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International Journal of Hematology Mar 2023Immune thrombocytopenia (ITP), thrombotic thrombocytopenic purpura (TTP), and vaccine-induced immune thrombotic thrombocytopenia (VITT) all have "thrombocytopenia" in... (Review)
Review
Immune thrombocytopenia (ITP), thrombotic thrombocytopenic purpura (TTP), and vaccine-induced immune thrombotic thrombocytopenia (VITT) all have "thrombocytopenia" in their name, and all but congenital TTP are caused by immune mechanisms, but these conditions are quite different in their clinical features and pathophysiology. This review series covers recent progress in pathophysiology and treatment of these diseases, as well as a recent epoch-making clinical trial of induced pluripotent stem cells (iPSC)-derived platelets for patients with thrombocytopenia.
Topics: Humans; Purpura, Thrombotic Thrombocytopenic; Purpura, Thrombocytopenic, Idiopathic; Blood Platelets
PubMed: 36656456
DOI: 10.1007/s12185-023-03542-w -
Pediatric Emergency Care Feb 2014Traumatic asphyxia is a rare condition in children that usually occurs after severe compression to the chest or abdomen. We report 3 cases in patients 18, 20, and 36...
Traumatic asphyxia is a rare condition in children that usually occurs after severe compression to the chest or abdomen. We report 3 cases in patients 18, 20, and 36 months of age who presented signs and symptoms of traumatic asphyxia after car accidents. Two clinical features were consistent in all 3 patients: multiple petechiae on the face and bulbar conjunctival hemorrhage; 2 patients had facial cyanosis, and 1 had facial edema.In children, the number of clinical manifestations that should be evident to diagnose traumatic asphyxia has not been ascertained. However, in any history of trauma with compression of the chest or abdomen and signs of increased intravenous craniocervical pressure, traumatic asphyxia should be suspected.
Topics: Asphyxia; Child, Preschool; Conjunctival Diseases; Eye Hemorrhage; Face; Female; Humans; Infant; Male; Purpura; Thoracic Injuries
PubMed: 24488162
DOI: 10.1097/PEC.0000000000000067 -
Cold Spring Harbor Molecular Case... Feb 2022Ethylmalonic encephalopathy (MIM #602473) is a rare autosomal recessive metabolic condition caused by biallelic variants in (MIM #608451), characterized by global...
Ethylmalonic encephalopathy (MIM #602473) is a rare autosomal recessive metabolic condition caused by biallelic variants in (MIM #608451), characterized by global developmental delay, infantile hypotonia, seizures, and microvascular damage. The microvascular changes result in a pattern of relapsing spontaneous diffuse petechiae and purpura, positional acrocyanosis, and pedal edema, hemorrhagic suffusions of mucous membranes, and chronic diarrhea. Here, we describe an instructive case in which ethylmalonic encephalopathy masqueraded as meningococcal septicemia and shock. Ultrarapid whole-genome testing (time to result 60 h) and prompt biochemical analysis facilitated accurate diagnosis and counseling with rapid implementation of precision treatment for the metabolic crisis related to this condition. This case provides a timely reminder to consider rare genetic diagnoses when atypical features of more common conditions are present, with an early referral to ensure prompt biochemical and genomic diagnosis.
Topics: Brain Diseases, Metabolic, Inborn; Humans; Mitochondrial Proteins; Nucleocytoplasmic Transport Proteins; Purpura; Sepsis
PubMed: 35165146
DOI: 10.1101/mcs.a006193 -
Archives of Disease in Childhood Apr 1988A four year old girl presented with varicella gangrenosa, and haematological investigations showed a disseminated intravascular coagulation. The child subsequently...
A four year old girl presented with varicella gangrenosa, and haematological investigations showed a disseminated intravascular coagulation. The child subsequently developed a unilateral deep venous thrombosis. She was treated with oral steroids and intravenous heparin and made a full recovery.
Topics: Chickenpox; Child, Preschool; Disseminated Intravascular Coagulation; Female; Gangrene; Humans; Purpura; Skin; Thrombophlebitis
PubMed: 3365017
DOI: 10.1136/adc.63.4.444 -
The Journal of the Arkansas Medical... Feb 2015
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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 -
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 -
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 -
Sultan Qaboos University Medical Journal Nov 2022
Topics: Humans; Purpura; Pigmentation Disorders; Exanthema; Eczema
PubMed: 36407701
DOI: 10.18295/squmj.1.2022.004