scholarly journals Immune Thrombocytopenia in Antiphospholipid Syndrome: Is It Primary or Secondary?

Biomedicines ◽  
2021 ◽  
Vol 9 (9) ◽  
pp. 1170
Author(s):  
Riccardo Tomasello ◽  
Giulio Giordano ◽  
Francesco Romano ◽  
Federica Vaccarino ◽  
Sergio Siragusa ◽  
...  
Keyword(s):  
High Risk ◽  
Antiphospholipid Syndrome ◽  
Antiphospholipid Antibodies ◽  
Immune Thrombocytopenia ◽  
Antiplatelet Agents ◽  
Warning Sign ◽  
Severe Thrombocytopenia ◽  
Appropriate Treatment ◽  
Hemorrhagic Risk ◽  
Antigenic Targets

Antiphospholipid syndrome (APS) is frequently associated with thrombocytopenia, in most cases mild and in the absence of major bleedings. In some patients with a confirmed APS diagnosis, secondary immune thrombocytopenia (ITP) may lead to severe thrombocytopenia with consequent major bleeding. At the same time, the presence of antiphospholipid antibodies (aPL) in patients with a diagnosis of primary ITP has been reported in several studies, although with some specific characteristics especially related to the variety of antigenic targets. Even though it does not enter the APS defining criteria, thrombocytopenia should be regarded as a warning sign of a “high risk” APS and thus thoroughly evaluated. The presence of aPL in patients with ITP should be assessed as well to stratify the risk of paradoxical thrombosis. In detail, besides the high hemorrhagic risk in secondary thrombocytopenia, patients with a co-diagnosis of APS or only antibodies are also at risk of arterial and venous thrombosis. In this narrative review, we discuss the correlation between APS and ITP, the mechanisms behind the above-reported entities, in order to support clinicians to define the most appropriate treatment strategy in these patients, especially when anticoagulant or antiplatelet agents may be needed.

scholarly journals Intracranial Hemorrhage in Egyptian Children with Primary Immune Thrombocytopenia (ITP): Report of 24 Cases in 20 Years

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 1080-1080
Author(s):  
Mohsen Saleh Elalfy ◽  
Mohamed Ahmed Badr ◽  
Ahmed Mansour ◽  
Tamer Hassan ◽  
Mohamed Meabed ◽  
...  
Keyword(s):  
High Risk ◽  
Head Trauma ◽  
Intracranial Hemorrhage ◽  
Immune Thrombocytopenia ◽  
Conflicts Of Interest ◽  
Complete Recovery ◽  
Published Data ◽  
Severe Thrombocytopenia ◽  
Egyptian Children ◽  
Neurosurgical Intervention

Background: 35 million children < 18 years; approximately 1800 new cases of ITP were diagnosed annually in Egypt. Intracranial hemorrhage (ICH) is a rare devastating complication of childhood immune thrombocytopenia (ITP). Incidence of ICH among children with ITP varies markedly in different studies from 0.2 up to 1.0 %. Intracranial hemorrhage after head trauma in children with ITP leads to significant morbidity and mortality. We published data during 1997 - 2007 (10 ICH) in children with primary ITP; risk factors and outcome. Aim & Methodology: A follow-up study to assess any change in outcome of ICH from last decade; whether new therapies might change the landscape of ICH. Centers treating > 25 child with prim ITP /year and offered a complete data of >150 children with ITP over 2 decades were enrolled. We compared 2008 - 2018 with the decade before it; variation of ICH reporting from center to center and outcome in relation to therapy. All children with ITP and ICH during study period had been treated, within < 24 hours and referred to neurosurgical hospital complex facility for consultation and intervention. Time elapsed till receiving platelet enhancing therapy and neurosurgical intervention was assessed, Outcome whether a complete recovery, permanent sequalae or death was reported. Results: Four thousand, three-hundred and forty primary ITP were evaluated, ( 380 were excluded due to incomplete data ) Twenty-four (0.6%) ICH were reported over 2 decades (14 in this decade with 20% increase incidence) and 48 matched ITP control subjects were evaluated. Platelet counts were less than 10 x 10(9)/L in 90% of children with ICH. Four (16.2%) children developed ICH within 14 days of diagnosis of ITP; one of these, was the presenting feature of ITP. four were from 3-12 months and sixteen (66.6%) of children had chronic ITP. Centers treating > 50 case/year had a higher frequency of reporting 0.8 % compared to 0.2 % in centers < 50 cases/year. Outcome is better on early intervention as well as aggressive platelet enhancing therapy with 70% complete recovery compared with 30% complete recovery on delayed intervention. Head trauma and hematuria and PC < 10 were the mostly associated with ICH, identified in 33%, 25% and 90% respectively of the patients with ICH and in 1, none and 50% of the controls (P < .001). Bleeding beyond petechiae and ecchymoses was also linked to ICH. Mortality was 25%; a further 25% had neurologic sequelae. Neurosurgical intervention was done in 25% of cases with good outcome. Reporting was more in this decade with better outcome in bigger centers. Conclusion: A rise in the incidence of ICH in Children with severe thrombocytopenia over last decade; high risk for ICH among those with PC< 10,000 plus head trauma and/or hematuria. Platelet enhancing agents whether HDMP or IVIG or TPO-RAs could not prevent ICH. However they had a good impact on survival and lessen sequalae if used in combination. Strategies by which high-risk children could be identified and well managed in small centers. Disclosures No relevant conflicts of interest to declare.

scholarly journals Intracranial hemorrhage (ICH) in children with immune thrombocytopenia (ITP): study of 40 cases

Blood ◽  
2009 ◽  
Vol 114 (23) ◽  
pp. 4777-4783 ◽  
Author(s):  
Bethan Psaila ◽  
Aleksandra Petrovic ◽  
Lemke K. Page ◽  
Jill Menell ◽  
Matthew Schonholz ◽  
...  
Keyword(s):  
High Risk ◽  
Head Trauma ◽  
Intracranial Hemorrhage ◽  
Immune Thrombocytopenia ◽  
The United States ◽  
Severe Thrombocytopenia ◽  
Aggressive Treatment ◽  
Neurologic Sequelae ◽  
Immune Thrombocytopenia Purpura ◽  
High Risk Children

Abstract Intracranial hemorrhage (ICH) is a rare but devastating complication of childhood immune thrombocytopenia purpura (ITP). A survey of ICH from 1987 to 2000 identified cases of ICH in childhood ITP in the United States. Forty patients with ICH and 80 matched ITP control subjects were accrued. The estimated incidence of ICH was 0.19% to 0.78%. Platelet counts were less than 20 × 109/L in 90% and less than 10 × 109/L in 75% of children with ICH. Eighteen (45%) children developed ICH within 7 days of diagnosis of ITP; for 10 of these, ICH was the presenting feature of ITP. Twelve (30%) children had chronic ITP. Head trauma and hematuria were the most prominent features associated with ICH, identified in 33% and 22.5% of the patients with ICH and 1 and none of the controls (both P < .001). Bleeding beyond petechiae and ecchymoses was also linked to ICH. Mortality was 25%; a further 25% had neurologic sequelae. Strategies by which high-risk children could be identified were considered, and the costs of preventive combination treatment were estimated. Children with severe thrombocytopenia plus head trauma and/or hematuria appeared to be at particularly high risk of ICH. Aggressive treatment of these children may be appropriate.

Clinical features and diagnosis of Ph - negative myeloproliferative neoplasms occurring in conjunction with the antiphospholipid syndrome

2019 ◽  
Vol 91 (7) ◽  
pp. 93-99
Author(s):  
A L Melikyan ◽  
I N Subortseva ◽  
E A Koloshejnova ◽  
E A Gilyazitdinova ◽  
K S Shashkina ◽  
...  
Keyword(s):  
High Risk ◽  
Clinical Features ◽  
Antiphospholipid Syndrome ◽  
Antiphospholipid Antibodies ◽  
Negative Impact ◽  
Myeloproliferative Neoplasms ◽  
Recurrent Thrombosis ◽  
Myeloproliferative Diseases ◽  
Thrombotic Complications ◽  
Clinical Cases

Thrombosis is a serious and extremely dangerous disease that has a negative impact on the quality and longevity. Antiphospholipid syndrome (APS) is a pathology characterized by recurring venous, arterial, microvasculature thrombosis, pregnancy pathology with loss of the fetus and the synthesis of antiphospholipid antibodies. A high risk of thrombotic complications is also observed in patients with myeloproliferative neoplasms (MPN). This article presents a description of three clinical cases of Ph - negative myeloproliferative diseases, occurring in conjunction with APS. In all cases, recurrent thrombosis allowed to suspect the presence of two diseases - MPN and APS.

Challenges in Treating Extensive Deep Vein Thrombosis with Severe Thrombocytopenia in Patients with Antiphospholipid Syndrome—A Follow-up of 2 Years

2019 ◽  
Author(s):  
Lee Kai Wei ◽  
Ashish Anil Sule
Keyword(s):  
Deep Vein Thrombosis ◽  
Antiphospholipid Syndrome ◽  
Lower Limb ◽  
Immune Thrombocytopenia ◽  
Femoral Vein ◽  
Severe Thrombocytopenia ◽  
Bleeding Events ◽  
Superficial Femoral Vein ◽  
Vein Thrombosis ◽  
Deep Vein

AbstractThrombocytopenia is one of the most common manifestations of antiphospholipid syndrome (APS). There is little evidence or definitive guidelines regarding the treatment of APS with thrombocytopenia. We describe a patient with APS and moderate-to-severe thrombocytopenia and the challenges of balancing anticoagulation with thrombocytopenia. A 19-year-old male patient presented with right lower limb swelling to the emergency department with a history of gradually worsening right leg swelling for 1 week and was diagnosed with right leg proximal deep vein thrombosis. Ultrasound Doppler of the right lower limb revealed complete venous thrombosis from the level of the popliteal vein to the distal superficial femoral vein. Subsequently, he was found to have triple-positive APS and moderate-to-severe immune thrombocytopenia, with a platelet count nadir of 31 × 10 to the ninth power/L. He was started on anticoagulation with warfarin. The severe thrombocytopenia was not treated with immunosuppressants and the platelets fluctuated in the range of moderate-to-severe thrombocytopenia but did not develop any rethrombotic or bleeding events. His platelets varied from 31 × 10 to the ninth power/L to 106 × 10 to the ninth power/L. This case report demonstrates that it may be safe to hold off treatment for thrombocytopenia in APS, even in cases of severe thrombocytopenia. Treatment with immunosuppressants may be instituted only when platelet levels fall below 20 × 10 to the ninth power/L or when there is clinically significant bleeding, as in primary immune thrombocytopenia.

ANTIPHOSPHOLIPID SYNDROME AND PREGNANCY

2020 ◽  
pp. 20-22
Author(s):  
Viktoriia Aleksandrovna Ilchenko ◽  
Yuliana Aleksandrovna Moshko
Keyword(s):  
Acetylsalicylic Acid ◽  
Antiphospholipid Syndrome ◽  
Postpartum Period ◽  
Antiphospholipid Antibodies ◽  
Cell Function ◽  
Clinical Manifestations ◽  
Antiplatelet Agents ◽  
The Body ◽  
Nerve Tissue ◽  
Endothelial Cell Function

The main issues of etiopathogenesis, clinical manifestations, diagnosis of antiphospholipid syndrome are considered. This syndrome is a state of the body whereat a thrombosis can occur, especially during pregnancy. This syndrome pathogenesis is characterized with an interaction of antiphospholipid antibodies with negatively charged phospholipids. They are the part of membranes of platelets, endotheliocytes, prothrombin−activating complex, beta−2−glycoprotein and nerve cells with impaired functions. Joining to phospholipids, the antiphospholipid antibodies lead to a sharp decrease in platelet count and impaired endothelial cell function. In nerve tissue disorders according to the structural and functional type occur, the formation and function of the humoral agents responsible for hemostasis are disrupted. The antiphospholipid syndrome in obstetrics and gynecology often manifests as spontaneous miscarriages, the effect of the "empty fetal egg", fetal growth retardation and even its antenatal death, as well as preeclampsia. In the treatment of pregnant women with antiphospholipid syndrome, mainly several protocols have been used, i.e. anticoagulants and antiplatelet agents in combination with glucocorticoids; combination of glucocorticoids with acetylsalicylic acid; monotherapy with sodium heparin or acetylsalicylic acid; anticoagulants and antiplatelet agents for hemostasis correction. The treatment protocols during pregnancy, childbirth and postpartum period have been presented. It is emphasized that the patients with antiphospholipid syndrome and vascular thrombosis should be monitored by rheumatologist, surgeon, obstetrician and gynecologist after successful child delivery. Duration of receiving antiplatelet and anticoagulant therapy has to be solved individually. Key words: antiphospholipid syndrome, antiphospholipid antibodies, cardiolipin antibodies, lupus anticoagulant, pregnancy, postpartum period.

A high-risk laboratory profile of antiphospholipid antibodies and thrombosis is associated with a large number of extra-criteria manifestations in obstetric antiphospholipid syndrome

2019 ◽  
Vol 67 (6) ◽  
pp. 478-485
Author(s):  
Sebastián Udry ◽  
José Omar Latino ◽  
Cristina Belizna ◽  
Silvia Perés Wingeyer ◽  
Diego Santiago Fernández Romero ◽  
...  
Keyword(s):  
High Risk ◽  
Antiphospholipid Syndrome ◽  
Antiphospholipid Antibodies ◽  
Obstetric Antiphospholipid Syndrome ◽  
Laboratory Profile

Current concepts on the pathogenesis of the antiphospholipid syndrome

Blood ◽  
2006 ◽  
Vol 109 (2) ◽  
pp. 422-430 ◽  
Author(s):  
Bill Giannakopoulos ◽  
Freda Passam ◽  
Soheila Rahgozar ◽  
Steven A. Krilis
Keyword(s):  
Experimental Evidence ◽  
Antiphospholipid Syndrome ◽  
Antiphospholipid Antibodies ◽  
Clinical Manifestations ◽  
Fetal Loss ◽  
Peripheral Tolerance ◽  
The Core ◽  
Glycoprotein I ◽  
Antigenic Targets ◽  
Acquired Thrombophilia

Abstract The antiphospholipid syndrome (APS) is an important cause of acquired thrombophilia. It is characterized by the core clinical manifestations of thrombosis, either venous or arterial, and in women it can also be associated with recurrent fetal loss. The detection of persistently elevated levels of antiphospholipid antibodies (aPL Abs) is a requisite laboratory feature for the diagnosis to be made. The dominant antigenic targets in APS are beta 2-glycoprotein I (β2-GPI) and prothrombin. There is an accumulating body of experimental evidence that suggests that specific subgroups of aPL Abs may directly contribute to disease pathogenesis. This review critically examines the experimental evidence underlying the various propositions made to explain how these antibodies may predispose to disease in humans. Furthermore, it also examines the evidence relating to the immunologic mechanisms that may contribute to the breakage of peripheral tolerance in this disorder. Delineating the strengths and limitations of the experimental evidence accumulated thus far will hopefully stimulate further experimentation toward achieving the ultimate goal of precisely defining the dominant pathogenic mechanisms operational in APS. This may pave the way for the development of improved therapies.

scholarly journals Antiphospholipid Antibodies and APS Nephropathy

2015 ◽  
Vol 8 (1) ◽  
pp. 10-17
Author(s):  
Rohan Willis ◽  
Emilio B Gonzalez
Keyword(s):  
Clinical Features ◽  
Antiphospholipid Syndrome ◽  
Antiphospholipid Antibodies ◽  
Pregnancy Loss ◽  
Recurrent Pregnancy Loss ◽  
Renal Involvement ◽  
Morphological Features ◽  
Occlusive Disease ◽  
Pathophysiological Mechanisms ◽  
Antigenic Targets

The presence of pathogenic antiphospholipid antibodies (aPL) is the characterizing feature of the antiphospholipid syndrome (APS), mediating the recurrent pregnancy loss and thrombosis typical of the disease through its action on various antigenic targets. APS nephropathy is the characteristic clinico-athological manifestation of renal involvement in APS and occurs as a result of vaso-occlusive disease in the intrarenal vasculature. The typical clinical features and morphological lesions of APS nephropathy have been well characterized and several studies have established a link between these features and the presence of various aPL. In this review, we outline the proposed pathophysiological mechanisms of aPL-mediated thrombosis, the characteristic clinical and morphological features of APS nephropathy and the evidence linking aPL action to the occurrence of APS nephropathy.

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