Hematological System Disorders

Prepared by Greg Zilberman, RN BSN

1
Overview of Anatomy and Physiology: Lymph nodes
Hematopoietic system includes:
 Blood
 Blood vessels
 Blood-forming organs

Spleen

Thymus gland

Liver

Major function of Blood

 Carry O2 and nutrients to cells
 Remove CO2 (carbon dioxide) and
metabolic waste products.
Bone marrow  Hormone transport
 Inflammatory and Immune responses
 Temperature regulation
 Fluid-electrolyte balance
 Acid-base balance

2
Bone Marrow:  Yellow marrow is red marrow that has
 Contained inside all bones changed to fat.
 Occupies interior of spongy bones and center of  Found in long bones
long bones All blood cells start as stem cells in the bone
 4-5 % of total body weight marrow.
 Primary function is hematopoiesis
 Two kinds of Bone marrow:
Red (functioning) marrow:

 Carries out hematopoiesis

 Production site of:
 Erythroid
 Myeloid
 Thrombocytic
 Found in ribs, vertebrae, other flat bones.

Yellow marrow:

3
Blood

4
Hematocrit:  Albumin:
Regulate intravascular volume. Maintains
osmotic pressure
 Globulins:
Alpha globulins transport:
Steroids
Lipids
Bilirubin
Beta globulins transport:
Iron and copper
Gamma globulins:
Immune response
Antibody function
 Fibrinogen play role in
Clot formation
Produces in the liver
 Prothrombin play role
Normal coagulation
 The hematocrit (Ht or HCT) Produced in the liver
 Packed cell volume (PCV) Depends on vitamin K
 Erythrocyte volume fraction (EVF) Cellular component:
 The proportion of blood volume that is  Erythrocytes (RBC)
occupied by red blood cells.
Normal Values
Male-39%-54%
Female-34%-47%

Plasma:
Liquid portion of the blood

 Responsible for O2 transport via Hgb.

 male 4.6-6.2 millions/mm³
 female 4.2-5.4 millions/mm³
 Hemoglobin (Hgb):
 male adult 13-18 g/dl
 female 12-16 g/dl
 Average life spans 120 days
 Serum (liquid portion of plasma)  Matured cells removed by liver and spleen.
 Fibrinogen  Normal Erythrocytes Sedimentation Rate (ESR)
 Proteins 0-10 mm/hr

5
  Leukocytes (WBC)
Normal Value Leukocytes
 Total leukocytes: 4.00-11.0 x 10 9/L
 Neutrophils: 2.5–7.5 x 10 9/L
 Lymphocytes: 1.5–3.5 x 10 9/L
 Monocytes: 0.2–0.8 x 10 9/L
 Eosinophils: 0.04-0.4 x 10 9/L
 Basophils: 0.01-0.1 x 10 9/L
Thrombocytes (Platelets)
Essential factor in coagulation via:
 Adhesion
 Aggregation
 Plug formation
Normal value:
150,000-400,000 cells/l
6
 Blood Groups
Erythrocytes carry antigen that determine different
blood groups.
 Antigens are labeled as A and B.
 Absence of both antigens is type O blood.
 Presence of both antigens is type AB blood.
 Presents of A antigen is type A blood.
 Presents of B antigen are type B blood.
 People who have type O blood
are- universal donors
 People who have type AB blood are
universal recipients.
 Rh Typing
 Rh blood type checks for the Rh antigen
(also called the Rh factor) on red blood
cells.
 If red blood cells have:
Rh antigen-blood is Rh-positive
 If red blood cells do not have the Rh antigen
is Rh-negative.
For example:
if you have the blood type A and
Rh antigens your blood type is:
A-positive (A+).
If your blood has the B antigen
but not the Rh antigen, your blood type is
B-negative (B–).
 Blood Coagulation
 Conversion of fluid blood into a solid
clot to reduce blood loss when blood
vessels are ruptured.

Systems that initiate clotting

 Intrinsic system:
By contact activation following
endothelial injury.
 Factor XII initiates as contact made
between damaged vessel and plasma
protein
 Factors VIII, IX, and XI activated.
 Extrinsic system initiates by tissue
thromboplastins, released from
injured vessels.
 Factor VII activated

7
Coagulation studies:
Activated Partial Thromboplastin Time aPTT 21-35 sec
Prothrombin time PT 10-14 sec
International Normalized ratio INR 1-2 standard
Warfarin therapy 2-3
Bleeding time 3-7 minutes
Thrombin time 11-15 sec

8
Disorders of the Hematologic System
 Iron deficiency anemia o Drug therapy:
This anemia is the result of insufficient iron intake  Ferrous sulfate (Feosol)
or chronic blood loss (GI bleed)  Z-track IM Iron
 Risk Factors:  Nursing Implementation
o Alcohol abuse o Intake and output
o Gastrectomy o Vital signs
o Acute or chronic bleeding o Respiratory status
o Poor nutrition o Cardiovascular status
o Menstruation o Signs or symptoms of bleeding
o Pregnancy o Administer oxygen therapy
o Vitamin B6 deficiency o Encourage PO fluids
o Common in developing countries o Activity as tolerated
and Tropical zone (blood sucking o Monitor patient for falls
parasites)  Client education
 Clinical Manifestations: o Take Iron supp with orange juice
o Dizziness o Can cause black stool/constipation
o Koilonychias abnormal thinness and o Foul aftertaste
concavity of fingernails (brittle hair and  Pernicious anemia
nail)  This disorder is a progressive, chronic
o Pallor
macrocytic anemia (large cell anemia). It is
o Cheilosis scaling and fissures at the
corners of the mouth
a result of deficiency of intrinsic factor.
o Dry and pale mucous membranes  Risk Factors:
o Cold intolerance o Bacterial or parasitic infections
o Stomatitis o Deficiency of intrinsic factors
o Pica (eating non-food substances) o Genetics
 Diagnostic tests: o Gastric mucosal atrophy
o Hemoglobin and hematocrit decreased o Autoimmune disease
o Microcytic and hypochromic RBCs o Small bowel resection or total
 Treatments: o Gastrectomy
o Packed RBCs transfusion o Prolonged iron deficiency
o High iron diet  Clinical Manifestations:
 Breads & cereals o Tachycardia and palpitations
 Chick peas o Depression
 Chicken liver (cooked) o Delirium
 Dark green leafy vegetables o Constipation or diarrhea
 Egg yolks o Mouth sores
 Kidney beans o Dyspnea
 Meats o Pallor
 Sea vegetables o Mild jaundice
 Soy beans o Paralysis
 Tofu o Paresthesia
o Weakness
o Vitamins:
o Fatigue
 Vitamin B6 o Weight loss
 Vitamin C
 Diagnostic tests:
o Bone marrow aspiration
o Bilirubin and lactate dehydrogenase- Increased

9
o Gastric analysis: hypochlorhydria Aplastic anemia
o Romberg test: positive  This anemia is associated with suppression,
o Schilling test: positive destruction or aplasia of the bone marrow.
o Upper GI series  The damage to the bone marrow results in a
 Schilling test: involves an oral and reduction of RBC, leukocytes and platelets.
intramuscular injection of cyanocobalamin,  Risk Factors:
followed by a 24-hour collection of urine for o Chemical exposure
cyanocobalamin. o Chemotherapy
 Pre-procedure: o Idiopathic unknown cases
NPO for 12 hours prior o Radiation
No laxative during test o Viral hepatitis
 Post-procedure: o Drug-induced:
Instruct patient to save all urine  Some antibiotics
 Some RA drugs
 Romberg test: Ask the patient to stand with
 Phenytoin (Dilantin)
feet together and arms at their side, ask  Clinical Manifestations:
patient to close eyes. If the patient loses o Dyspnea and Tachypnea
balance or sways from side to side, the test o Epistaxis (nosebleed)
is positive. o Fatigue
 Treatments: o Anorexia
o Transfusion of packed RBCs o Headache
o High iron and protein diet-bland o Gingivitis
 Fish o Melena (black tarry feces)
 Meat o Palpitations and tachycardia
 Milk o Fever and infections
 Eggs o Petechiae
o Vitamins: o Purpura
 Vitamin B6  Diagnostic tests:
 Vitamin C o Bone marrow biopsy
 Vitamin B12 o Hematology study
 Folic acid o Peripheral blood smears:
o Drug therapy: o Pancytopenia (↓ Granulocytes, Platelets,
Ferrous sulfate (Feosol) Erythrocytes)
Vitamin B12 injection monthly o Fecal occult blood
Transfusion therapy
 Treatments:
o Monitor:
o Transfusions of packed RBC and platelets.
 Vital signs
o Diet
 Stool:
 High protein
Color
 High calorie and vitamin diet.
Amount
 Drug therapy:
Consistency
May not be indicated for all patients
 Neurological status
o Antithymocyte globulin (ATG)
 Laboratory results
o Antilymphocyte globulin (ALG)
 Activity as tolerated
Stimulate the production of blood cells by suppressing
 Mouth care pre- and post- meals
the immune system
 Soft toothbrushes
o Cyclophosphamide immunosuppressant
 Warm environment
o Corticosteroids
 Proper skin care
o Bone marrow transplant
 Monitor:
o Vital signs
10
 o Respiratory status  Nursing Intervention:
o Cardiovascular status o Monitor for:
o Assess for any signs of bleeding  S/S of Hypoxia
o Early S/S and Dx and Treatment of infections  Confusion
o Administer oxygen therapy  Cyanosis
o Encourage coughing and deep breathing  SOB
o Activity as tolerated  Tachycardia
o Proper mouth and skin care  Palpitation
o Avoid IM injections o Frequent patient repositioning
o No hard toothbrushes or razors o Meticulous skin care
 Hemolytic Anemia o Avoid soap if jaundice
 Abnormal rate of RBCs distraction primarily by  Splenectomy
the spleen. o Pre-op care
o Congenital
o Pneumococcal vaccine (high risk for pneumonia
Sickle cell anemia for several years)
Thalassemia o Post-op care
o Acquired o Monitor for:
Autoimmune anemia
o Hemorrhage and Shock
Malaria o T˚
Lead poisoning o Abdominal exam (distension)
Transfusion incapability o Early ambulation
Erythrocytes life span is shorten (15-16 days) and
hemolysis occurs at a rate that the bone marrow
cannot compensate Sickle cell anemia
 Assessment:
o Pallor
o Icteric sclera
o Can be jaundice
o Chills
o Fever
o Irritability
o Back pain
o Abdominal pain
o N/V
o Diarrhea
o Melena
o Hematuria
o Splenomegaly
o Hepatomegaly
o Cholelithiasis (gallstones)  Congenital abnormality.
It is a recessive gene that causes a change in the
 Diagnosis:
hemoglobin chain of the RBCs.
o Hgb/ Hct ↓
 Most common inherited disorder in US in
o Reticulocyte ↑
African-American population.
o Coomb’s test –pos (autoimmune)
 Hgb S (abnormal Hgb) replaces all or part of the
o Bilirubin (indirect) ↑
hemoglobin on the RBCs
 Treatment:  Sickle RBCs live 6-20 days instead of 120,
o Corticosteroid causing hemolytic anemia.
o Blood transfusion  Risk Factors:
o Splenectomy (if drugs not effected) o Genetic inheritance
o Immunosuppressant (Delagil)
11
  Clinical Manifestations:
 General:
 Tachycardia
 Jaundice
 Dyspnea
 Chronic fatigue
 Family history
 Frequent infections
Sickle cell crisis:
Patient education:
Treatments:
 Warm compress for pain relief
 Blood transfusion
 Drug therapy:
 Morphine or Demerol
 Iron and folic acid supplements
 Hydration:
 IV fluids and PO
 Bed rest during crisis
 Oxygen therapy
 Allow patient to verbalize feeling
 Genetic counseling
 Avoid tight clothing (circulation impairment)
 Good skin care (especially legs)
 Avoid active sports
 Avoid flying in airplanes

Hemophilia
 Genetic, an X-linked disorder, affects males
inheriting from their mothers, and females
inherit the carrier status from their fathers.
 Hemophilia characterized by producing mild
to severe abnormal bleeding.
 Risk Factors:
o A recessive trait on the X chromosome

 Severe pain
 ematuria
 Lethargy
 Irritability
 Pale skin
Painful crisis:
 Increase in jaundice
 Low grade fever
 Dark urine
 Necrosis of tissue
Aplastic crisis:
 Possible coma
 RBC hemolysis
 Lethargy
 Pallor
 Dyspnea
Diagnostic tests:
 Hgb usually ↓ (6-9 g/dl)
 Hemoglobin electrophoresis

12
  Recombinant factor VII and purified
factor IX
o Notify primary health care provider ASAP of any
injury
o Avoid IM injections
o Avoid aspirin
o Emotional support
o Genetic counseling
o Support groups
Bleeding into joints:

 Clinical Manifestations:
o Severe hemophilia:
 Excessive bleeding following
circumcision
 Large subcutaneous or deep
intramuscular hematomas
 Spontaneous bleeding
o Moderate hemophilia:
 Occasional spontaneous bleeding
o Range of motion (ROM) - 48 hrs after bleeding
 Subcutaneous or deep intramuscular controlled
hematomas o Non-weight bearing until bleeding stops and
o Mild hemophilia: swelling of the joint subsides
 No spontaneous bleeding  During bleeding episodes:
 Prolonged bleeding following a o Direct pressure to the affected area
minor trauma o Cold compress
 Diagnostic tests: o Administer clotting factor
o Prolonged PTT  After bleeding episodes:
o Platelet count and function within normal o Monitor for any sign of bleeding
limits o Monitor PTT
o Hemophilia A: 0-25% of normal Factor VIII o Assess neurological status (↑ Risk for
o Hemophilia B: Abnormal Factor IX Intracranial hemorrhage)
 Treatments:  Client education:
o Hemophilia A: o Avoid all contact sports.
 Cryoprecipitate antihemophilic o Instruct parents to obtain a Medic Alert
factor (AHF) bracelet for the child.
o Hemophilia B:
13
Disseminated Intravascular Coagulation (DIC)  Nursing Intervention:
 Secondary coagulation disorder involving o Monitor V/S
widespread clotting in the small vessels. o Assess for bleeding & shock
o Avoid IM
 Hemorrhage occurs from: o Apply pressure for bleeding sites
o Kidneys o Gently handle the patient
o Brain o Non-traumatic mouth care
o Oxygen
o Heart
o Emotional support
o Other organs
Idiopathic Thrombocytopenic Purpura (ITP)
 May occur in clients, who are critically ill A disease characterized by destruction of platelets,
with: resulting in decrease platelets count of less than
o Obstetrics 100,000/mm³.
o Surgical
o Neoplastic disease
 Risk factors:
o Hemolytic process
o Vascular damage to endothelium
o Shock
o Sepsis
o OB hemorrhage
o Abruptio placentae  Characterized by Petechial and Ecchymotic skin
o Intrauterine death of the fetus lesions.
o Pregnancy –induced Hypertension  Risk factors:
 Assessment: o Stress
o Petechial and ecchymotic rash on the skin and o Infection
mucus membrane o Trauma
o Prolonged bleeding after injection (IV; IM) o Autoimmune
o Severe and uncontrollable bleeding during child  Lab results:
birth or surgical procedure o Thrombocytopenia
o Oliguria o Anemia
o Acute renal failure  Medical management:
o Convulsions o Steroids
o Coma o Platelets transfusion
o Death o Splenectomy
 Lab Analysis:  Nursing Intervention:
o PT/PTT-prolonged o Control bleeding
o Clotting time-prolonged o Protect from infection
o Fibrinogen –depressed  Client education:
o Thrombocytopenia o Padding in crib
 Medical Management: o Soft toys
o Blood transfusion: o Protective headgear during
 Whole blood toddlerhood
 Packed RBCs o Soft toothbrush
 Platelets o Stool softeners to prevent straining
 Plasma o Avoid contact sports
 Cryoprecipitate o Suggest
 Heparin (inhibits thrombin; preventing further  Swimming
clot formation; allowing coagulation factors to  Golf
accumulate) 

14
Leukemia  Chlorambucil (Leukeran)
 This disorder is the over production of WBC o Antimetabolites:
 Fluorouracil (Adrucil)
precursors, that do not mature. The four
 Methotrexate (Folex)
types of leukemia are: o Antineoplastic:
o Acute myeloid leukemia  Vincristine (Oncovin)
o Acute lymphocytic leukemia  Vinblastine (Velban)
o Chronic myeloid leukemia o Hematopoietic growth factor:
o Chronic lymphocytic leukemia  Epoetin alfa (Epogen)
 Risk Factors:  Nursing Implementation
 Genetics o Vital signs
 Exposure to certain chemicals o Respiratory status
 Radiation o Cardiovascular status
 Altered immune system o Intake and output
 Virus o Daily weights
 Clinical Manifestations: o Laboratory results
o Frequent infections Assess for Infection
o Weakness and fatigue o Encourage PO fluids
o Generalized pain o Semi-fowler’s position
o Enlarged o Cough and deep breath
 Lymph nodes o Turn and position every 2 hours
 Spleen o Report temperature over 101˚
 Liver o Proper skin and mouth care
o Fever o Avoid IM injections
o Jaundice Lymphoma
o Hematemesis
 This is a disease of the lymphatic system.
o Pain in:
It is classified as either Hodgkin’s disease or
 Joints
 Abdomen non-Hodgkin’s’ disease.
 Bone  Hodgkin’s disease (Reed-Sternberg cell): is
o Hypotension associated with the proliferation of cells
o Night sweats from a single node.
o Prolonged menses  Non-Hodgkin’s disease (malignant
o Tachycardia lymphoma):
o Petechiae It is associated with tumors occurring
 Diagnostic tests: throughout the lymphatic system.
o Bone marrow biopsy It can be categorized as:
o Decrease in Hgb and Hct
 Lymphocytic
 Treatments:
 Histiocytic
o High protein, vitamin and mineral diet
o Transfusion of:
 Mixed cell type
 Whole blood  Risk Factors:
 Packed RBCs o Environmental
 Platelets o Genetic
 Stem cell transplant o Immunologic
 Drug therapy: o Viral
o Antibiotics:  Clinical Manifestations:
 Plicamycin (Mithracin) o Cough
 Doxorubicin (Adriamycin) o Weight loss and anorexia
o Alkylating: o Night sweats
 Busulfan (Myleran) o Hepatomegaly
15
 o Recurrent infections and intermittent fever  Treatment:
o Splenomegaly o Supportive
 Hodgkin’s: o Goal is to maintain normal Hgb level by blood
o Dyspnea transfusion.
o Dysphagia o Bone marrow transplantation
o Bone pain  Assessment:
o Enlarged, non-tender, firm and movable o Severe anemia
lymph nodes of the cervical region. o Pallor
o Predictable pattern o Failure to thrive
o Hepato/Splenomegaly
 Non-Hodgkin’s:
o Microcytic and Hypochromic RBCs
 Painless, generalized lymphadenopathic disease
 Nursing Implementation:
of lymph node
o Administer Folic acid (Vitamin B9)
 Less predictable pattern
o Blood transfusions
 Diagnostic tests: o Monitor for:
o Bone marrow biopsy  Fe overload (antidote Deferoxamine
o Increased alkaline phosphatase Desferal)
o Chest X-ray o Genetic counseling
o Decreased Hb and HCT
Acquired Immunodeficiency Syndrome
 Treatments:
o Radiation
(AIDS)
o Transfusion of packed RBCs  Disease that is caused by the human
o High protein, calorie, vitamins, minerals, iron immunodeficiency virus (HIV) that affects the T-
and calcium diet; cell-mediated immunity.
o Bland, soft food  Risk Factors:
o Drug therapy: o Exposure to body fluids containing HIV
o Monitor:  Clinical Manifestations:
o Vital signs o Anorexia
 Intake and output o weight loss
 Laboratory results o recurrent diarrhea
 Bleeding o Fever
 Infections o Malnutrition
 Jaundice o Fatigue and weakness
 Semi-fowler’s position o Night sweats
 Oxygen therapy o Opportunistic infections
 Proper skin and mouth care o Pallor
 Turn and position Q 2 H  Diagnostic tests:
 Encourage PO fluids o CD4 T-cell less than 200cells/ul
 Activity as tolerated o ELISA positive HIV antibody titer
 Allow patient to verbalize feelings o Western blot: positive
B-Thalassemia Major o Decrease in:
 WBC
 An autosomal recessive disorder
 RBC
 Also called Cooley’s anemia.
 Platelets
 Characterized by the reduced production of the
globin-chain in the synthesis of hemoglobin.
 Treatments:
o High calorie, protein diet in small frequent
 Common in Mediterranean.
meals
o Activity as tolerated

16
 o Drug therapy: Whole Blood
o Antibiotic therapy:
 Trimethoprim
 Sulfamethoxazole (Bactrim)
o Antiemetic:
 Prochlorperazine (Compazine)
 Ondansetron (Zofran)
o Antifungal:
 Fluconazole (Diflucan)
o Antiviral:
 Didanosine (Videx)
 Zidovudine (Retrovir)
 Rarely use
 Acyclovir (Zovirax)
 Unit contains 500 ml
 Pentamidine (Pentam 300)
 Rh and ABO compatibility required
o Good oral hygiene
 Infusion should not exceed 4 hr.
o Allow patient to express feelings
Red Blood Cells
o Community support
o Early diagnosis and treatment for infection
o Teach patient the importance of proper
medication administration
o Prevention of disease transmission

Blood and Blood Products

 Improve O2 transport
 Volume expansion  RBC replacement
o Whole blood  Blood loss in pre and post-op clients
o Plasma  Rh and ABO computability required
o Albumin  Supply in:
 Provision of proteins o 250 ml
o 350 ml
o Fresh frozen plasma
o 400 ml
o Albumin  Transfusion takes 2-4 hr
 Provision of coagulation factors Platelets
o Cryoprecipitate
o Fresh whole blood
o Fresh frozen blood
 Provision of platelets
o Platelet concentrate
o Fresh whole blood

 Used for Thrombocytopenia

 Units
o 50-70 ml
o 200-400 ml
o Administer immediately
o Given rapidly over 25-30 min

17
Fresh Frozen Plasma
Cryoprecipitate

 Used for Clotting factor

 Contains no platelets
 Infused over 6 hr of thawing Used for
 Rh and ABO are required  Clotting factor (VIII)
 Unit 200-250 ml  Fibrinogen
 Infused rapidly

 Nursing Intervention
Albumin  Assess for Hx of blood transfusion and any
reaction
 Informed consent
 Check MD order
 At least two nurses should verify:
o ABO and Rh type
o Cross-match record
Used for o Client name and blood number
 Hypovolemic shock o Expiration date
 Hypoalbunemia o if any discrepancies
 Prepared from plasma DO NOT ADMINISTER
 Stored for 5 years  Administer immediately after receiving from
the blood bank
 Do NOT add any medication
 DO NOT warm blood in a microwave or hot
water
 Inspect the bag
 Obtain V/S
 Initiate infusion with 18-20 gauge needles and
begin with NS infusion
 DO NOT USE D5W (cause hemolysis)
 Start transfusion slowly 100 ml/hr
 Stay with client for the first 15 min and take V/S
 Monitor V/S frequently
 Maintain transfusion at prescribed rate.

18
19
20
21
22
23
24
25
26
27
28
29
30
31