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Hematologic Emergencies.2
Hematologic Emergencies.2
Hematologic Emergencies.2
Hematologic Emergency
❖ Outline Of Lecturer
1. Abnormalities of haemostasis
5. Acute Leukemia
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Haemostasis
This is the process of clot formation in the walls of damaged blood vessels to prevent
blood loss, whilst, at the same time, maintaining blood in a fluid state within the
vascular system.
Blood disorders occur when there is an imbalance in haemostasis.
1. If blood becomes too thin, it loses the ability to form the blood clots that stop
bleeding.
2. If blood becomes too thick, the risk of blood clots developing within the blood
vessels I, creating a potentially life-threatening condition such as Disseminated
intravascular coagulation DIC
Abnormalities of haemostasis
▪ People with bleeding disorders bleed easily and may lose excessive amounts of blood as a
result of injuries, surgery, or dental surgery.
▪ Severe bleeding disorders may even cause spontaneous bleeding without any injury.
▪ Injury to a blood vessel initiates a series of events that result in the formation of a clot
(hemostasis).
Pathophysiology of Abnormalities of haemostasis
▪ Abnormalities of hemostasis disrupt the body's ability to regulate blood clotting.
▪ Defects in platelets, coagulation factors, or blood vessels can contribute to these
abnormalities.
Types of Abnormalities
1. Bleeding Disorders:
✓ Hemophilia: Inherited deficiency of clotting factors, leading to prolonged bleeding.
✓ von Willebrand disease: Deficiency or dysfunction of von Willebrand factor, affecting
platelet function and clotting.
2. Clotting Disorders:
1. Deep Vein Thrombosis (DVT): Formation of blood clots in deep veins, commonly in
the legs.
2. Pulmonary Embolism (PE): Blockage of pulmonary arteries by blood clots originating
from DVT or other sources.
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3. Disseminated Intravascular Coagulation (DIC): Widespread activation of clotting
factors, causing excessive clotting and subsequent bleeding.
Clinical Features Abnormalities of haemostasis
✓ Bleeding Disorders: Easy bruising, prolonged bleeding after injury or surgery, excessive
bleeding from minor cuts, nosebleeds, heavy menstrual bleeding.
✓ Clotting Disorders: Pain, swelling, redness in the affected area (DVT), sudden shortness
of breath, chest pain, coughing up blood (PE), signs of organ dysfunction (DIC).
Diagnosis Abnormalities of haemostasis
1. Obtain medical history and conduct a physical examination.
2. Perform laboratory tests like Complete Blood Count (CBC), coagulation profile (PT,
aPTT, INR), von Willebrand factor assay, specific factor assays, D-dimer test, etc.
3. Utilize imaging studies such as ultrasound, CT scan, or MRI to diagnose DVT or PE.
Prognosis Abnormalities of haemostasis
Prognosis varies based on the specific disorder and its severity.
With proper management, many patients can lead relatively normal lives, while severe
cases may require ongoing medical intervention and monitoring.
Management Abnormalities of haemostasis
1. Bleeding Disorders:Hemophilia:
1. Replacement therapy with deficient clotting factors, such as factor VIII for Hemophilia A
or factor IX for Hemophilia B.
2. Regular prophylactic infusions of clotting factors to prevent bleeding episodes.
3. von Willebrand disease:
4. Desmopressin (DDAVP) administration to release stored von Willebrand factor and
improve platelet function.
5. Replacement therapy with von Willebrand factor concentrates if desmopressin is
ineffective.
2. Clotting Disorders:
- Deep Vein Thrombosis (DVT) and Pulmonary Embolism (PE):
✓ Anticoagulant therapy with medications such as heparin or low molecular weight
heparin initially, followed by oral anticoagulants like warfarin or direct oral
anticoagulants (DOACs).
✓ Thrombolytic therapy may be considered in severe cases.
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- Disseminated Intravascular Coagulation (DIC):
✓ Address and treat the underlying cause.
✓ Transfusion of blood products (platelets, fresh frozen plasma, cryoprecipitate) to correct
coagulation abnormalities.
✓ Supportive care for organ dysfunction and bleeding complications.- Surgical
Management:
✓ Surgical interventions may be necessary, such as removing blood clots or repairing
damaged blood vessels.
- Nursing Management:
1. Assessment:
✓ Perform a comprehensive assessment to identify the specific bleeding or clotting
disorder, its severity, and any associated symptoms or complications.
✓ Monitor vital signs, including blood pressure, heart rate, and respiratory rate.
✓ Assess for signs of bleeding, such as petechiae, purpura, ecchymosis, or excessive
bleeding from wounds or mucous membranes.
✓ Assess for signs of clotting, such as swelling, redness, warmth, or pain in the affected
area.
2. Medication Administration:
✓ Administer prescribed medications as ordered, such as clotting factor replacements,
anticoagulants, or thrombolytics.
✓ Monitor the effectiveness of medications and document any adverse reactions or
complications.
✓ Educate the patient and their family about the purpose, dosage, and potential side effects
of prescribed medications.
3. Monitoring and Interventions:
✓ Monitor laboratory values, including coagulation profiles (PT, aPTT, INR), platelet
counts, and fibrinogen levels.
✓ Assess and document the patient's response to treatment, including any improvement or
worsening of symptoms.
✓ Implement measures to prevent bleeding or injury, such as providing a safe environment,
using gentle handling techniques, and ensuring the patient receives appropriate protective
equipment.
✓ Apply pressure or use appropriate measures to control bleeding if necessary.
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✓ Monitor for signs of complications, such as excessive bleeding, hematoma formation, or
signs of thrombosis.
4. Education and Support:
✓ Provide patient and family education on the specific bleeding or clotting disorder,
including its causes, treatment options, and potential complications.
✓ Teach the patient about self-care measures, such as managing bleeding episodes,
administering medications, and recognizing signs of bleeding or clotting.
✓ Promote adherence to the treatment plan, including regular follow-up visits and
laboratory monitoring.
✓ Offer emotional support and counseling to help the patient cope with the challenges of
living with a bleeding or clotting disorder.
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Disseminated Intravascular Coagulation (DIC)
DIC is a condition where the coagulation and fibrinolytic systems are activated, leading to clot
formation and breakdown. It can be caused by infections, malignancies, trauma, liver disease,
or environmental factors. DIC can be acute and life-threatening or chronic and compensated.
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Differential Diagnosis DIC:
Differential diagnosis aims to exclude other conditions that may cause similar symptoms, such
as hypovolemic shock, decreased platelet count, and clotting disorder-related skin diseases.
Treatment for DIC usually involves several aspects, including:
1. Addressing the underlying cause or triggering factor, such as treating infections or
managing complications.
2. Replacing clotting factors and platelets through transfusions.
3. Administering anticoagulant medications to prevent further clot formation.
4. Supportive care measures, such as maintaining fluid and electrolyte balance and
managing organ dysfunction.
DIC can cause serious complications, including:
1. Organ failure: Different organs in the body may be affected due to the formation of
blood clots in the blood vessels.
2. Severe bleeding: Excessive bleeding may occur in the body due to the depletion of
clotting factors in the blood.
3. Blood clots: Blood clots may form in small and large blood vessels in the body.
DIC Prevention
It is difficult to completely prevent DIC, but some measures can be taken to reduce the
risk, such as:
1. Maintaining good health by avoiding infections and following a healthy lifestyle.
2. Avoiding severe injuries and exposure to extreme trauma.
3. Taking medications cautiously according to medical guidance.
DIC Follow-up:
After treatment and improvement of DIC, regular medical follow-up may be necessary to
ensure the patient's stability and recovery.
Nursing diagnosis and intervention for DIC may involve:
1. Impaired Tissue Perfusion related to microvascular thrombosis:
▪ Monitor vital signs frequently and assess tissue perfusion.
▪ Administer prescribed blood products, such as fresh frozen plasma, platelets, or
cryoprecipitate, to support clotting factor levels.
▪ Position the patient to promote optimal tissue perfusion.
▪ Monitor for signs of bleeding and promptly report any changes.
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2. Risk for Bleeding related to consumption of clotting factors and platelets:
▪ Implement bleeding precautions, such as using a soft-bristle toothbrush and avoiding
invasive procedures if not necessary.
▪ Monitor laboratory values, including platelet count, fibrinogen levels, and coagulation
profile, to detect any changes.
▪ Administer prescribed anticoagulant therapy carefully, considering the balance
between preventing clotting and avoiding bleeding complications.
▪ Educate the patient and family about signs and symptoms of bleeding, emphasizing
the importance of reporting any unusual bleeding or bruising.
3. Acute Pain related to tissue ischemia and microvascular thrombosis:
▪ Assess pain level regularly using a pain scale and document the findings.
▪ Administer prescribed pain medications, such as analgesics or anticoagulants, as
appropriate.
▪ Provide comfort measures, such as positioning, warm compresses, or relaxation
techniques, to alleviate pain.
▪ Educate the patient about pain management strategies and encourage the use of non-
pharmacological pain relief methods.
4. Risk for Infection related to invasive procedures and immunosuppression:
▪ Implement strict infection control measures, such as hand hygiene, proper use of personal
protective equipment, and aseptic techniques during procedures.
▪ Monitor for signs and symptoms of infection, such as fever, increased white blood cell
count, or localized redness and swelling.
▪ Administer prescribed prophylactic antibiotics as ordered.
▪ Educate the patient and family about the importance of infection prevention strategies
and the need for prompt reporting of any signs of infection.
5. Impaired Gas Exchange related to microvascular thrombosis and tissue ischemia:
▪ Monitor respiratory status closely, including oxygen saturation levels and
respiratory rate.
▪ Administer supplemental oxygen as prescribed to maintain adequate oxygenation.
▪ Encourage deep breathing exercises and frequent position changes to optimize lung
function.
▪ Provide emotional support and reassurance to reduce anxiety, which can affect
respiratory function.
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Immune Thrombocytopenic Purpura
Immune Thrombocytopenic Purpura (ITP): ITP is an autoimmune disorder where the immune
system mistakenly destroys platelets, leading to a decrease in their count. This can cause
excessive bleeding and bruising.
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Acute Leukemia
Acute Leukemia: Acute leukemia is a type of cancer that affects the blood and bone marrow. It
leads to the rapid production of abnormal white blood cells, inhibiting the production of healthy
blood cells.
Pathophysiology of Acute Leukemia:
▪ Acute leukemia is a type of cancer that affects the blood and bone marrow, causing
abnormal production of immature white blood cells.
▪ It results from genetic mutations in the stem cells of the bone marrow, leading to
uncontrolled growth and accumulation of immature cells (blasts).
▪ These abnormal cells interfere with the production of healthy blood cells, leading
to symptoms and complications.
Types of Acute Leukemia:
1. Acute lymphoblastic leukemia (ALL): Most common in children, characterized by the
overproduction of immature lymphoid cells.
2. Acute myeloid leukemia (AML): More common in adults, characterized by the
overproduction of immature myeloid cells.
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Thrombotic Thrombocytopenic Purpura (TTP)
Thrombotic Thrombocytopenic Purpura (TTP): TTP is a rare blood disorder characterized by
the formation of blood clots in small blood vessels throughout the body. This can lead to organ
damage and low platelet counts.
Pathophysiology Thrombotic Thrombocytopenic Purpura (TTP)
1. TTP is a rare disorder characterized by the formation of blood clots in small blood
vessels, leading to tissue damage.
2. It often occurs due to a deficiency in the ADAMTS13 protein, which helps break down
excessive von Willebrand factor (vWF) in the blood.
Types Thrombotic Thrombocytopenic Purpura (TTP)
- TTP can be divided into two main types:
1. Hereditary TTP
2. Acquired TTP.
Clinical features Thrombotic Thrombocytopenic Purpura (TTP)
▪ Common signs and symptoms of TTP include low platelet count
(thrombocytopenia), low red blood cell count (anemia), and enlarged spleen
(splenomegaly).
▪ Evidence of microvascular clotting can appear throughout the body, including the
skin (purpura) and internal organs such as the kidneys and brain.
Diagnosis Thrombotic Thrombocytopenic Purpura (TTP)
- Diagnosis involves blood tests to determine platelet count, kidney function, and other
evaluations of body functions. A detailed diagnosis usually requires a skin biopsy and
examination.
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✓ Coordinate with the healthcare team to develop a discharge plan that includes
follow-up appointments, medication management, and ongoing support.
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Acute Hemolytic Anemia
▪ This condition involves the rapid destruction of red blood cells, leading to a sudden
drop in hemoglobin levels. It can be caused by various factors, such as incompatible
blood transfusions or autoimmune reactions
▪ Anemia is a condition that causes a rapid decrease in the hemoglobin levels in the
blood.
▪ Anemia can result from factors such as iron deficiency, hemoglobin disorders, or folate
deficiency.
▪ It can lead to the destruction of red blood cells due to inadequate nutrition or immune
reactions.0
▪ Severe cases of anemia can result in frequent blood transfusions.
▪ Anemia should be diagnosed and treated by specialized medical professionals.
Normal Red Blood Cell (RBC) Values for Adults. A
Measure Male Female
RBC count (million/μL) 4.5–6.0 4.0–5.5
Hemoglobin (g/dL) 14–17 12–15
Hematocrit (%) 42–52 36–48
Mean corpuscular volume (FL) 78–100 78–102
Mean cellular hemoglobin (pg./cell) 25–35 25–35
Mean corpuscular hemoglobin concentration (g/dL) 32–36 32–36
Red cell distribution width (%) 11.5–14.5 11.5–14.5
Reticulocyte count (%) 0.5–2.5 0.5–2.5
Classification of Anemias.
Mean Mean Corpuscular
Type Corpuscular Hemoglobin Cause Other Hematologic Findings
Volume (fL) Concentration (g/dL)
Low reticulocytes, low serum ferritin and iron,
<80 <32 Iron deficiency high total iron-binding capacity
Macrocytic >101 (usually Vitamin B12 or Macro elliptocytes, hyper segmented neutrophils
>36
>130) folate deficiency
>101–120 >36 Liver disease Decreased platelets
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Acute Hemolytic Anemia
Acute Hemolytic Anemia is a condition characterized by the rapid destruction of red
blood cells, leading to a sudden decrease in overall blood count. This occurs due to an
immune reaction between antibodies and red blood cells, resulting in their breakdown
and destruction.
1. Immune Hemolytic Anemia:- This occurs when the immune system of the recipient
recognizes transfused red blood cells as foreign and targets them for destruction.
2. Drug-induced Hemolytic Anemia: -Certain medications can cause immune reactions
that lead to the destruction of red blood cells.
3. Genetic Factors: Some inherited genetic conditions can result in enzyme deficiencies in
red blood cells, making them more susceptible to hemolytic destruction.
1. Complete Blood Count (CBC) to evaluate red blood cell count, white blood cells, and
platelets.
2. Urine analysis to detect abnormal color changes.
3. Advanced blood tests (electrophoresis, genetic analysis) to diagnose genetic hemolytic
anemias.
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4. Eye examination (retinal examination) to identify changes in blood vessels.
Nursing Interventions:
- Monitor vital signs regularly.
- Assess and document the patient's symptoms and response to treatment.
- Administer prescribed medications and transfusions.
- Provide emotional support and education to the patient and their family.
- Monitor for potential complications and report any abnormalities to the healthcare
team.
~Waheed Abkr, [8/31/2023 1:05 AM]
2. Abnormalities of haemostasis Pathophysiology, types, Clinical
Acute hemolytic anemia is a condition characterized by the rapid destruction of red blood
cells (hemolysis), leading to a decrease in the number of circulating red blood cells.
Here's a breakdown of the key points related to the topic:
✓ Acute hemolytic anemia can be classified based on the underlying cause and the
specific type of hemolysis (intravascular or extravascular).
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✓ Clinical features may include symptoms of anemia, such as fatigue, weakness,
pallor, and shortness of breath.
✓ Other possible signs and symptoms include jaundice, dark urine (due to increased
bilirubin), enlarged spleen, and, in severe cases, signs of organ dysfunction or
failure.
Management:
a. Medical Management:
✓ Treatment aims to address the underlying cause, stop hemolysis, and manage
symptoms.
✓ Specific interventions may include discontinuing medications or addressing
infections, autoimmune disorders, or transfusion reactions.
✓ In severe cases, blood transfusions or the administration of immunosuppressive
medications may be required.
b. Surgical Management:
✓ Surgery is typically not the primary treatment for acute hemolytic anemia unless it is
necessary to address an underlying cause, such as removing an enlarged spleen in
certain cases.
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c. Nursing Management Acute Hemolytic Anemia
✓ Monitor vital signs, oxygen saturation, and assess for signs of worsening anemia or
organ dysfunction.
✓ Administer prescribed medications, including immunosuppressive agents or iron
supplementation.
✓ Implement appropriate infection control measures if the cause is infectious.
✓ Educate the patient and their family about the condition, treatment plan, signs
of
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Chronic Hemolytic Anemia
Chronic Hemolytic Anemia is a blood disorder characterized by the continuous
destruction of red blood cells, either due to their premature breakdown or faster
destruction in the bloodstream than their replacement.
1. Genetic factors: The most c0ommon cause is a genetic mutation that affects the blood's
hemoglobin.
2. Immune disorders: Immune disorders can lead to the destruction of red blood cells.
3. Splenic disorders: The spleen can cause the destruction of red blood cells due to
excessive sequestration.
Risk factors:
Diagnosis:
Management:
Medical Administration:
1. Blood transfusion: In cases of severe anemia, blood transfusions may be needed
to increase red blood cell count and relieve symptoms.
2. Medications: A variety of medications may be prescribed depending on the
underlying cause of symptom management or targeting the erythrolysin
mechanism (e.g. corticosteroids, immunosuppressive drugs).
3. Folic acid supplements: Folic acid supplements may be recommended to support
red blood cell production.
a. Hydroxyurea: This drug is commonly used to manage diabolic anemia by
reducing the frequency and severity of pain crises.
b. Fat treatment: For people with chronic anemia who need frequent blood
transfusions, grease therapy can be used to reduce iron buildup.
Surgical Management:
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- Respiratory failure.
- Liver fibrosis.
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Sickle cell anemia
Sickle cell anemia is a genetic blood disorder that affects the shape and function of red
blood cells. It is caused by a mutation in the gene responsible for producing hemoglobin,
a protein that carries oxygen in the blood. This mutation leads to the production of
abnormal hemoglobin called hemoglobin S, which causes red blood cells to become rigid
and take on a sickle shape
Causes:
Sickle cell anemia is an inherited disorder caused by a mutation in the gene responsible for
hemoglobin production. This mutation leads to the production of abnormal hemoglobin called
hemoglobin S. When oxygen levels decrease or there is increased stress on the red blood cells,
hemoglobin S causes the red blood cells to become rigid and assume a sickle shape.
Pathophysiology:
The sickle-shaped red blood cells are prone to getting trapped in small blood vessels, leading to
vaso-occlusive crises. This can cause tissue ischemia, pain, and organ damage. Additionally, the
lifespan of sickle cells is shorter than normal red blood cells, leading to chronic anemia.
Types:
There are different types of sickle cell disease, including sickle cell anemia (HbSS), sickle cell
trait (HbAS), and various other genotypes such as HbSC and HbS beta thalassemia. Sickle cell
anemia (HbSS) is the most severe form.
Clinical Features:
Clinical features of sickle cell anemia include:
1. Anemia-related symptoms: Fatigue, weakness, and pallor.
2. Vaso-occlusive crises: Severe pain, usually in the bones, joints, and abdomen.
3. Acute chest syndrome: Chest pain, cough, and difficulty breathing due to pulmonary
complications.
4. Splenic sequestration crisis: Sudden enlargement of the spleen, leading to abdominal pain
and anemia.
5. Stroke: Due to the occlusion of blood vessels in the brain.
6. Gallstones: Caused by the breakdown of red blood cells.
7. Delayed growth and development in children.
8. Leg ulcers: Chronic non-healing ulcers on the lower legs.
9. Priapism: Prolonged and painful erections in males.
Diagnosis:
Diagnosis of sickle cell anemia involves:
a. Hemoglobin electrophoresis: Determines the presence of abnormal hemoglobin S.
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b. Complete blood count (CBC): Reveals anemia and other blood abnormalities.
c. Sickle cell solubility test: Detects the presence of sickle hemoglobin.
d. Genetic testing: Confirms the presence of the sickle cell gene.
Prognosis:
The prognosis of sickle cell anemia varies. While some individuals experience frequent
complications and have a shortened lifespan, others may have milder symptoms and lead
relatively normal lives. Early diagnosis, regular medical care, and proper management can
improve outcomes.
Management:
The management of sickle cell anemia includes:
1. Medical Management:
- Pain management: Analgesics and, in severe cases, opioids.
- Hydroxyurea: Reduces the frequency of painful crises.
- Blood transfusions: To manage severe anemia and prevent complications.
- Antibiotics and vaccinations: To prevent infections.
2. Surgical Management:
- Splenectomy: In some cases of recurrent splenic sequestration crises.
- Cholecystectomy: For gallstone-related complications.
3. Nursing Management:
- Pain assessment and management.
- Hydration and electrolyte balance.
- Monitoring for complications and providing supportive care.
- Education on self-care, disease management, and genetic counseling.
- Psychosocial support for patients and families.
3. Bleeding management
✓ Apply direct pressure to the bleeding site, elevate the affected limb if applicable, and
consider the use of hemostatic agents or tourniquets if necessary.
✓ Administer blood products (such as packed red blood cells, platelets, or fresh frozen
plasma) as ordered by the healthcare provider.
4. Fluid resuscitation:
✓ Administer intravenous fluids as prescribed to maintain adequate circulating volume and
prevent hypovolemia.
✓ Monitor fluid balance closely and assess for signs of fluid overload.
5. Pain management: Hematological emergencies, such as sickle cell crisis or acute leukemia,
can be associated with severe pain.
✓ Administer appropriate analgesics as ordered and reassess pain levels regularly.
✓ Provide comfort measures, such as positioning, heat packs, or distraction techniques, to
help alleviate pain.
1. Monitoring and laboratory tests:
✓ Continuously monitor the patient's vital signs, oxygen saturation, and level of
consciousness.
✓ Perform frequent laboratory tests, including complete blood counts, coagulation profiles,
and other relevant tests, to assess the patient's hematological status and response to
treatment.
2. Psychosocial support: Hematological emergencies can be emotionally and psychologically
challenging for patients and their families.
✓ Provide emotional support, education, and clear communication to help alleviate
anxiety and promote coping strategies.