Recurrent Thromboembolism, Disseminated Intravascular Coagulation, and Coumarin-induced Skin Necrosis Associated with Protein C Deficiency

Severe and recurrent purpura fulminans developed in a Chinese boy at one day of age. Results of coagulation studies performed on the patient during attacks were compatible with the diagnosis of disseminated intravascular coagulation. Subsequent investigations have revealed that the patient is homozygous and that his parents are heterozygous for protein C deficiency. Cryoprecipitate and fresh frozen plasma induced a remission, and administration of warfarin has been successful in preventing recurrence of attacks for as long as 8 months without infusion of any plasma components. None of the family members who are heterozygous for protein C deficiency have had thrombotic episodes.

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NECROSE CUTANEE REVELANT UN DEFICIT CONGENITAL EN PROTEINE C : A PROPOS DE 3 CAS

International Journal of Advanced Research ◽

10.21474/ijar01/12557 ◽

2021 ◽

Vol 9 (03) ◽

pp. 80-83

Author(s):

S. Halouani ◽

◽

W. Kojmane ◽

F. Hmami ◽

S. Atmani ◽

...

Keyword(s):

Early Diagnosis ◽

Replacement Therapy ◽

Disseminated Intravascular Coagulation ◽

Skin Necrosis ◽

Protein C ◽

Autosomal Recessive ◽

Autosomal Recessive Disorder ◽

Protein C Deficiency ◽

Neonatal Skin ◽

Protein C Activity

Neonatal skin necrosis in the context of a congenital homozygous protein C deficiency is a rare inherited autosomal recessive disorder, it is characterized by rapidly extensive necrotic patches occurring a few hours after birth in a newborn who doesnt present any hemodynamic disorder. The diagnosis is based on the assay of protein C activity which is collapsed or even undetectable. Early diagnosis and replacement therapy are the mainstays of management before the onset of disseminated intravascular coagulation. We report three cases of newborns presenting with DIC in the context of protein C deficiency and the course of which was fatal.

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Severe Acquired Protein C Deficiency in Purpura Fulminans Associated With Disseminated Intravascular Coagulation: Treatment With Protein C Concentrate

PEDIATRICS ◽

10.1542/peds.91.2.418 ◽

1993 ◽

Vol 91 (2) ◽

pp. 418-422

Author(s):

WILLIAM T. GERSON ◽

JOSEPH D. DICKERMAN ◽

EDWIN G. BOVILL ◽

ELIZABETH GOLDEN

Keyword(s):

Disseminated Intravascular Coagulation ◽

Protein C ◽

Purpura Fulminans ◽

Protein S ◽

Clinical Settings ◽

Protein C Deficiency ◽

Intravascular Coagulation ◽

Hemorrhagic Necrosis ◽

Protein C Concentrate ◽

History Of

Purpura fulminans (PF) defines an acute, often lethal syndrome of disseminated intravascular coagulation (DIC) with rapidly progressive hemorrhagic necrosis of the skin due to dermal vascular thrombosis.1-7 It is indicative of a severe disturbance in hemostasis now recognized to involve the protein C system in many cases.1,2,5,8-12 Purpura fulminans is usually seen in three clinical settings: (1) in the newborn period as a manifestation of homozygous protein C deficiency, or rarely protein S deficiency13,14; (2) in individuals with acute, severe viral or bacterial infection where an acquired deficiency in protein C activity is documented1-3,5-8,10,12,15; and (3) as a rare, postinfectious syndrome with a history of an antecedent" preparatory disease," most commonly a viral or bacterial illness involving the skin (eg, varicella or scarlet fever), with the sudden development, during an otherwise unremarkable convalescence, of progressive purpura and necrosis.3-5,7,10,12

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Chronic Disseminated Intravascular Coagulation and Childhood-Onset Skin Necrosis Resulting From Homozygosity for a Protein C Gla Domain Mutation, Arg15Trp

Journal of Pediatric Hematology/Oncology ◽

10.1097/00043426-200211000-00018 ◽

2002 ◽

Vol 24 (8) ◽

pp. 685-688 ◽

Cited By ~ 5

Author(s):

Mara Steinkamp ◽

Alon Geva ◽

Steven Joffe ◽

Catherine N. Lapp ◽

Ellis J. Neufeld

Keyword(s):

Disseminated Intravascular Coagulation ◽

Skin Necrosis ◽

Protein C ◽

Childhood Onset ◽

Intravascular Coagulation ◽

Gla Domain

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Disseminated intravascular coagulation in meningococcal sepsis

Hämostaseologie ◽

10.1055/s-0037-1619581 ◽

2003 ◽

Vol 23 (03) ◽

pp. 125-130 ◽

Cited By ~ 2

Author(s):

S. Zeerleder ◽

R. Zürcher Zenklusen ◽

C. E. Hack ◽

W. A. Wuillemin

Keyword(s):

Organ Dysfunction ◽

Disseminated Intravascular Coagulation ◽

Skin Necrosis ◽

Multiple Organ ◽

Meningococcal Sepsis ◽

Multiple Organ Dysfunction ◽

Intravascular Coagulation ◽

Therapeutic Concepts ◽

New Therapeutic Concepts ◽

Coagulation And Fibrinolysis

SummaryWe report on a man (age: 49 years), who died from severe meningococcal sepsis with disseminated intravascular coagulation (DIC), multiple organ dysfunction syndrome and extended skin necrosis. We discuss in detail the pathophysiology of the activation of coagulation and fibrinolysis during sepsis. The article discusses new therapeutic concepts in the treatment of disseminated intravascular coagulation in meningococcal sepsis, too.

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Safety of plasma-derived protein C for treating disseminated intravascular coagulation in adult patients with active cancer

American Journal of Hematology ◽

10.1002/ajh.22238 ◽

2012 ◽

Vol 87 (2) ◽

pp. 230-232 ◽

Cited By ~ 1

Author(s):

Alessandra Malato ◽

Giorgia Saccullo ◽

Lucio Lo Coco ◽

Clementina Caracciolo ◽

Simona Raso ◽

...

Keyword(s):

Disseminated Intravascular Coagulation ◽

Protein C ◽

Adult Patients ◽

Intravascular Coagulation ◽

Active Cancer

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Protein C activity value as a prognostic factor in sepsis-induced disseminated intravascular coagulation

Journal of the Japanese Society of Intensive Care Medicine ◽

10.3918/jsicm.21.614 ◽

2014 ◽

Vol 21 (6) ◽

pp. 614-622

Author(s):

Toshihiro Sakurai ◽

Shu Yamada ◽

Maki Kitada ◽

Satoshi Hashimoto ◽

Shoko Hashimoto ◽

...

Keyword(s):

Prognostic Factor ◽

Disseminated Intravascular Coagulation ◽

Protein C ◽

Intravascular Coagulation ◽

Protein C Activity

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Endothelial Dysfunction Is Associated with Mortality and Severity of Coagulopathy in Patients with Sepsis and Disseminated Intravascular Coagulation

Clinical and Applied Thrombosis/Hemostasis ◽

10.1177/1076029619852163 ◽

2019 ◽

Vol 25 ◽

pp. 107602961985216 ◽

Cited By ~ 7

Author(s):

Amanda Walborn ◽

Matthew Rondina ◽

Michael Mosier ◽

Jawed Fareed ◽

Debra Hoppensteadt

Keyword(s):

Disseminated Intravascular Coagulation ◽

Protein C ◽

High Mobility ◽

Severity Of Illness ◽

Endothelial Activation ◽

Endothelial Damage ◽

Intravascular Coagulation ◽

Angiopoietin 2 ◽

Von Willebrand ◽

Relationship Of

The role of the endothelium in sepsis-associated disseminated intravascular coagulation (DIC) is multifaceted and may contribute substantially to disease severity and outcome. The purpose of this study was to quantify measures of endothelial function, including markers of activation (endocan, Angiopoietin-2 [Ang-2], and von Willebrand Factor), endogenous anticoagulants (tissue factor pathway inhibitor and protein C), and damage-associated factors (High Mobility Group Box 1 [HMGB-1]) in the plasma of patients with sepsis and DIC, and to determine the relationship of these factors with severity of illness and outcome. Plasma samples were collected from 103 adult patients with sepsis within 48 hours of intensive care unit admission. Biomarker levels were measured using commercially available, standardized methods. Disseminated intravascular coagulation was diagnosed according to the International Society of Thrombosis and Hemostasis scoring algorithm. Twenty-eight-day mortality was used as the primary end point. In this study, endothelial damage and dysfunction were associated with the severity of coagulopathy and mortality in DIC patients. Loss of the endogenous anticoagulant protein C and elevation in the vascular regulator Ang-2 were associated with the development of overt DIC. In addition to Ang-2 and protein C, endocan, a biomarker of endothelial activation, and HMGB-1, a mediator of endothelial damage and activation, were significantly associated with mortality. This underscores the contribution of the endothelium to the pathogenesis of sepsis-associated DIC.

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