 11.8±3.7 years (from 3 to 17 years) and an average disease duration of 4.1±2.4 years. 79.5% pts had acute cutaneous lupus at the onset, 46.1% - nonscarring alopecia, 71.8% - arthritis, 23.1% - oral and nasal ulcers, 23.1% - serositis, 43.6% - renal involvement, 35.9% –neuropsychiatric disorders. Leucopenia/lymphopenia was found in 71.8% of pts, thrombocytopenia – in 23,1%. ANA were detected in 100% pts, anti-dsDNA – in 79.5%, anti-Sm – in 31.6%, antiphospholipid antibodies - in 7,3%, hypocomplementemia – in 61.5%, positive direct Coombs test – in 35.9 %. Macrophage activation syndrome at the onset was documented in 15.4 % of pts. The distribution of rs7574865 genotypes in the control group showed no significant deviations from the Hardy-Weinberg equilibrium. The distribution of genotype frequencies among pts had statistically significant differences compared to the control (χ2=12.95, p=0.0015): GG-30.8% and 63.1% (p=0.001), GT-56.4% and 33.0% (p=0.018), TT-12.8% and 3.9% (p=0.114), GT+TT - 69.2% and 36.9% (p=0.0005). The frequency of the mutant STAT 4 allele T (polymorphism), was significantly higher in the SLE group than in the control group (41% and 20.4%, respectively; p=0.0007). We identified an association of the T allele with some clinical, laboratory, and immunological disorders in SLE: arthritis (OR 3.9, p=0.0002), acute cutaneous lupus (OR 2.47, p=0.003), nonscarring alopecia (OR 3.12, p=0.002), renal involvement (OR 2.42, p=0.022), leucopenia (OR 2.72, p=0.003), thrombocytopenia (OR 4.88, p=0.002), anti-dsDNA (OR 2.82, p=0.0006), hypocomplementemia (OR 2.34, p=0.012), positive direct Coombs test (OR 3.38, p=0.002).Conclusion:Our pilot study confirmed that the STAT4 rs7574865 G/T polymorphism was associated with the risk of SLE in children and some of SLE manifestations.Disclosure of Interests:None declared

scholarly journals An Early Unexpected Immune Thrombotic Thrombocytopenic Purpura Relapse Associated with SARS-CoV-2 Infection: A Case Report and Literature Review

2021 ◽  
pp. 1-5
Author(s):  
Maya Kornowski Cohen ◽  
Liron Sheena ◽  
Yair Shafir ◽  
Vered Yahalom ◽  
Anat Gafter-Gvili ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Case Report ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Lupus Erythematosus ◽  
Response To Therapy ◽  
Intravascular Hemolysis ◽  
Systemic Lupus ◽  
Thrombocytopenic Purpura ◽  
History Of ◽  
Triggering Factor

SARS-CoV-2 has been reported as a possible triggering factor for the development of several autoimmune diseases and inflammatory dysregulation. Here, we present a case report of a woman with a history of systemic lupus erythematosus and antiphospholipid syndrome, presenting with concurrent COVID-19 infection and immune thrombotic thrombocytopenic purpura (TTP). The patient was treated with plasma exchange, steroids, and caplacizumab with initial good response to therapy. The course of both TTP and COVID-19 disease was mild. However, after ADAMTS-13 activity was normalized, the patient experienced an early unexpected TTP relapse manifested by intravascular hemolysis with stable platelet counts requiring further treatment. Only 3 cases of COVID-19 associated TTP were reported in the literature thus far. We summarize the literature and suggest that COVID-19 could act as a trigger for TTP, with good outcomes if recognized and treated early.

scholarly journals Thrombotic thrombocytopenic purpura and systemic lupus erythematosus.

1986 ◽  
Vol 45 (4) ◽  
pp. 319-322 ◽  
Author(s):  
D A Fox ◽  
J D Faix ◽  
J Coblyn ◽  
P Fraser ◽  
B Smith ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Thrombocytopenic Purpura

scholarly journals Thrombotic Thrombocytopenic Purpura in a Patient with Systemic Lupus Erythematosus

2021 ◽  
Vol 8 (1) ◽  
Author(s):  
Guavita-Navarro Diana ◽  
Cajamarca-Baron Jairo ◽  
Buitrago-Bohorquez Jhon ◽  
Gallego-Cardona Laura ◽  
Guevara Diana ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Thrombocytopenic Purpura

THROMBOTIC THROMBOCYTOPENIC PURPURA: A DIAGNOSTIC CHALLENGE IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS

2021 ◽  
Author(s):  
Rubens Bonfiglioli ◽  
Vanessa Félix Nascimento Coelho ◽  
Andreza Lamonica ◽  
Thais de Campos Ferreira Pinto
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Diagnostic Challenge ◽  
Thrombocytopenic Purpura

, , , ,

Systemic Lupus Erythematosus with Secondary Thrombotic Thrombocytopenic Purpura and Acute Parkinsonism: A Case Report

2022 ◽  
Vol 71 (12) ◽  
Author(s):  
Pooja Deepak ◽  
Roha Saeed Memon ◽  
Fizza Tariq ◽  
Hassan Ahmed ◽  
Shaheen Bhatti
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Lupus Erythematosus ◽  
Late Onset ◽  
Case Reports ◽  
Gastrointestinal Symptoms ◽  
Signs And Symptoms ◽  
Urgent Care ◽  
Systemic Lupus ◽  
Thrombocytopenic Purpura

Systemic lupus erythematosus (SLE) is an autoimmune disease that has certain characteristic features but can also present with misleading signs and symptoms especially when it is of late-onset. Various case reports address its association with thrombotic thrombocytopenic purpura (TTP), however, its association with parkinsonism remains unclear. We present the case of a 58-year-old male who reported with acute-onset parkinsonism along with some gastrointestinal symptoms. Detailed laboratory investigations unmasked the underlying SLE with an overlapping picture of TTP. This unusual presentation in a resource-constrained setting created challenges and subsequent delays in the diagnosis and management of the patient. Despite urgent care, the patient’s age, presence of overlapping conditions, and multi-organ involvement were some of the factors due to which the treatment failed and he could not survive. We report the association of SLE with secondary TTP and parkinsonism.

Sign in / Sign up

Export Citation Format

Share Document