hematologic system - uokirkuk.edu.iq

College of Nursing 2019-2020, Lecture Three

University of Kirkuk By: Bestoon Ahmed, PhD Pediatric Nursing

Alterations in Hematologic System Functions

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Hematologic System




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KEY TERMS

Hematology is the study of the blood and blood products.

Ecchymosis: A bruise.

Epistaxis: bleeding from nose

Hemolysis: destruction or decrease in RBC

Leukopenia: A lower than normal WBC count.

Petechiae: pinpoint red lesions

Purpura: bleeding under the skin and mucous membrane (red to purple).

Thrombocytopenia: A deficiency of platelet.

Erythropoiesis: is the process by which red blood cells (erythrocytes) are produced

Hematopoiesis: the formation and development of blood cells.

Extramedullary: that occurring outside the bone

Hemochromatosis: excessive accumulation of iron in tissues

Hemarthrosis - Bleeding into the joint.

Ischemia - Poor tissue perfusion.

Hematology is the study of the blood and blood products.

Diseases:

Iron-deficiency anemia

Thalassemia

Sickle cell disease (SCD)

Hemophilia

Acute lymphoblastic leukemia (ALL)




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Anatomy and Physiology

The hematologic system refers to the blood and blood-forming organs. The formation of red blood

cells, white blood cells, and platelets begins in the bone marrow.

Stem cells are produced in the bone marrow. Initially, these cells are not differentiated and may

become red blood cells (RBCs), white blood cells (WBCs), or platelets. In the next stage of

development, the stem cell becomes committed to a particular precursor cell, to become either a

myeloid or lymphoid type of cell and will differentiate into a particular cell type when in the presence

of a specific growth factor.

The spleen; is found in the left upper quadrant of the abdomen.

Function:

The spleen filters whole blood. It removes old and imperfect white blood cells, lymphocytes and

macrophages, and RBCs. The spleen also breaks down hemoglobin and stores of RBCs and platelets.

The liver; is found in the right upper quadrant of the abdomen and is the main production site for many

of the clotting factors, including prothrombin. Normal liver function is important for vitamin K

production in the intestinal tract. Vitamin K is necessary for clotting factors VII, IX, X, and

prothrombin.

1. ANEMIA

A low hemoglobin or red blood cell (RBC) count results in decreased oxygen carrying capability of

the blood.

Causes:

This may be due to:

• Blood loss.




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• Damage to the red blood cells due to altered hemoglobin or destruction (Hemolysis).

• Nutritional deficiency (iron, vitamin B12, and folic acid).

• Lack of RBC production.

• Bone marrow failure.

• Some patients have a family history of anemia due to genetic transmission, such as thalassemia

or sickle cell.

PROGNOSIS

Anemia is a symptom of something else happening. The cause of the anemia needs to be determined in

order to correct the anemia and its symptoms.

1. Iron Deficiency Anemia:

A blood disorder caused by insufficient iron intake in which the body produces inadequate

quantities of hemoglobin. Most common types of anemia, most common nutritional deficiencies in

children.

TREATMENT

Correction of the underlying cause is necessary. Treatment may include dietary modifications and

supplementations.

Causes:

o Inadequate intake of Iron

o Inadequate storage of Iron.

o Excessive loss of Iron.

o Some combination of these conditions.




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NURSING DIAGNOSIS:

Activity intolerance and fatigue.

Imbalanced nutrition, less than body requirement.

NURSING INTERVENTION

1. Check vital signs for changes.

2. Monitor CBC, hemoglobin, RBC,

3. Plan nursing care based on patient tolerance of activity.

4. Monitor for angina.

2. Thalassemia

It is a blood disorder passed down through families (inherited) in which the body makes an abnormal

form or inadequate amount of hemoglobin. Normal hemoglobin is composed of 2 alpha and 2 beta

globins. Thalassemia syndromes are a heterogeneous group of inherited anemia characterized by

reduced or absent synthesis of either alpha or Beta globin chains of Hb A.

Most common single gene disorder 2 types of thalassemia: alpha and beta.

Inheritance

• Autosomal recessive

• Beta thal - point mutations on chromosome 11

• Alpha thal - gene deletions on chromosome 16

PATHOPHYSIOLOGY

Anemia result from lack of adequate Hb A → tissue hypoxia→↑ Erythropoietin (EPO) production → ↑

erythropoiesis in the marrow and sometimes extramedullary → expansion of medullary cavity of

various bones. Liver spleen enlarge → extramedullary hematopoiesis




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EFFECTS OF BONE MARROW EXPANSION

• Deformities of skull and face

• Sinus and middle ear infection due to ineffective drainage

• Folate deficiency

• Hypermetabolic state -> fever, wasting

• Increased absorption of iron from intestine

Hepatomegaly

• Extra medullary erythropoiesis

• Iron released from breakdown of endogenous or transfused RBCs cannot be utilized for Hb

synthesis – hemosiderosis

• Hemochromatosis

• Infections – transfusion related - Hep B, C, HIV

• Chronic active hepatitis

Spleenomegaly

• Extra medullary hematopoiesis

• Work hypertrophy due to constant hemolysis

• Hypersplenism (progressive spleenomegaly)

Infections

• Poor nutrition

• Increased iron in body

• Blockage of monocyte-macrophage system

• Hypersplenism- leukopenia




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• Infections associated with transfusions

Jaundice

• Unconjugated hyperbilirubinemia - hemolysis

• Hepatitis - transfusion, hemochromatosis

• GB stones - obstructive jaundice

• Cholangitis

Accumulation of Iron

Lungs: restrictive lung defects

Adrenal insufficiency

Hypothyroidism, hypoparathyroidism

Clinical Features THAL Major

INFANTS:

Age of presentation: 6-9 mo (Hb F replaced by Hb A)

Progressive pallor and jaundice

Cardiac failure

Failure to thrive, gross motor delay

Feeding problems

Bouts of fever and diarrhea

Hepatosplenomegaly

BY CHILDHOOD:

• Growth retardation

• Severe anemia-cardiac dilatation

• Transfusion dependent

• Icterus

• Changes in skeletal system

Diagnosis

1. BLOOD Picture

• Hb – reduced (3-9mg/dl)




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• RBC count – increased

• WBC, platelets – normal

• RBC indices – MCV & MCH, MCHC reduced, RDW normal

2. X-RAY

• Radiological changes

TREATMENT

• Hb to be maintained –

• Hypertransfusion : >10 gm/dl

• Supertransfusion : >12 gm/dl

• If regular transfusions- no hepatomegaly, no facies

• 10-15ml/kg PRBC raises Hb by 3-5gm/dl –

Chelating Therapy

(1 unit of blood contains 250 mg iron)

• Iron-chelating agents: desferrioxamine-

• Dose: 30-60mg/kg/day

• IV / s/c infusion pump over 12 hr period 5-6 days /wk

SPLENECTOMY

• Deferred as long as possible. At least till 5-6 yrs age

Splenectomy (indications):

• Massive splenomegaly causing mechanical discomfort

• Progressively increasing blood transfusion requirements (>180-200 ml/kg/yr) packed RBC

BONE MARROW TRANSPLANTATION

Newer therapies: • GENE MANIPULATION AND REPLACEMENT

OTHER SUPPORTIVE MEASURES

• Tea – thebaine and tannins– chelate iron

• Vitamin C – increases iron excretion

• Restrict Fe intake – decrease meat, liver, spinach

• Folate – 1 mg/day




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• Genetic counselling

• Psychological support

• Hormonal therapy – GH, estrogen, testosterone, L-thyroxine

Prognosis • Life expectancy: 15-25 yrs

• Untreated: < 5 yrs

Prevention • Antenatal diagnosis

• Termination of pregnancy if Thal major

• Preventing marriage b/w traits

3. Sickle Cell:

An autosomal recessive hereditary disease in which red blood cells become malformed, causing

them to clump, leading to thromboses, decreased tissue perfusion, and organ damage. Sickle Cell

has sickled shaped red blood cells, which causes complications because the blood cells are not able

to reach certain parts of the body.

Pathophysiology:

An abnormal gene causes damage to the RBC membrane. The abnormal hemoglobin within the red

blood cell is called hemoglobin S. Dehydration or drying of the RBC makes it more vulnerable to

sickling (forming a crescent-like shape), as do hypoxemia and acidosis. Hemolytic anemia results as

RBCs are destroyed due to the damage to the outer membrane. The sickled cells can also clump

together, causing difficulty getting through the smaller vessels.

Prognosis:

People can live a relatively normal life with Sickle Cell if they have the proper treatment.

The average life expectancy for males is 42.

The average life expectancy for females is 48.

In 2003 the oldest patient to have Sickle cell was an 85-year-old Jamaican woman.




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Genetics of Sickle Cell

➢ Sickle cell is an autosomal recessive disease.

➢ Therefore, the child can only get Sickle cell if both parents are carriers, not if only one is and

the other is normal. They have a 25% chance of getting it if both are carriers

Nursing Diagnosis

➢ Impaired Gas Exchange:

➢ Ineffective Tissue Perfusion:

➢ Acute/chronic Pain

➢ Deficient Knowledge [Learning Need]

➢ Delayed Growth and Development

➢ Risk for sedentary Lifestyle:

➢ Compromised family Coping

4. Hemophilia

Is a group of hereditary illnesses characterized by slowed to markedly altered blood clotting

resulting from a deficiency of one of the factors necessary for blood coagulation.

A. Incidence.

1. Von Willebrand disease: one in 100 to 1 in 10,000 individuals; most common bleeding disorder.

2. Hemophilia A: one in 4,000 to 1 in 5,000 males.

3. Hemophilia B: approximately 1 in 20,000 males.

B. Etiology.

1. Von Willebrand disease.

a. Three types: type 1, autosomal dominant inheritance; type 3, autosomal recessive inheritance;

type 2 may be either autosomal dominant or recessive inheritance.

b. Th e mutated gene results in a deficiency or altered functioning of the Von Willebrand factor.




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2. Hemophilia A: also called classic hemophilia.

a. X-linked recessive inheritance.

b. Th e mutated gene results in factor VIII

deficiency or an altered form of factor VIII.

3. Hemophilia B: also called Christmas disease.

a. X-linked recessive inheritance.

b. Th e mutated gene results in either factor IX deficiency or an altered form of factor IX.

D. Diagnosis.

1. Clinical picture and family history.

2. Prolonged partial thromboplastin time (PTT).

3. Genetic testing: prenatal testing is available.

4. Factor assay assessment.

Nursing Diagnosis:

1. Risk for deficient Fluid Volume [isotonic]:

2. Risk for acute/chronic Pain:

3. Risk for impaired physical Mobility

4. Ineffective Protection may be related to abnormal blood profile, possibly evidenced by altered

clotting.

5. Compromised family Coping:

6. Nursing Intervention

• No IM injections.

• No aspirin.




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• To stop bleeding:

• Elevate site.

• Apply direct pressure to the site.

Explain to the patient:

• Wear a medical alert identification.

• Contact physician for any injury.

• Avoid situations where injury might occur.

5. Acute lymphoblastic leukemia (ALL)

Also called acute lymphocytic leukemia, ALL is the most common form of childhood cancer and is

characterized by a proliferation of lymphoblasts, or immature white blood cells. Acute

lymphoblastic leukemia (ALL) is characterized by a proliferation in the production of

lymphoblasts, or immature white blood cells, in the bone marrow.

A. Incidence.

1. Leukemia is the most common form of cancer in children, and ALL is the most common

leukemia seen in children.

2. Cancer is very rare in children: only 1% of new cancers each year are in children. However,

cancer is the leading cause of death from disease in children.

3. ALL is more common in males than in females.

4. Peak age of onset of ALL is between 2 and 6 years of age.

5. Currently, there is a 90% cure rate in children under 15 years of age.

B. Etiology.

1. Unknown, but chromosomal anomalies predispose some children to leukemia.




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a. Trisomy 21 (Down syndrome): 15 times the risk of general population.

b. Translocation of chromosomes 7 and 14: frequently seen in children with ALL.

Nursing Diagnosis:

Activity Intolerance:

Acute Pain

Fatigue:

Imbalanced Nutrition:

Risk for deficient Fluid Volume:

Nursing Intervention

1. Monitor for bleeding—platelet count may be decreased.

2. Monitor for infection—patients have increased susceptibility to infection.

3. Monitor pain control.

4. Small, frequent meals.

Teach patients about infection control:

5. Avoid others with infection.

6. Report signs of infection, sore throat, fevers, etc.

Explain to the patient:

7. Use an electric razor.

8. Use soft toothbrush.

9. Watch for bleeding or bruising.

hematologic system - uokirkuk.edu.iq

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