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Disseminated Intravascular Coagulation

Acquired Purpura Fulminans


Presentation

The following organ systems heralds the following manifestation during a disseminated intravascular coagulation:

  • Heart: Chest pain may be seen due to the presence of clot formation in the heart where a heart attack is also possible.
  • Lungs: Shortness of breath is seen if clots are present in the lungs.
  • Kidney and other organs: Clots present here can result in kidney failure or failure of the organ where the clot is present.
  • Brain: Blood clots in the brain will lead to into stroke. Headache, paralysis, dizziness, changes in speech, and alike are observable in stroke patients. Severe bouts of headaches and double vision may be caused by bleeding in the brain.
  • Limbs: Swelling, pain, redness and warmth in the limb where a blood clot is present.
  • Internal bleeding: Blood in urine and in the stools.
  • External bleeding: Bleeding under the skin or in body cavities or at sites of intravenous injection.
Easy Bruising
  • Other symptoms are: blood clots decreased blood pressure easy bruising rectal or vaginal bleeding red dots on the surface of the skin ( petechiae ) If you have cancer, DIC generally begins slowly, and clotting in the veins is more common than excessive[healthline.com]
HELLP Syndrome
  • Nevertheless, it has been suggested that in most cases these women also had a HELLP syndrome and that the occurrence of DIC in women who had only preeclampsia without manifestations of the HELLP syndrome is rare.[ncbi.nlm.nih.gov]
  • KEYWORDS: Acute fatty liver of pregnancy; Endothelial dysfunction; HELLP syndrome; Hemorrhage; Score; Trophoblast[ncbi.nlm.nih.gov]
  • syndrome Amniotic fluid embolism Disseminated Intravascular Coagulation Injuries Untreated, DIC can cause serious and potentially fatal injuries, including: Hemorrhage (excessive bleeding) Shock Maternal death Fetal death Preventing and Treating DIC[birthinjuryjustice.org]
  • HELLP syndrome: HELLP syndrome: Hemolysis, Elevated Liver enzymes, Low platelet count Syndrome. AFLP: Acute Fatty Liver pregnancy. (DOCX) Author Contributions Conceptualization: MJ FF. Data curation: MJ. Formal analysis: MJ FF AD. Investigation: MJ.[journals.plos.org]
  • Obstetric causes include complications such as amniotic fluid embolism, pre-eclampsia and eclampsia, HELLP syndrome (haemolysis, elevated liver enzymes and low platelets ), placental abruption and placenta praevia, intrauterine infection and death of[cochrane.org]
Fever
  • There was no other explanation for the fever and rash, including infection, malignancy, and collagenosis.[ncbi.nlm.nih.gov]
  • Literature review revealed that successful conservative management of a patient with asymptomatic DIC and subsequent fever without infection is extremely rare.[ncbi.nlm.nih.gov]
  • BACKGROUND: The clinical presentation of thyroid storm includes fever, tachycardia, hypertension, and neurological abnormalities. It is a serious condition with a high mortality rate.[ncbi.nlm.nih.gov]
  • CASE PRESENTATION: A 49-year-old immunocompetent Caucasian male presented with sudden onset of fever and DIC due to primary CMV infection, which was treated with Valganciclovir.[ncbi.nlm.nih.gov]
  • CASE PRESENTATION: An 86-year-old Japanese woman was admitted to our hospital because of low back pain and fever. A careful evaluation led to a diagnosis of acute obstructive pyelonephritis due to a ureteral stone.[ncbi.nlm.nih.gov]
Anemia
  • And, early diagnosis with appropriate intervention is important because mortality from ES is known to be greater than that of isolated immune hemolytic anemia and probably worse in the presence of DIC.[ncbi.nlm.nih.gov]
  • DIT results in TTP-like syndrome with hematologic phenotype of consumptive thrombocytopenia, microangiopathic hemolytic anemia, and multiorgan dysfunction syndrome.[ncbi.nlm.nih.gov]
  • In light of developing fluctuant subgaleal fluid associated with pallor, anemia, metabolic acidosis, and respiratory distress, immediate blood transfusion is warranted. In the presence of DIC, transfusion of FFP is beneficial.[ncbi.nlm.nih.gov]
  • Grade 3 adverse effects included neutropenia, anemia, hyponatremia, catheter-related infection and diarrhea (maximum: 2 patients each).[ncbi.nlm.nih.gov]
  • Microangiopathic hemolytic anemia and thrombocytopenia without coagulopathy is not DIC.[medicalmediareview.com]
Malaise
  • A 66-year-old woman presented to an urgent care clinic for 2 to 3 weeks of general malaise, nausea/vomiting, night sweats, and dyspnea.[ncbi.nlm.nih.gov]
  • Acute DIC Chronic DIC Multiple bleeding sites Bruising of skin, mucous membranes Internal bleeding Lack of blood supply to tissues ( ischaemia ) Sudden onset of high fever, severe general malaise, and extensive purpura of the extremities Petechiae, purpuric[dermnetnz.org]
Patient Appears Acutely Ill
  • On physical examination, the patient appeared acutely ill, but her heart sounds were normal. A neurologic examination revealed no abnormalities. Laboratory test results are shown in Table 1.[kjim.org]
Cough
  • Clinical history can include epistaxis, gingival bleeding, haematuria, oliguria, cough, dyspnoea, fever, delirium, and coma.[bestpractice.bmj.com]
  • You cough up blood. Your arm or leg feels warm, tender, and painful. It may also look swollen and red.[drugs.com]
  • […] organ dysfunction caused by blood clots blocking blood flow and oxygen to organs such as the liver and kidney, leading to liver and kidney failure Blackening of the skin caused by blockage from blood clots and poor blood flow to the skin Chest pain, coughing[labtestsonline.org]
  • She presented with a week’s history of shortness of breath and productive cough, confirmed microbiologically to be due to Streptococcus pneumoniae.[anesthesiology.pubs.asahq.org]
Dyspnea
  • A 66-year-old woman presented to an urgent care clinic for 2 to 3 weeks of general malaise, nausea/vomiting, night sweats, and dyspnea.[ncbi.nlm.nih.gov]
  • , rales Pulmonary thromboembolism : dyspnea, chest pain, hemoptysis Deep vein thrombosis : lower limb edema Neurological dysfunction: altered mental status, stroke Purpura fulminans: DIC with extensive skin necrosis Waterhouse Friderichsen syndrome :[amboss.com]
  • […] red, pinpointlike dots (petechiae) Vomiting of blood Bloody or black stools Blood in the urine Vaginal bleeding Unexplained bruises Severe abdominal or back pain Seizures or loss of consciousness in advanced cases (rare) Nausea Difficulty breathing (dyspnea[healthcommunities.com]
Hemoptysis
  • […] hematochezia Collection of blood in body cavities: features of hemoperitoneum, hemothorax Thrombotic manifestations Acute renal failure : oliguria Hepatic dysfunction : jaundice ARDS : dyspnea, rales Pulmonary thromboembolism : dyspnea, chest pain, hemoptysis[amboss.com]
Hematemesis
  • References: [3] [4] Clinical features Bleeding manifestations Petechiae, purpura, ecchymoses Oozing of blood from surgical wounds, intravenous lines Hematuria Hematemesis, hematochezia Collection of blood in body cavities: features of hemoperitoneum,[amboss.com]
Bleeding Gums
  • Signs and symptoms that appear gradually are prolonged bleeding from a venipuncture site, bleeding gums, nosebleeds, and bruising easily as well as the presence of minute, pinpoint red spots caused by bleeding under the layer of the skin.[encyclopedia.com]
Oral Bleeding
  • KEYWORDS: chronic disseminated intravascular coagulation; oral bleeding; thoracoabdominal aortic aneurysm; tooth extraction[ncbi.nlm.nih.gov]
Acrocyanosis
  • Consider heparin (300-500 U/hr) for DIC manifested by thrombosis or acrocyanosis in the absence of active bleeding. 5 Anti-fibrinolytics (tranexamic acid and ε-amioncaproic acid) are generally contraindicated, except in the setting of life-threatening[emdocs.net]
Tachycardia
  • BACKGROUND: The clinical presentation of thyroid storm includes fever, tachycardia, hypertension, and neurological abnormalities. It is a serious condition with a high mortality rate.[ncbi.nlm.nih.gov]
Purpura
  • The DIC was rapidly improved; however, the purpura and coagulopathy recurred after two months, and repeated rhsTM treatments were required.[ncbi.nlm.nih.gov]
  • 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.[ncbi.nlm.nih.gov]
  • Abstract A 78-year-old woman was admitted to our hospital complaining of anorexia and purpura of the extremities. She presented with prominent peripheral eosinophilia and disseminated intravascular coagulation (DIC).[ncbi.nlm.nih.gov]
  • Purpura fulminans is a severe and rapidly fatal form of acute DIC. It is sometimes associated with symmetrical peripheral gangrene (tissue death affecting hands and feet).[dermnetnz.org]
  • References: [3] [4] [5] Differential diagnoses Severe hepatic dysfunction : clinical features, and/or laboratory findings of liver injury, normal factor VIII assay Thrombotic thrombocytopenic purpura / immune thrombocytopenic purpura : no consumption[amboss.com]
Petechiae
  • Two weeks later, he was transferred to the West China Hospital with nasal hemorrhage for 1 week, hematochezia, hematuria, and petechiae for 5 days. DIAGNOSES: Laboratory data and symptoms on admission indicated DIC.[ncbi.nlm.nih.gov]
  • Physical examination may reveal petechiae, ecchymosis, gangrene, mental disorientation, hypoxia, hypotension, and GI bleeding.[bestpractice.bmj.com]
  • Clinical Symptoms These are noted due to spontaneous primary bleeding, including petechiae, ecchymoses, mucosal bleeding or secondary bleeding into body cavities e.g. haemoabdomen.[en.wikivet.net]
  • The bleeding can also be external or seen just under the skin causing purpura, which looks like bruising, and petechiae, which look like small red dots on the skin.[study.com]
Epistaxis
  • Clinical history can include epistaxis, gingival bleeding, haematuria, oliguria, cough, dyspnoea, fever, delirium, and coma.[bestpractice.bmj.com]
  • […] of inflammatory pathways via cytokines Suppression of physiologic anticoagulant pathways Activation and/or impairment of fibrinolysis Clinical Presentation Generally occurs in the setting of a risk factor listed above Hemorrhage – petechiae, purpura, epistaxis[arupconsult.com]
  • In the chronic DIC associated with underlying malignancy, patients may develop epistaxis, purpura, or inter-current thrombocytopenia. In patients without a prior diagnosis of a malignancy, these events may eventuate in the discovery of cancer.[cancertherapyadvisor.com]
Hematuria
  • Two weeks later, he was transferred to the West China Hospital with nasal hemorrhage for 1 week, hematochezia, hematuria, and petechiae for 5 days. DIAGNOSES: Laboratory data and symptoms on admission indicated DIC.[ncbi.nlm.nih.gov]
  • References: [3] [4] Clinical features Bleeding manifestations Petechiae, purpura, ecchymoses Oozing of blood from surgical wounds, intravenous lines Hematuria Hematemesis, hematochezia Collection of blood in body cavities: features of hemoperitoneum,[amboss.com]
  • […] malignancy, liver disease, retained dead fetus syndrome, abdominal aortic aneurysm, giant hemangioma and head trauma Bleeding can present as surgical site, venipuncture site or mucocutaneous bleeding (most common) Gastrointestinal bleeding, CNS bleeding, hematuria[pathologyoutlines.com]
Oliguria
  • Clinical history can include epistaxis, gingival bleeding, haematuria, oliguria, cough, dyspnoea, fever, delirium, and coma.[bestpractice.bmj.com]
  • […] manifestations Petechiae, purpura, ecchymoses Oozing of blood from surgical wounds, intravenous lines Hematuria Hematemesis, hematochezia Collection of blood in body cavities: features of hemoperitoneum, hemothorax Thrombotic manifestations Acute renal failure : oliguria[amboss.com]
  • […] mucocutaneous bleeding (most common) Gastrointestinal bleeding, CNS bleeding, hematuria or ecchymoses Thrombosis can present as purpura fulminans (manifestation of subdermal microthrombi with skin necrosis) Cold, pulseless limb Sudden loss of vision Oliguria[pathologyoutlines.com]
Vaginal Bleeding
  • Symptoms of Disseminated Intravascular Coagulation Abnormal bleeding from anywhere in the body, possibly at several sites at once Tiny, red, pinpointlike dots (petechiae) Vomiting of blood Bloody or black stools Blood in the urine Vaginal bleeding Unexplained[healthcommunities.com]
  • Other symptoms are: blood clots decreased blood pressure easy bruising rectal or vaginal bleeding red dots on the surface of the skin ( petechiae ) If you have cancer, DIC generally begins slowly, and clotting in the veins is more common than excessive[healthline.com]
Dark Urine
  • […] pink or dark urine. On dipstick, the urine will be positive for “blood,” but there will be no red blood cells on microscopy.[medicalmediareview.com]
Confusion
  • Abstract Heat stroke is a life-threatening condition which is characterised by nausea, vomiting, confusion, disorientation and coma. Aggressive treatment in the form of intravenous fluids along with other symptomatic management can be life saving.[ncbi.nlm.nih.gov]
  • However, some confusion still exists in the definition and management of DIC since various specialists understands the mechanisms involved in DIC from different perspectives.[ncbi.nlm.nih.gov]
  • […] injury (as in burns and head injury) Large hemangioma (a blood vessel that is not formed properly) Symptoms of DIC may include any of the following: Bleeding, from many sites in the body Blood clots Bruising Drop in blood pressure Shortness of breath Confusion[nlm.nih.gov]
  • Confusion or disorientation. Fever. Signs of haemorrhage. Signs of adult respiratory distress syndrome (ARDS). Skin may show various signs including: Petechiae. Purpura. Haemorrhagic bullae. Acral cyanosis.[patient.info]
Seizure
  • Treatment of seizures Treatment of seizures depends on the underlying disorder causing the DIC.[epilepsy.com]
  • Although one episode of seizures was noted, electroencephalogram was normal. With the application of obstetric vacuum, we recommend that the neonatal health care professionals frequently evaluate the infant's condition.[ncbi.nlm.nih.gov]
  • […] or loss of consciousness in advanced cases (rare) Nausea Difficulty breathing (dyspnea) Seizures Organ failure Prevention of Disseminated Intravascular Coagulation Disorders that could result in DIC require prompt treatment.[healthcommunities.com]
  • […] common) Gastrointestinal bleeding, CNS bleeding, hematuria or ecchymoses Thrombosis can present as purpura fulminans (manifestation of subdermal microthrombi with skin necrosis) Cold, pulseless limb Sudden loss of vision Oliguria Mental status changes, seizures[pathologyoutlines.com]
  • Typical thrombotic features of DIC are thrombophlebitis developing at unusual sites; respiratory distress syndrome; development of renal impairment without obvious explanations; central nervous system disturbances such as confusion and seizures, typically[anesthesiology.pubs.asahq.org]
Altered Mental Status
  • mental status, stroke Purpura fulminans: DIC with extensive skin necrosis Waterhouse Friderichsen syndrome : adrenal infarcts adrenal insufficiency The clinical features of DIC may appear acutely (e.g., following trauma, sepsis ), or may appear subacutely[amboss.com]
  • Following a prolonged extrication, the patient was intubated in the field secondary to altered mental status (GCS 6).[emdocs.net]

Workup

The first observation, in order to diagnose DIC is to ask for the patient’s medical history. History of stroke, uncontrolled bleeding, et cetera may collaborate a diagnosis of DIC. Apart from this, other tests may be done to confirm a diagnosis of DIC.

  • Complete blood count and blood smear: This gives the RBC count, WBC count, hemoglobin count, hematocrit, platelet count, etc.
  • Fibrin degradation: Fibrin degradation products are those that are left behind after the blood clot dissolves. This test measures the amount of this substance in the blood.
  • Fibrinogen/CRP ratio: This ratio gives a predictive outcome of DIC can guide the surgeon about his treatment options [7].
  • Partial thromboplastin time: This test measures the time taken for blood to clot.
  • Serum fibrinogen: This test measures the amount of fibrinogen present in the blood
Decreased Platelet Count
  • Blood tests revealed a decreased platelet count, disseminated intravascular coagulation (DIC), renal dysfunction, hemolysis, and infection.[ncbi.nlm.nih.gov]
  • Diagnosis is based on presence of 1 known underlying conditions causing DIC plus abnormal global coagulation tests: decreased platelet count, increased prothrombin time, elevated fibrin-related maker (D-dimer/fibrin degradation products), and decreased[bestpractice.bmj.com]
Fibrinogen Decreased
  • Decreased in DIC May not be accurate in early disease because it is an acute phase reactant Clotting times and coagulation factors may be inaccurate in the presence of interfering substances, including drugs administered for anticoagulation Refer to[arupconsult.com]
Prothrombin Time Increased
  • time (increase in seconds) 3-6 6 FEU fibrinogen equivalent units Toh, ISTH, 2007 Laboratory Testing D-dimer – increased in acute and chronic DIC (best single test) Normal D-dimer essentially rules out DIC (excellent negative predictive value) Low specificity[arupconsult.com]

Treatment

The treatment for DIC depends on the severity of the condition and also on the underlying disease. Effective treatment for the underlying condition is the most effective way of treating DIC.
In case of acute DIC blood transfusion may allay the situation. Acute bleeding with chronic leukemia may be stabilized with the infusion of plasma extenders and tranexamic acid [8].

This is done to compensate the excessive loss of blood. Platelets (platelet concentrate) and clotting factors may be given to the patient via rapid blood transfusion [9]. Patients complicated with sepsis may benefit from the administration of activated C-protein which is the only approved therapeutic approach recognized in the US for these cases [10]. In case of chronic DIC blood thinners such as heparin may be given in order to prevent clot formation.

Prognosis

The prognosis of a patient with disseminated intravascular coagulation depends on the severity of the inciting condition and the very coagulation in itself. DIC can be handled effectively with the proper treatment of the primary underlying condition.

Consequently with DIC, fibrin depositions in vessels of some organs can result in ischemia. The deposition of fibrin can also lead multiple organ failure and can evidently lead to death. DIC can increase the chances of mortality in patients with sepsis and severe trauma.

Complications

The following are a few of the complications associated with DIC:

  • Bleeding: The excessive, uncontrollable bleeding is life threatening and can result in death. The underperfused liver due to DIC may result in the impaired production of Antithrombin which aggravates the bleeding problems [6].
  • Lack of oxygen: The presence of clots in the blood vessels reduces the blood supply hence the lack of oxygen supply to the organs which can lead to organ damage.
  • Stroke: Formation of blood clots in the vessels of the brain can lead to stroke.

Etiology

As mentioned earlier, DIC in itself is not a disease but a manifestation in itself of other illnesses. Some of these illnesses have been shown to cause DIC:

Epidemiology

Disseminated intravascular coagulation is a rare condition that occurs in only 1% of all hospitalized patients [3]. The risk for DIC may be higher for those with ongoing sepsis which can reach up to 30-50% of cases [4].

The mortality rate for DIC is 50-75%. No evidence has been raised that DIC is influenced by genetics, geography, race, gender, age or socioeconomic status.

Sex distribution
Age distribution

Pathophysiology

Disseminated intravascular coagulation is caused when the control mechanism of hemostasis is disrupted. This leads to widespread intravascular coagulation. When the hemostatic system is system is disturbed, inflammatory cytokines are released.

This leads to uncontrolled thrombin generation. The disturbance in the hemostatic system can be due to thrombin generation which is mediated by tissue factors or imbalance in thrombin generation which may be triggered by inflammation. The uncontrolled thrombin generation leads to microvascular thrombosis and impaired anticoagulant pathways. In patients with DIC the levels of antithrombin (an inhibitor of thrombin) in the plasma is considerably less; thus, thrombin overproduction remains unopposed. The reduced level of antithrombin correlates with an increased rate of mortality, especially in patients with sepsis or septic shock.

Tissue damage leads to fibrin deposition. This releases and activates plasminogen which in turn initiates plasmin generation. The generation of a plasmin inhibitor α2 antiplasmin is also reduced into a much lower level than that of plasmin.
Plasmin degrades clotting factors VIII, V and I where fibrinogen degradation products are also produced. All of these products, along with fibrin which isn’t polymerized completely, lead to impaired platelet function and abnormal fibrin polymerization causing uncontrolled bleeding.

Microvascular thrombosis causes ischemia, necrosis, release of tissue factors, and organ dysfunction. Intravascular coagulation is consequently accelerated due to this phenomenon.
In acute DIC consumption of platelets and clotting factors occur faster than they can be replaced which leads to uncontrolled bleeding. Trauma patients presents with an acute phase fibrinolysis causing a consumptive type of coagulation disorder leading to uncontrolled bleeding [5].

Whereas in chronic DIC the time and intensity of trigger varies rendering the regulatory mechanism of coagulation with more control comparatively.

Prevention

The best way to prevent DIC is the timely treatment of the conditions that can lead to DIC. This condition can be managed with proper medication. Since DIC is not an illness in itself, the only prevention method available is to treat the condition that can lead to DIC.

Summary

Disseminated intravenous coagulation (DIC) is a condition in which the excessive release of thrombin and other clotting factors cause clotting in the small vessels of the body. This leads to the excessive generation and systemic deposition of fibrin. This in turn leads to Multiple Organ Dysfunction Syndrome (MODS) or multi-organ failure that eventually causes death [1].

Disseminated intravascular coagulation is not a disease in itself but it is a complication which presents itself as an illness progresses.

There are two types of DICs, the acute and the chronic type. Acute DIC progresses in a very short duration of time and immediate treatment is required. The progression of chronic DIC is slower compared to the acute form. Chronic DIC presents symptoms later than acute DIC and is usually caused by cancer. Solid tumors and large aortic aneurysm may also lead to chronic DIC [2].

Patient Information

In case of unstoppable bleeding, go to the doctor immediately
When signs of internal or external bleeding are observed, seek medical attention immediately.

References

Article

  1. Vincent JL, De Backer D. Does disseminated intravascular coagulation lead to multiple organ failure?. Crit Care Clin. Jul 2005;21(3):469-77.
  2. Levi M. Disseminated intravascular coagulation: What's new?. Crit Care Clin. Jul 2005;21(3):449-67.
  3. Matsuda T. Clinical aspects of DIC--disseminated intravascular coagulation. Pol J Pharmacol. Jan-Feb 1996;48(1):73-5.
  4. Fourrier F, Chopin C, Goudemand J, Hendrycx S, Caron C, Rime A, et al. Septic shock, multiple organ failure, and disseminated intravascular coagulation. Compared patterns of antithrombin III, protein C, and protein S deficiencies. Chest. Mar 1992;101(3):816-23.
  5. Hayakawa M, Sawamura A, Gando S, Kubota N, Uegaki S, Shimojima H, Sugano M, Ieko M. Disseminated intravascular coagulation at an early phase of trauma is associated with consumption coagulopathy and excessive fibrinolysis both by plasmin and neutrophil elastase. Surgery. 2011; 149(2):221-30 (ISSN: 1532-7361)
  6. Carey MJ, Rodgers GM. Disseminated intravascular coagulation: clinical and laboratory aspects. Am J Hematol. Sep 1998;59(1):65-73
  7. Windsperger K, Lehner R. The fibrinogen/CRP ratio as a new parameter for the diagnosis of disseminated intravascular coagulation in patients with HELLP syndrome and as a predictive factor for neonatal outcome. Am J Obstet Gynecol. 2013; 208(2):118.e1-7 (ISSN: 1097-6868).
  8. Martí-Carvajal AJ, Simancas D, Cardona AF. Treatment for disseminated intravascular coagulation in patients with acute and chronic leukemia. Cochrane Database Syst Rev. 2011; (6):CD008562 (ISSN: 1469-493X)
  9. Gando S, Sawamura A, Hayakawa M. Trauma, shock, and disseminated intravascular coagulation: lessons from the classical literature. Ann Surg. 2011; 254(1):10-9 (ISSN: 1528-1140)
  10. Hook KM, Abrams CS. The loss of homeostasis in hemostasis: new approaches in treating and understanding acute disseminated intravascular coagulation in critically ill patients. Clin Transl Sci. 2012; 5(1):85-92 (ISSN: 1752-8062).

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Last updated: 2018-06-21 17:21