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British Journal of Haematology Nov 2023The objective of this guideline is to provide healthcare professionals with clear, up-to-date and practical guidance on the management of thrombotic thrombocytopenic...
The objective of this guideline is to provide healthcare professionals with clear, up-to-date and practical guidance on the management of thrombotic thrombocytopenic purpura (TTP) and related thrombotic microangiopathies (TMAs), including complement-mediated haemolytic uraemic syndrome (CM HUS); these are defined by thrombocytopenia, microangiopathic haemolytic anaemia (MAHA) and small vessel thrombosis. Within England, all TTP cases should be managed within designated regional centres as per NHSE commissioning for highly specialised services.
Topics: Humans; Purpura, Thrombotic Thrombocytopenic; Thrombotic Microangiopathies; Hemolytic-Uremic Syndrome; Anemia, Hemolytic; Hematology
PubMed: 37586700
DOI: 10.1111/bjh.19026 -
Hematology. American Society of... Dec 2023While immune thrombocytopenia often presents with mild bleeding manifestations or surprising findings of thrombocytopenia on routine complete blood counts in patients... (Review)
Review
While immune thrombocytopenia often presents with mild bleeding manifestations or surprising findings of thrombocytopenia on routine complete blood counts in patients without symptoms, some patients can present with new thrombocytopenia and life-threatening bleeding. Emergent assessment and treatment are needed to prevent substantial morbidity and even mortality. These patients present to the emergency room with bleeding, and hematologists are subsequently consulted. Understanding the approach to making the diagnosis and excluding other life-threatening illnesses is essential, as is rapid initiation of treatment in the bleeding patient even when the diagnosis of immune- mediated thrombocytopenia is tentative. Using a case-based format, we review how to approach and treat patients presenting with new thrombocytopenia and bleeding.
Topics: Humans; Purpura, Thrombocytopenic, Idiopathic; Hemorrhage; Thrombocytopenia; Platelet Count; Emergency Service, Hospital
PubMed: 38066888
DOI: 10.1182/hematology.2023000478 -
Innere Medizin (Heidelberg, Germany) Feb 2024The immune-mediated small vessel vasculitis is known as Schoenlein-Henoch purpura predominantly from pediatrics and in these cases occurs more frequently after... (Review)
Review
The immune-mediated small vessel vasculitis is known as Schoenlein-Henoch purpura predominantly from pediatrics and in these cases occurs more frequently after infections of the upper airways. In adults, immunoglobulin A (IgA) vasculitis often proceeds more severely und recurrently with the classical tetrad of skin manifestations in the sense of leukocytoclastic vasculitis, joint affection, gastrointestinal involvement and IgA nephritis, in contrast to the mostly mild and self-limiting course in children. The background of this systemic vasculitis with formation of IgA immune complexes is considered to be an altered glycosylation of IgA, as this causes the exposure of binding sites for autoantibodies so that an immune complex reaction can be elicited. This ultimately leads to perivascular deposition of IgA and a further activation of neutrophils. Groundbreaking in the diagnostics is the histological detection of leukocytoclastic vasculitis and in cases of renal manifestations a kidney biopsy with characteristic deposits of immune complexes, which cannot be clearly differentiated from IgA nephropathy. The treatment is aimed at the respective manifestation and is mostly based on consensus recommendations due to the lack of randomized studies. In addition to immunosuppressive medication, in the presence of a chronic kidney disease general nephroprotection is becoming increasingly more important also by inhibition of sodium-glucose transporter 2 (SGLT2). The type and extent of kidney involvement and also rare cardiac manifestations are the main determinants of the prognosis. Continuous medical accompaniment of those affected is necessary due to the possible progression of the disease and the risk of recurrence.
Topics: Adult; Humans; Child; IgA Vasculitis; Antigen-Antibody Complex; Immunoglobulin A; Vasculitis; Polyarteritis Nodosa; Vasculitis, Leukocytoclastic, Cutaneous
PubMed: 38236411
DOI: 10.1007/s00108-023-01650-7 -
British Journal of Haematology Apr 2024Immune thrombocytopenia (ITP) in pregnancy is challenging for both mother and fetus. Understanding the pathophysiology, treatments, and risks to the mother and fetus... (Review)
Review
Immune thrombocytopenia (ITP) in pregnancy is challenging for both mother and fetus. Understanding the pathophysiology, treatments, and risks to the mother and fetus leads to proper management resulting in successful pregnancy and delivery in almost all cases. ITP in a pregnant woman has many similarities to ITP not in pregnancy although gestational thrombocytopenia can be confused with ITP. However, recognizing differences is instrumental in avoiding bleeding complications and toxicities of treatment. This Nutshell review focuses on the natural history of ITP in pregnancy, its treatment, and dilemmas.
Topics: Pregnancy; Female; Humans; Purpura, Thrombocytopenic, Idiopathic; Platelet Count; Pregnancy Complications, Hematologic; Thrombocytopenia
PubMed: 38263610
DOI: 10.1111/bjh.19230 -
Zeitschrift Fur Rheumatologie Sep 2023IgA vasculitis (IgAV) is an immune complex-mediated vasculitis characterized by IgA1-dominant immune deposits in small vessels. It is the most common systemic vasculitis...
IgA vasculitis (IgAV) is an immune complex-mediated vasculitis characterized by IgA1-dominant immune deposits in small vessels. It is the most common systemic vasculitis in childhood with a mostly uncomplicated and self-limiting course. Adults are less affected but the course is frequently more complicated and more frequently accompanied by renal involvement. IgAV characteristically manifests itself on the skin with palpable purpura and in joints, the kidneys and the gastrointestinal tract. In cases of incomplete or atypical symptoms a differential diagnostic work-up is required. A number of triggers have been suggested, especially infections and drugs. Disease management is tailored to organ manifestations and the severity of the symptoms. For children, optimized supportive care and targeted symptom relief are usually sufficient. Management of renal and gastrointestinal manifestations follows recommendations for ANCA-associated vasculitis and IgA nephropathy. Treatment options include glucocorticoids and immunosuppressive agents with varying and mostly insufficient evidence.
Topics: Adult; Child; Humans; IgA Vasculitis; Glomerulonephritis, IGA; Immunoglobulin A; Skin; Immunosuppressive Agents; Polyarteritis Nodosa; Giant Cell Arteritis; Granulomatosis with Polyangiitis
PubMed: 37266676
DOI: 10.1007/s00393-023-01355-0 -
Blood Transfusion = Trasfusione Del... May 2024Immune thrombocytopenia (ITP) is an acquired immune-mediated bleeding disorder characterized by isolated thrombocytopenia. Its estimated yearly incidence in the...
BACKGROUND
Immune thrombocytopenia (ITP) is an acquired immune-mediated bleeding disorder characterized by isolated thrombocytopenia. Its estimated yearly incidence in the pediatric population is 1.9-6.4/100,000. ITP in children is usually a self-limiting and benign disorder. The clinical management of children with ITP often remains controversial, as robust randomized trials on the management of this disorder are lacking. Treatments vary widely in clinical practice and existing guidelines from hematology societies on clinical management offer indications based largely on expert opinion rather than strong evidence.
MATERIALS AND METHODS
The Coagulative Disorder Working Group of the Italian Association of Pediatric Hematology and Oncology (AIEOP) developed this document to collect shared expert opinions on the management of newly diagnosed ITP, updating previous guidelines and providing recommendations to pediatricians. Each statement has been given a score expressing the strength of evidence, appropriateness and agreement among participants.
RESULTS
Clear-cut definitions of the clinical phases of the disease and clinical response are stated. Recommendations are given regarding the classification of bleeding symptoms, evaluation of bleeding risk, diagnosis, and prognostic factors. Specific recommendations for treatment include indications for first-line (intravenous immunoglobulins, steroids) and second-line (combined therapy, thrombopoietin receptor agonists, immunosuppressive drugs, rituximab) therapeutic agents, as well as hemorrhagic emergency and supportive treatment, including emergency splenectomy. The optimal follow-up schedule, the relation between ITP and vaccines and health-related quality-of-life issues are also discussed.
DISCUSSION
The panel achieved broad consensus on issues related to how to treat children with newly diagnosed ITP, providing a comprehensive review of all relevant clinical aspects.
Topics: Humans; Child; Purpura, Thrombocytopenic, Idiopathic; Italy; Hemorrhage; Immunoglobulins, Intravenous; Child, Preschool; Female; Male; Acute Disease; Hematology
PubMed: 37677093
DOI: 10.2450/BloodTransfus.501 -
La Tunisie Medicale Jun 2024Thrombotic thrombocytopenic purpura (TTP) is a rare but potentially fatal hematological disorder that requires urgent treatment. Once the diagnosis has been made, plasma...
INTRODUCTION
Thrombotic thrombocytopenic purpura (TTP) is a rare but potentially fatal hematological disorder that requires urgent treatment. Once the diagnosis has been made, plasma exchange (PE) must be started immediately and until a response is obtained.
AIM
Evaluate PE in terms of responses and complications in the treatment of TTP.
METHODS
This was a monocentric, descriptive, retrospective study including patients in whom TTP was diagnosed and treated with plasmapheresis in the clinical hematology department at Aziza Othmana Hospital, between January 2010 and December 2020.
RESULTS
Our study included 26 patients. PE was initiated within a median of 1 day. The rhythm of exchanges was daily in 22 patients. Twenty PE-related complications were noted, hypocalcemia being the most frequent (30%). CR was achieved in 15 patients after PE alone. Nine patients were refractory, and six received 2nd-line treatment, with CR achieved in five patients. Relapse was noted in six patients (40%). They were treated by PE and only one patient received rituximab. Four patients had a response. The overall response rate was 69% and overall mortality was 30%. OS at 2 years was 68,3% and RFS was 84,4%. Factors associated with the achievement of CR were the fall in LDH at D5 of treatment (p=0,027,OR=0,59 ;IC 95%[0,32-1,08]) and the daily rhythm of PE (p=0,005, OR=0,35; IC 95%[0,14-0,91]).
CONCLUSION
Our results were comparable to those of the literature, but the rate of refractory disease was higher. Rituximab may enhance our results.
Topics: Humans; Purpura, Thrombotic Thrombocytopenic; Retrospective Studies; Plasma Exchange; Female; Male; Middle Aged; Adult; Young Adult; Treatment Outcome; Aged; Recurrence; Plasmapheresis; Adolescent; Rituximab
PubMed: 38864197
DOI: 10.62438/tunismed.v102i6.4614 -
American Family Physician Mar 2024
Topics: Humans; Purpura; IgA Vasculitis; Skin Neoplasms
PubMed: 38574218
DOI: No ID Found -
Cutis Jan 2024
Topics: Humans; Factitious Disorders; Psychotic Disorders; Autoimmune Diseases; Purpura; Skin Diseases, Vascular
PubMed: 38478944
DOI: 10.12788/cutis.0957 -
British Journal of Haematology Oct 2023Refractory immune thrombocytopenia (ITP) is a challenging disease that can be defined by refractoriness to second-line treatments. In this review, we list and comment... (Review)
Review
Refractory immune thrombocytopenia (ITP) is a challenging disease that can be defined by refractoriness to second-line treatments. In this review, we list and comment available evidence about clinical and biological factors associated with refractoriness to splenectomy, thrombopoietin receptor agonists (TPO-RAs), rituximab and fostamatinib, as well as those associated with multirefractory ITP (active disease with failure of rituximab, TPO-RAs and splenectomy).
Topics: Humans; Purpura, Thrombocytopenic, Idiopathic; Receptors, Fc; Receptors, Thrombopoietin; Recombinant Fusion Proteins; Rituximab; Splenectomy; Thrombopoietin; Biomarkers
PubMed: 38019080
DOI: 10.1111/bjh.19076