-
Journal of the American Academy of... Apr 2020Retiform purpura is a specific morphology within the spectrum of reticulate eruptions of vascular origin. It develops when blood vessels serving the skin are compromised... (Review)
Review
Retiform purpura is a specific morphology within the spectrum of reticulate eruptions of vascular origin. It develops when blood vessels serving the skin are compromised resulting in downstream cutaneous ischemia, purpura, and necrosis. Identifying retiform purpura is important particularly in the acutely ill patient. It may elucidate the underlying diagnosis, provide prognostic information, and suggest a treatment approach. The differential diagnosis of retiform purpura is vast, reflecting the myriad conditions that can lead to cutaneous vessel wall damage or lumen occlusion. In this article, we give an overview of the differential diagnosis of this cutaneous morphology, provide an approach to workup, and highlight updates in treatment of some of the more common conditions that manifest as retiform purpura.
Topics: Biopsy; Clinical Laboratory Techniques; Diagnosis, Differential; Humans; Medical History Taking; Physical Examination; Purpura; Skin Diseases, Vascular
PubMed: 31479689
DOI: 10.1016/j.jaad.2019.07.112 -
Blood Advances Dec 2019Despite an increase in the number of therapies available to treat patients with immune thrombocytopenia (ITP), there are minimal data from randomized trials to assist...
BACKGROUND
Despite an increase in the number of therapies available to treat patients with immune thrombocytopenia (ITP), there are minimal data from randomized trials to assist physicians with the management of patients.
OBJECTIVE
These evidence-based guidelines of the American Society of Hematology (ASH) are intended to support patients, clinicians, and other health care professionals in their decisions about the management of ITP.
METHODS
In 2015, ASH formed a multidisciplinary guideline panel that included 8 adult clinical experts, 5 pediatric clinical experts, 2 methodologists with expertise in ITP, and 2 patient representatives. The panel was balanced to minimize potential bias from conflicts of interest. The panel reviewed the ASH 2011 guideline recommendations and prioritized questions. The panel used the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach, including evidence-to-decision frameworks, to appraise evidence (up to May 2017) and formulate recommendations.
RESULTS
The panel agreed on 21 recommendations covering management of ITP in adults and children with newly diagnosed, persistent, and chronic disease refractory to first-line therapy who have non-life-threatening bleeding. Management approaches included: observation, corticosteroids, IV immunoglobulin, anti-D immunoglobulin, rituximab, splenectomy, and thrombopoietin receptor agonists.
CONCLUSIONS
There was a lack of evidence to support strong recommendations for various management approaches. In general, strategies that avoided medication side effects were favored. A large focus was placed on shared decision-making, especially with regard to second-line therapy. Future research should apply standard corticosteroid-dosing regimens, report patient-reported outcomes, and include cost-analysis evaluations.
Topics: Hematology; History, 21st Century; Humans; Purpura, Thrombocytopenic, Idiopathic; United States
PubMed: 31794604
DOI: 10.1182/bloodadvances.2019000966 -
Blood Advances Nov 2019Over the last decade, there have been numerous developments and changes in treatment practices for the management of patients with immune thrombocytopenia (ITP). This...
Over the last decade, there have been numerous developments and changes in treatment practices for the management of patients with immune thrombocytopenia (ITP). This article is an update of the International Consensus Report published in 2010. A critical review was performed to identify all relevant articles published between 2009 and 2018. An expert panel screened, reviewed, and graded the studies and formulated the updated consensus recommendations based on the new data. The final document provides consensus recommendations on the diagnosis and management of ITP in adults, during pregnancy, and in children, as well as quality-of-life considerations.
Topics: Clinical Decision-Making; Combined Modality Therapy; Decision Trees; Disease Management; Humans; Purpura, Thrombocytopenic, Idiopathic; Severity of Illness Index; Treatment Outcome
PubMed: 31770441
DOI: 10.1182/bloodadvances.2019000812 -
Hematology. American Society of... Dec 2022Hematologists are often consulted for thrombocytopenia in pregnancy, especially when there is a concern for a non-pregnancy-specific etiology or an insufficient platelet... (Review)
Review
Hematologists are often consulted for thrombocytopenia in pregnancy, especially when there is a concern for a non-pregnancy-specific etiology or an insufficient platelet count for the hemostatic challenges of delivery. The severity of thrombocytopenia and trimester of onset can help guide the differential diagnosis. Hematologists need to be aware of the typical signs of preeclampsia with severe features and other hypertensive disorders of pregnancy to help distinguish these conditions, which typically resolve with delivery, from other thrombotic microangiopathies (TMAs) (eg, thrombotic thrombocytopenic purpura or complement-mediated TMA). Patients with chronic thrombocytopenic conditions, such as immune thrombocytopenia, should receive counseling on the safety and efficacy of various medications during pregnancy. The management of pregnant patients with chronic immune thrombocytopenia who are refractory to first-line treatments is an area that warrants further research. This review uses a case-based approach to discuss recent updates in diagnosing and managing thrombocytopenia in pregnancy.
Topics: Female; Pregnancy; Humans; Purpura, Thrombocytopenic, Idiopathic; Pregnancy Complications, Hematologic; Purpura, Thrombotic Thrombocytopenic; Thrombotic Microangiopathies; Platelet Count
PubMed: 36485110
DOI: 10.1182/hematology.2022000375 -
Expert Review of Hematology Jun 2019: Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, consumption... (Review)
Review
: Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, consumption thrombocytopenia, and organ injury. TTP pathophysiology is based on a severe ADAMTS13 deficiency, the specific von Willebrand factor (VWF)-cleaving protease. : VWF/ADAMTS13 interaction is specific, and ADAMTS13 conformation has been elucidated recently. ADAMTS13 deficiency is congenital or acquired. Several targets are used in TTP treatment. : Differential diagnosis of TTP may be challenging and ADAMTS13 investigations are needed. ADAMTS13 conformation could be a promising parameter for TTP diagnosis and prognosis in the coming years. Plasma therapy remains the first-line treatment of the acute phase of TTP. Additional curative and preemptive rituximab are used in acquired TTP. Clinical trials dedicated to innovative drugs are promising.
Topics: Humans; Purpura, Thrombotic Thrombocytopenic
PubMed: 31107120
DOI: 10.1080/17474086.2019.1611423 -
Blood Reviews Sep 2023There are not many publications that provide a holistic view of the management of primary and secondary ITP as a whole, reflecting the similarities and differences... (Review)
Review
There are not many publications that provide a holistic view of the management of primary and secondary ITP as a whole, reflecting the similarities and differences between the two. Given the lack of major clinical trials, we believe that comprehensive reviews are much needed to guide the diagnosis and treatment of ITP today. Therefore, our review addresses the contemporary diagnosis and treatment of ITP in adult patients. With respect to primary ITP we especially focus on establishing the management of ITP based on the different and successive lines of treatment. Life-threatening situations, "bridge therapy" to surgery or invasive procedures and refractory ITP are also comprehensively reviewed here. Secondary ITP is studied according to its pathogenesis by establishing three major differential groups: Immune Thrombocytopenia due to Central Defects, Immune Thrombocytopenia due to Blocked Differentiation and Immune Thrombocytopenia due to Defective Peripheral Immune Response. Here we provide an up-to-date snapshot of the current diagnosis and treatment of ITP, including a special interest in addressing rare causes of this disease in our daily clinical practice. The target population of this review is adult patients only and the target audience is medical professionals.
Topics: Adult; Humans; Purpura, Thrombocytopenic, Idiopathic; Thrombocytopenia; Platelet Count; Receptors, Thrombopoietin; Thrombopoietin
PubMed: 37414719
DOI: 10.1016/j.blre.2023.101112 -
Romanian Journal of Internal Medicine =... Dec 2019Immune thrombocytopenia is an autoimmune hematological disorder characterized by severely decreased platelet count of peripheral cause: platelet destruction via... (Review)
Review
Immune thrombocytopenia is an autoimmune hematological disorder characterized by severely decreased platelet count of peripheral cause: platelet destruction via antiplatelet antibodies which may also affect marrow megakaryocytes. Patients may present in critical situations, with cutaneous and/or mucous bleeding and possibly life-threatening organ hemorrhages (cerebral, digestive, etc.) Therefore, rapid diagnosis and therapeutic intervention are mandatory. Corticotherapy represents the first treatment option, but as in any autoimmune disorder, there is a high risk of relapse. Second line therapy options include: intravenous immunoglobulins, thrombopoietin receptor agonists, rituximab or immunosuppression, but their benefit is usually temporary. Moreover, the disease generally affects young people who need repeated and prolonged treatment and hospitalization and therefore, it is preferred to choose a long term effect therapy. Splenectomy - removal of the site of platelet destruction - represents an effective and stable treatment, with 70-80% response rate and low complications incidence. A challenging situation is the association of ITP with pregnancy, which further increases the risk due to the immunodeficiency of pregnancy, major dangers of bleeding, vital risks for mother and fetus, potential risks of medication, necessity of prompt intervention in the setting of specific obstetrical situations - delivery, pregnancy loss, obstetrical complications, etc. We present an updated review of the current clinical and laboratory data, as well as a detailed analysis of the available therapeutic options with their benefits and risks, and also particular associations (pregnancy, relapsed and refractory disease, emergency treatment).
Topics: Diagnosis, Differential; Emergency Treatment; Female; Humans; Pregnancy; Pregnancy Complications; Purpura, Thrombocytopenic, Idiopathic
PubMed: 31199777
DOI: 10.2478/rjim-2019-0014 -
Hematology. American Society of... Nov 2018Immune thrombocytopenia (ITP) has historically been thought to occur in 2 distinct forms: childhood ITP and adult ITP. This division is based largely on the presumption... (Review)
Review
Immune thrombocytopenia (ITP) has historically been thought to occur in 2 distinct forms: childhood ITP and adult ITP. This division is based largely on the presumption that childhood ITP is often benign and self-limited, whereas ITP in adults tends to be more chronic and difficult to treat. Although data exist to justify a different approach to the diagnosis and treatment in young children and the elderly, ITP in older children, adolescents, and younger adults is likely to share more similar pathology. This article will highlight the most recent data describing the natural history, diagnostic approach, management strategies, and disease-related outcomes in children and adults with ITP. These data reveal many unexpected similarities between the 2 groups, while confirming some of the more well-described differences. Discussion of these findings aims to highlight similarities and differences between ITP in children and adults, which will underscore important areas of future research and/or changes in management guidelines.
Topics: Adolescent; Adult; Age Factors; Child; Child, Preschool; Humans; Infant; Practice Guidelines as Topic; Purpura, Thrombocytopenic, Idiopathic
PubMed: 30504339
DOI: 10.1182/asheducation-2018.1.405 -
Blood Reviews Sep 2021Immune thrombocytopenia (ITP), resulting from antibody-mediated platelet destruction combined with impaired platelet production, is a rare cause of thrombocytopenia in... (Review)
Review
Immune thrombocytopenia (ITP), resulting from antibody-mediated platelet destruction combined with impaired platelet production, is a rare cause of thrombocytopenia in both children and adults. The decision to treat newly diagnosed patients is based on several factors, including the desire to increase platelet count to prevent bleeding, induce remission, and improve health-related quality of life (HRQoL). At present, standard first-line therapy is corticosteroids. While this treatment does increase the platelet count in many patients, a high percentage still relapse after discontinuation of therapy. For this reason, alteration or intensification of first-line therapy that results in superior long-term remission rates is desirable. The objective of this review is to outline different upfront strategies for newly diagnosed patients with ITP in an effort to potentially enhance remission rates and prevent relapse, taking into account an assessment of the risks and benefits of each approach. We primarily focus on adults with ITP, highlighting pediatric data and practice when applicable.
Topics: Disease Management; Genetic Variation; Humans; Prognosis; Purpura, Thrombocytopenic, Idiopathic
PubMed: 33736875
DOI: 10.1016/j.blre.2021.100822 -
Anales de Pediatria Aug 2019Primary immune thrombocytopenia, formerly known as immune thrombocytopenic purpura, is a disease for which the clinical and therapeutic management has always been...
Primary immune thrombocytopenia, formerly known as immune thrombocytopenic purpura, is a disease for which the clinical and therapeutic management has always been controversial. The ITP working group of the Spanish Society of Paediatric Haematology and Oncology has updated its guidelines for diagnosis and treatment of primary immune thrombocytopenia in children, based on current guidelines, bibliographic review, clinical assays, and member consensus. The main objective is to reduce clinical variability in diagnostic and therapeutic procedures, in order to obtain best clinical results with minimal adverse events and good quality of life.
Topics: Child; Humans; Purpura, Thrombocytopenic, Idiopathic; Quality of Life
PubMed: 31178291
DOI: 10.1016/j.anpedi.2019.04.014