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Haematologica Feb 2023
Topics: Humans; Purpura, Thrombotic Thrombocytopenic
PubMed: 35488359
DOI: 10.3324/haematol.2022.281095 -
Tidsskrift For Den Norske Laegeforening... Apr 2024
Topics: Humans; Purpura; Male
PubMed: 38651721
DOI: 10.4045/tidsskr.23.0604 -
The Journal of Pediatrics Dec 2019
Topics: Child, Preschool; Exercise; Humans; Male; Orbital Diseases; Purpura
PubMed: 31472928
DOI: 10.1016/j.jpeds.2019.07.046 -
The Journal of Pediatrics Feb 2020
Topics: Child; Cough; Diagnosis, Differential; Early Diagnosis; Exanthema; Female; Humans; Orbit; Purpura
PubMed: 31685229
DOI: 10.1016/j.jpeds.2019.09.080 -
The Canadian Journal of Neurological... Jan 2018
Topics: Epilepsy; Female; Humans; Purpura; Young Adult
PubMed: 28978365
DOI: 10.1017/cjn.2017.235 -
Journal of Thrombosis and Haemostasis :... Aug 2021Targeted therapy of immune thrombotic thrombocytopenic purpura (iTTP) requires acurate and prompt diagnosis and differentiation from complement-mediated hemolytic uremic... (Review)
Review
Targeted therapy of immune thrombotic thrombocytopenic purpura (iTTP) requires acurate and prompt diagnosis and differentiation from complement-mediated hemolytic uremic syndrome and other causes of thrombotic microangiopathy. ADAMTS-13 (A Disintegrin And Metalloprotease with ThromboSpondin-1 Domain, member 13) evaluation (activity and inhibitors or anti-ADAMTS-13 IgG) is the key for diagnosis and further management of patients with suspected iTTP during acute episode and in clinical response or remission. Clinical trial results and real-world data have demonstrated the efficacy and safety of the triple therapy consisting of therapeutic plasma exchange, caplacizumab, and immunosuppressives (e.g., corticosteroids and rituximab) for acute iTTP. Such a therapeutic strategy has significantly accelerated the normalization of platelet counts, decreased the length of stays in the intensive care unit and the hospital, but most importantly reduced the mortality rate. The present review highlights some of the important advancements for the diagnosis and management of iTTP and proposes triple therapy as the standard of care for acute iTTP today.
Topics: ADAMTS13 Protein; Humans; Plasma Exchange; Purpura, Thrombocytopenic, Idiopathic; Purpura, Thrombotic Thrombocytopenic; Standard of Care; Thrombospondin 1
PubMed: 34060225
DOI: 10.1111/jth.15406 -
Archives of Disease in Childhood.... Feb 2018A healthy 15-month-old girl presented to the emergency department with a 24-hour history of fever and rash. The initial blanching rash developed into non-blanching areas...
CASE HISTORY
A healthy 15-month-old girl presented to the emergency department with a 24-hour history of fever and rash. The initial blanching rash developed into non-blanching areas with associated leg swelling. She had received no recent medications, had no known drug allergies and no unwell contacts.On examination, she was feverish at 38.6°C, capillary refill time was <2 s with warm peripheries, heart rate 169 bpm and blood pressure 94/59 mm Hg. A palpable purpuric rash was evident on all four limbs and face (figure 1) although the trunk was spared. Her legs were tense and oedematous to the knee.edpract;103/1/25/EDPRACT2016311782F1F1EDPRACT2016311782F1Figure 1Rash at presentation.Initial investigations: Haemoglobin level: 131 g/L, white cell count: 16.6×10/L, neutrophils: 11.1×10/L and platelets: 407×10/LCoagulation screen: normalC reactive protein level: 20 mg/LLactate level: 1.7 mmol/LIntravenous ceftriaxone was commenced following blood culture and meningococcal PCR. The following day, while remaining systemically well, she developed a vesicular rash on her trunk and back (figure 2).edpract;103/1/25/EDPRACT2016311782F2F2EDPRACT2016311782F2Figure 2Vesicular rash.
QUESTIONS
What is the diagnosis? Henoch-Schonlein purpura (HSP)Meningococcal septicaemiaAcute haemorrhagic oedema of infancy (AHOI)Vasculitic urticariaGianotti-Crosti syndromeWhat further investigation is required? Check viral serology including Epstein-Barr virus and hepatitis B virusComplement levels and autoimmune screenSkin biopsyLumbar puncture and audiologyNo further investigationHow should this child be managed? Complete 7 days of ceftriaxone treatmentOral aciclovirOral steroidsRegular follow-up with urinalysis and blood pressure monitoringStop antibiotics if cultures were negative at 48 hours and discharge▪▪.
Topics: Edema; Female; Fever; Humans; Infant; Purpura; Treatment Outcome
PubMed: 27799151
DOI: 10.1136/archdischild-2016-311782 -
The Pan African Medical Journal 2015
Topics: Biopsy; Disease Progression; Female; Humans; Leg; Middle Aged; Pigmentation Disorders; Purpura
PubMed: 27386021
DOI: 10.11604/pamj.2015.20.144.3021 -
Transfusion and Apheresis Science :... Apr 2023
Review
Topics: Humans; Prognosis; Biomarkers; Purpura, Thrombotic Thrombocytopenic
PubMed: 36890096
DOI: 10.1016/j.transci.2023.103676 -
Pediatrics in Review Jul 2022
Topics: Eczema; Humans; Infant; Purpura; Sudden Infant Death
PubMed: 35773537
DOI: 10.1542/pir.2020-004900