scholarly journals Antiphospholipid Antibody Syndrome Presenting with Hemichorea

2012 ◽  
Vol 2012 ◽  
pp. 1-2 ◽  
Author(s):  
Yezenash Ayalew ◽  
Fazlihakim Khattak

A 25-year-old Bangladeshi lady presented to neurology with a three-month history of involuntary movements of her right arm, associated with loss of power. There was progression to the right leg, and she subsequently developed episodes of slurred speech and blurred vision. At the time of presentation, she was 12 weeks pregnant and the symptoms were reported to have started at conception. Past medical history was unremarkable apart from one first trimester miscarriage and there was no significant family history suggestive of a hereditary neurological condition. MRI of the head revealed no abnormalities but serology showed positive antinuclear antibodies (ANAs) at a titre of 1/400. Further investigations revealed strongly positive anticardiolipin antibodies (>120) and positive lupus anticoagulant antibodies. The patient had a second miscarriage at 19 weeks gestation strengthening the possibility that the chorea was related to antiphospholipid antibody syndrome and she was started on a reducing dose of Prednisolone 40 mg daily and aspirin 300 mg daily. Six months later, she had complete resolution of neurological symptoms. There are several reports of chorea as a feature of antiphospholipid syndrome, but no clear consensus on underlying pathophysiology.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 4089-4089 ◽  
Author(s):  
Muhammad S. Shurafa ◽  
Nidhika Babbar ◽  
Quresh Khairullah

Abstract Antiphospholipid antibody syndrome (APS) is a thrombophilic disorder defined by the presence of recurrent thrombosis associated with anticardiolipin antibodies (ACA). To date, there is no effdective therapy for this condition. Rituximab, a chimeric monoclonal antibody B Cells has been shown to be effective in autoimmune disorders. Sporadic abstracts have reported the disappearance of ACA after Rituximab treatment. We have successfully treated a patient with primary catastrophic APS and renal failure with Rituximab. Case Report: A thirty seven year old male with history of DVT, ischemic cardiomyopathy and cerebrovascular accident was admitted to the Hospital for the treatment of acute renal failure and thrombocytopenia. Physical examination was significant for cognitive deficits, mild hepatomegaly, generalized livido reticulairs, and acrocyanosis. Laboratory data revealed platelet count of 97000, creatinine 3.4 mg/dl, IgG ACA titer of 43.8 and negative IgM ACA and ANA. He had proteinuria with benign urinary sediment. Kidney biopsy showed thrombotic microangiopathy. Plasmaphersis was initiated and hemodialysis for the rapidly progressing renal failure. He continued his treatment in an outpatient hemodialysis unit while on Coumadin. Four weekly cycles of Rituximab were given as an outpatient. Serial laboratory investigations showed gradual normalization of the platelet count and disappearance of the IgG ACA. Gradually renal function recovered and hemodialysis was discontinued after three months. He remains in remission with no further thrombotic events.Conclusion: Rituximab is promising in the treatment of APS and must be considered in the first;line treatment of catastrophic APS. A clinical trial is necessary to test the feasibility of discontinuing anticoagulation in these patients. PERTINENT LABORATORY DATA DATE HEMOGLOBIN PLATELETS CREATININE ACA TITER 03/21/02 1.4 11/15/03 14.4 97 3.4 11/21/03 12.5 100 6.2 43.8 12/01/03 12.2 222 ON DIALYSIS 16.1 01/01/04 14.1 276 ON DIALYSIS 20.9 03/17/04 14.1 276 ON DIALYSIS 05/05/04 11.3 330 3.3 NEGATIVE


1998 ◽  
Vol 79 (02) ◽  
pp. 282-285 ◽  
Author(s):  
Josep Ordi-Ros ◽  
Francesc Monegal-Ferran ◽  
Nuria Martinez ◽  
Fina Cortes-Hernandez ◽  
Miquel Vilardell-Tarres ◽  
...  

SummaryObjective: To evaluate the usefulness of IgA antiphospholipid antibodies as markers of thrombosis and/or antiphospholipid antibody syndrome. Patients and Methods: A cross-sectional study design in a tertiary, university-based, autoimmune reference hospital. Seven-hundred ninety-five patients classified into five different groups – autoimmune diseases (255), deep vein thrombosis (153), transitory ischemic attacks (108), obstetric complications (196), infectious diseases (83) and controls (81) – were tested for IgA, IgG and IgM aPL, and lupus anticoagulant. Plasma and serum samples were drawn for detection of aPL using an internationally standardized ELISA method and LA was carried out using coagulometric assays. Results: True IgA aPL were found only in two patients with systemic lupus erythematosus; these patients were also positive to IgG aPL. Conclusion: The incidence of true positivity to IgA anticardiolipin antibodies is extremely low. Their determination was not helpful in diagnosing the antiphospholipid syndrome or in explaining thrombotic events or aPL related manifestations – fetal loss – in the groups studied.


2020 ◽  
Author(s):  
metin kaya gürgöze ◽  
Aslıhan Kara ◽  
Mehmet yusuf sarı ◽  
İlknur Çalık ◽  
Saadet Akarsu

Abstract Background: Although pyoderma gangrenosum (PG) -like lesions have been rarely described in adults with the antiphospholipid antibody syndrome (APS) and systemic lupus erythematosus (SLE), the occurrence of PG as a preceding manifestation of APS in children with SLE has not been reported until. We present a young girl with SLE and APS who developed progressive extstensive ulcerations that were consistent with PG.Case presentation: A 14-year-old girl with a 2-year history of SLE was admitted to our department, complaining painful crusted ulcerations on her legs. Skin biopsy was reported as PG. However, she did not respond to immunosuppressive therapy administered. When her skin biopsy findings is reassessed in keeping with the positive anticardiolipin antibody results, superficial small vessel microthrombosis was observed. Diagnosis of APS and PG developing secondary to SLE were made. It was resulted in marked clinical improvement with anticoagulation therapy in addition to immunosuppressives as is recommended in APS. Conclusions: Based in clinical, pathological and response to proposed treatment, we can state that PG -like lesions in children with SLE could be considered as a secondary form of APS.


1970 ◽  
Vol 11 (2) ◽  
pp. 205-206
Author(s):  
MP Ranjith ◽  
Ranjith Divya ◽  
S Meera ◽  
Shabu Bahuleyan ◽  
Roney Joseph Kuryan

Antiphospholipid antibody syndrome is an autoimmune disease characterized by thrombosis, both arterial andvenous, recurrent spontaneous abortion and the persistence of positive antiphospholipid antibodies. Placentalthrombosis is believed to be the cause of recurrent abortions, characteristic of the syndrome. We report a pregnantwith antiphospholipid antibody syndrome patient with history of recurrent miscarriages and managed successfullywith high dose heparin.Keywords: Antiphospholipid antibody syndrome; recurrent intra uterine death; HeparinDOI: 10.3329/jom.v11i2.5476J MEDICINE 2010; 11 : 205-206


Blood ◽  
1993 ◽  
Vol 82 (9) ◽  
pp. 2730-2741 ◽  
Author(s):  
KR McCrae ◽  
AM DeMichele ◽  
P Pandhi ◽  
MJ Balsai ◽  
P Samuels ◽  
...  

Abstract Women with anticardiolipin antibodies (ACLA) are at increased risk for fetal loss. One potential explanation for this outcome is that sera from these individuals contain antibodies reactive with trophoblast cells, which are involved in the establishment of the uteroplacental vasculature and maintenance of placental blood fluidity. To examine this hypothesis, we compared the incidence of trophoblast-reactive antibodies in 27 patients with ACLA and a history of fetal loss with that in 29 normal pregnant women. Sera from 20 patients, but only one control, contained trophoblast-reactive antibodies (P < .001). These antibodies were not directed against major histocompatibility class I antigens, and reacted with both term and first-trimester trophoblast cells. In most cases, sera from which ACLA were adsorbed by cardiolipin- containing liposomes maintained reactivity against cells. In addition, patient Ig fractions immunoprecipitated an approximately 62-kD protein from the trophoblast cell surface, stimulated the release of arachidonic acid and thromboxane A2 by trophoblasts, and inhibited the binding of prourokinase to trophoblast urokinase receptors. These observations show that sera from women with ACLA and a history of fetal loss contain antitrophoblast antibodies. These antibodies may be serologically distinct from ACLA, and may contribute to the pathogenesis of fetal demise.


2013 ◽  
Vol 109 (04) ◽  
pp. 669-675 ◽  
Author(s):  
Siva Ketha ◽  
Patrick Smithedajkul ◽  
Adrian Vella ◽  
Rajiv Pruthi ◽  
Waldemar Wysokinski ◽  
...  

SummaryAdrenal haemorrhage (AH) is a rare but potentially devastating complication of heparin-induced thrombocytopenia (HIT). Neither the prevalence nor the natural history of AH due to HIT are known. The objectives of this study were to identify the spectrum of AH causes, to characterise the frequency of AH due to HIT and determine the natural history of HIT-associated AH. All patients with incident adrenal haemorrhage from January 2002 through June 2012 seen at the Mayo Clinic were identified. Over this time frame, there were a total of 115 patients with AH of which 11 cases (10%; mean age 67 ± 8 years; 73% female) were associated with HIT. Of these, all but one occurred in the postoperative setting and involved both adrenal glands (89%) with acute adrenal insufficiency at the time of diagnosis. Cases were found incidentally during an evaluation for fever, shock, abdominal pain or mental status changes. All HIT patients experienced venous thrombosis at other locations including deep venous thromboses (n=14), pulmonary emboli (n= 4) and arterial thrombosis (n=2). Four patients undergoing total knee arthroplasty had “spontaneous HIT” with AH in the absence of identifiable heparin exposure. Other causes of AH included trauma (29%), sepsis (15%), antiphospholipid antibody syndrome (10%), and metastatic disease (12%). In conclusion, AH is an important but seldom recognised presumed thrombotic complication of HIT, which usually occurs in the postoperative period, especially after orthopaedic procedures. This syndrome can occur in the apparent absence of heparin exposure, especially following major joint replacement surgery.


Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 1054-1054 ◽  
Author(s):  
K. Firozvi ◽  
P. Acs ◽  
S. Baidas ◽  
R. Deveras ◽  
C. M. Kessler

Abstract Introduction: rFVIIa has been touted as a pancoagulant to reverse untoward hemorrhage in various clinical situations. We describe 3 hypercoagulable patients with enox-induced bleeding treated successfully and safely with rFVIIa. Case Summaries: Patient 1, a 58 year old female, received enox 60mg SQ q12 h for a left femoral DVT. On day 2, a bleeding right femoral pseudoaneurysm was detected. On day 3, the patient’s hematocrit fell from 37.5% to 22%, as swelling and pain ensued in the right thigh 4h after receiving her AM dose of enox. The concurrent PT, INR and aPTT were 18.1, 1.72 and 34.2 sec respectively. rFVIIa (20μg/kg) was administered intravenously with rapid cessation of bleeding. Patient 2, a 42 year old male, with a history of SLE, antiphospholipid antibody syndrome, and a distant history of a distal DVT was admitted for acute renal failure (creatinine of 4.2) secondary to lupus nephritis. One day after a kidney biopsy, the patient was placed on coumadin 5mg and continuous infusion of unfractionated heparin which was then changed to enox 70 mg SQ q 12. Both coumadin and enox were held after 4 d, once his PT, INR and aPTT reached 30, 3.97 and 56.2 sec respectively. The next day, a CT scan to evaluate a new abdominal pain revealed a large bleed at the kidney biopsy site. Despite transfusions of 6 bags of red blood cells, 4 bags of fresh frozen plasma, and 10mg of SQ vitamin K1, his hematocrit dropped to 19% and his PT, INR, and aPTT remained elevated at 28, 3.49, and 60.8 sec respectively. Thromboembolization was achieved to terminate bleeding from 2 of his 3 renal biopsy sites, the last of which was technically inaccessible. rFVIIa (30μg/kg) was administered as an intravenous bolus with immediate cessation of active bleeding. The next day, the antifactor Xa level was 0.12 anti-Xa U/ml and the PT, INR, and aPTT were 13.7, 1.09 and 45.1 sec, respectively and remained at these levels for the next 4 days. Patient 3, a 56 year old female with a prior history of multiple PEs and proximal DVTs due to protein S deficiency, was admitted for total knee arthroplasty. Admission labs were all within normal limits. Enox 80mg sq was initiated 24 h post-operatively for DVT prophylaxis. Four h later, brisk bleeding developed acutely from the surgical site. The simultaneous antifactor Xa level was 0.49 anti-Xa U/ml. rFVIIa (20μg/kg) was administered as an intravenous bolus and bleeding from the JP drain ceased instantly. All 3 patients stabilized within hours following administration of rFVIIa for their acute bleeding events; all required multiple transfusions of FFP and packed RBCs before rFVIIa; and all resumed anticoagulation without further bleeding. Discussion: Many clinicians fear that the rare untoward hemorrhage associated with any low molecular weight heparin (LMWH) preparation cannot be efficiently or rapidly reversed as there is no specific or reliable antidote. rFVIIa concentrate has reversed the anti-Xa properties of LMWH in ex vivo plasma-spiking experimental models but experience with use of rFVIIa to reverse LMWH-induced bleeding in vivo is lacking. Conclusion: This report suggests that rFVIIa administered in low doses (20–30μg/kg) reverses clinically significant LMWH-induced bleeding complications effectively, rapidly, and safely and should be considered as an adjunct in the treatment of LMWH-induced bleeding in patients with either hypercoagulable conditions or acute VTE. Clinical trials are needed to confirm the effectiveness of rFVIIa in this clinical scenario.


Author(s):  
Sushma

Acharya Kashyapa in Revatikalpa adhyaya explained about the Jataharinies, as a group of demons who has divine vision, attack women to destroy or to produce serious problems of menstrual cycle, recurrent abortions, still birth, intrauterine fetal death, neonatal deaths or severe disorders which decreases the life span of the fetus. As recurrent miscarriage, intrauterine death of an infant or death at birth has always been a devastating experience for the mother and of concern in clinical practice. All these mortality remains a challenge in the care of pregnant women worldwide, particularly for those who had history of adverse outcome in previous pregnancies (BOH). Antiphospholipid antibody syndrome (Hughes syndrome) is an autoimmune, hypercoagulable state caused by antiphospholipid antibodies. Pregnancy related complications are miscarriage, still birth, preterm delivery, IUD etc. This is one of the few treatable causes of pregnancy loss, and successful pregnancy rates of 70% or more can be achieved with appropriate treatment. Description of Jataharinies resemble with APLA, hence critical analysis will help to emphasize on management of Jataharinies by Ayurvedic treatment principles.


2019 ◽  
Vol 12 (10) ◽  
pp. e226494
Author(s):  
Amisha Jakharia-Shah ◽  
Hugh Wheatley ◽  
Matthew Beesley

A 59-year-old woman presented to an otolaryngology clinic with an 8-week history of a painless lump over her left parotid gland. Her medical history included an invasive ductal carcinoma (33 mm) and a ductal carcinoma in situ (70 mm) of the right breast, for which she had a mastectomy and various adjuvant therapies. The primary tumour presented 8 years prior to the metachronous metastasis. This patient was a non-smoker and had no significant family history. Post-superficial parotidectomy pathology revealed the parotid gland tumour to be oestrogen receptor-positive and HER2 receptor-positive, thus ruling out the initial differential diagnosis of a pleomorphic adenoma. A consequential total parotidectomy with a posterolateral neck dissection was performed with sparing of the facial nerve. The patient recovered well having only encountered a self-resolving salivary fistula. She portrayed no signs of facial nerve palsy and subsequent imaging scans showed no abnormalities.


2018 ◽  
Vol 2 (02) ◽  
pp. 51-58
Author(s):  
Md. Motahar Hossain ◽  
Md. Akhter Hossain ◽  
Yasmin Rahman ◽  
Md. Kamrul Hasan

Antiphospholipid syndrome (APS) is an autoimmune disease characterized by venous thromboembolism, arterial thrombosis, and obstetric morbidities in the setting of persistently positive levels of antiphospholipid antibodies. It may be primary or secondary. The latest classification criteria (Sydney 2006) recognize just three tests to define this syndrome- lupus anticoagulant, anticardiolipin antibodies and anti-?2-glycoprotein-1 antibodies. Treatment of thrombotic events involves lifelong anticoagulation with vitamin K antagonist warfarin. Antiphospholipid antibody syndrome (APS) with only pregnancy morbidity is treated with thromboprophylaxis with heparin during pregnancy and postpartum for 6 weeks. In this review we discuss the pathogenesis, diagnosis, treatment and prognosis of the APS.


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