Disorders of the Hematopoietic System

3/11/2009 Disorders of the Hematopoietic System Chapter 37 Blood Disorders Blood Dyscrasias: abnormalities in the numbers and types of red blood cel...
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3/11/2009

Disorders of the Hematopoietic System Chapter 37

Blood Disorders Blood Dyscrasias: abnormalities in the numbers and types of red blood cells  Coagulopathies: bleeding disorders that involve platelets or clotting factors 

Anemia Deficiency of erythrocytes or hemoglobin  Reduction in amount of O2 carried to cells  Causes 

  

Blood loss Inadequate or abnormal erythrocyte production Destruction of normally formed RBCs

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Anemia Lab Interpretation 

MCV: size   



Microcytic Normocytic Macrocytic



Hemoglobin



Hematocrit





Carries O2 Volume of RBCs

MCHC: color   

Hypochromic Normochromic Hyperchromic

Hypovolemic Anemia 

Occurs when there is a loss in blood volume  



Acute Chronic

Assessment Findings 





Severe: EXTREME pallor, tachycardia, hypotension, ↓urine output, altered LOC Chronic: pallor, fatigue, chills, postural hypotension, tachycardia, tachypnea CBC: ↓Hgb, ↓Hct

Hypovolemic Anemia 

Medical Management   



IV fluids Blood transfusions Treatment of underlying conditions

Nursing Management

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Hypovolemic Anemia 

Medical Management  



Blood transfusions Restore blood volume

Nursing Management   

Vital signs Control acute bleeding Limit activity that increases O2 consumption

Iron Deficiency Anemia 

Insufficient iron for hemoglobin production Blood loss Inadequate dietary intake  Malabsorption  Hemolysis  

Iron Deficiency Anemia 

Assessment Findings Reduced energy, fatigue Feel cold all the time  Dyspnea with minor exertion  Rapid heart rate  



Diagnostic Findings 

CBC, serum iron, blood smear

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Iron Deficiency Anemia 

Medical Management  



Iron: Oral, IM Iron rich foods in diet: meat, egg yolks, shellfish, whole grains, nuts, legumes

Nursing Management 

Patient Teaching  Dilute oral iron, drink through straw  Orange juice to promote absorption  Change in stool color  Avoid antacid, coffee, tea, milk for 2 hr between iron use

Z-Track Technique

Sickle Cell Anemia Hereditary disease Primarily in African Americans; can occur in Meditarranean & Middle Eastern countries  Sickle Cell Disease 







Inherit 2 defective genes (one from each parent); all Hgb is abnormal

Sickle Cell Trait 

Inherit 1 defective gene

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Sickle Cell Anemia Sickle Cell Crisis  Vascular occlusions occur rapidly under hypoxic conditions  SX: Severe pain in ischemic tissue, necrotic tissue, fever, swelling in joints  Complications: Stroke, pulmonary infarct, shock, renal failure

http://library.thinkquest.org/06aug/0044 0/images/sicary.jpgklecellincapill http://learn.genetics.utah.edu/units/disorders/whataregd/sicklecell/images/sicklecell.jpg

Sickle Cell Anemia Assessment Findings  Jaundice  Gallstones  Predisposition to infection  Chronic leg ulcers  Priapism  Acute chest syndrome

Diagnostic Findings  Sickledex Test  ↓ Hgb

http://student.bmj.com/issues/03/12/education/images/view_20.jpg

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Sickle Cell Anemia 

Medical Management    

Supportive treatment Regular blood transfusions Bone marrow transplantation Antibiotic therapy

Sickle Cell Crisis 

Management    

Narcotic analgesia O2, bed rest IV fluids Desferal

Sickle Cell Anemia Nursing Management  Health history  Physical assessment  Patient teaching     

Adequate hydration Avoid vigorous exercise Avoid vasoconstriction, stop smoking Pneumonia vaccine Seek immediate medical attention for signs of infection

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Hemolytic Anemia Erythrocytes are destroyed prematurily  Spleen becomes obstructed and damaged resulting in an increased risk of infection  Persistent anemia causes: 

  

Tachycardia Dyspnea Cardiomegaly & dysrhythmias

http://www.wadsworth.org/chemheme/heme/microscope/pix/schistocyte_nw.jpg

http://www.medvet.umontreal.ca/clinpath/banq-im/Images/acanschzf.jpg

Hemolytic Anemia 

Assessment Findings   



Jaundice Enlarged spleen Shock

Diagnostic Findings  

Microscopic examination Positive Coombs test

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Hemolytic Anemia 

Medical Management    



Remove the cause Corticosteroids Blood transfusion Splenectomy

Nursing Management  

Health history Supportive care

Thalassemias Genetic disorder that results in abnormal Hgb synthesis  Hereditary  Alpha-thalassemias 





asymptomatic

Beta-thalassemias  

Mild: asymptomatic Severe: Cooley’s anemia

Thalassemias Diagnosis  Hgb electrophoresis  Medical Management 

 



Frequent blood transfusions Cooley’s: iron chelating therapy

Nursing Management  

Bedrest Protect from infection http://www.scinfo.org/chelate/img023.gif

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Pernicious Anemia Lack of intrinsic factor necessary for absorption of vitamin B12  Assessment 

   



Glossitis (inflammation of tongue) Stomatitis Digestive disturbances Neuromuscular changes

Diagnostic findings 

Schilling test

http://content.answers.com/main/content/img/elsevier/dental/f0031-01.jpg

Pernicious Anemia 

Medical Management



Nursing Management





 

Vitamin B12 injections for life If glossitis and stomatitis are present: soft, bland diet Small frequent meals Oral care

Folic Acid Deficiency Anemia 

Characterized by immature erythrocytes

Four common causes 1. Poor nutrition 2. Malabsorption syndromes 3. Drugs that impede absorption & use of folic acid 4. Alcohol abuse & anorexia

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Folic Acid Deficiency Anemia 

Assessment Findings 

Similar to pernicious anemia



Smooth, beefy-red tongue



Absence of neurologic problems



Medical Management



Nursing Management



  

Folic acid supplement Dietary choices high in folic acid Good oral hygiene Rest periods if fatigued easily http://www.doctorspiller.com/images/OralAnatomy/AtrophicGlossitis.jpg

Polycythemia Vera Greater than normal number of erythrocytes, leukocytes & platelets  Assessment Findings 



Reddish-purple face and lips



Fatigue, dizziness, exertional dyspnea, Pruritis Excessive bleeding Hemorrhoids Splenomegaly Swollen joints

    

Polycythemia Vera 

Medical Management    



Phlebotomy Anticoagulants Radiation therapy Antineoplastic drugs

Nursing Management  

Patient education Observe client for complications

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Leukemia Any malignant blood disorder in which rapid reproduction of leukocytes (usually immature) is unregulated  Cause is unknown  Predisposing factors 



Genetics Exposure to chemicals/radiation



Viruses



Leukemia 

Hallmark Assessment Findings   



Infection Severe anemia with fatigue Easy bruising

Diagnostic Findings 

↓ leukocytes, erythrocytes, platelets

Leukemia 

Medical Management   



Antineoplastic drugs Transfusions Bone marrow & stem cell transplants

Nursing Management   

Risk for infection Risk for hemorrhage Disturbed body image, anxiety, fear

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Multiple Myeloma 

Malignancy involving plasma cells (Blymphocytes) in the bone marrow.  

Poor prognosis Survival of 1-5 years

http://lh6.ggpht.com/_p163cKQmQ3k/RqCs5euktOI/AAAAAAAAAo8/Tem3O_R9NXw/Multiple+Myeloma+punch+out+lesions.jpg

Multiple Myeloma 

Assessment Findings    



Skeletal pain Pathologic fractures Infection Anemia

Diagnostic Findings   

X-ray: honeycomb bone lesions ↑ serum calcium, low blood counts Bence Jones protein

Multiple Myeloma 

Medical Management   

 

Chemotherapy Radiation Bone marrow & stem cell transplant Analgesics Antibiotics



Nursing Management    

Pain Hydration Signs of infection Safety to prevent injury/fracture

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Agranulocytosis 



Most common cause: toxicity from drugs 

Antibiotics, anti-thyroid agents, psychotropic agents, anti-convulsants



Chemotherapy agents

Assessment Findings   

Infection, fever, chills General malaise, fatigue Opportunistic infections: mouth, throat, nose, rectum, and vagina

Agranulocytosis 

Medical Management  



Removal of the cause Neupogen

Nursing Management    

Medication history Protective isolation if leukocyte count is low Good hand washing Restriction of visitors and staff

Aplastic Anemia Insufficient number of erythrocytes, leukocytes, and platelets  Consequence of inadequate stem cell production in the bone marrow  Exact cause is unknown 



Congenital: autoimmune



Acquired: exposure to toxic chemicals, radiation, and drug therapy

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Aplastic Anemia 

Assessment Findings  

 



Weakness, fatigue, Unusual bleeding, petechiae, ecchymosis Enlarged spleen Opportunistic infections

Diagnostic Findings  

CBC: insufficient blood cells Bone marrow aspiration

Aplastic Anemia 

Medical Management    



Withdrawal of the causative agent Transfusions Bone marrow & stem cell transplants Antibiotics

Nursing Management  

Assess for anemia, infection, bleeding Soft foods and oral hygiene to avoid bleeding from gums

Coagulopathies A term used to describe a condition in which a component that is necessary to control bleeding is either missing or inadequate  Examples: 

 

Thrombocytopenia Hemophilia

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Thrombocytopenia 

Platelet manufacture by bone marrow is decreased  Aplastic anemia 



Platelet destruction by spleen is increased

Hematologic malignant disorders



Pregnancy



Infection



Drug induced



Severe burns



Chronic alcoholism



HIV



Chemotherapy or radiation



Splenomegaly

Thrombocytopenia 

Assessment Findings 

Purpura



Bleeding

http://podiatry.files.wordpress.com/2007/03/backtcp.jpg

Thrombocytopenia 

Medical/Surgical Management   



Eliminate cause when possible Platelet transfusions Splenectomy

Nursing Management 

Minimize risk for bleeding

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Hemophilia A disorder involving clotting factors  Hereditary: sex-linked recessive characteristic  Three types 

  

Hemophilia A (von Willebrand’s) Hemophilia B (Christmas disease) Hemophilia C (Rosenthal’s disease)

Hemophilia Clinical manifestations: 

Slow, persistent bleeding from minor trauma and small cuts



Bleeding into joints



Delayed bleeding after minor injury



Uncontrollable hemorrhage after dental extraction



Epistaxis



GI bleeding



Hematuria

http://www.accessmedicine.com/loadBinary.aspx?name=licha&filename=licha_XI.050.jpg

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Hemophilia 

Medical Management   



Transfusions Thrombin application Direct pressure/cold compress/ice to bleeding site

Nursing Management  

Health history, last bleeding episode Patient teaching

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