Patho 2 final (hemo, GI, renal, endo)

What is the range of hemoglobin M+F

• M - 140-180

• F -120-160

What is the range for Plt (x10⁹ L)

150-400

RBC M + F x 10¹²

• RBC Female 4.2-5.4 × 10¹²/L

• RBC Male 4.7-6.2 × 10¹²/L

HCT M + F

percentage of RBC in blood

• Hematocrit Female 37-47%

• Hematocrit Male 42-52%

EPO production

Kidneys produce erythropoetin - hormone used to stimulate bone marrow to produce RBC

HOWEVER - NEEDS IRON//FERRITIN AS BUILDING MATERIAL

cant signal without the building blocks

Normal RBC production

• produced in bone marrow

• stimulated by erythropoietin (EPO)

• Developing RBCs need » Iron Vitamin B12 Folate

• Iron is incorporated into the heme portion of hemoglobin.

• Hemoglobin fills the RBC and carries oxygen

reticulocytes

Immature RBC - no nucleus but not fully formed and ready to be released

anemia general

• Reduction in total RBC volume or Hgb concentration leading to reduced oxygen- carrying capacity.

• Measured using MCV

microcytic » RBC are smaller than normal

normocytic » RBC normal size

macrocytic » RBC larger than normal

BODY COMPS WITH TACHYCARDIA + TACHYPNEA - PUSH MORE O2 OUT TO TISSUES

Can occur in 3 ways

• decreased production

• increased destruction

• blood loss

iron deficiency anemia - IDA general//patho

insufficient iron availability - unable to meet body’s demands

• Decreased Iron Stores = Low ferritin level

• Reduced iron for EPO → (↓ Hemoglobin production)

• Production of Microcytic, Hypochromic RBC UNABLE TO CARRY O2 NOT FULLY DEVELOPED

• ↓O2 capacity = Tissue Hypoxia

Due to

• Low iron intake//impaired absorption » celiac disease, chronic diarrhea, malabsorption syndrome

• increased iron loss » heavy menstruation, GI bleed, PUD, trauma

• higher iron demand » rapid growth, pregnancy, lactation

• poor dietary intake, alc use disorder

EncourageActivity-Rest balance to reduce fatigue andoxygen demand

Transfusion of packed RBCs

treat underlying cause

nutrional therapy

IDA peds

• Most common nutritional deficiency worldwide in children

• Highest risk from birth to age 2 years due to rapid growth and increased iron demands.

• Adolescents are also at increased risk because of » Rapid growth spurts, Poor dietary habits Menstruation (females)

• Potential Consequences in Children » Developmental delays, Behavioral and cognitive concerns , Fatigue and decreased activity tolerance

peds IDA symptoms

• Lethargy and listlessness

• Pallor

• Reduced interest in play and physical activity

• ↑ sleep

• Weakness and decreased endurance

• Severe IDA: tachycardia and systolic murmur » can go away or permenant

• Irritability and behavioral changes

• Potential cognitive and developmental delays

• *Adolescents may demonstrate poorer cognitive and motor performance

• Long-term severe deficiency may contribute to irreversible cognitive impairment

IDA symp early vs late

early

• Fatigue

• Weakness

• Irritability

• Decreased activity tolerance

• Mild pallor or ashy (earlobes, palms)

• Shortness of breath with exertion

late

• Tachycardia

• palpitations/ systolic murmur

• Glossitis (smooth, beefy red tongue)

• Pica (craving ice, clay, starch)

• Brittle, concave spoon nails (koilonychia)

• Cheilitis (fissures at corners of mouth)

• dysphagia

BAD

• chest pain

• syncope

• resp depression

IDA hypoxia and HF progression

• tissue hypoxia due to low hgb and low o2 - muscle cramps weakness fatigue ↑ RR exertional dyspnea

• compensatory mechanisms » ↑ HR o2 demand, SV RASS (na and h2o retention)

• chronic anemia can lead to HF ↑ demand

ferritin vs transferrin vs TIBC

• ferritin » protien that stores iron inside your cells

• transferrin » transport protien that carries iron in bloodstream

• TIBC » binding sites on transferrin available

IDA diagnostics and labs

• CBC » ↓Hgb, ↓Hct, microcytic/hypochromic RBCs

• ferritin » ↓ - less iron to store in cells

• serum iron » ↓

• TIBC ↑ - more sites available on transferrin

• transferrin saturation » ↓ - no iron to transport

• reticulocytes » Normal or ↓before treatment; may ↑after iron therapy as RBC production improves

• peripheral blood smear » Small, pale RBC

• bone marrow biopsy Decreased iron stores in bone marrow

IDA treatment

IRON REPLACEMENT (IV or oral) - ferrous sulf or gluc

Vit c » helps with iron absorption from diet

hemolytic anemia - general

• intrinsic RBC defect or extrinsic destruction

• ↓ circulating RBC lifespan

• Bone marrow increases RBC production - (↑ Reticulocytes)

• If destruction > production - Anemia develops

Symp
Fatigue, Jaundice, Pallor
Splenomegaly/Gallstones
Tea coloured urine (hemoglobinuria)
Back/abdominal pain (acute hemolysis)
Tachycardiac, dyspnea

Labs - Hemolysis Panel
Hemoglobin ↓
↑ Reticulocytes > 2%
↑ LDH - enzyme released in RBC breakdown
↑ Unconjugated bilirubin - released in RBC breakdown (jaundice)

unconjugated bilirubin

BREAKDOWN PRODUCT OF HEMOGLOBIN (hgb)

occurs when rbc are broken down faster than liver can process it

can lead to jaundice

Total Bilirubin 3-22 mcmol/L Elevated causes jaundice

spleen function

• helps filter out old blood cells and debris

• recycles iron - sends it back to bone marrow to make new hgb

• 1/5 of blood and plts stored there

• produces WBC - B and T cells

• CONSIDERED NON VITAL

sickle trait vs sickle cell

sickle trait

• 1 sickle and one normal gene

• usually no symp, anemia is absent

• sickling is rare - under extreme stress

• pain crisis rare

• minimal complications risk

• usually no treatment needed, just monitoring

sickle cell

• 2 genes

• chronic symp and complications

• chronic hemolytic anemia

• sickling rare and pain crises common

• significant risk of organ damafe

• lifelong monitoring and treatment

risk factors for SCD

• Family history of SCD// sickle cell trait carrier » ignificance Inherited autosomal recessive disorder - Child may inherit SCD

• Ancestry from malaria-endemic regions » SCD as malarial protection (virus skips sickle cells)

• African, Caribbean, Middle Eastern, Mediterranean, South American, and South Asian ancestry Increased prevalence of sickle cell genes

SCD patho

• Genetic mutation → Hemoglobin S (HbS) - autosomal recessive

• HbS polymerization - RBCs become rigid & sickle-shaped (crescent) shape when oxygen levels decrease.

• Sickled cells are fragile, break down prematurely, and impair blood flow.

• Repeated cycles of sickling and return to normal shape damage the red blood cell membrane.

• Damaged red blood cells undergo premature destruction (hemolysis).

• Premature RBC destruction leads to chronic anemia.

• Sickled cells may temporarily return to a normal shape with rehydration and reoxygenation.

• To compensate for ongoing hemolysis, the bone marrow increases red blood cell production (erythropoiesis)

SCD manifestations

• Chronic hemolytic anemia

• Fatigue and weakness

• Pallor

• Mild jaundice and scleral icterus

• Delayed growth and development in children// Delayed puberty in adolescents Recurrent infections » due to affected spleen

• Splenomegaly (more common in young children)

• Chronic organ damage (e.g., kidneys, lungs, heart)

• Leg ulcers (may occur in adults) - blocakge and hypoxemia

• Retinopathy and vision changes » blockage of retinal vessels

• neuro concerns

• RISK FOR MI DVT PE STROKE PRIAPISM

ALL DUE TO LOW O2 AND HYPOXEMIA OF TISSUES

SCD triggers

3 D - deoxygenation dehydration disease

• low o2 » causes sickling

• dehydration » Increases blood viscosity and sickling

• infection » Increases inflammation and oxygen demand

• stress

• extreme temp » May promote vaso-occlusion

• acidosis » Increases HbS polymerization and sickling

• high altitude » Reduces oxygen availability

• strenuous excercise» Can cause hypoxia and dehydration

vaso-occlusive// pain crisis

• MOST COMMON - caused by low o2

• causes organ damage and pain - sickled cells block capillaries

• leads to severe ischemia and pain

• Severe pain in bones, joints, chest, abdomen, or back; tenderness, swelling, decreased mobility

acute chest syndrome

symp

• Chest pain

• Fever

• Hypoxia

• dyspnea

• tachypnea

• Pulmonary infiltrates - abnormal material filling alveoli
  
  

SYMP ARE SIMILAR TO HEART ATTACK - NEEDS O2 - caused by low oxygenation

aplastic crisis

• bone marrow stops producing RBC

• ↓↓ drop in RBC reticulocytes hct

• profound fatigue, pallor, weakness, worsening anemia, low reticulocyte count

• often associated with parvovirus B19 infection

splenic sequestration

• MOST DANGEROUS - spleen can rupture

• sickle cells block capillaries to and inside spleen

• primarily affects young children (2 and under)

• Rapid splenic enlargement + severe anemia; pallor, weakness, tachycardia, hypotension, signs of hypovolemic shock

diagnostics + labs SCD

• CBC » ↓ hgb hct

• ↑ Reticulocytes » Body attempting to rebuild lost RBC after intense breakdown//hemolysis

• ↓ Reticulocytes (sudden decrease

• ↑ Bilirubin » from hemolysis » Aplastic crisis

• ↓ O₂ saturation » Acute chest syndrome

• ↑ Creatinine kidney damage

• ↑ WBC » Infection trigger

• LDH ↑ - hemolysis marker

• hgb electrophorisis » Presence of HbS; confirms diagnosis

• ↑ Peripheral smear – Sickled cells

• genetic testing » confirms sickle trait carrier
  
  
  

SCD management // treatment

• Encourage adequate hydration to reduce blood viscosity

• Teach avoidance of crisis triggers: Dehydration, Infection, Hypoxia, High altitudes, Extreme temperatures, Stress

• Monitor for chronic complications (e.g., vision changes, organ damage) Promote routine screening and follow-up care

• Ensure recommended immunizations are up to date

• Encourage prompt treatment of infections

Sickle crisis priority assessments 5

• • Pain assessment

• Respiratory status and oxygen saturation

• Neurological status

• Hydration status

• Signs of infection

Sickle crisis management

• Administer oxygen as prescribed for hypoxia

• Provide IV and/or oral fluids to reduce blood viscosity and maintain renal perfusion

• Encourage rest to decrease metabolic demands

• Monitor closely for acute chest syndrome and other crises

• Monitor for worsening anemia and other complications

• Implement DVT prophylaxis as prescribed

• EFFECTIVE PAIN MANAGEMENT HM AND MORPHINE - DOSES WILL BE SUPER HIGH COMPARED TO NORM

hematomochrosis - primary vs secondary

HIGH FE IN TISSUES

primary

• autosomal recessive

• Clinical manifestations reflect the organs affected by iron deposition

• Liver is typically affected first; can progress to cirrhosis, liver failure, and death

• Iron also commonly accumulates in the pancreas, heart, joints, and endocrine glands

secondary

• DUE TO ANOTHER CONDITION

• Excessive iron supplementation or iron injections

• Repeated blood transfusions

• Chronic liver diseases (e.g., alcoholic liver disease, chronic hepatitis, NASH)

hemochromatosis patho

• hepcidin - regulates entry of iron into circ

• deficiency results in excess iron absorbtion from GI tract

• Progressive iron overload causes tissue damage through iron deposition in the liver, pancreas, heart, and endocrine glands

hemochromatosis symp expected vs critical

expected

• fatigue

• abdo pain

• arthralgia

• elevated liver enzymes and hepatomegaly

• bronze or grey pigmentation

• DM - deposistion of iron into panc

critical

• cirrhosis

• liver failure

• hepatocellular carcinoma

• progressive cardiomypathy - leads to HF

hemachromatosis labs and diagnostics

• serum ferritin » ↑Elevated

• transferrin sat » ↑Elevated

• serum iron » ↑Elevated

• TIBC » ↓Low to normal - more iron available seats are full

• LFT » ↑May show liver injury

• genetic » mutation in primary

• MRI - Liver + heart » Detects iron deposition

• liver biopsy » Confirms iron overload and assesses liver damage

hemachromatosis management

• Monitor iron studies (e.g., ferritin levels) to evaluate iron overload and treatment effectiveness.

• Support and educate patients undergoing therapeutic phlebotomy the primary treatment for reducing excess iron stores. Support and educate

• Assess for signs of organ involvement, including: Liver dysfunction (cirrhosis, liver failure), Heart failure,Diabetes mellitus Assess

• Reinforce dietary modifications: Avoid iron supplements,Avoid vitamin C supplements (may increase iron absorption),Limit iron-rich foods as advised,

• Promote adherence to ongoing monitoring and follow-up appointments.

• Educate patients that early diagnosis and treatment can prevent serious complications and help maintain a normal life expectancy.

• meds » iron chelating agent - helps remove iron from blood and excrete in urine or bile

relative polycythemia

• Normal number of RBCs, but decreased plasma volume

• Blood becomes more concentrated (hemoconcentration) •

• Common causes:  Dehydration  Diarrhea  Vomiting  Excessive diuretic use

• Usually resolves when the underlying cause is treated and fluids are replaced

absolute polycythemia

True increase in RBC production

Primary Polycythemia (Polycythemia Vera)

• Bone marrow disorder, Often caused by a JAK2 mutation

• Uncontrolled production of RBCs (may also increase WBCs and platelets)

• ↑ Blood volume and viscosity → ↑ clotting risk

Secondary Polycythemia- Occurs as a response to chronic hypoxia or excess erythropoietin (EPO)

• Increased EPO stimulates RBC production

• Common causes: High altitude  Smoking  COPD  Heart failure  EPO-secreting tumors

polycythemia vera patho

• myleoproliferative neoplasm - uncontrollable bone marro production of bone cess

• typically RBC but can also impact WBC and plt

• JAK2 mutation → abnormal stimulation of hematopoietic stem cells o

• Bone marrow produces excessive RBCs (often WBCs and platelets as well)

• Increased RBC mass leads to: ↑ Blood volume ↑ Blood viscosity (thicker blood) ↓ Tissue perfusion ↑ Risk of thrombosis

• Long-term complications may include » Bone marrow fibrosis , Cytopenias, Progression to acute leukemia

polycythemia vera manifestations

• Headache

• Dizziness

• Fatigue

• Visual disturbances

• Hypertension

• Ruddy (reddish) complexion (plethora) » face ears hands feet

• Pruritus (especially after warm baths/showers) » due to nerve activation and histamine response ( ↑ mast cells and basophils) - doesnt responf to antihistamines

• Splenomegaly

• Thrombotic events (DVT, *Stroke, MI)

labs and diagnostics polycythemia

• CBC » ↑Hemoglobin (Hb), ↑Hematocrit (Hct), ↑RBC count; may show microcytosis

• EPO » ↓or normal (secondary polycythemia typically has ↑EPO)

• WBC » ↑WBC count (leukocytosis), often with basophilia

• Plt » ↑Platelets (thrombocytosis); platelet dysfunction may be present

• uric acid » ↑due to increased RBC growth and cell turnover; may contribute to gout

• histamine » ↑(associated with pruritus)

• vit b12 » ↑ - due to excess RBC

• bone marrow examination » Hypercellularity with increased RBC, WBC, and platelet production

• JAK 2 mutation testing » Positive in most patients; supports diagnosis

EVERYTHING IS UP

polycythemia nursing management

• Monitor for signs of hyperviscosity and thrombosis: Headache, Dizziness,Visual disturbances, Chest pain,Unilateral swelling or pain, Neurological changes

• Assist with and educate patients about therapeutic phlebotomy as prescribed.

• Monitor hydration status and accurately measure intake and output

• Encourage adequate fluid intake to help reduce blood viscosity.

• Assess for signs of fluid overload or dehydration during hydration therapy.

• Promote mobility and circulation: Encourage ambulation, Perform active/passive leg exercises

• mplement thrombosis prevention measures as ordered Implement

• Monitor CBC results (Hgb, Hct, RBCs, WBCs, platelets) and report significant changes.

• r Assess nutritional status and collaborate with dietitians if decreased intake occurs due to early satiety, abdominal discomfort, or dyspepsia

remove excess blood via prophylactic phlebotomy, stay hydrated to dilute blood viscosity without fluid overloading, watch for clots

thrombocythemia

• Platelet count > 400 × 10⁹/L

• May be primary (essential thrombocythemia) or secondary (reactive thrombocytosis)

• Often asymptomatic until platelet counts become markedly elevated (> 1,000 × 10⁹/L)

• increase the risk of » Thrombosis (blood clot formation) Hemorrhage (bleeding)

platelet consumption

Common in thrombocytopenia, polycythemia, DIC, HIT

• Clinical manifestations result from abnormal platelet function and impaired blood flow

BLEEDING RISK BECAUSE OF DEFECTIVE PLATELTS AND PLATELET COMSUMPTION

• THEY ALL GET USED UP SO WHEN A MAJOR TRAUMA ACTUALLY HAPPENS, THERES NO GOOD ONES AROUND AND YOU HAVE AN EVEN GREATER RISK OF BLEEDING

Secondary (Reactive) Thrombocytosis

• Increased platelet production in response to another condition

• Common causes: • Inflammation or infection • Malignancy • Splenectomy

thrombocythemia patho

• Chronic myeloproliferative neoplasm causing excessive platelet production

• Commonly associated with JAK2 or CALR gene mutations

• Excess platelets increase the risk of » Thrombosis (clot formation) Bleeding due to platelet dysfunction

• May also cause microvascular occlusion and reduced blood flow

thrombocythemia clinical manifestations (normal vs critical)

Expected

• Headache, dizziness

• Paresthesia's (numbness/tingling)

• Visual disturbances

• Pruritis (itching)

• Mild splenomegaly

• Fatigue

BAD

• Stroke/TIA

• Myocardial infarction

• *DVT or PE •

• Acute limb ischemia» Gangrene

• Severe hemorrhage (including intracranial and GI bleed

thrombocythemia diagnostics

• CBC » ↑ Platelet count (>450 × 10⁹/L)

• bone marrow biopsy » Increased mature megakaryocytes

• Genetic testing » JAK2 or CALR mutation may be present

• diagnostic eval » Rule out reactive thrombocytosis and other myeloproliferative disorders

thrombocythemia managment and treatment

• Monitor for signs of thrombosis and bleeding

• Assess for headache, visual changes, paresthesia's, and erythromelalgia - red painful burning skin

• Encourage mobility and hydration to reduce thrombotic risk

• Monitor CBC and platelet trends

• Educate patients to report symptoms of clotting or bleeding immediately

thrombocythemia pharm

• Low-dose aspirin (reduces microvascular symptoms and clotting risk)

• Hydroxyurea(reduces platelet production)

• Interferon or anagrelide as indicated

thrombocytopenia

Platelet count < 150 × 10 /L LOW Usually not clinically significant until < 100 × 10

• < 50 ×10 /L: Increased bleeding risk with trauma or procedures

• 10-15 × 10 /L: Risk of spontaneous bleeding (petechiae, purpura, mucosal bleeding)

• < 10 ×10 /L: High risk of life-threatening hemorrhage (GI, respiratory, or CNS bleeding)

thrombocytopenia

• develops when platelet loss exceeds platelet production:

↓Platelet Production (congenital or acquired)

• Viral infections (e.g., HIV, EBV, CMV)

• Bone marrow disorders (aplastic anemia, cancer infiltration) • Chemotherapy or radiation therapy

• Vitamin B₁₂or folate deficiency

• Chronic kidney disease

• Certain medications and alcohol

• 
  

↑Platelet Consumption/Destruction(most common cause)

• Immune thrombocytopenia (ITP)

• Heparin-induced thrombocytopenia (HIT)

• thrombotic thrombocytopenic purpura (TTP)

• Disseminated intravascular coagulation (DIC)

thrombocytopenia symp expected vs critical

expected

• Petechia, purpura, ecchymosis » pinpricks vs spots vs larger bruise

• Prolonged bleeding from minor cuts

• Epistaxis » nose bleed

• • Bleeding gums

• Menorrhagia

• Fatigue w/ mild blood loss

Critical

• GI bleeding

• Intracranial hemorrhage (changes in LOC)

• Pulmonary hemorrhage » Respiratory distress

• Uncontrolled bleeding

• Hypovolemic Shock

thrombocytopenia diagnostics

• CBC » ↓ Platelet count (<150 × 10⁹/L)

• Peripheral Blood Smear » Confirms thrombocytopenia; rules out platelet clumping (pseudothrombocytopenia)

• Coagulation Studies (PT/INR, aPTT) » Helps identify underlying disorders (e.g., DIC)

• Additional tests » Determines cause (e.g., B12, folate, infection, bone marrow disorders)

trhombocytopenia nursing management and meds

• Monitor platelet count and bleeding trends

• Assess for signs of bleeding (petechiae, bruising, epistaxis, GI bleeding)

• Implement bleeding precautions: Soft toothbrush, Electric razor, Avoid IM injections when possible, Apply prolonged pressure after venipuncture

• Prevent injury and falls

• Treat the underlying cause

• Educate patient to report new bleeding, bruising, or neurological symptoms

• Administer prescribed therapies» Corticosteroids/IVIG (immune causes) Platelet transfusions (severe bleeding or critically low platelets

heparin induced thrombocytopenia (HIT

• Immune-mediated adverse reaction to heparin

• More common with unfractionated heparin (UFH) than low-molecular-weight heparin (LMWH)

• Typically develops 5-10 days after heparin initiation

• Characterized by a significant drop in platelet count (>50% from baseline)

1. Heparin binds to Platelet Factor 4 (PF4)

2. Immune system produces IgG antibodies against the heparin-PF4 complex

3. Antibodies bind and activate platelets

4. Activated platelets are consumed, causing thrombocytopenia

5. Platelet activation also promotes thrombin formation and clotting

BLEEDING IS UNCOMMON DESPITE THROMBOCYTOPENIA

Key concept: ↓ Platelets + ↑ Platelet Activation = Increased Risk of Thrombosis

venous vs arterial complications in HIT

Venous Thrombosis (most common)

• Deep vein thrombosis (DVT)

• Pulmonary embolism (PE)

Arterial Thrombosis

• Limb ischemia

• Stroke

• Myocardial infarction (MI)

• Renal or mesenteric ischemia

HIT diagnostics

• Platelet Count » ↓ Platelets (typically >50% decrease from baseline)

• CBC » Thrombocytopenia, often nadir around 60 × 10⁹/L

• Anti-PF4/Heparin Antibody Test » +ve

• Serotonin Release Assay (SRA) » +ve - confirmatory test

• Doppler Ultrasound» DVT

• CT Pulmonary Angiography May identify PE

HIT nursing management and treatment

• Immediately discontinue all heparin products

• • Monitor platelet count and trend

• • Assess for signs of thrombosis: DVT (leg pain, swelling, redness), PE (chest pain, dyspnea, hypoxia), Stroke (neurological deficits), Limb ischemia (pain, pallor, cool extremity)

• Administer prescribed alternative anticoagulants - DONT GIVE WARFARIN UNTIL >150 (worsen coagulability and gangrene

• Avoid future heparin exposure; document heparin allergy/HIT history •

• Monitor for improvement in platelet count after heparin is stopped •

• Educate patient to report symptoms of thrombosis

DIC general

• Acquired disorder characterized by widespread activation of the coagulation system

• Causes microvascular fibrin clots to form throughout the body, reducing blood flow and potentially leading to organ ischemia and organ failure •

• Ongoing clot formation consumes platelets and clotting factors, resulting in a paradoxical risk of severe bleeding ("consumptive coagulopathy") •

• Can occur as an acute, life-threatening emergency or a more chronic, low-grade process

• Always occurs secondary to an underlying condition that activates the clotting cascade Common triggers: Sepsis, severe trauma, major surgery, burns, malignancy, pregnancy complications, and transfusion reactions.

CLOTTING BEFORE BLEEDING - PLT CONSUMPTION

DIC patho

• Underlying trigger(e.g., sepsis, trauma, malignancy, obstetric complications) causes excessive tissue factor (TF)release/exposure

• TF activates Factor VII, leading to thrombin generation and widespread fibrin clot formation

• Natural anticoagulant systems (antithrombin, Protein C, TF pathway inhibitor) become overwhelmed or impaired •

• Microvascular fibrin clots obstruct blood flow, causing tissue ischemia, organ dysfunction, and potential organ failure •

• Platelets and clotting factors are rapidly consumed (consumptive coagulopathy) •

• Fibrinolysis is activated, producing fibrin degradation products (FDPs), which further impair clot formation

• Result is the hallmark of DIC: simultaneous thrombosis and bleeding

• Key mechanism:Excessive tissue factor exposure → widespread clot formation →consumption of platelets/clotting factors →bleeding + organ damage

DIC HTT + critical

• Integ » widespread hemorrhage and lesions, OOZING FROM PUNCTURE SITES, gangrene (excess clotting factors)

• CNS » subarachnoid hemorrhage, changes in LOC

• MSK » DVT and limb ischemia

• GI » GI bleed, abdo distension, malaise, weakness,

• Resp » ARDS, pulmonary infection, cyanosis tachypnea hypoxemia (blood unable to pass through veins and arteries)

• Renal » hematuria oliguria renal failure

Critical

• Massive or uncontrolled bleeding

• Signs of shock (hypotension, tachycardia, poor perfusion)

• Altered level of consciousness or sudden neurologic changes

• Intracranial hemorrhage

• Significant GI bleeding or hematuria

• Acute respiratory distress / hypoxemia

• Oliguria or anuria (acute kidney injury)

• Evidence of multiple organ dysfunction/failure

DIC diagnostics + pharm

• Platelet Count » Platelets (thrombocytopenia)

• PT/INR, aPTT ↓Prolonged

• D-dimer/FDPs Elevated - prescence of clots

• Fibrinogen » Decreased

• Clinical Assessment

Pharmacology Review: Evidence of bleeding and/or thrombosis

Heparin: May be used in selected cases where thrombosis predominates •

Antifibrinolytics (rare): May be considered for life threatening bleeding not controlled with replacement therapy

DIC management and treatment

• Treat the underlying cause (e.g., sepsis, trauma, malignancy) •

• Monitor for bleeding, thrombosis, and signs of organ dysfunction

• Perform frequent assessment of vital signs, level of consciousness (LOC), respiratory status/oxygenation, urine output, and tissue perfusion •

• Monitor trends in platelet count, PT/INR, aPTT, fibrinogen, and D-dimer levels

• Maintain adequate circulating volume with IV fluids

• Support oxygenation and organ perfusion

• Administer blood products as prescribed: » platelets • Fresh Frozen Plasma (FFP) • Cryoprecipitate

heparin

• MOA » inhibits thrombin (activates fibrinogen→fibrin) and factor Xa

PREVENTS CLOT FROM GETTING BIGGER

• indication » ACUTE DVT PE, ACS, bridge to DOAC

• subQ (PREVENTATIVE) or IV infusion (ACUTE) ONLY

• monitor aPPT

• antidote is protamine sulfate (fully reverses)

Adverse Effects:

•Bleeding//hemorrhage

•Heparin-induced thrombocytopenia (HIT)

•Injection site irritation

•Osteoporosis (long-term use)

Contraindications:

•Active bleeding

•History of HIT

•Severe thrombocytopenia

warfarin

• MOA » inhibits vit K clotting synthesis factor

Vit K is used to create clotting factors needed to make stable clots

• indication» reduce risk of embolism post MI, treat and prevention of clotting disorders (DVT PE) and complications from afib or valve replacement

• REQUIRES INR MONITORING

• long term anticoag

• adverse effects » can cause major or fatal bleeding, skin necrosis

• contraindications» pregnancy, active bleeding, low compliance

• Bioavailability» near 100% oral bioavailability

• onset» Initial anticoagulant effect on INR may be seen as soon as 24 to 72 hour

• peak» plasma: 90 minutes to 4 hours.~4 hours

• duration» 2 to 5 days

• half life » 20 to 60 hours; Mean: 40 hours

• Route » oral - qd same time

Pharmokin

• absorption» : Rapid, complete

• distribution» 0.14 L/kg

  Protein binding: 99%

• metabolism» Hepatic

• excretion» Urine (92%, primarily as metabolites; minimal as unchanged drug)

• Pt teaching » limit Foods high in vitamin K (eg, leafy green vegetables) inhibit anticoagulant effect, along with alc consumption

• adverse affects » skin necrosis, decreased bone mineral density, microemboli vessel occlusion or emboloisation

Coag 3 (what do they do)

• PT/INR » ↑ WARFARIN, liver disease (EXTRINSIC PATH)

• aPTT » ↑ in HEPARIN, hemophilla (INTRINSIC PATH)

• D-Dimer » fibrin degradation, ↑ in DVT PE

'

PT 11-12.5 sec Increased with warfarin therapy
aPTT 25-40 sec Monitors heparin therapy
D-dimer <3.0 nmol/L DVT, PE, DIC

• BOTH ARE MARKERS OF TIME IT TAKES BLOOD TO CLOT

• D-DIMER IS PRESCENCE OF CLOTS IN BLOOD
  
  

intrinsic vs extrinsic pathway

intrinsic

• Activated by damage within the vessel or exposure to collagen

• Uses Factors XII, XI, IX, VIII

• Slower pathway; amplifies clot formation

• Assessed by aPTT

• Defective in Hemophilia A (Factor VIII deficiency)

• Defective in Hemophilia B (Factor IX deficiency)

extrinsic

• Activated by tissue injury and release of Tissue Factor (Factor III)

• Uses Factor VII Faster pathway

• initiates clot formation

• Assessed by PT/INR

• Major initiating pathway in DIC

• Rapidly activates the coagulation cascade

clotting factors review

1. fibrinogen » activated by thrombin and turns to fibrin (netting)

2. Prothrombin » activated by xx and helps activate Factor I

3. Tissue factor » sparks clotting cascade from extrinsic side, found outside the circ system in the tissues

5. joins factor X_a to activate Factor II to prothrombin

7. joins tissue factor to start extrinsic pathway and clotting cascade

8. antihemophiliic factor HELPER FACTOR - binds to IX - together they activate X_a . deficient in hemophilia A

9. christmas factor binds with VIII, together they activate X_a . deficient in hemophilia B

10. Starts common//shared pathway, changes II to thrombin

13. helps stabilize fibrin mesh

von wilibrand’s » stabilizes factor VIII as it goes to join IX

von willebrand disease - general

• Most common inherited bleeding disorder

• Caused by a deficiency or dysfunction of von Willebrand factor (vWF) •

• vWFis required for platelet adhesion and helps protect Factor VIII from breakdown

• Affects both males and females

• Typically presents with mucosal bleeding, such as epistaxis, easy bruising, heavy menstrual bleeding, and prolonged bleeding after procedures

hemophillia general

• Inherited hemorrhagic disorder caused by deficiencies of specific coagulation factors, resulting in impaired blood clot formation

• Hemophilia A: Deficiency of Factor VIII (most common form)

• Hemophilia B: Deficiency of Factor IX

• Both are X-linked recessive disorders, primarily affecting males

• Characterized by prolonged bleeding and an increased risk of spontaneous or excessive bleeding following injury or procedures

Severity depends on the amount of functional clotting factor present

• Severe: <1% factor activity

• Moderate: 1-5% factor activity

• Mild: >5-40% factor activity

hemophilia patho

• Inherited X-linked recessive disorders caused by mutations in the genes responsible for producing clotting factors VIII (Hemophilia A) or IX (Hemophilia B)

• Deficient or dysfunctional Factor VIII or Factor IX impairs the intrinsic coagulation pathway

• Reduced activation of Factor X leads to decreased thrombin generation and impaired fibrin clot formation

• As a result, bleeding is prolonged, and even minor injury can lead to excessive hemorrhage

hemophilia symp expected vs critical

expected

Ecchymosis (bruising) • Prolonged bleeding after injury/procedures • Hemarthrosis (joint bleeding) • Joint pain, swelling, and decreased ROM

critical

• • Intracranial hemorrhage • Internal bleeding • Compartment syndrome/neurovascular compromise • Hypovolemic shock • Muscle hematomas

hemophilia diagnostics + pharm

• Coagulation Studies » ↑ aPTT (PTT), normal PT/INR

• Factor assays » ↓ Factor VIII (Hemophilia A) or ↓ Factor IX (Hemophilia B)

• History & Assessment » Personal/family history of prolonged bleeding, bruising, or hemarthrosis

• Genetic Testing Pharmacology Review

Treat with

• Recombinant Factor VIII replacement (Hemophilia A)

• • Recombinant Factor IX replacement (Hemophilia B)

hemophilia nursing management

• Prevent injury and minimize risk of bleeding

• Assess for bleeding episodes, hemarthrosis, pain, swelling, and neurovascular compromise

• Monitor for signs of internal or intracranial bleeding

• Apply pressure to bleeding sites and avoid unnecessary invasive procedures when possible

• Promote joint protection and mobility; refer to physiotherapy as appropriate

• Educate patients/families on bleeding precautions and when to seek emergency care