Hematologic Disorders
Transcript of Hematologic Disorders
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Management of Clients with Hematologic
DisordersAlapan, NikkiBermudez, Igi
Buenaventura, HayzelGomez, Lyle
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Hematologic Disorders
1.Disorders affecting Red Blood Cells2.Disorders affecting White Blood Cells3.Disorders of Platelets and Clotting Factors
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Disorders Affecting Red Blood Cells
1. AnemiaA. Anemia caused by Decreased Eryt
hrocyte ProductionB. Anemia caused by Increased Eryth
rocyte Production
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Anemia• Anemia - a clinical condition that
results from an insufficient supply of healthy red blood cells (RBCs), the volume of packed RBCs, and/or the quantity of hemoglobin.
• Hypoxia - results when the body’s tissues are not adequately oxygenated.
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Etiologic Categories of Anemia
1. Decreased RBC Production2.
Increased RBC Desruction (Hemolysis)
3. Blood Loss
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Decreased RBC Production Defective DNA Synthesis
Cobalamin/Vitamin B12 Deficiency Folic Acid Deficiency
Decreased Hemoglobin Synthesis Iron Deficiency Thalassemia (Decreased Globin Synthesis) Sideroblastic Anemia (Failure To Completely Form Heme)
Decreased Number of Erythrocyte Precursors Aplastic Anemia Anemia of Leukumia and Myelodysplasia Chronic Diseases
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Increased RBC DestructionIntrinsic
Abnormal Hemoglobin (Sickle Cell Anemia) Enzyme Deficiency Membrane Abnormalities
Extrinsic Physical Trauma Antibodies (Autoimmune and Isoimmune) Infectious Agents Toxins (Snake Venom; Chemotherapy)
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Blood Loss
Acute Trauma Blood Vessel Rupture
Chronic Gastritis Hemorrhiods Menstruation
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AnemiaPathophysiology
Transport of oxygen is impairedHemoglobin is lacking or the number of
RBCs is too low to carry adequate oxygen to tissues
Hypoxia developsIncreasing the rate of RBC production
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AnemiaPathophysiology
Increasing cardiac output by increasing stroke volume or heart rate
Redistributing blood from tissues of low oxygen needs to tissues with high oxygen needs
Shifting the oxygen-hemoglobin dissociation curve to the right
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Anemia caused by Decreased Erythrocyte Production
1. Iron Deficiency Anemia2. Thalassemia3. Megaloblastic Anemia
A. Pernicious Anemia or B12 DeficiencyB. Folic Acid Deficiency Anemia
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Iron Deficiency Anemia
chronic, hypochromic, microcytic anemia
results from insufficient supply of iron in the body
without iron, hemoglobin concentration in the RBCs is reduced
the cells are unable to oxygenate the body tissues adequately
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Iron Deficiency AnemiaPathophysiology
Iron is present is in all RBCs as heme in Hb Iron concentration in the body is regulated
by the absorptive cells in the proximal small intestines
Errors in this balance leads to anemia
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Iron Deficiency AnemiaDiagnostic Test
CBC Radiographic Studies Stool examination Esophagoscopy Gastroscopy Sigmoidoscopy
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Iron Deficiency AnemiaClinical Manifestation
MILD CASE OF IDA AsymptomaticSEVERE CASE OF IDA Fatigue Headache Dyspnea Palpitations
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Iron Deficiency AnemiaClinical Manifestation
SEVERE CASE OF IDA Pallor in the face, palm of hand,
nail bed and mucous membranes of the mouth and conjuctiva
Angular Stomatitis – inflammaton of the mucosa of the mouth
Glossititis – inflammation of the tongue
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Iron Deficiency AnemiaClinical Manifestation
SEVERE CASE OF IDA Cheilitis – inflammation of the lips
Brittle nails
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Iron Deficiency Anemia
Nursing Intervention Monitor the client to ensure
compliance in taking supplemental iron
Diets high in iron should e planned with the client and family members.
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Thalassemia an autosomal-recessive genetic disorder
results in inadequate normal Hb production
disrupts the synthesis of globin
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ThalassemiaPathophysiology
ALPHA-THALASSEMIA mutation of alpha-globin gene(s)THALASSEMIA MINOR 1 beta-globin gene is mutated minor disruptions in beta-globin
synthesis
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ThalassemiaPathophysiology
THALASSEMIA MAJOR mutation exists in both beta-genes significant impairment of beta-globin
synthesis marked reduction in hemoglobin production profound anemia
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ThalassemiaPathophysiology
HEMOLYSIS from an imbalance in the alpha- and beta-
globin chains which are normally paired excess unpaired alpha- or beta-globin chains
aggrevate and form a precipate damages RBC membranes intravascular hemolysis
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Thalassemias
Diagnostic Test CBC Amniocentesis
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ThalassemiaClinical Manifestation
ALPHA-THALASSEMIA AsymptomaticTHALASSEMIA MINORSymptoms of mild to moderate Anemia
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ThalassemiaClinical Manifestation
THALASSEMIA MAJOR pain failure to thrive diarrhea splenomegaly hepatomegaly Jaundice Bone marrow hyperplasia
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Thalassemia
Nursing Intervention Educate the patient about the disease
Give the patient a list of medications to avoid
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Megaloblastic Anemia
1. Pernicious Anemia or B12 Deficiency2. Folic Acid Deficiency Anemia
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Pernicious AnemiaCobalamin/B12 Deficiency
autoimmune disorder Absence of intrinsic factor in gastric secretions
malabsorption of Cobalamin
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Pernicious AnemiaB12 Deficiency
Diagnostic TestSchilling Test- patient receives small
oral dose of radioactive Vitamin B12 followed in a few hours by a large nonradioactive Vitamin B12 . If the Vitamin B12 is absorbed more than 8% will be excreted in the urine within 24 hours.
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Pernicious AnemiaB12 Deficiency
PathophysiologyFaulty absorption from the GI tractAbsence of Intrinsic FactorNo Vitamin B12 absorptionErythrocyte production is
eventually diminished
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Pernicious AnemiaB12 Deficiency
Clinical ManifestationClassic signs of anemiaNeurologic manifestations with
severe anemia (<7 g/dl) Vitamin B12 is necessary for the
synthesis of myelin Parasthesias of feet and fingers Ataxia Loss of position and vibration sense Spasticity
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Pernicious AnemiaB12 Deficiency
Clinical ManifestationSore tongue, beefy red (unknown cause)Hyperbilirubinemia
Jaundice/icterus “Lemon yellow” skin – combination of
pallor and jaundiceHepatosplenomegaly – enlarged liver
and spleen
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Pernicious AnemiaB12 Deficiency
Nursing InterventionMonitor B12 levelsMonitor Hemogram and reticulocytesGive Folic acid with B12 for severe
anemiaFolic acid caution
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Folic Acid Deficiency Anemia
Folic acid deficiency anemia happens when your body does not get enough folic acid.
Folic acid is one of the B vitamins, and it helps your body make new cells, including new red blood cells
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Folic Acid Deficiency Anemia
Pathophysiology Malaabsorptive diseases of the small bowel, such as sprue, may not absorb folic acid normally
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Folic Acid Deficiency AnemiaDiagnostic Test
CBCClinical ManifestationsFolic acid levelsBone marrow aspiration and
analysis
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Folic Acid Deficiency AnemiaClinical Manifestation
Classic signs of anemiaNo neurologic manifestationsManifestations related to
malnourishment painful ulcerations of cheeks and tongue fissuring of lips and mouth
Gastrointestinal symptoms Dysphagia Flatulence Watery diarrhea
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Folic Acid Deficiency AnemiaNursing Intervention
– Monitor CBC and reticulocytes– If malabsorption is cause, diet alone will
not be sufficient– Oral is preferred
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Anemia caused by Increased Erythrocyte Production
1. Hemolytic Anemia2. Sickle Cell Disease3. Polycythemia Vera4. Hemochromatosis
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Hemolytic Anemia
An end result of condition that lead to hemolysis
Hemolysis- premature destruction of erythrocytes
Anemia occurs when the bone marrow fails to replace RBCs at the rate they are destroyed
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Hemolytic AnemiaDiagnostic Test
CBCPeripheral smearMorphologic examinationRBC indicesIncreased RBC distribution width (RDW)Reticulocyte count
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Hemolytic AnemiaDiagnostic Test
Lactic acid dehydrogenase levelSerum haptoglobin levelIndirect bilirubin level
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Hemolytic AnemiaClinical Manifestation
WeaknessFatigueHemochromatosis-Bronze skin
tone and diabetesHemoglobinuria – dark urineJaundiceElevation in Bilirubin levelsEnlargement of spleen and liver
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Sickle Cell Disease
Group of inherited disorders of mutant hemoglobin
Sickling of RBCsLow oxygenationAbnormally shaped RBCsSickle Cell Anemia –autosomal-
recessive disorderSickle Cell Trait – much milder form
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Sickle Cell Disease
PathophysiologyDefective hemoglobin exposed to a
decrease 02Decreased solubilitySickle Cell becomes rigid, sticky and fragileSickle cell agglutinate and impede
circulation in the capillariesMicroinfarction tissue hypoxia and further
sickling
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Sickle Cell DiseaseDiagnostic Test
Low HematocritSickled Cells on SmearHemoglobin Electrophoresis - a
blood test done to evaluate the different types of hemoglobin in the bloodstream.
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Sickle Cell DiseaseClinical Manifestation
Jaundice – is characteristic and is usually obvious in the sclerae
Enlargement of the face and skullTachycardiaCardiac murmursCardiomegaly – enlarged heartDysrhythmiasHeart Failure
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Sickle Cell DiseaseNursing Intervention
Manage PainPrevent/Manage InfectionPromote Coping SkillsMinimize Deficient Knowledge
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Polycythemia Vera
Primary polycythemiaProfilerative disorderMyeloid stem cells seem to have
escaped normal control mechanisms
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Polycythemia VeraPathophysiology
The bone marrow is hypercellular, and the erythrocyte, leukocyte, and plateple counts in the peripheral blood are elevated
Spleen resumes its embryonic function of hematopoiesis and enlarges
The bone marrow becomes fibrotic, with a resultant inability to produce as many cells
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Polycythemia VeraDiagnostic Test
CBCElevated erythrocyte massLeukocyte and Platelet countErythropoietin levelElevated hematocrit
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Polycythemia VeraClinical Manifestation
Ruddy complexionSplenomegalyIncreased Blood Volume
Headache Dizziness Tinnitus Fatigue Paresthesias Blurred Vision
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Polycythemia VeraClinical Manifestation
Increased Blood Viscosity Angina Claudication Dyspnea Thrombophlebitis
Elevated Blood pressure
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Polycythemia VeraNursing Intervention
The nurse’s role primarily that of educator. Risk factors for thrombotic complications Avoid tight and restrictive clothing Avoid crossing the legs Sedentary behavior should be encouraged Minimize alcoholism Proper Diet
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HemochromatosisIron overload diseaseInherited metabolic disorderIncreased absorption of iron that is
deposited in the body tissues and organs particularly the liver, heart and pancreas
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HemochromatosisDiagnostic Test
LaboratoryBlood TestLiver BiopsyTransferring Saturation Test – calculate how
much iron is bound to the protein that carries iron to the blood
Serum Ferratin Test – shows the level of iron in the liver
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HemochromatosisClinical Manifestation
Joint PainFatigueLack of energyIrritabilityDepressionLoss of body hairAbdominal painLose of sexual driveHeart Problems
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HemochromatosisNursing Intervention
Should not take Iron supplementsAvoid Alcoholic Beverages Dietary intake of iron is limited
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Disorders Affecting White Blood Cells
1. Agranulocytosis2. Multiple Myeloma3. Infectious Mononucleosis4. Splenic Rupture and Hypersplenism
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Agranulocytosis(granulocytopenia, malignant neutropenia) is an acute, potentially fatal blood
dyscrasiaprofound neutropenia (a reduced number
of circulating neutrophils) Because neutrophils make up roughly 93%
of all granulocytes, the terms neutrophenia and agranulocytosis are often used interchangeably.
Agranulocytosis is a fairly rare condition. For unknown reasons, females are much
more susceptible to this condition than males, although even among females, it’s relatively rare.
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AgranulocytosisPathophysiology
Failure to produce adequate numbers of WBC
prevents normal surveillance and phagocytosis;
infection from the neutropenia is a common sequel.
Neutrophils constitute a swift and powerful defense against invading microorganisms.
Consequently, decreases in their number result in a greater susceptibility to bacterial invation, especially when the client’s absolute neutrophil count drops below 500/mm3.
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AgranulocytosisDiagnostic Test
Complete blood count- leucopenia, evidenced by WBC counts of 500 to 3000/mm3 with extreme reduction in polymorphonuclear cells (0 to 2%)
Bone marrow examinationUrine culture, blood and ulcerative lesions
in the throat and mouth that are positive for bacteria, usually gram-positive cocci.
A history of exposure to an offending agent as well as all the aforementioned findings
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AgranulocytosisClinical Manifestation
Fatigue and weaknessSore throat Ulcerations of the pharyngeal and
buccal mucosa, DysphagiaHigh feverWeak and rapid pulse, and sever
chills
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Multiple Myelomaa B- cell neoplastic condition abnormal malignant proliferation
of plasma cells secreting a monoclonal paraprotein, accumulation of mature plasma cells in the bone marrow,
Risk factors include an increase incidence in some families, ionizing radiation and occupational chemical exposure.
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Multiple MyelomaPathophysiology
an abnormal proliferation of plasma cells.
With this overproduction of plasma cells, bone destruction also occurs.
disruption of RBC, leukocyte and platelet production, with result from plasma cells crowding the bone marrow.
Impaired production of these cells forms causes anemia, increased vulnerability to infection and bleeding tendencies, respectively.
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Multiple MyelomaDiagnostic Test
Radiographic Studies- reveal diffuse lesions in the bone, widespread demineralization and osteoporosis.
Urine examination- Bence- Jones protein in the urine, consisting of monoclonal immunoglobulin light chains.
Bone marrow biopsy- the bone marrow contains large numbers of immature plasma cells. Normally, plasma cells constitute 5% of the bone marrow cell population.
Blood examination- blood samples sent for plasma electrophoresis reveal a large amount of abnormal immunoglobulin.
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Multiple MyelomaClinical Manifestation
Back pain or bone pain that worsen with movement.
Pathologic fractures accompanied by severe pain.
Sternum and rib cage deformities.Diffuse osteoporosis.
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Multiple MyelomaNursing Intervention
Patients also need to be instructed about the signs and symptoms of hypercalcemia.
Maintaining mobility and hydration is important to diminish exacerbations.
Renal function should also be monitored closely.
Maintaining high urine output (3 L/day) can be very useful in preventing this complication.
Patients need to be educated about activity restrictions (e. g., lifting no more than 10 pounds, use of proper body mechanics).
Pain management. NSAIDs can be very useful for mild pain or in combination with opioid analgesics.
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Infectious MononucleosisInfectious mononucleosisglandular disease or the “kissing
disease”, a self- limiting condition characterized by painful enlargement of
the lymph nodes, lymphocytosis, sore throat and fever.
Primarily a disease of the young, it usually strikes children between the ages of 3 and 5 years and young adults between the ages of 15 and 25 years old.
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Infectious MononucleosisThe greatest incidence occurs among
college students, medical students and nurses.
Although this disease usually occurs sporadically, epidemic forms may sweep through colleges and children’s homes.
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Infectious MononucleosisPathophysiology
The cause of infectious mononucleosis is Epstein- Barr virus (EBV), a herpes virus.
mode of transmission remains unknown,
the disease may be transmitted through the oropharyngeal route during close contact, as with kissing.
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Infectious MononucleosisDiagnostic Test
Paul- Bannel testComplete Blood Count- WBC count
usually ranges from 12,000 to 20,000/mm3, of which 50% are lymphocytes and monocytes and 10% to 20% are large, atypical lymphocyte.
The mono spot test is also performed with a throat swab- it detects anti- EBV antibodies and is positive in 50% of cases within the first week and 90% of cases in the fourth week.
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Infectious MononucleosisClinical Manifestation
FatigueHeadachesMalaiseMyalgiasTemperature up to 39° C (102.2°F)Pharyngitis
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Infectious MononucleosisNursing Intervention
Bed rest is recommended until fever is resolved.
Acetaminophen, cool sponge bath, and a large fluid intake help control fever.
Warm saline throat irrigation may relieve the sore throat
Aspirin is avoided because of risk of Reye’s syndrome
Contact sports must be avoided to reduce the risk of splenic rupture.
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Splenic Rupture and Hypersplenism
The most frequent indication for splenectomy is rupture of the spleen complicated by severe hemorrhage. Causes of splenic rupture include: Trauma Accidental tearing of the splenic capsule during
surgery on neighboring organs. Disease of the spleen that causes softening or
damagehypersplenism, a second important
indication for splenectomy, the spleen destroys, in excessive numbers, one of the blood cell types
Primary hypersplenism occurs in idiopathic thrombocytopenic purpura and congenital spherocytosis.
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Splenic Rupture and Hypersplenism
Diagnostic TestLaboratory indication for
splenectomy include granulocytopenia of less than 500/mm3 and thrombocytopenia of less than 20,000/mm3.
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Splenic Rupture and Hypersplenism
Clinical ManifestationsModerate to massive splenomegalyAnemiaLeucopenia or thrombocytopeniaA compensatory increase in the
production of the affected cell line by the bone marrow.
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Disorders of Platelets and Clotting Factors
Disorders of hemostasis that affect platelets and clotting factors
1. Hemorrhagic Disorders2. Immune Thrombocytopenic Purpura3. Coagulation Disorders4. Disseminated Intravascular Coagulation5. Hemophilia
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Hemorrhagic DisordersNormal clot formation and lysis depend on 1.Intact blood vessels2.An adequate number of functioning
platelets3.Sufficient amounts of the 12 clotting
factors4.A well-controlled fibrinolytic system
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Hemorrhagic Disorders
Four basic problems underlying hemorrhagic disorders:
1.weak, damaged vessels2.Platelet deficiency result from
hypoprofileration, excessive pooling of platelet in spleen, excessive platelet destruction
3.Deficiency or total lack of one of the clotting factors
4.Excessive or insufficient fibrinolysis
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Immune Thrombocytopenic Purpura
Most commmon thrombocytopenic disorder
Idiopahitc thrombocytopenic purpuraCause was unknownHemorrhagic autoimmune diseaseDestruction of platelets
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Immune Thrombocytopenic PurpuraDiagnostic Test
A platelet count below 100,000/mmᵌProlonged bleeding time with normal
coagulation timeIncreased capillary fragility as
demonstrated by the torniquet testPositive platelet anitbody screeningBone marrow aspirate containing
normal or increased numbers of megakaryocytes
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Coagulation Disorders
defect in clotting mechanism one or more of the clotting factors is
depleted or absent
1.Disseminated intravascular coagulation
2.Hemophilia
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Disseminated Intravascular Coagulation
loss of balance between clotting and lysing systems
caused by the simultaneous presence of thrombin and plasmin
Fibrinolysis – too much plasmin Thrombosis – too much thrombinConsumptive Coagulopathy- clotting
factors are consumed to such an extent that generalized bleeding occurs
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Disseminated Intravascular Coagulation
PathophysiologyExcessive clotting can be
precipitated through intrinsic or extrinsic coagulation pathways
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Disseminated Intravascular Coagulation
Clinical ManifestationBleeding from mucous membranes
venipuncture sites and GI and urinary tracts.
Organ dysfunction Renal failure
Hemorrhages
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Disseminated Intravascular Coagulation
Nursing Intervention Monitor and manage potential
complications Target potiential sites of end-organ damage
• Kidneys• Lungs• Brain• Skin
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Hemophilia X-linked genetic disorderA deficiency of coagulation factorsHEMOPHILIA A Deficiency of Factor VIIIHEMOPHILIA B Christmas DiseaseDeficiency of factor IX
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HemophiliaAssessment
HemorrhageBleeding into joints
KneesElbowsAnklesShouldersWristHips
Ankylosis (fixation) of the joint
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HemophiliaNursing Intervention
Assist in coping Teach about activity restrictions Avoid any agents that interfere with platelet
aggregation Dental hygiene is important Nasal packing should be avoided All injections should be avoided