Management of Hematologic Disorders Anemia

Basophil (WBC)

BY/ Dr Hend elshenawie

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

Platelet (thrombocyte)

RBCs Basophil (WBC)

Neutrophil (WBC)

B-Lymphocyte (WBC)

Monocyte (WBC) Eosinophil (WBC)

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 deficiency 2-Vitamin B12 deficiency (megaloblastic) Folate deficiency 3-Decreased erythropoietin production (eg, from renal dysfunction ,Cancer /inflammation

2-Bleeding (Resulting From RBC Loss

Bleeding 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 anemia 4-Autoimmune anemia 5-Mechanical heart valverelated anemia

Clinical manifestation
1-CVS:decrase blood volume------shift fluid toward organ-----stimulate rennin angiotensin II----salt & water retention Tachycardia, murmur, angina, palpitation 2-RESP:extertional dyspena, tachyapnea 3-CNS:headache,dizznes,fainting,fatigue, Numbness, cold sensitivity, loss of concentration 4-skin:pale skin, conjunctiva, impaired healing 5-GIT :aneroxia, nausea, constipation 6-GENITOURINARY:amenorrhea

nail bed in a patient with anemia

Pallor -

Medical Management
Management 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 ANEMIA

Assessment: *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 Interventions
1-Managing fatigue 2-Maintaining adequate nutrition 3- 2-Maintaining adequate PERFUSION 4- Monitoring & managing : POTENTIAL COMPLICATIONS (heart failure, paresthesias, and confusion)

Hypoproliferative Anemias 1-IRON DEFICIENCY ANEMIA

It is alack of iron for hemoglobin production *The body can store about one fourth to one third of its iron,

Causes
1- Decrease HB synthesis or disorder of iron metabolism 1-Dietery insufficiency 2- Menorrhea 3-pregnancy 4- Hiatus hernia 5- Parasitic infestation 6- Decrease absorption as gasterostomy 7- hemorrhoid, ulcerative colitis& GIT bleeding

Pathophysiology
1- 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 manifestations
1-pallor , weakness, fatigue 2- shortness of breath , palpitation 3- Nail brittle, spoon shaped , concave 4- tongue red, sore, painful 5- wide pulse pressure, tachycardia ,murmurs, irritability 6- headache ,angular stomatitis ,menstrual disturbance

ANGULAR CHEILITIS AND SMOOTH TONGUE IN IRON DEFICIENCY

Diagnosis
1- blood test: decrease(HB, HCT ,serum iron, serum ferritin) 2-bone marrow biopsy (serum ferritin)

Medical managements
1- 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 Management
1- encourage patient to take Food sources high in iron include organ meats (beef or calfs 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 DISEASE

the chronic diseases of inflammation, infection, and malignancy cause this type of anemia which characterized by low serum in iron Many chronic inflammatory diseases are 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 ANEMIA
It 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)

Causes
1-Exposure to high dose of radiation 2- treatment of cancer 3- chemotherapy 4- complication of viral hepatitis 5- drugs of rheumatoid arthritis 6-autoimmune disease

Clinical manifestation
1-fatigue, pallor, dyspnea). 2-Purpura (bruising), ecchymosis, bleeding orfice 3-repeated throat infections, cervical lymphadenopathy ,splenomegaly sometimes occur&. Retinal hemorrhages are common Diagnostic finding: A bone marrow aspirate shows an extremely hypoplastic or even aplastic (very few to no cells) marrow replaced with fat.

Medical Management
1-bone marrow transplantaton (BMT) 2-peripheral stem cell transplantation (BSCT). 3- immunosuppressive therapy 4-RBCs,platelet transfusion. Nursing Management: 1- Assess carefully for signs of infection and bleeding 2-Encourge bed rest

MEGALOBLASTIC ANEMIAS
It iscaused by deficiencies of vitamin B12 or folic acid, identical bone marrow and peripheral blood changes occur ,because both vitamins are essential for normal DNA synthesis the RBCs that are produced are abnormally large and 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 Anemia

It is lack of erythropoises, premature cell death It is absorbed in small intestine and stored in liver - it is important in synthiesis of DNA,RNA quickly depleted when the dietary intake of folate is deficient (within 4 months) *Folate is found in green vegetables and liver

Causes
1- vit loss in cooking 2- mal nutrition 3- malabsorpation syndrome 4-Alcohilsm 5-pregnancy 6- mal nutrition in elderly 7- colorectal cancer

Clinical manifestation: malnourished appearance stomatitis Dysphagia watery diarrhea

Medical management
1-daily oral administration of folate preparation 2-Use food contain cereal grain product 3-Parenteral administration of folic acid

Vitamin B12 deficiency anemia pernicious anemia

It 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).

Causes
1- congenital or acquired deficiency of intrinsic factors needed to absorb it 2- gastrectomy 3- hereditary atrophic gastritis 4- mal absorption syndrome Clinical manifestation (neurological assessment) 1- weak muscles 2-numbness 3- tingling in hands & feet * damage in spinal cord 4- difficulty walking, parathesia 5-Wt loss 6-abdominal pain 6-nausea,diarrhea 7-Beefy tongue liver& spleen enlarged

Diagnostic finding:
1- blood test 2- bone marrow examination 3- serolgic studies( antibodies in gastric juice) 4- Gastric biobsy 5- Schilling test, Medical Management increasing the amount of folic acid in the diet and administering 1 mg of folic acid daily Nursing 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 patients with 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 ACID DEFICIENCY 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 themselves Inherited as sickle cell , thalessemia ( RBCs does nt live as long as normal RBCs

Hemolytic Anemia
2-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 low oxygen tension. *The oxygen level in venous blood can be low enough 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 region or an organ may be reduced * If ischemia or infarction results, the patient may have pain, swelling, and fever.

Clinical Manifestations
1-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 ankles 3-tissue hypoxia 4- hematuria 5- enlargement of the bones of the face and skull. 6-tachycardia, cardiac murmurs, and often an enlarged heart (cardiomegaly). Dysrhythmias and heart failure, 7-bone pain, infarction 8- fever, infection, jaundice

THE PATHOGEN ESIS AND CLINICAL CONSEQUE NCES OF SICKLE

CELL DISEASE

Sickle Cell (hand and foot syndrome

Summary of Complications in Sickle Cell Anemia

organ
spleen

mechanism
Primary site of sickling infarctions phagocytic function of macrophages Infection Infarctionpulmonary pressure pulmonary hypertension Infarction Sicklingdamage to renal medulla Anemia

S&S
Abdominal pain; fever, signs of Infection Chest pain; dyspnea

lungs

Centeral nervous system kideny heart

Weakness (if severe); learning difficulties (if mild Dehydration

Weakness, fatigue, dyspnea

bone
liver

Erythroid production
Infarction of bone Hemolysis Viscosity /stasis infarction skin ulcers Infarction

Ache

Bone pain, especially hips Abdominal pain

Skin&peripherial vascular eye penin

Vision; blindness Sickling Pain, impotenc

Assessment and Diagnostic Findings

1- Blood analysis: low hematocrit 2- microscopic examination: sickled cells on the smear.

Medical Management
1-PHARMACOLOGIC THERAPY Hydroxyurea (Hydrea), a chemotherapy agent, to increasing hemoglobin F levels, &decreasing the permanent formation of sickled cells. 2- TRANSFUSION THERAPY 3- 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 pain 3- 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 CRISIS

Assessment: 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 DIAGNOSES
1-Acute pain related to tissue hypoxia due to agglutination of sickled cells within blood vessels 2-Risk for infection 3- Risk for powerlessness related to illness-induced helplessness 4- Deficient knowledge regarding sickle crisis prevention

Planning and Goals

decreased incidence of crisis, enhanced sense of selfesteem and power, and absence of complication

Nursing intervention
1- Managing pain 2-preventive & managing infevction 3- Promoting coping skills 4-Monitor& managing complications: Hypoxia, ischemia, infection, and poor wound healing leading to skin breakdown and ulcers Dehydration (CVA, brain attack, stroke) Anemia Renal dysfunction Heart failure, pulmonary hypertension, and acute chest syndrome Impotence Poor compliance Substance abuse related to poorly managed chronic pain

THALASSEMIA
The 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 chains within 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-PHOSPHATE DEHYDROGENASE DEFICIENCY

The 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