HEMATOLOGIC DISORDERS

ANEMIAS
Clinical condition that results from
1. Insufficient supply of healthy RBC
2. Volume of packed RBC
3. Quantity of hemoglobin
HYPOXIA develops
Not a disease in itself!

Risk Factors:
Prevalence increases with age (>65 y/o)
Sickle cell disease & Thalassemia high in African Americans
Thalassemia also high in Mediterranean
Pernicious Anemia high in Scandinavians and African Americans

Etiology
Decreased RBC production
o Defective DNA synthesis
Cobalamin/ Vitamin B12 deficiency
Folic Acid deficiency
o Decreased hemoglobin synthesis
Iron deficiency
Thalassemia (decreased globin synthesis)
Sideroblastic anemia (failure to completely form heme)
o Decreased Number of Erythrocyte Precursors
Aplastic anemia
Anemia of leukemia and myelodysplasia
Chronic diseases
Increased RBC destruction (Hemolysis)
o Intrinsic
Abnormal hemoglobin (Sickle cell anemia)
Enzyme deficiency
Membrane abnormalities
o Extrinsic
Physical trauma
Antibodies (autoimmune)
Infectious agents
Toxins (snake venom; chemotherapy)
Blood loss
o Acute
Trauma
Blood vessel rupture
o Chronic
Gastritis
Hemorrhoids
Menstruation
Morphologic Classification
Normocytic/normochromic normal size and color
Macrocytic/normochromic large size, normal color
Microcytic/hypochromic small size, pale color

S/sx:
MILD ANEMIA (Hb levels: 10-14 g/dl)
o Usually asymptomatic; s/sx occur following a strenuous exertion
MODERATE ANEMIA (Hb levels: 6-10 g/dl)
o Chornic fatigue
o Dyspnea (SOB)
o Diaphoresis with exertion
o Palpitations
SEVERE ANEMIA (Hb <6 g/dl)
o Pallor, severe fatigue, malaise, weakness, lightheadedness, fever, exertional dyspnea,
H/A, vertigo, sensitivity to cold, weight loss

MX MNGT: (Goals)
Alleviate and Control the causes
o Supplemental iron therapy
o Nutritional therapy
o Surgery to repair sites of hemorrhage
o Splenectomy
o Removal of toxic agents that cause aplasia
o Stem cell or bone marrow transplantation
o Corticosteroid therapy
o Immunosuppressive therapy
Relieve manifestations
o O2 therapy
o Erythropoietin
Action: Increases the production of RBC
SQ injections
Prerequisites:
Client must have bone marrow capable of producing RBCs
Sufficient nutrients for the production of RBCs
o Iron replacement
Oral form: given for mild anemia
Ferrous sulfate (Feosol), Ferrous gluconate (Fergon), ferrous fumarate
With or after meals
Take with Vitamin C or orange juice aids in the absorption of iron
Clients usually receive iron supplements for at least 6 months for
repletion of the body stores
Do not administer with milk, antacid will inhibit absorption
SE: N/V, constipation or diarrhea, and blackened stools
o NC:
Drink ginger tea or suck on ginger candy to avoid or
minimize N/V
Stool softeners to avoid constipation
Oral liquid form should be administered with straw to prevent
permanent staining of the teeth
Parenteral iron therapy
Iron dextran (Imferon), sodium ferric gluconate, iron sucrose
Z-track (Deep IM)
Do not massage site of injection to prevent leakage of medication
into SQ layer
Does not induce GI effects
Associated with hypersensitivity reactions
Blood component therapy
o Blood transfusion

ANEMIA CAUSED BY DECREASED ERYTHROCYTE PRODUCTIOON
A. IRON DEFICIENCY ANEMIA (IDA)
Chronic, hypochromic, microcytic anemia resulting from an insufficient supply of iron in
the body either inadequate absorption or excessive loss of iron
WITHOUT IRON! hemoglobin concentration in the RBCs is reduced & RBCs are unable
to oxygenate the bodys tissue
National Academy of Sciences RECOMMENDS iron intake
o 15 mg/day for women
o 10 mg/day for men
ETIOLOGY & RISK FACTORS
Insufficient dietary intake of iron
o Poor countries, diets lack meat
Blood loss
o Chronic blood loss of as little as 2-4 ml/day can result to IDA
o ! every 2 ml of blood=1 mg of iron
Impaired absorption of iron
o Chronic diarrhea
o Tannates (tea & coffee), carbonates, antacid (Magnesium trisilicate) hinder
non-heme iron absorption
Excessive demands for RBC production as a result of hemolysis
Women: most common etiologies! menstruation & pregnancy
o Normal iron excretion: <1 mg/day
o Iron is excreted in urine, sweat, bile, feces
o Woman loses 15 mg/month during menses
o About 500 mg of iron is lost during a normal pregnancy
PATHOPHYSIOLOGY
Iron is present in all RBCs as HEME in hb
Iron is vital for DNA synthesis & electron transport; regulated by the absorptive cells in
the proximal small intestine
o HEME 2/3 of the bodys iron
o FERRITIN, an Iron-phosphorus-protein complex 1/3 of the bodys iron (non-
heme)
S/SX
Mild cases asymptomatic
Severe cases
o Angular Stomatitis (inflammation of the mucosa of the mouth)
o Brittle nails
o Cheilitis (inflammation of the lips)
o Dypsnea
o Fatigue
o Glossitis (inflammation of the tongue)
o Headache
o Palpitations, Pallor in the face, Palm of the hand, nail bed, conjunctiva
o VINSON-PLUMMERS SYNDROME (SAD)
Stomatitis
Atrophic Glossitis
Dysphagia
MNGT
Diagnosis of and correction of the underlying cause
o GIT series, stool examination for occult blood, endoscopy to identify site of
blood loss
o Correction of alcoholism, malnutrition, hemorrhage
Promote rest reduce O2 demands
Provide good oral care prevent/relieve stomatitis
Treatment through diet & supplemental iron preparation
o Almonds, asparagus
o Bran, beans
o Carrots, cauliflower, celery
o Dandelions
o Egg yolk
o Graham bread
o Kidney
o Lettuce, liver
o Oysters, oatmeal
o Soybeans, Spinach
o Whole wheat
B. B THALASSEMIA (COOLEY ANEMIA)
Autosomal-recessive genetic disorder that results in inadequate normal Hb production
IDA affects heme synthesis, thalassemia disrupts the synthesis of globin
ETIOLOGY & RISK FACTORS
Found in people of Mediterranean, African & Southeast Asian origin
Alpha-thalassemia inherit an alpha-gene
Thalassemia minor one beta-gene
Thalassemia major both beta-genes
S/SX
Bone marrow hyperplasia
Diarrhea
Failure to thrive, frequent infections
Hepatomegaly
Jaundice from RBC hemolysis
o Hemolysis results from an imbalance in alpha & beta globin chains, which are
normally paired
o Excess unpaired alpha & beta globin chains aggregate and form a precipitate
that damages RBC membranes
Pain
Splenomegaly
DX
Fetal diagnosis through amniocentesis after 8 weeks of gestation
MNGT
Provide adequate normal Hb for erythropoeisis
o Chronic transfusions to correct the anemia with targeted Hb level at 9-10
g/dl
Alleviate effects of iron overload (HEMOSIDEROSIS)
o Iron chelation with Deferoxamine to prevent iron overload
Given IV or SQ
Iron supplementation SHOULD NOT be used!
Splenectomy to decrease transfusion requirements
o Because RBCs may be sequestered in the spleen
o To increase the lifespan of supplemental RBCs
Genetic counseling and testing for families should be encouraged
C. MEGALOBLASTIC ANEMIA
Caused by impaired DNA synthesis resulting in defective, large RBCs (megaloblasts)
Caused by deficiencies of Vitamin B12 (Cobalamin) & Folic Acid
1. COBALAMIN/B12 DEFICIENCY (PERNICIOUS ANEMIA)
PA an autoimmune disorder characterized by absence of IF (intrinsic Factor) in
gastric secretions leading to malabsorption of cobalamin
At risk for Gastric Carcinoma!
ETIOLOGY & RISK FACTORS
90% have antibodies against parietal cells where IF is produced
60% have anti-IF antibodies
Associated with Human Leukocyte Antigen (HLA) & in type A blood groups
40-70 y/o (peak: 70 y/o)
Prevalent in people of Celtic and Scandinavian society
PATHOPHYSIOLOGY
Cobalamin (Extrinsic factor) released from its ingested protein-bound state by
gastric acid binds to IF, a glycoprotein produced by parietal cells absorbed in
the terminal ileum binds with protein TCII (transcobalamin II) transported to
storage sites (liver)
COBALAMIN responsible for DNA synthesis and cell replication
o RBC production
o DNA synthesis of maturing RBC (megaloblastic cells)
o Impairment of integrity of cells
S/SX
beefy, red, inflamed tongue (most common manifestation)
Presence of anemia: Hb, hct, RBC levels
Achlorhydria (absence of HCl) * HCl & IF are produced by the parietal cells
GI manifestations (wt loss, appetite loss, N/V, abdominal distention, diarrhea,
constipation, steatorrhea)
Neurologic disorders (paresthesias of feet and hands [tingling, numbness], poor
gate, memory loss, cognitive problems, depression)
DX
SCHILLING TEST definitive test for PA
o Measures the absorption of orally administered radioactive Vitamin B12
(tagged with Cobalt 60) BEFORE & AFTER parenteral administration of IF
o IM nonradioactive Vitamin B12 eliminates the radioactive Vitamin B12
into the urine
o Collect 24-hour urine specimen
o excretion of Vitamin B12 (+) PA
Gastric Secretion Analysis
o To check for the presence of free HCl
o Most with PA have low volume gastric secretions with high pH and free HCl
acid levels
o These findings DO NOT change, even after administration of HISTAMINE,
which normally stimulates gastric secretion
MX MNGT
Cobalamin/Vitamin B12 therapy
o Parenteral administration of Cobalamin (Cyanocobalamin or
hydorxocobalamin) 1000 mcg/day for 2 weeks then weekly until Hct
returns to normal. Once Hct is normal, monthly injections for life are
required
o Intranasal form of cyanocobalamin (Nascobal) available as a nasal gel
that is self-applied weekly
Digestants
o Enhance the metabolism of vitamins
o Ex. HCl acid may be diluted in water and given with meals during the first
few weeks of Vitamin B12 therapy
Iron supplements
Diet
o Red meats, especially liver
o Eggs
o Dairy products
2. FOLIC ACID DEFICIENCY ANEMIA
Aka macrocytic, hypochromic anemia
ETIOLOGY & RISK FACTORS
Poor dietary intake
Pregnancy
Chronic alcohol
Hemodialysis folic acid is lost during dialysis
Malabsorption syndromes (celiac disease, steatorrhea)
Medications
o Long term use of anticonvulsants (phenytoin, phenobartbital)
o Antimetabolites (purine, pyrimidine analogs) to clients with cancer
o OC
S/SX
Slow and insidious onset
Cracked lips and sore tongue
Same with PA but does not cause neurologic manifestations
DX
Serum folate < 4 ng/ml (Normal: 7-20 ng/ml)
MGNT
Parenteral folic acid - IM
o Folinic acid (Leucovorin calcium injection)
Oral folic acid
o 0.1 5 mg/day
Vitamin C
Diet
o Green leafy vegetables, liver, citrus fruits, nuts, grains & yeast

ANEMIA CAUSED BY INCREASED ERYTHROCYTE DESTRUCTION
A. SICKLE CELL DISEASE (SCD)
Group of inherited disorders of mutant hemoglobin (hemoglobin S [HbS]) that causes
the characteristic sickling of RBCs
Sickle cell anemia an autosomal recessive disorder (homozygous for Hbs) most
common variant
Sickle cell trait heterozygous, milder form, carrier state
SCD is lifelong condition
ETIOLOGY & RISK FACTOR
Found in races of people where malaria is endemic (Mediterranean, Middle East, Africa
& India)
Life expentancy: Male 42 years; females 48
Leading cause of death: acute chest syndrome
PATHOPHYSIOLOGY
Defective hemoglobin (Hbs) when exposed to decrease O2 becomes vioscous, has
decreased solubility & forms a gel-like substance containing hemoglobin crystals within
the affected RBCs crystals will clump together which disrupts the membrane cell
assumes a crescent or sickle shape (rigid, sticky & fragile) impede circulation in
capillaries resulting to microinfarcts, tissue hypoxia & further sickling tissue
necrosis of vital organs
Sickle cell crisis: acute episodic exacerbation of the disorder
o Causes: high altitudes, exercising strenuously, respiratory infection, anesthesia
o Or is the results of vaso-occlusive crisis (pain crisis)
Induced by stress, exposure to cold water or temp, infection
Organ damage syndromes hallmark of SCD
S/SX
Chronic hemolytic anemia
o Pallor, fatigue, exercise intolerance, jaundice
Organ damage syndrome
o Pulmonary, cerebrovascular, cardiovascular, renal complications
Painful sickle cell crisis
o Requires immediate attention
o Severe pain in long bones, joints, the chest, the back and the abdomen
o Hand-foot syndrome: painful bone infarction (first symptom of SCD)
o Fever, malaise, & leukocytosis
Aplastic crisis
o Serious complication caused by infection with parvovirus B19
o The virus infects red cell progenitors in the bone marrow cessation of
erythropoiesis
o Self-limiting because bone marrow recovers in 7-10 days
Sequestration crisis
o Sudden & massive trapping of destroyed RBC components by visceral organs;
splenic sequestration (common & painful complication) splenomegaly &
hepatomegaly
Hemolytic crisis
o Abnormal destruction of RBC (hemolysis)
o Client is febrile
Acute chest syndrome
o Chest pain, fever, cough, tachypnea, leukocytosis, & pulmonary infiltrates
Hyperactivity of the bone marrow
o Spindly legs, short trunk & a tower-shaped skull
Impaired circulation
o Edema of hands and legs, ulcers
DX
Blood smear presence of sickle cells
Sickle-turbidity tube test blood is mixed with sickledex solution in a test tube
o 5 minutes later: + cloudiness presence of Hbs
Hemoglobin electrophoresis diagnostic test for Hbs
MX MNGT
Reduce pain
o Morphine sulfate (drug of choice) per IV
o Analgesics & NSAIDS
o Nonpharmacological measures
Physical therapy, acupuncture, hypnosis
Pharmacologic agents
o Hydroxyurea
Increases the production of HbF, which retards sickling, Hb level
increases, & vaso-occlusive crises becomes less frequent
SE: leucopenia, thrombocytopenia
o Sodium cromoglycate
Reduces the percentage of sickle cells in venous blood
Given by inhalation
o Folic acid & Iron supplementation
Blood transfusions
o Goal of transfusion is reduce the level of HbS to 30% or less
Prevent infections
o Penicillin prophylaxis
o Pneumococcal vaccination at 2 years with booster at 5 years
Vaso-occlusive crisis
o Treated with hydration & analgesics
Explain how to prevent crises
o Avoiding lower oxygen tension
o Taking caution against becoming dehydrated
NX MNGT
Emphasize importance of adequate hydration to prevent sickling
Heat application to affected area is soothing; cold compresses are not applied because
this enhances sickling & vasoconstriction
Passive ROM
B. POLYCYTHEMIA VERA
Excessive production of erythrocytes, leukocytes, & platelets
Caused by excessive activation of pluripotent stem cells in the bone marrow
1. Increase in blood viscosity
2. Increase in total blood volume
3. Severe blood congestion of all tissues and organs
ETIOLOGY & RISK FACTORS
50-70 y/o
S/SX
Dizziness, headache, vertigo, tinnitus, visual disturbance, angina pectoris
Ruddy complexion & dusky red mucosa
Hypertension & heart failure (SOB)
Increased clotting
Bleeding
Hepatosplenomegaly
Skeletal gout (painful swollen joints, usually the big toe)
DX
RBC count: 8-12 M/mm3
Hb level: 18-25 g/dl
Hct: >54% in men > 49% in women
Uric acid level 3-4x normal
MNGT
Reduction of blood volume & viscosity
o Phlebotomy
Administration of myelosuppresive agents (hydroxyurea) combined with aspirin for
platelets to avoid thrombotic or hemorrhagic complications
Radiation therapy (Na Phosphate/IV)
Allupurinol for hyperurecemia
Avoid high altitude, low O2 levels tend to initiate kidneys to increase secretion of
erythropoietin