hematologic disorders

Management of Clients with Hematologic

DisordersAlapan, NikkiBermudez, Igi

Buenaventura, HayzelGomez, Lyle

Hematologic Disorders

1.Disorders affecting Red Blood Cells2.Disorders affecting White Blood Cells3.Disorders of Platelets and Clotting Factors

Disorders Affecting Red Blood Cells

1. AnemiaA. Anemia caused by Decreased Eryt

hrocyte ProductionB. Anemia caused by Increased Eryth

rocyte Production

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.

Etiologic Categories of Anemia

1. Decreased RBC Production2.

Increased RBC Desruction (Hemolysis)

3. Blood Loss

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

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)

Blood Loss

Acute Trauma Blood Vessel Rupture

Chronic Gastritis Hemorrhiods Menstruation

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

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

Anemia caused by Decreased Erythrocyte Production

1. Iron Deficiency Anemia2. Thalassemia3. Megaloblastic Anemia

A. Pernicious Anemia or B12 DeficiencyB. Folic Acid Deficiency Anemia

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

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

Iron Deficiency AnemiaDiagnostic Test

CBC Radiographic Studies Stool examination Esophagoscopy Gastroscopy Sigmoidoscopy

Iron Deficiency AnemiaClinical Manifestation

MILD CASE OF IDA AsymptomaticSEVERE CASE OF IDA Fatigue Headache Dyspnea Palpitations


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


SEVERE CASE OF IDA Cheilitis – inflammation of the lips

Brittle nails

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.

Thalassemia an autosomal-recessive genetic disorder

results in inadequate normal Hb production

disrupts the synthesis of globin

ThalassemiaPathophysiology

ALPHA-THALASSEMIA mutation of alpha-globin gene(s)THALASSEMIA MINOR 1 beta-globin gene is mutated minor disruptions in beta-globin

synthesis


THALASSEMIA MAJOR mutation exists in both beta-genes significant impairment of beta-globin

synthesis marked reduction in hemoglobin production profound anemia


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

Thalassemias

Diagnostic Test CBC Amniocentesis

ThalassemiaClinical Manifestation

ALPHA-THALASSEMIA AsymptomaticTHALASSEMIA MINORSymptoms of mild to moderate Anemia

ThalassemiaClinical Manifestation

THALASSEMIA MAJOR pain failure to thrive diarrhea splenomegaly hepatomegaly Jaundice Bone marrow hyperplasia

Thalassemia

Nursing Intervention Educate the patient about the disease

Give the patient a list of medications to avoid

Megaloblastic Anemia

1. Pernicious Anemia or B12 Deficiency2. Folic Acid Deficiency Anemia

Pernicious AnemiaCobalamin/B12 Deficiency

autoimmune disorder Absence of intrinsic factor in gastric secretions

malabsorption of Cobalamin

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.


PathophysiologyFaulty absorption from the GI tractAbsence of Intrinsic FactorNo Vitamin B12 absorptionErythrocyte production is

eventually diminished


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


Clinical ManifestationSore tongue, beefy red (unknown cause)Hyperbilirubinemia

Jaundice/icterus “Lemon yellow” skin – combination of

pallor and jaundiceHepatosplenomegaly – enlarged liver

and spleen


Nursing InterventionMonitor B12 levelsMonitor Hemogram and reticulocytesGive Folic acid with B12 for severe

anemiaFolic acid caution

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

Folic Acid Deficiency Anemia

Pathophysiology Malaabsorptive diseases of the small bowel, such as sprue, may not absorb folic acid normally

Folic Acid Deficiency AnemiaDiagnostic Test

CBCClinical ManifestationsFolic acid levelsBone marrow aspiration and

analysis

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

Folic Acid Deficiency AnemiaNursing Intervention

– Monitor CBC and reticulocytes– If malabsorption is cause, diet alone will

not be sufficient– Oral is preferred

Anemia caused by Increased Erythrocyte Production

1. Hemolytic Anemia2. Sickle Cell Disease3. Polycythemia Vera4. Hemochromatosis

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

Hemolytic AnemiaDiagnostic Test

CBCPeripheral smearMorphologic examinationRBC indicesIncreased RBC distribution width (RDW)Reticulocyte count

Hemolytic AnemiaDiagnostic Test

Lactic acid dehydrogenase levelSerum haptoglobin levelIndirect bilirubin level

Hemolytic AnemiaClinical Manifestation

WeaknessFatigueHemochromatosis-Bronze skin

tone and diabetesHemoglobinuria – dark urineJaundiceElevation in Bilirubin levelsEnlargement of spleen and liver

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

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

Sickle Cell DiseaseDiagnostic Test

Low HematocritSickled Cells on SmearHemoglobin Electrophoresis - a

blood test done to evaluate the different types of hemoglobin in the bloodstream.

Sickle Cell DiseaseClinical Manifestation

Jaundice – is characteristic and is usually obvious in the sclerae

Enlargement of the face and skullTachycardiaCardiac murmursCardiomegaly – enlarged heartDysrhythmiasHeart Failure

Sickle Cell DiseaseNursing Intervention

Manage PainPrevent/Manage InfectionPromote Coping SkillsMinimize Deficient Knowledge

Polycythemia Vera

Primary polycythemiaProfilerative disorderMyeloid stem cells seem to have

escaped normal control mechanisms

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

Polycythemia VeraDiagnostic Test

CBCElevated erythrocyte massLeukocyte and Platelet countErythropoietin levelElevated hematocrit

Polycythemia VeraClinical Manifestation

Ruddy complexionSplenomegalyIncreased Blood Volume

Headache Dizziness Tinnitus Fatigue Paresthesias Blurred Vision

Polycythemia VeraClinical Manifestation

Increased Blood Viscosity Angina Claudication Dyspnea Thrombophlebitis

Elevated Blood pressure

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

HemochromatosisIron overload diseaseInherited metabolic disorderIncreased absorption of iron that is

deposited in the body tissues and organs particularly the liver, heart and pancreas

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

HemochromatosisClinical Manifestation

Joint PainFatigueLack of energyIrritabilityDepressionLoss of body hairAbdominal painLose of sexual driveHeart Problems

HemochromatosisNursing Intervention

Should not take Iron supplementsAvoid Alcoholic Beverages Dietary intake of iron is limited

Disorders Affecting White Blood Cells

1. Agranulocytosis2. Multiple Myeloma3. Infectious Mononucleosis4. Splenic Rupture and Hypersplenism

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.

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.

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

AgranulocytosisClinical Manifestation

Fatigue and weaknessSore throat Ulcerations of the pharyngeal and

buccal mucosa, DysphagiaHigh feverWeak and rapid pulse, and sever

chills

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.

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.

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.

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.

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.

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.

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.

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.

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.

Infectious MononucleosisClinical Manifestation

FatigueHeadachesMalaiseMyalgiasTemperature up to 39° C (102.2°F)Pharyngitis

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.

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.


Diagnostic TestLaboratory indication for

splenectomy include granulocytopenia of less than 500/mm3 and thrombocytopenia of less than 20,000/mm3.


Clinical ManifestationsModerate to massive splenomegalyAnemiaLeucopenia or thrombocytopeniaA compensatory increase in the

production of the affected cell line by the bone marrow.

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

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

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

Immune Thrombocytopenic Purpura

Most commmon thrombocytopenic disorder

Idiopahitc thrombocytopenic purpuraCause was unknownHemorrhagic autoimmune diseaseDestruction of platelets

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

Coagulation Disorders

defect in clotting mechanism one or more of the clotting factors is

depleted or absent

1.Disseminated intravascular coagulation

2.Hemophilia

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


PathophysiologyExcessive clotting can be

precipitated through intrinsic or extrinsic coagulation pathways


Clinical ManifestationBleeding from mucous membranes

venipuncture sites and GI and urinary tracts.

Organ dysfunction Renal failure

Hemorrhages


Nursing Intervention Monitor and manage potential

complications Target potiential sites of end-organ damage

• Kidneys• Lungs• Brain• Skin

Hemophilia X-linked genetic disorderA deficiency of coagulation factorsHEMOPHILIA A Deficiency of Factor VIIIHEMOPHILIA B Christmas DiseaseDeficiency of factor IX

HemophiliaAssessment

HemorrhageBleeding into joints

KneesElbowsAnklesShouldersWristHips

Ankylosis (fixation) of the joint

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

hematologic disorders

Documents

pernicious

anemia caused

globin gene

insufficient

intrinsic

oxygen

iron

tissues