Hematologic Disorders

Hematologic disorders
Assignment
• Arrhythmias :
• Atherosclerosis and arteriosclerosis:
• Varicose vein:
• Embolism:
• Thrombosis :
CARDIOVASCULAR DISORDERS 2
Anemia
 Anemia is a low red cell count and a below normal
hemoglobin or hematocrit level.
 It exists when there is an insufficient amount of
hemoglobin to deliver oxygen to the tissues
Causes
1. Inadequate production of RBC
 Inadequate stimulation of bone marrow
2. Bone marrow suppression
Premature or excessive distraction of RBC
 May be due to abnormal RBC produced bone marrow
3. Blood loss
From any source such as the GIT, the uterus, the nose or a wound
Hereditary factors
Associated with chronic disease
Classification
 Morphologic:
Normocytic, normochromic,
 Microcytic hypo chromic
 Macrocytic
Classification …
Physiologic
Hypo proliferation,
Excessive distraction,
 Maturation abnormality
Diagnostic approach:
Hematological studies:
Hgb, Hct, RBC indices WBC, Serum iron level,
Iron binding capacity, folate level, vit B12 level
 Platelate count, bleeding time, PT, PTT
 Bone marrow aspiration and biopsy
 Iron deficiency anemia:
• Is a condition in which the body iron content is

decreased below the normal level
 Results when the intake of dietary iron is

inadequate for hemoglobin synthesis
 It is the most common type of anemia in all

age group
Clinical pictures of IDA
Etiology:
• Bleeding from ulcer, gastritis, tumors
• Malabsorption of iron
• Menorrhogia (excessive menstrual bleeding)
• Chronic alcoholism
• Inadequate iron intake
• Pregnancy, hookworm
 Clinical picture:
 Pallor, weakness and dyspnea
 If the deficiency is sever- smooth, sore tongue
 Brittle and ridged nails (spoon shaped nails
called Koilonchia)
 Lab. Findings:
 Decrease Hct, Hgb, RBC
 RBC morphology abnormalities

 Decrease serum iron concentration
 WBC and platelate usually are normal
 Management:
• Treat underlying cause
• The hemoglobin level may increase only a few weeks and the
anemia can be corrected in a few months, and
• Iron store replenishment takes much longer, so it is important that
the patient continue taking the iron for as long as 6- 12 months.
• In some case oral iron is poorly absorbed or poorly tolerated
or iron supplementation is needed in large amounts, in these
situation IV or IM administration of iron dextran may be
needed
Management…
• Drugs include:
=Ferrous sulfate, ferrous gluconate, ferrous fumerate

= Iron injection- iron dextran
• The IM injection causes some local pain and can stain the skin.
• These side effects are minimized by sing the
• Z -track technique for administering iron dextran deep in to

the gluteus maximums muscle, and
• avoid vigorously rubbing the injection site after the injection
Aplastic anemia:
caused by
• Decrease in precursor cells in the bone marrow and
replacement of the marrow with fat
 decrease in or damage to marrow stem cells, damage to the
microenvironment within the marrow and replacement of the
marrow with fat
Aplastic anemia…
• It results in bone marrow aplasia (markedly reduced
hematopoiesis.
Therefore in addition to sever anemia, significant
neutropenia and thrombocytopenia
Aplastic anemia…
Cause: idiopathic
 Infection and pregnancy
 Medications, chemicals, or radiation
 Anti-tumoric agent
 Antihistamines, analgesics, sedatives

Clinical pictures:
Onset is gradual
characterized by weakness, pallor, breathlessness,

exertion and other manifestation of anemia.
• In 1/3 of the patient there is abnormal bleeding
• Fever and sepsis, Pancytopenia
• Adenopathy (enlargement of the gland) and

hepatomegally may be seen in rarely case
 Diagnosis:
bone marrow aspiration and biopsy and CBC
 Management:
• Aplastic anemia have poor prognosis
There are two methods
Bone marrow transplantation and
 Administrations of immune suppressive therapy
 Supportive therapy: discontinuing the offending
agents
: Transfusion of packed RBC and plate late

 Prevention:
 Avoid the potentially bone marrow depressant
drugs
 Assess the patient about the sign of infection
tissue hypoxia, bleeding from any wound,
abrasion and ulcer of mucous membrane
Megoloblastic anemia
 Is caused by deficiencies of the Vit B12 and folic acid.
 B12 and folic acid are essential vitamins for normal DNA
synthesis.
 RBCs are abnormally large and are called megaloblastic RBCs.
Vitamin B12
• Can occur mainly in vegeterians, who consume no meet or dairy
products, and inadequate dietary intake
 There is pancytopenia (a decrease in all myeloid derived cells)
 Hemoglobin level is low as 4-5 g/dl, and WBC count is 2000 to 3000/mm3
 Platelate count is < 50,000mm3
 Poikilocytosis (a widely vary shape of RBC)
 It is also caused by absence of intrinsic factor, which is normally secreted

by cells of the stomach; anemia, which results from this condition, is
called pernicious anemia
Pernicious anemia:
 Tend to run in families
 Adults particularly the elderly are affected

 The stomach wall atrophies and fails to secrete
intrinsic factor so, the absorption of vit B 12 is
significantly impaired
Clinical picture:
 Sign of anemia-pallor, weak and lethargic

 Smooth, sore red tongue and mild diarrhea
 Confusion and parasthesia of extremities and
 Patient die after several years due to CHF
secondary to anemia
 Diagnosis…
Management:
• Replacement of vitamin B12 or fortified
soymilk
Hemophilia
 Hemophilia is a hereditary disease in which the blood dose not clots
properly.
Coagulation disorders may be inherited or acquired.
 Most of the inherited plasma coagulation disorders are due to defects

in single coagulation proteins with two X linked disorders, factor VIII
and factor IX deficiency,
 The two inherited bleeding disorders are Hemophilia A and

hemophilia B.
 It is caused by a deficiency in one of the substance (clotting factors)

needed to normal clotting.
Types
Pathophysiology
Hemophilia A
• Occurs 1 in 10,000 male births ( X linked)
 More common than hemophilia B
 Females are asymptomatic and remain as a carrier

 Patients may be symptomatic when the factor VIII level is
below 5 %( normal 25%)
 The majority of patient with hemophilia have factor VIII level

below 5%
 The disease is recognized in early child hood
 Clinical picture
Bleeding in soft tissue, muscles and weight bearing joints

 Based on the level of factor VIII the bleeding severity may be
described.
 If the level of the factor is <1% they bleed frequently even
without noticeable trauma.
 If the level of the factor is between 1-5% the patient have less
frequent bleeding episode.
 If the level of the factor is > 5% the patients have infrequent
bleeding, that is usually secondary to trauma.
 Clinical picture…
• 75% of all bleeding in patient with hemophilia occurs in to the
joints (knees, ankles, shoulders, wrists and hips).
 Pain followed by swelling in a weight bearing joints and
limitation of movement is common.
Ankylosis, weakness sensation and atrophy of the joints area due

to hematoma.
 Synovial inflammation and GI bleeding.
 The most feared complications of hemophilia are orophangeal

and CNS bleeding.
 Diagnosis:
Reduced level of factors and  PTT time is prolonged
 Management:
 Concentrate factor VIII and factor IX, usually when patients
are actively bleeding and for when before traumatic procedures
 Desmopressin: produce the amount of blood products required
 Nursing management:
 Education about the disease process

 Prevent unnecessary trauma that can cause bleeding
 Avoid drugs such as NSAID, and alcohol because these
interfere with platelate aggregation and injections
 Dental hygiene to prevent dental extractions

 Post op care
 The patient should wear an identity tag or carry a card stating
that they suffer from hemophilia
Thrombocytopenia
Is a decrease in normal number of platelets,
which are the particles in the blood that are
essential for clotting.


Etiologies
 Decreased production of platelate as a result of leukemia,
aplastic anemia, infection, medication
 Increase distraction of platelate due to infection, lymphoma,
LES, ITP (Idiopathic thrombocytopenic purpura is the most
common cause of excessive platelate distraction)
 Increase consumption of platelate
Clinical picture:
 Bleeding when the plate late count is < 50,000/mm3 and during
surgery
Petechiae when the plate late number is< 20,000/mm3 (bleeding
below the skin producing bruises and small hemorrhages)
 Excessive menstrual bleeding and excessive bleeding after

surgery or dental extrication
 When the plate late number is below5000/mm3 fatal bleeding
will result
Diagnostic:
Bone marrow aspiration and biopsy
Management:
Treat the underlying cause
 Platelet transfusion to raise the plate late
count, if plate late production is impaired
Nursing management
Avoid aspirin and aspirin containing medications
and also avoid IM injections.
 Take no rectal temperature; do not give
suppositories, enemas
 Avoid constipations, apply pressure to venipuncture

site for 5 min or until bleeding has stopped
 Use only soft bristled toothbrush for mouth care

Nursing management…
For epistaxis position patient in high fowlers position apply ice
pack to back of neck and direct pressure to nose
Notify prolonged bleeding (unable to stop within 10 min)
Administer platelets, fresh frozen plasma, packed red blood
cells as prescribed.
Polycythemia
 Polycythemia (Erythrocytosis) is an increased volume of RBC
 When the hematocrit is elevated to more than 55% in male and
more than 50% in female then the term polycythemia is used.
Types
• There are two types of polycythemia: primarily
and secondary.
 Primarily polycythemia (polycythemia Vera)
 A proliferative disease in which the myeloid
stem cells seem to have escaped normal control
mechanisms
Clinical manifestation:
 Splenomegally
Headache, dizziness, tinnitus, fatigue, due to the increased

blood volume
 Angina, dyspnea, thrombophelbitis due to increased blood

viscosity
 Pruritis caused by histamine released due to the increased

number of basophils
 Burning sensation in the fingers and toes
 Diagnosis:
 Elevated RBC mass

 Enlarged spleen
 WBC and plate late count
Complication:
• Thromboses which leads to cerbrovascular accident like brain
attack, stroke, or heart attack
Thrombotic complications are the most frequent cause of death
Bleeding
Management
Goal: to reduce the high blood cell mass
 Phlebotomy- is an important part of therapy and can be
performed repeatedly to keep the hematocrit within normal
range, these is achieved by removing enough blood (initially
500ml once or twice a week)
= Avoid iron supplementation
= Chemotherapeutic agent to suppress marrow function

 Nursing management
 Assess risk factors for thrombotic complications

 Education to the patient about sign and symptoms of thrombosis
 Avoid drugs, which alter platelets function
 Bathing in tepid or cool water and lotion for pruritus
Leukemia
Leukemia: Unregulated proliferation and accumulation of WBC
in the bone marrow, replacing normal marrow elements
 There is also proliferation of WBC in the liver, spleen and
lymph nodes
 Can be classified according to the cell line involved either
lymphocytic or myelocitc and according to maturity of malignant
cells either acute (immature) or chronic

CARDIOVASCULAR (differentiated) cells
DISORDERS 57
Acute myeloid leukemia (AML)
 Due to defect in stem cell that differentiates
in to myeloid cells, monocytes, granulocytes,
erythrocytes and platelets
Common at age of 60
AML…
 Potentially curable disease, and sometimes with worse
prognosis.
 Patient with secondary AML (due to other cancer) have
a much worse prognosis, with treatment these type of
patient survive an average of < one year
Clinical picture
• Fever, Hyperplasia of the gum
• Infection results from neutropenia
• Weakness and fatigue from anemia
• Bleeding tendency from thrombocytopenia
• Hepato-splenomegally
 Bleeding and infections are the major causes of death in
AML CARDIOVASCULAR DISORDERS 65

Diagnosis:
• CBC (complete blood count)
: Bone marrow analysis
Management
 Induction therapy;
 Aggressive administration of chemotherapy, to eradicate the
leukemic cells
 Side effect include; Severely Neutropenic, anemic,
thrombocytpenic, infections
 Consolidation Therapy:
 To eliminate any residual leukemia cells that are not clinically
detectable
Management…
• Bone marrow transplantation/ peripheral blood steam cell
transplantation
 Supportive care alone for patients with significant comorbdity
such as extremely poor cardiac, pulmonary, renal or hepatic
function.
 70% of patients with AML experience a relapse, a recurrent

study of long term survival of patients with AML found that
only 11% survived 10 year or longer.
Acute lymphocytic leukemia (ALL)
 Is due to uncontrolled proliferation of immature cells

(lymphoblasts) derived from the lymphoid stem cell
 Common in young children, peak incidence is 4 year

of age and it attack more male than female.
 80% of children survive at least 5 year
 Frequently involves the CNS
Clinical Pictures
◦ Reduced number of leukocytes, erythrocytes and
plat elates
◦ Hepatosplenomegally
◦ Bone pain
◦ Headache and vomiting
Management;
• Corticosteroids
• Cranial irradiation
• Intra-thecahl chemotherapy
Nursing intervention
• Assess history (weakness and fatigue)
 Monitor the results of lab. Studies
 Maintain adequate nutrition, activity and ability to self-care
 Assess the oral mucosa for possible lesions, color change due to the effect of
medications
 Avoid using lemon glycerin swabs for oral hygiene due to the tendency to dry
the tissue, in stead use simple rinses with saline
 Use stool softeners with monitoring and control fever and pain
 Maintain fluid and electrolyte balance

Hematologic Disorders

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Hematologic Disorders

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Hematologic disorders

• Atherosclerosis and arteriosclerosis:

 Anemia is a low red cell count and a below normal

hemoglobin or hematocrit level.

 It exists when there is an insufficient amount of

hemoglobin to deliver oxygen to the tissues

Hgb, Hct, RBC indices WBC, Serum iron level,

Iron binding capacity, folate level, vit B12 level

 Platelate count, bleeding time, PT, PTT

 Bone marrow aspiration and biopsy

• Is a condition in which the body iron content is

 Results when the intake of dietary iron is

 It is the most common type of anemia in all

• Bleeding from ulcer, gastritis, tumors

• Menorrhogia (excessive menstrual bleeding)

• Inadequate iron intake

 Pallor, weakness and dyspnea

 If the deficiency is sever- smooth, sore tongue

 Brittle and ridged nails (spoon shaped nails

 Decrease Hct, Hgb, RBC

 RBC morphology abnormalities

=Ferrous sulfate, ferrous gluconate, ferrous fumerate

• Z -track technique for administering iron dextran deep in to

• Decrease in precursor cells in the bone marrow and

replacement of the marrow with fat

 decrease in or damage to marrow stem cells, damage to the

microenvironment within the marrow and replacement of the

marrow with fat

Therefore in addition to sever anemia, significant

neutropenia and thrombocytopenia

 Infection and pregnancy

 Medications, chemicals, or radiation

 Antihistamines, analgesics, sedatives

characterized by weakness, pallor, breathlessness,

• Fever and sepsis, Pancytopenia

• Adenopathy (enlargement of the gland) and

bone marrow aspiration and biopsy and CBC

• Aplastic anemia have poor prognosis

There are two methods

Bone marrow transplantation and

 Administrations of immune suppressive therapy

 Supportive therapy: discontinuing the offending

: Transfusion of packed RBC and plate late

 Avoid the potentially bone marrow depressant

 Assess the patient about the sign of infection

tissue hypoxia, bleeding from any wound,

abrasion and ulcer of mucous membrane

 There is pancytopenia (a decrease in all myeloid derived cells)

 Platelate count is < 50,000mm3

 Poikilocytosis (a widely vary shape of RBC)

 It is also caused by absence of intrinsic factor, which is normally secreted

 Tend to run in families

 Adults particularly the elderly are affected

 Sign of anemia-pallor, weak and lethargic

• Replacement of vitamin B12 or fortified

Coagulation disorders may be inherited or acquired.

 Most of the inherited plasma coagulation disorders are due to defects

 The two inherited bleeding disorders are Hemophilia A and

 It is caused by a deficiency in one of the substance (clotting factors)

 Females are asymptomatic and remain as a carrier

 The majority of patient with hemophilia have factor VIII level

Bleeding in soft tissue, muscles and weight bearing joints

Ankylosis, weakness sensation and atrophy of the joints area due

 Synovial inflammation and GI bleeding.