Management of Hematologic Disorders Anemia

BY/ Dr Hend elshenawie

Basophil (WBC)

Neutrophil (WBC)

Intended learning outcomes*Identify anemia

*Identify causes of anemia

*Describe pathophysiolgy of anemia

* Differentiate between the hypoproliferative and the hemolytic

anemia

*compare and contrast the physiologic mechanisms,

clinical manifestations, medical management, and nursing interventions

for each type of anemia.

*Use the nursing process as a framework for care of patients with

anemia.

*Compare the leukemias, their incidence, physiologic alterations,

clinical manifestations, management, and prognosis.

* Use the nursing process as a framework for care of patients with

leukemia.

Normal types of blood cells

Basophil (WBC)

Platelet(thrombocyte

)

B-Lymphocyte

(WBC)

Eosinophil (WBC)

Monocyte (WBC)

Neutrophil (WBC)

RBCs

Introduction*Hgb: measures the concentration of the major

oxygen carrying pigment in whole blood*Hct: percent of a sample of whole blood occupied

by intact RBCs*RBC Count: number of RBCs contained in a

specified volume of whole blood

*All factors are dependent on the RBC mass and the plasma volume

Anemia It is qualitative or quantitative deficiency of

hemoglobin, since HB carries oxygen from lungs to the tissues, anemia leads to hypoxia in organs

Causes of anemia

Classification of Anemias

1-Hypoproliferative (Resulting From Defective RBC Production)

1-Iron deficiency2-Vitamin B12 deficiency (megaloblastic)Folate deficiency3-Decreased erythropoietin production (eg,

from renal dysfunction ,Cancer /inflammation

2-Bleeding (Resulting From RBC LossBleeding from gastrointestinal tract,

menorrhagia(excessive menstrual flow),epistaxis (nosebleed),trauma

3-Hemolytic (Resulting From RBC Destruction)

1-Altered erythropoiesis (sickle cell anemia, thalassemia, other hemoglobinopathies)

2-Hypersplenism (hemolysis)3-Drug-induced anemia4-Autoimmune anemia5-Mechanical heart valve–related anemia

Clinical manifestation1-CVS:decrase blood volume------shift fluid

toward organ-----stimulate rennin angiotensin II----salt & water retention

Tachycardia, murmur, angina, palpitation2-RESP:extertional dyspena, tachyapnea3-CNS:headache,dizznes,fainting,fatigue,Numbness, cold sensitivity, loss of

concentration4-skin:pale skin, conjunctiva, impaired healing5-GIT :aneroxia, nausea, constipation6-GENITOURINARY:amenorrhea

Pallor - nail bed in a patient with anemia

Medical ManagementManagement of anemia is directed toward

correcting or controlling the cause of the nemia;

if the anemia is severe, the RBCs that are lost or destroyed may be replaced with a transfusion of packed RBCs (PRBCs).

NURSING PROCESS:THE PATIENT WITH ANEMIAAssessment:*Health history*physical examination(patient complains)*lab investigation

NURSING DIAGNOSES*Activity intolerance related to weakness,

fatigue, and general malaise• Imbalanced nutrition, less than body

requirements, related to inadequate intake of essential nutrients

• Ineffective tissue perfusion related to inadequate blood volume or hematocrit

• Noncompliance with prescribed therapy

Goals*increased tolerance of normal activity,* attainment or maintenance of adequate

nutrition,*maintenance of adequate tissue perfusion,

compliance with prescribed therapy, and absence of complications.

Nursing Interventions1-Managing fatigue2-Maintaining adequate nutrition3- 2-Maintaining adequate PERFUSION4- Monitoring & managing :POTENTIAL COMPLICATIONS(heart failure, paresthesias, and confusion)

Hypoproliferative Anemias1-IRON DEFICIENCY ANEMIAIt is alack of iron for hemoglobin production*The body can store about one fourth to one

third of its iron,

Causes1- Decrease HB synthesis or disorder of iron

metabolism1-Dietery insufficiency2- Menorrhea3-pregnancy4- Hiatus hernia5- Parasitic infestation6- Decrease absorption as gasterostomy7- hemorrhoid, ulcerative colitis& GIT bleeding

Pathophysiology1- Body iron stores for erythropiotin

depleted----- erythropoisis proceed normally with HB remain normal

2-Iron transportation to bone marrow decreased & decrease iron erythropoietin

3-Small HB deficient --- decrease number of RBCs ---- decrease HB production

Clinical manifestations1-pallor , weakness, fatigue2- shortness of breath , palpitation3- Nail brittle, spoon shaped , concave4- tongue red, sore, painful5- wide pulse pressure, tachycardia ,murmurs,

irritability6- headache ,angular stomatitis ,menstrual

disturbance

ANGULAR CHEILITIS AND SMOOTH TONGUE IN IRON DEFICIENCY

Diagnosis1- blood test: decrease(HB, HCT ,serum iron,

serum ferritin)2-bone marrow biopsy (serum ferritin)

Medical managements1 -Identify site of blood loss & correct it

2 -Iron replacement therapy 150-200 mg ferrous sulphate orally/3 times per day for three weeks

3 -Iron rich food

Nursing Management1- encourage patient to take Food sourceshigh in iron include organ meats (beef or calf’s

liver, chicken liver),other meats, beans pinto, leafy green vegetables, and molasses.

2-Taking iron-rich foods with a source of vitamin C enhances the absorption of iron

•

PATIENT EDUCATION

1-Take iron on an empty stomach (1 hour before or 2 hours after a meal). Iron absorption is reduced with food, especially dairy products.

2- To prevent gastrointestinal distress, the following schedule may work better if more than one tablet a day is prescribed:

Start with only one tablet per day for a few days, then increase to two tablets per day, then three tablets per day.

PATIENT EDUCATION

3- Increase the intake of vitamin C (citrus fruits and juices, strawberries, tomatoes, broccoli), to enhance iron absorption.

4- Eat foods high in fiber to minimize problems with constipation.

5- Remember that stools will become dark in color.

ANEMIA OF CHRONIC DISEASEthe chronic diseases of inflammation, infection, and

malignancy cause this type of anemia which characterized by low serum in iron

Many chronic inflammatory diseasesare associated with a normochromic, normocytic

anemia.During disease: iron &transferin taken up by

reticuloendothelial cell which account for plasma level

Ex:(rheumatoid arthritis; severe, chronic infections; and many cancers renal failuire).

It develops gradually over 6 to 8 weeks and then stabilizes at a HCT seldom less than 25%. The HBrarely falls below 9 g/dL,

APLASTIC ANEMIAIt is in sufficient erythropoiesis* It a decrease in or damage to marrow stem

cells, and replacement of the marrow with fat. It results in bone marrow aplasia( reduced in RBCs,WBCs,Platelet)

Causes1-Exposure to high dose of radiation2- treatment of cancer3- chemotherapy4- complication of viral hepatitis5- drugs of rheumatoid arthritis6-autoimmune disease

Clinical manifestation1-fatigue, pallor, dyspnea). 2-Purpura (bruising), ecchymosis, bleeding

orfice3-repeated throat infections, cervical

lymphadenopathy ,splenomegalysometimes occur&. Retinal hemorrhages are

commonDiagnostic finding: A bone marrow aspirate shows an extremely

hypoplastic or even aplastic (very few to no cells) marrow replaced with fat.

Medical Management1-bone marrow transplantaton (BMT) 2-peripheral stem cell transplantation (BSCT). 3- immunosuppressive therapy4-RBCs,platelet transfusion.Nursing Management:1- Assess carefully for signs of infection and

bleeding2-Encourge bed rest

MEGALOBLASTIC ANEMIAS It iscaused by deficiencies of vitamin B12 or folicacid, identical bone marrow and peripheral blood

changes occur ,because both vitamins are essential for normal DNA synthesis

the RBCs that are produced are abnormally largeand are called megaloblastic RBCs. Other cells derived from the myeloid stem cell

(nonlymphoid WBCs, platelets) are also abnormal, the precursor erythroid and

myeloid cells are large and bizarre in appearance

FOLIC ACID DEFICIENCY AnemiaIt is lack of erythropoises, premature cell deathIt is absorbed in small intestine and stored in

liver- it is important in synthiesis of DNA,RNAquickly depleted when the dietary intake of

folateis deficient (within 4 months)*Folate is found in green vegetables and liver

Causes1- vit loss in cooking 2- mal nutrition3- malabsorpation syndrome4-Alcohilsm 5-pregnancy 6- mal nutrition in elderly 7- colorectal

cancer

Clinical manifestation: malnourished appearance stomatitisDysphagia watery diarrhea

Medical management1-daily oral administration of folate preparation2-Use food contain cereal grain product3-Parenteral administration of folic acid

Vitamin B12 deficiency anemiapernicious anemiaIt is alack of vit B12 for erythropoises-Abnormal DNA, RNA synthesis in erythroblast &

premature cell death

(Intrinsic factor is normally secreted by cells within the gastric mucosa; normally it binds with the dietary vitamin B12 and travels with it to the ileum, where the vitamin is absorbed. Without

intrinsic factor, orally consumed vitamin B12 cannot be absorbed, and RBC production is eventually diminished).

Causes1- congenital or acquired deficiency of intrinsic

factors needed to absorb it2- gastrectomy 3- hereditary atrophic gastritis4- mal absorption syndrome Clinical manifestation (neurological assessment)1- weak muscles 2-numbness3- tingling in hands & feet * damage in spinal

cord 4- difficulty walking, parathesia5-Wt loss 6-abdominal pain 6-nausea,diarrhea 7-Beefy tongue liver& spleen enlarged

Diagnostic finding:1- blood test 2- bone marrow examination3- serolgic studies( antibodies in gastric juice)4- Gastric biobsy 5- Schilling test,Medical Managementincreasing the amount of folic acidin the diet and administering 1 mg of folic acid dailyNursing Management- assess patient : skin and mucous membranes.-Mild jaundice may be apparent and is best seen in the sclera-. Vitiligo (patchy loss of skin pigmentation)- premature graying of the hair are often seen in patientswith pernicious anemia. -The tongue is smooth, red, and sore, loss of skin

pigmentation

Vitamin B-12 Deficiency acanthtosis (hypopigmentation)

COMPARISON OF FEATURES OF VITAMIN B12 AND FOLIC ACIDDEFICIENCY STATES

Hemolytic Anemia it is caused by increased RBCs break down or

hemolytic , erythrocyte survival is shortened to less than 15-20 days

Two types:1- intrinsic: destruction of RBCs ----defect within

RBCs themselvesInherited as sickle cell , thalessemia( RBCs does n’t live as long as normal RBCs

Hemolytic Anemia2-Extrinsic: RBCs produce healthy but destroyed

by trapped in spleen from infection, drugs or autoimmune disorders

SICKLE CELL ANEMIA it is gentic inhirted disorder in that the body

produces RBCs that shaped like sickle ( hard, sticky& not move easily through blood vessels

they tend to stuck& flow to limb & organs

pathophsiology

*The sickle hemoglobin(HbS) acquires a crystal-like formation when exposed to lowoxygen tension. *The oxygen level in venous blood can be lowenough to cause this change; *consequently, the RBC containing (HbS) loses its round,

very pliable, biconcave disk shape and becomes deformed, rigid, and sickle-shaped (Fig. 33-5).

*These long, rigid RBCs can adhere to the endothelium of small vessels;

*when they pile up against each other, blood flow to a regionor an organ may be reduced* If ischemia or infarction results, the patient may have pain,swelling, and fever.

Clinical Manifestations1-The HbS gene is inherited in people of African

descent and to a lesser extent in people from the Middle East

2-chronic leg ulcer arround ankles3-tissue hypoxia 4- hematuria5- enlargement of the bones of the face and skull.6-tachycardia, cardiac murmurs, and often anenlarged heart (cardiomegaly). Dysrhythmias and

heart failure,7-bone pain, infarction 8- fever, infection,

jaundice

THE PATHOGENESIS AND CLINICAL CONSEQUENCES OF SICKLECELL DISEASE

Sickle Cell (hand and foot syndrome

Summary of Complications in Sickle Cell Anemia

S&S mechanism organAbdominal pain; fever, signs ofInfection

Primary site of sickling →infarctions→↓ phagocytic function of macrophages

spleen

Chest pain; dyspnea InfectionInfarction→↑pulmonary pressure→pulmonary hypertension

lungs

Weakness (if severe); learningdifficulties (if mild

Infarction Centeral nervous system

Dehydration Sickling→damage to renal medulla kideny

Weakness, fatigue, dyspneaAnemia heart

Ache ↑ Erythroid production bone

Bone pain, especially hipsInfarction of boneHemolysis

liver

Abdominal pain ↑ Viscosity /stasis →infarction→skin ulcers

Skin&peripherial vascular

↓ Vision; blindnessInfarction eye

Pain, impotenc Sickling penin

Assessment and Diagnostic Findings1- Blood analysis: low hematocrit2- microscopic examination: sickled cells on the

smear.

Medical Management1-PHARMACOLOGIC THERAPYHydroxyurea (Hydrea), a chemotherapy agent, toincreasing hemoglobin F levels, &decreasing the

permanent formation of sickled cells.2- TRANSFUSION THERAPY3- daily folic acid replacements to maintain the

supply required for increased erythropoiesis from hemolysis

4- Incentive spirometry has been shown to decrease

the incidence of pulmonary complication.5-Antibiotics

SUPPORTIVE THERAPY

1-maintain adequate amounts of fluids; intravenous hydration withdextrose 5% in water (D5W) or dextrose 5% in 0.25 normal salinesolution (3 L/m2/24 hours)

2-Opioid analgesic to reduce pain3- Aspirin is very useful in diminishing mild to

moderate pain; inflammation and potential thrombosis (due to its ability to diminish platelet adhesion).

4- Nonsteroidal anti-inflammatory drugs (NSAIDs

NURSING PROCESS:THE PATIENT WITH SICKLE CELL CRISISAssessment:1- Pain levels should always be

monitored(joints, abdomen,);2- The respiratory system must be assessed

carefully, including auscultation of breath sounds, measurement of oxygen saturation levels, and signs of cardiac failure, such as the presence and extent of dependent edema, an increased point of maximal impulse, and

cardiomegaly

Assessment:

3- assessed for signs of dehydration by a history of fluid intake and careful examination of mucous membranes, skin turgor, urine output,

and serum creatinine and blood urea nitrogen values.

4-neurological , chest examination

NURSING DIAGNOSES1-Acute pain related to tissue hypoxia due to

agglutination of sickled cells within blood vessels

2-Risk for infection3- Risk for powerlessness related to illness-

induced helplessness4- Deficient knowledge regarding sickle crisis

prevention

Planning and Goalsdecreased incidence of crisis, enhanced sense

of self-esteem and power, and absence of complication

Nursing intervention1- Managing pain2-preventive & managing infevction3- Promoting coping skills4-Monitor& managing complications:Hypoxia, ischemia, infection, and poor wound healing leadingto skin breakdown and ulcers• Dehydration• (CVA, brain attack, stroke)• Anemia • Renal dysfunction• Heart failure, pulmonary hypertension, and acute chestsyndrome• Impotence • Poor compliance• Substance abuse related to poorly managed chronic pain

THALASSEMIAThe thalassemias are a group of hereditary disorders

associated with defective hemoglobin-chain synthesis abnormal decrease in the hemoglobin content of RBCs), extreme microcytosis

(smaller-than-normal RBCs), destruction of blood

elements (hemolysis), and variable degrees of anemia*the production of one or more globulin chainswithin the hemoglobin molecule is reduced.The alpha thalassemiasoccur mainly in people from

Asia and the Middle East; the beta-thalassemias are most prevalent in

Mediterranean populations

GLUCOSE-6-PHOSPHATEDEHYDROGENASE DEFICIENCYThe abnormality in this disorder is in the G-6-PD

gene; this gene produces an enzyme within the RBC that is essential for membrane stability.

A few patients have inherited an enzyme so defective that they have a chronic hemolytic anemia