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HEMATOLOGIC

DISORDERS

AND ITS MANAGEMENT


ANEMIA

Is a condition in which hemoglobin


concentration is lower than normal
It reflects the presence of fewer the normal
number of erythrocytes within the circulation.
CLASSIFICATION OF ANEMIA

Anemia is classified according to:


Deficiency of erythrocytes caused by a defect in the
production (Hypoproliferative anemia)
Destruction of erythrocytes (Hemolytic anemia)
Or by their loss (bleeding)
FACTORS AFFECTING ERYTHROCYTES PRODUCTION

The bone marrow’s inability to respond to decreased


erythrocytes
The degree to which your erythrocytes proliferate in the bone
marrow and the manner in which they mature
The presence and absence of end products of erythrocytes
destruction withing the circulation
ASSESSMENT AND DIAGNOSTIC FINDINGS

Initial evaluation:
Hemoglobin, hematocrit, reticulocytes count, RBC
RBC indices (MCV, RDW)
IRON
B12 and Folate levels
Other tests:
Haptoglobin and erythropoietin
CBC
COMPLICATIONS

Heart failure
Paresthesia
delirium
MEDICAL MANAGEMENT:
NURSING PROCESS:
ASSESSMENT: PHYSICAL ASSESSMENT

Assess for weakness, fatigue and general malaise


Pallor of the skin and mucosal membrane are common.
Jaundice, angular cheilosis
Brittle ridged and concave nails
Tongue maybe beefy and sore
pica and restless leg syndrome
ASSESSMENT: HEALTH HISTORY

Medications
Alcohol intake
Genetic predisposition of the disease
NUTRITIONAL ASSESSMENT:

Important for indications of deficiencies such as


iron, vitamin B12 and folate
Strict vegetarian diet
CARDIAC STATUS:

Assess for tachycardia, palpitations, dyspnea,


dizziness, orthopnea and exertional dyspnea
ASSESSMENT OF GI

Nausea and vomiting (coffee grounds)


Diarrhea, anorexia, glossitis
Melena
Assess for menstrual period for girls
ASSESS FOR NEUROLOGIC EXAM:

Peripheral numbness, ataxia, paresthesia, poor coordination and


confusion
DIAGNOSIS:

Fatigue
Nutrition less than body requirements
Activity intolerance
Non-compliance to therapy
PLAN AND INTERVENTION:

Managing fatigue
Prioritize activities and establish a balance
between activity and rest
Maintaining adequate nutrition:
Promoting adherence with prescribed therapy
Managing potential complications
Heart failure
Angina
HYPOPROLIFERATIVE ANEMIA
IRON DEFICIENCY ANEMIA

 Iron intake is inadequate


 Common causes:
 Postmenopausal bleeding, Ulcer, gastritis and inflammatory dieases
 Menorrhagia, pregnancy
 Chronic alcoholism
 NSAIDs and Aspirin
 Iron malabsorption problems (gastrectomy and bariatric surgery)
CLINICAL MANIFESTATION

If prolonged:
ASSESSMENT AND DIAGNOSTIC FINDINGS:

Bone marrow aspiration


Iron stores and hemoglobin levels
Hematologic results are low
Red blood cell count
Male: 4.35-5.65 trillion cells/L* (4.32-5.72 million cells/mcL**)
Female: 3.92-5.13 trillion cells/L (3.90-5.03 million cells/mcL)

Hemoglobin
Male: 13.2-16.6 grams/dL*** (132-166 grams/L)
Female: 11.6-15 grams/dL (116-150 grams/L)
Hematocrit Male: 38.3-48.6 percent
Female: 35.5-44.9
percent

White blood cell 3.4-9.6 billion cells/L


count (3,400 to 9,600
cells/mcL)

Platelet count Male: 135-317 billion/L


(135,000 to
317,000/mcL)
Female: 157-371
billion/L
(157,000-371,000/mcL)
Normal value range is: Iron: 60 to 170
micrograms per deciliter (mcg/dL), or 10.74 to
30.43 micromoles per liter (micromol/L)
Total iron binding capacity (TIBC): 240 to 450
mcg/dL, or 42.96 to 80.55 micromol/L.
Transferrin saturation: 20% to 50%
MEDICAL MANAGEMENT:
NURSING MANAGEMENT

Preventive education
Medication compliance and adverse reactions
ANEMIA IN RENAL DISEASE

 When you have kidney disease, your kidneys cannot


 make enough EPO. Low EPO levels cause your
 red blood cell count to drop and anemia to develop.
ANEMIA OF INFLAMMATION:

A general term used to describe anemia associated with


chronic disease with inflammation, infection or malignancy.
Usually this anemia has normochromic and normocytic.
Hgb level rarely falls under 9 g/dl
However, EPO is low because or erythroid cells.
APLASTIC ANEMIA
DEFINITION:

Caused by decrease or damage to the marrow


stem cells, to the microenvironment of the
marrow or the replacement of bone marrow with
fat.
CLINICAL MANIFESTATION:

Symptoms of anemia
Bruising
Repeated throat infection
Cervical lymphadenopathy
spleenomegaly
ASSESSMENT AND DIAGNOSTIC

Ingestion od toxic chemicals


CBC reveals pancytopenia
Neutrophil count less than 1500/ul
Hemoglobin less than 10g/dl
Platelets 50,000 /ul
Bone marrow aspiration has very few to no cells presnted
MEDICAL MANAGEMENT
Hematopoietic stem cell transplant
Hematopoietic stem cell transplantation (HSCT)
involves the intravenous (IV) infusion of
autologous or allogeneic stem cells to reestablish
hematopoietic function in patients whose bone
marrow or immune system is damaged or
defective.
MEDICAL MANAGEMENT

Immunosuppressive drugs
Cyclosporine or androgens
Atithymocyte which is obtained from horses or rabbit
Then, client is supported with PRBC and PLATELET
NURSING MANAGEMENT

Assess for signs of infection and bleeding


WOF: Side effects of cyclosporine
DEFINITION:

This is caused by a deficiency in B12 and folic


acid which are important in DNA synthesis
BONE MARROW ANALYSIS

Reveals abnormal increase in number of cells


Large and bizzare appearance of cells
PANCYTOPENIA
FOLIC ACID DEFICIENCY
VITAMIN B12 DEFICIENCY

 Pernicious anemia
VITAMIN B12 FOLIC ACID
CLINICAL MANIFESTATION
Neurologic manifestation  
GI manifestations GI Manifestation
Smooth, sore and red Smooth, sore and red tongue
tongue
Mild diarrhea Mild diarrhea
Extremely pale Extremely pale
Confused with paresthesia  
MANIFESTATION:

Mild jaundice, vitiligo, premature graying of hair


ASSESSMENT AND DIAGNOSTIC STUDIES

Serum levels of both vitamins


Schilling test
B12 assay test
Antibody test for to detect intrinsic factor
Homocysteine and methylmalonate, or methylmalonic acid. These
substances can build up in your body if you have a B-12 or folate
deficiency.
If the test is done on your blood plasma, a normal
range for folate is 2 to 10 ng/mL. If the test is done
on red blood cells, a normal range is 140 to 960
ng/mL. If the test is done on your blood plasma,
a normal range for folate is 2 to 10 ng/mL. If
the test is done on red blood cells, a normal
range is 140 to 960 ng/mL.
SCHILLING TEST

The Schilling test is a medical procedure used to determine


whether you’re absorbing vitamin B-12 properly. Your doctor
may choose to order this test if you have vitamin B-12
deficiency, or pernicious anemia. The Schilling test usually
involves up to four stages. It also involves analyzing your urine
samples to help determine the cause of the vitamin deficiency.
MEDICAL MANAGEMENT

Administration of folic acid 1 mg daily


Administer IM folic to patients with malabsorption
Small dose of Vitamin B12
4-8 weeks blood counts return to normal
(manifested by good tongue appearance but neurologic
manifestation requires more time to recover)
NURSING MANAGEMENT

Monitor for neurologic complications


Monitor for ambulation and should assess gait,
stability and the use of assistive device
Nutritional intake
HEMOLYTIC ANEMIA
HEMOLYTIC ANEMIA

The erythrocytes have a shortened lifespan , thus their number


in circulation is reduced.
Fewer erythrocytes cause decreased in oxygen, causing hypoxia
which stimulates erythropoietin releases from the kidney.
The bone marrow then releases reticulocytes.
Because of the breaking down, bilirubin levels increases.
SICKLE CELL ANEMIA
Can cause severe hemolytic anemia that results form
inheritance of sickle cell hemoglobin (HBS gene)
HbS gene can loses its round, pliable, biconcave
disc shape and become dehydrated, rigid and sickle
cell.
CLINICAL MANIFESTATION

 Anemia with hemoglobin at 5 to 11 g/dl


 Tachycardia, cardiac murmurs and often enlarged heart, Dysrythmia
 Jaundice
 Any organ can have thrombosis
 Hypoxic damage or ischemic necrosis
SICKLE CELL CRISIS

Acute vaso-occlusive crisis


Results from the entrapment of erythrocytes and leukocytes in
the microcirculation causing hypoxia, inflammation and
necrosis.
Sequestration crisis
Other organs pooled the sickled cells.
Hemoglobin level falls
Aplastic crisis
From infection with the human parvo
ACUTE CHEST SYNDROME

As manifested by fever, respiratory distress


(tachypnea, cough and wheezing) and new
infiltrates seen on the chest x-ray
TREATMENT:

Incentive spirometry
Bronchoscopy
Corticosteroids
transfusion
ACUTE CHEST SYNDROME

Medical management:
Transfusion of red cell
Antimicrobial therapy
Bronchodilators
Oxide therapy
Pulmonary hypertension
Stroke
Reproductive problems
ASSESSMENT AND DIAGNOSTIC

Normal hemoglobin level, normal hematocrit, normal


blood smear. (trait)
Sickle cell disease has low hematocrit and
sickled cells on the smear
The WBC and Platelet counts are often
higher than normal
MEDICAL MANAGEMENT

Hematopoietic stem cell transplant


PHARMACOLOGIC TREATMENT

Hydroxyurea
A chemo drug used to increase fetal hemoglobin
levels in patient with SCD, thereby decreasing the
production of sickled cells
Side effects: chronic suppression of leukocytes,
teratogenesis
Required daily folic acid replacement to maintain
the supply required for increased erythropoiesis
from hemolysis.
Infections can be treated with antibiotics.
SUPPORTIVE THERAPY
 Pain management
 Aspirin and NSAID
 WOF: Thrombosis
 Neuropathic pain
 Serotonin and norepinephrine, TCA or gabapentinoids
 Adequate hydration
 IV FLUIDS
NURSING PROCESS
ASSESSMENT:
Assess for familial history of the disease
Assess for pain, swelling and ischemia
Assess for cardiovascular function
Signs of dehydration
Neurologic examination and cognitive dysfunction
infections
DIAGNOSIS

Acute pain
Fatigue
Risk of infection
Deficient knowledge
DEFINITION:

A group of hereditary anemia that causes


hypochromia (an abnormal decrease in the
hemoglobin content of erythrocytes) extremely
microcytic (smaller than normal) hemolysis.
Defective synthesis of hemoglobin
2 MAJOR TYPES
ALPHA THALASSEMIA
ALPHA THALASSEMIA

Milder than beta thalassemia.


The erythrocytes are microcytic but the anemia if
present is milder
BETA THALASSEMIA

Fatal in the first few years of life


CHARACTERISTICS

Anemia, marked hemolysis, ineffective


erythropoiesis
Organ dysfunction can occur due to iron
overloading of PRBS Transfusion
Regular cheilation
DEFINITION:

It refers to the increased volume of RBC.


Hematocrit is elevated due to dehydration
SECONDARY

Excess in production of EPO


Therapeutic phlebotomy
NEUTROPENIA
DEFINITION:

A neutrophil count less than 2000/mm3.


It results from decreased production of neutrophils or
increased destruction of these.
CLINICAL MANIFESTATION

There are no definite symptoms of neutropenia


until the patient develops an infection
A CBC with differential can be helpful
MEDICAL MANAGEMENT:

Treatment depends on the cause of the condition.


If neutropenia is caused by medications, offending agent is
withheld.
In the case of chemo drugs, reducing the drugs may help
alleviate the levels of neutrophils
DIAGNOSTICS

Cultures of blood, urine and sputum as well as


chest xray are obtained in case the client develops
fever.
Broad spectrum antibiotics are used as soon as
cultures are obtained.
NURSING MANAGEMENT:

Preventing and managing neutropenia.


THROMBOCYTOPENIA
DEFINITION:

Low platelet levels caused by:


Decreased production of platelet in the bone
marrow
Increased destruction of platelet
Increased consumption of platelet
CLINICAL MANIFESTATIONS:

Bleeding and petechia do not usually develops with the level


greater than 50,000/mm3.
When the petechial level falls below 20,000/mm3, petechia,
excessive bleeding, nasal, gingival bleeding, excess bleeding
after surgery can occur.
If it is below 5000/mm3 spontaneous fatal CNS, GI
hemorrhaging
MEDICAL MANAGEMENT

Treatment of underlying cause


If platelet production is impaired, platelet transfusion is needed.
NURSING MANAGEMENT

Education about the condition


Fall prevention especially in older adults
Medications
HEMOPHILIA
2 TYPES

 Type A  Type B is called Christmas


 Caused by genetic defect that is disease
caused by defective in factor VII  Stems from a genetic defect of
 More common than type B factor IX
 Most affected in males  Most affected in males

 Females carriers  Females are only carriers


CLASSIFICATIONS:

Severe disease (plasma factor less than 1IU/dl OR


LESS THAN 1% normal factor VII
Moderate disease reflects level of 1-5 IU/dl or 1%-
5% normal
Mid above 5 IU/dl or above 5%
CLINICAL MANIFESTATIONS

Severe hemorrhaging into various parts of the body


75% of bleeding occurs in joints commonly in the
knees, elbows, ankles, shoulders, wrist and hips.
MEDICAL MANAGEMENT:
Recombinant forms of factors VII and X
concentrates should be available
Prophylactic use, children receives 3-4 times each
week
FFP
NURSING MANAGEMENT

 Preventing trauma and bleeding episodes


 medication therapy
 Avoiding agents that interfere with platelet aggregation
 Applying pressure to wounds
 Analgesics for pain
 Frequent VS if clients have hemorrhagic episodes
DISSEMINATED INTRAVASCULAR COAGULATION
 This is not an actual disease but rather a sign of underlying condition
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