RÉSUMÉ
A 42-year-old woman with a history of acute myeloid leukaemia status postallogeneic stem cell transplant presented with fevers, altered mental status, pulmonary infiltrates and septic shock that further progressed to thrombocytopenia and purpura fulminans. Laboratory studies were consistent with a diagnosis of thrombotic thrombocytopenic purpura (TTP). Blood cultures grew Streptococcus pneumoniae On chart review, our patient had a history of low immunoglobulin levels following stem cell transplant, which may have predisposed her to pneumococcal infection. The patient responded to therapy with ceftriaxone, plasma exchange, rituximab and caplacizumab. This is the fourth-documented case of pneumococcal induced TTP and, to the best of our knowledge, the first-describing pneumococcal induced TTP with purpura fulminans. We conclude that patients with TTP should be evaluated for infectious aetiologies and empiric antibiotics should be considered. Clinicians should be aware of the possibility for TTP to lead to purpura fulminans.
Sujet(s)
Bactériémie/complications , Infections à pneumocoques/complications , Purpura thrombotique thrombocytopénique/étiologie , Choc septique/complications , Adulte , Antibactériens/usage thérapeutique , Bactériémie/sang , Bactériémie/thérapie , Ceftriaxone/usage thérapeutique , Diagnostic différentiel , Femelle , Fibrinolytiques/usage thérapeutique , Doigts/anatomopathologie , Doigts/chirurgie , Gangrène , Glucocorticoïdes/usage thérapeutique , Maladie du greffon contre l'hôte/traitement médicamenteux , Humains , Facteurs immunologiques/usage thérapeutique , Leucémie aigüe myéloïde/thérapie , Nez/anatomopathologie , Échange plasmatique , Infections à pneumocoques/sang , Infections à pneumocoques/thérapie , Purpura fulminans/sang , Purpura fulminans/diagnostic , Purpura thrombotique thrombocytopénique/sang , Purpura thrombotique thrombocytopénique/thérapie , Rituximab/usage thérapeutique , Choc septique/sang , Choc septique/thérapie , Anticorps à domaine unique/usage thérapeutique , Transplantation de cellules souches , Orteils/anatomopathologie , Orteils/chirurgieSujet(s)
Séquence nucléotidique , Exons , Protéine C , Purpura fulminans , Délétion de séquence , Substitution d'acide aminé , Tests de coagulation sanguine , Enfant d'âge préscolaire , Femelle , Humains , Nouveau-né , Protéine C/génétique , Protéine C/métabolisme , Purpura fulminans/sang , Purpura fulminans/génétique , Purpura fulminans/anatomopathologie , Purpura fulminans/thérapieSujet(s)
Antibactériens/administration et posologie , Débridement/méthodes , Gangrène , Purpura fulminans , Biopsie/méthodes , Coagulation intravasculaire disséminée , Diagnostic précoce , Femelle , Gangrène/étiologie , Gangrène/chirurgie , Humains , Adulte d'âge moyen , Purpura fulminans/sang , Purpura fulminans/diagnostic , Purpura fulminans/physiopathologie , Purpura fulminans/thérapie , Ulcère cutané/étiologie , Ulcère cutané/anatomopathologie , Délai jusqu'au traitementSujet(s)
Infections à cytomégalovirus , Cytomegalovirus/métabolisme , Herpèsvirus humain de type 3/métabolisme , Protéine S/métabolisme , Purpura fulminans , Infection à virus varicelle-zona , Adulte , Infections à cytomégalovirus/sang , Infections à cytomégalovirus/anatomopathologie , Infections à cytomégalovirus/thérapie , Infections à cytomégalovirus/virologie , Femelle , Humains , Purpura fulminans/sang , Purpura fulminans/anatomopathologie , Purpura fulminans/thérapie , Purpura fulminans/virologie , Infection à virus varicelle-zona/sang , Infection à virus varicelle-zona/anatomopathologie , Infection à virus varicelle-zona/thérapie , Infection à virus varicelle-zona/virologieRÉSUMÉ
Neonatal purpura fulminans (PF) is a life-threatening disorder caused by congenital or acquired deficiencies of protein C (PC) or S. PF presents as a cutaneous manifestation of disseminated intravascular coagulation. We describe a case of PF in a newborn with left leg ischemia and undetectable PC levels soon after birth. Despite anticoagulation therapy and PC concentrate, left foot amputation was required. Genetic testing of PROC for congenital PC deficiency was normal. This case highlights the course of PF due to acquired PC deficiency in a newborn treated with PC concentrate which is rarely described in the literature.
Sujet(s)
Maladies néonatales , Déficit en protéine C , Purpura fulminans , Humains , Nouveau-né , Maladies néonatales/sang , Maladies néonatales/génétique , Mâle , Déficit en protéine C/sang , Déficit en protéine C/génétique , Purpura fulminans/sang , Purpura fulminans/génétiqueRÉSUMÉ
Purpura fulminans (PF) is a dreadful and frequent complication of Neisseria meningitidis invasive infection, and is associated with a high mortality rate. This syndrome begins with dermal microvessels thrombosis that rapidly lead to hemorrhagic skin necrosis. In this review, we discuss the prothrombotic events occurring during meningococcal infection. Moreover, recent data from an experimental mouse model have highlighted the critical role of the meningococcus adhesion to the endothelium in the development of PF lesions, thus opening new therapeutic perspectives.
Sujet(s)
Infections à méningocoques/complications , Infections à méningocoques/microbiologie , Purpura fulminans/étiologie , Coagulation sanguine , Prédisposition génétique à une maladie , Interactions hôte-pathogène/génétique , Interactions hôte-pathogène/immunologie , Humains , Infections à méningocoques/immunologie , Neisseria meningitidis/immunologie , Purpura fulminans/sangRÉSUMÉ
HISTORY AND CLINICAL FINDINGS: A 51-year-old female patient with history of longterm drug abuse, was admitted to our hospital with large, stocking-shaped areas of painful, non-displaceable confluent bruising reaching up to the groin. INVESTIGATIONS: The emergency laboratory tests showed leucopenia, thrombocytopenia and anemia as well as a distinct protein C deficiency. DIAGNOSIS, TREATMENT AND COURSE: Purpura fulminans was diagnosed and treated with an initial dose of protein C. The patient survived and the skin necrosis can be treated. CONCLUSION: Purpura fulminans is an internistic and dermatological emergency situation which can lead to shock through consumptive coagulopathy. The serious course of disease can be prevented by rapid treatment with protein C.
Sujet(s)
Urgences , Déficit en protéine C/diagnostic , Purpura fulminans/diagnostic , Diagnostic différentiel , Femelle , Humains , Adulte d'âge moyen , Pronostic , Protéine C/administration et posologie , Déficit en protéine C/sang , Déficit en protéine C/traitement médicamenteux , Purpura fulminans/sang , Purpura fulminans/traitement médicamenteux , Troubles liés à une substance/complicationsRÉSUMÉ
Purpura fulminans (PF) is a life-threatening hemorrhagic condition. Because of the rarity and randomness of the disease, no improvement in treatment has been made for a long time. In this study, we assessed the serum proteome response to PF by comparing serum proteins between healthy controls and PF patient. Liquid chromatography with tandem mass spectrometry (LC-MS/MS) approach was used after depleting 6 abundant proteins of serum. In total, 262 proteins were confidently identified with 2 unique peptides, and 38 proteins were identified significantly up- (≥ 2) or downregulated (≤ 0.5) based on spectral counting ratios (SpCPF/N). In the 38 proteins with significant abundance changes, 11 proteins were previously known to be associated with burn or sepsis response, but 27 potentially novel proteins may be specifically associated with PF process. Two differentially expressed proteins, alpha-1-antitrypsin (SERPINA1) and alpha-2 antiplasmin (SERPINF2), were validated by Western blot. This is the first study where PF patient and healthy controls are compared in a proteomic study to elucidate proteins involved in the response to PF. This study provides an initial basis for future studies of PF, and the differentially expressed proteins might provide new therapeutic targets to decrease the mortality of PF.
Sujet(s)
Brûlures/sang , Purpura fulminans/sang , Sepsie/sang , alpha-1-Antitrypsine/sang , alpha-2-Antiplasmine/métabolisme , Marqueurs biologiques/sang , Brûlures/complications , Études cas-témoins , Femelle , Expression des gènes , Gene Ontology , Humains , Adulte d'âge moyen , Protéome/génétique , Protéome/métabolisme , Purpura fulminans/microbiologie , Sepsie/microbiologie , alpha-1-Antitrypsine/génétique , alpha-2-Antiplasmine/génétiqueRÉSUMÉ
Acute perturbations in the hemostatic balance of anticoagulation and procoagulation antecede the manifestation of purpura fulminans, a rare syndrome of intravascular thrombosis and hemorrhagic infarction of the skin. Hallmarks include small vessel thrombosis, tissue necrosis and disseminated intravascular thrombosis. The course may be rapidly fulminant resulting in multiorgan failure with thrombotic occlusion of the vasculature, leading to distal extremity ischemia and necrosis. Depletion of protein C (PC) has been emphasized in the pathogenesis. Early intravenous antibiotic administration and hemodynamic support are cornerstones in management. Herein, we report a case of pneumococcal sepsis-induced purpura fulminans limited to the skin in an asplenic adult patient without the development disseminated intravascular coagulation.
Sujet(s)
Infections à pneumocoques/étiologie , Infections à pneumocoques/thérapie , Purpura fulminans/microbiologie , Purpura fulminans/thérapie , Sepsie/complications , Sepsie/thérapie , Administration par voie intraveineuse , Amputation chirurgicale , Antibactériens/usage thérapeutique , Ceftriaxone/usage thérapeutique , Débridement , Femelle , Doigts/chirurgie , Main/chirurgie , Humains , Jambe/chirurgie , Adulte d'âge moyen , Infections à pneumocoques/complications , Infections à pneumocoques/microbiologie , Purpura fulminans/sang , Purpura fulminans/anatomopathologie , Purpura thrombopénique idiopathique/étiologie , Transplantation de peau , Splénectomie , Streptococcus pneumoniae/isolement et purification , Streptococcus pneumoniae/physiologie , Résultat thérapeutiqueRÉSUMÉ
The association of idiopathic purpura fulminans (PF) and venous thrombosis (VT) seldom reveals constitutional thrombophilia in an infant. We report a case of PF in an 18-month-old infant. Laboratory tests showed disseminated intravascular coagulation (DIVC) with normal rates of C and S proteins and antithrombin. The echo-Doppler examination conveyed venous thrombosis of the lower limbs, while the genetic study showed heterozygous mutation of Factor II (G 20210A). Precocious and multidisciplinary management included frozen fresh plasma supplementation and necrosectomy with skin grafts. The diagnosis and therapeutic problems posed by PF combined with deep venous thrombosis are discussed.