Disorders of the Hematopoietic

1
Disorders of the Hematopoietic Lymphatic Systems
2
Disorders of the Hematopoietic System

• Anemia
• A term used that refers to a deficiency of either
  erythrocytes (RBCs) or hemoglobin
• (See Table 39-1)
• Result from
• Blood loss
• Inadequate or abnormal erythrocyte production
• Destruction of normally formed RBCs

3
Disorders of the Hematopoietic System

• Anemia (cont)
• Hypovolemic Anemia
• Loss of blood volume fewer blood cells
• Acute
• Trauma
• Chronic
• Gastric bleeding from a peptic ulcer

4
Disorders of the Hematopoietic System

• Anemia (cont)
• Hypovolemic Anemia (cont)
• When the blood is lost, the bone marrow responds
  by increasing its production of erythrocytes
• These cells are smaller and contain less heme
  (the pigmented, iron-containing portion of
  hemoglobin)
• The RBCs are microcytic hypochromic (See Table
  39-1)
• If the formation of new RBCs cannot compensate
  for the loss
• Cellular function is compromised from an
  inadequate oxygen supply accumulated CO2

5
Disorders of the Hematopoietic System

• Anemia (cont)
• Hypovolemic Anemia (cont)
• Hypovolemic Shock
• Pallor
• Fatigue
• Chills
• Postural hypotension
• Rapid heart rate
• Rapid respiratory rate

6
Disorders of the Hematopoietic System

• Anemia (cont)
• Hypovolemic Anemia (cont)
• Laboratory
• CBC
• Decreased erythrocytes
• Increased reticulocytes (eerythrocytes in the
  process of maturation)
• Low hemoglobin (hgb) and hematocrit (hct) levels
  (See Table 39-2)
• Mean cell volume is low
• Result of smaller size of the erythrocytes
• Mean cell hemoglobin concentration is low
• Reflectin the reduced hemoglobin level

7
Disorders of the Hematopoietic System

• Anemia (cont)
• Hypovolemic Anemia (cont)
• Medical Management
• Replacement of blood by transfusions
• If chronic condition, treat the cause
• Administration of oral, IV, or IM iron
• Helps the body compensate for the lost hgb
• Oxygen therapy

8
Disorders of the Hematopoietic System

• Anemia (cont)
• Hypovolemic Anemia (cont)
• Assessment
• Obtain data from patient regarding reason for
  presenting symptoms
• V/S
• Review lab tests
• Prepare pt. For diagnostic tests
• Endoscopy
• Perform a physical exam
• To detect sources of bleeding

9
Disorders of the Hematopoietic System

• Anemia (cont)
• Sickle Cell Anemia
• Erythrocytes become sickle (crescent) shaped
  when oxygen supply in the blood is inadequate
  (See Fig. 39-2)
• Normally, hgb. A replaces fetal hgb. F about 6
  months after birth
• In sickle cell anemia, an abnormal form of hgb.,
  hgb. S, replaces hgb.F
• Hgb. F causes the RBCs to become sickle-shaped

10
Disorders of the Hematopoietic System

• Anemia (cont)
• Sickle Cell Anemia (cont)
• Hereditary disease
• African Americans, as well as people from the
  Mediterranean Middle Eastern countries
• Person must inherit two defective genes
• If the person inherits only one defective gene,
  he/she is a carrier of the sickle cell trait
• Many more people are carriers than have sickle
  cell disease

11
Disorders of the Hematopoietic System

• Anemia (cont)
• Sickle cell Anemia (cont)
• Episodes of sickle cell crisis
• Vascular occlusion
• Develops rapidly in periods of hypoxia
• Sickle-shaped cells lodge in small blood vessels,
  where they block the flow of blood and oxygen to
  the affected tissue
• Severe pain in the ischemic tissue
• Stroke is a common complication, even in young
  children
• Chronic hemolytic anemia

12
Disorders of the Hematopoietic System

• Anemia (cont)
• Sickle cell Anemia (cont)
• Assessment
• Jaundice
• Hyperbilirubinemia (excess bilirubin pigment in
  the blood)
• (See Fig. 39-3
• Gall stones
• Predisposition to infection
• When the spleen becomes dysfunctional
• Anaerobic metabolism
• Compromises growth

13
Disorders of the Hematopoietic System

• Anemia (cont)
• Sickle cell Anemia (cont)
• Assessment (cont)
• Chronic leg ulcers
• Blockage of the small blood vessels of the legs
• Priapism (prolonged erection)
• Delayed emptying of thick blood from the penis
• S/S anemia
• The reduced blood flow during sickle cell crisis
• Localized ischemia
• Severe pain
• Possible tissue infarction (necrosis)
• Can lead to CVA, pulmonary infarction, shock,
  renal failure

14
Disorders of the Hematopoietic System

• Anemia (cont)
• Sickle cell Anemia (cont)
• Diagnostic Findings
• Sickledex test
• Determines if hemoglobin S is present
• Hemoglobin electrophoresis
• Determines whether the person has sickle cell
  disease or carries the trait
• Hgb levels tend to run 7 10 g/dl
• Secretory phospholipase A
• A predictor of Acute Chest Syndrome
• A type of pneumonia triggered by decreased Hgb
  infiltrates in the lungs

15
Disorders of the Hematopoietic System

• Anemia (cont)
• Sickle cell Anemia (cont)
• Medical Management
• Treatment is supportive rather than curative
• Regular blood transfusions
• Decrease the risk for stroke and other
  complications of infarction
• However, this increases blood viscosity
  (thickness)
• Can potentially do more harm than good
• Bone marrow transplantation
• Has cured a few people
• During crisis
• Narcotic analgesics, oxygen, bed rest, hydration
  with IVs, possibly be given transfusions

16
Disorders of the Hematopoietic System

• Anemia (cont)
• Sickle cell Anemia (cont)
• Assessment
• Health history focusing on previous episodes of
  sickle cell crisis complications
• V/S (Ie fever infection)
• S/S dehydration (can trigger crisis)
• Check lungs heart acute chest syndrome/heart
  fail.
• Pain assessment
• Check skin sclera jaundice/ulcerations

17
Disorders of the Hematopoietic System

• Anemia (cont)
• Sickle cell Anemia (cont)
• Assessment (cont)
• Check joints swelling
• U/A concentrated/blood cells renal damage
• Mental status, verbal ability, motor strength -
  CVA

18
Disorders of the Hematopoietic System

• Leukocytosis
• An increased number of leukocytes
• Normally an increased number serve as a
  protective mechanism
• In a disease condition, the proliferation of
  leukocytes is detrimental

19
Disorders of the Hematopoietic System

• Leukocytosis (cont)
• Leukemia
• Any malignant blood disorder in which
  proliferation of leukocytes, usually in an
  immature form, is unregulated
• Decrease in production of erythrocytes platelets

20
Disorders of the Hematopoietic System

• Leukocytosis (cont)
• Leukemia (cont)
• 4 general types of leukemia (See Table 39-3)
• Acute lymphocytic leukemia (ALL)
• Chronic lymphocytic leukemia (CLL)
• Acute myelogenous leukemia (AML)
• Chronic myelogenous leukemia (CML)

21
Disorders of the Hematopoietic System

• Leukocytosis (cont)
• Leukemia (cont)
• Lymphocytic leukemias
• Result from bone marrow dysfunction that affects
  lymphoid stem cells
• Myelogenous leukemias
• Result from bone marrow dysfunction that affects
  myeloid stem cells (See Fig. 39-4)

22
Disorders of the Hematopoietic System

• Leukocytosis (cont)
• Leukemia (cont)
• The cause of leukemia is unknown
• Toxic chemicals, radiation, viruses, certain
  drugs
• Possible genetic correlation
• The increase in leukocytes is rampant
• There are many more immature than mature cells
• Ineffective at fighting infections
• Decreased production of erythrocytes and
  platelets
• The patient eventually develops severe anemia,
  and the reduction in platelets leads to bleeding

23
Disorders of the Hematopoietic System

• Leukocytosis (cont)
• Leukemia (cont)
• Assessment
• Infections
• Fatigue from anemia
• Easy Bruising
• In ALL, Fever
• Enlarged spleen lymph nodes, internal or
  external bleeding
• The leukocyte count is low, normal, or high, but
  the number of normal leukocytes is decreased
• The number of erythrocytes platelets decreases,
  too

24
Disorders of the Hematopoietic System

• Leukocytosis (cont)
• Leukemia (cont)
• Medical management
• Drug therapy is the primary weapon for arresting
  leukemia
• Erythrocyte platelet transfusions
• Antibiotics, when secondary infections develop
• Bone marrow stem cell transplantation
• Have increased survival for some pts.
• Autologous from oneself
• Allogenic from another

25
Disorders of the Hematopoietic System

• Leukocytosis (cont)
• Leukemia (cont)
• Assessment
• Obtain history of symptoms
• Weakness fatigue, frequent infections,
  nosebleeds or other prolonged bleeding events,
  joint pain Also headache / confusion
• Examine the pts body
• Bruising, upon palpation of the abdomen attempt
  to determine enlargement tenderness over the
  liver spleen
• Assess labs
• Assess the outcome of bone marrow aspiration

26
Disorders of the Hematopoietic System

• Multiple Myeloma
• A malignancy involving plasma cells, which are
  B-lymphocyte cells in bone marrow.
• Prognosis is poor
• Estimated survival of 1 5 years after diagnosis

27
Disorders of the Hematopoietic System

• Multiple Myeloma
• Unknown cause
• Is associated with aging, recurrent infections,
  drug allergies, exposure to occupational toxins
  radiation
• Onset is rare before age 40
• The abnormal plasma cells proliferate in the bone
  marrow
• They release osteoclast activating factor
• This breaks down bone cells
• Increased blood calcium pathologic fractures
• They also form single or multiple osteolytic
  (bone-destroying) tumors
• Produce a punched-out or honeycombed
  appearance in bones

28
Disorders of the Hematopoietic System

• Multiple Myeloma (cont)
• The malignant plasma cells release 2 types of
  abnormal proteins
• Bence Jones proteins impair renal tubules
• Leads to renal failure
• M-type globulin
• Compromises production of functional
  immunoglobulins (antibodies)
• Interferes with an optimal immune response
• The excess production of plasma cells reduces the
  formation of erythrocytes and platelets
• Causes anemia increases the risk for bleeding

29
Disorders of the Hematopoietic System

• Multiple Myeloma (cont)
• Assessment
• Signs symptoms
• First symptom is usually a vague pain in the
  pelvis, spine, or ribs
• As it progresses, the pain becomes more severe
  and localized
• When tumors replace bone marrow, pathologic
  fractures occur
• High incidence of infection, especially
  pneumonia, caused by a decreased production of
  appropriate antibodies

30
Disorders of the Hematopoietic System

• Multiple Myeloma (cont)
• Assessment
• Signs symptoms
• May experience symptoms associated with anemia
• Weakness, fatigue, chills
• Decreased platelets
• Bruising nosebleeds
• Hypercalcemia renal failure
• Renal calculi may develop

31
Disorders of the Hematopoietic System

• Multiple Myeloma (cont)
• Diagnostic Findings
• X-Rays
• Characteristic bone lesions (See Fig. 39-5)
• Blood cell counts
• Calcium levels are elevated from bone destruction
• Urine samples
• Positive for Bence Jones protein
• Bone marrow aspiration
• Increased atypical plasma cells
• Uric acid levels is elevated
• From cellular destruction

32
Disorders of the Hematopoietic System

• Multiple Myeloma (cont)
• Medical Management
• Steroids
• Anticancer drugs
• Radiation
• Remission occurs in approx. 60 of pts.
• Anemia is treated with blood transfusions
• Infections are managed with antibiotics
• Bone marrow and peripheral stem cell
  transplantation

33
Disorders of the Hematopoietic System

• Multiple Myeloma (cont)
• Nursing Management
• Check for pain, signs of infection, excessive
  fatigue, bleeding, thrombus formation, changes
  in the quantity/quality of urine production
• Administer analgesics
• Assist with ambulation immobility can lead to
  more calcium loss from the bones
• Provide up to 4000 ml of fluids
• Report signs of calculus formation
• Safety is very important movement can lead to a
  fracture

34
Disorders of the Lymphatic System

• Lymphedema
• Is a condition that results from obstructed lymph
  circulation
• Primary lymphedema
• Inherited
• Secondary lymphedema
• From treatment such as mastectomy or radiation
  for cancer
• Leads to an occlusion of affected lymphatic
  vessels, which impairs lymph circulation and
  causes lymph to accumulate in the lymphatic system

35
Disorders of the Lymphatic System

• Lymphedema (cont)
• Assessment
• Skin in the affected area swells
• Pitting is evident
• Becomes firm, tight, shiny
• Elevation does not diminish the swelling
• Skin also becomes thickened, rough, and
  discolored
• Tissue nutrition is impaired from the stagnation
  of lymph fluid
• Ulcers and infection can develop

36
Disorders of the Lymphatic System

• Lymphedema (cont)
• Medical Management
• Usually symptomatic
• Elevate
• Pt wears an elastic stocking or sleeve when in a
  dependent position
• Massage
• Mechanical pulsating compression device
• Surgery may be used
• To relieve the obstruction of lymphatics

37
Disorders of the Lymphatic System

• Lymphedema (cont)
• Nursing management
• Inspect and measure the area
• Enc. Pt. To move and exercise the arm or leg
• Insturcts pt. to elevate extremities how to
  apply elastic garments mechanical devices
• Emotional support
• Self-esteem is often decreased
• Social withdrawal
• Support self-image by suggesting certain styles
  of clothing that conceals

38
Disorders of the Lymphatic System

• Infectious Mononucleosis
• A viral disease that affects lymphoid tissues
  such as the tonsils spleen
• It can involve other organs such as the brain,
  meninges, liver

39
Disorders of the Lymphatic System

• Infectious Mononucleosis (cont)
• The Epstein-Barr virus causes this disorder
• Contagious spreads by direct contact with
  saliva pharyngeal secretions from an infected
  person
• Kissing
• Coughing
• Talking
• Sneezing
• Sharing food, cigarettes, etc.
• Incubation period
• As long as 30 50 days
• Most commonly affects young adults
• In close quarters I.e. armed services
  college dorms

40
Disorders of the Lymphatic System

• Infectious Mononucleosis (cont)
• At the time of infection
• Macrophages engulf the virus
• A display of the antigen on the cell surface
• The production of T-lymphocytes results
• T lymphocytes trigger the production of B-cell
  lymphocytes antibodies
• Infiltration of tissue occurs, particularly the
  spleen
• Force to the abdomen can cause the spleen to
  rupture when it is enlarged

41
Disorders of the Lymphatic System

• Infectious Mononucleosis (cont)
• Symptoms resolve in approximately 1 2 weeks,
  unless complications develop
• One episode produces subsequent immunity
• The virus remains in the body for the persons
  lifetime
• The Epstein-Barr virus is believed to trigger
  Hodgkins lymphoma in approximately 40 of people
  with this disease

42
Disorders of the Lymphatic System

• Infectious Mononucleosis (cont)
• Assessment
• Fatigue
• Fever
• Sore throat
• Headache
• Cervical lymph node enlargement
• Tonsils with white or greenish gray exudate
• Pharyngeal swelling
• Can compromise swallowing breathing
• Faint reddish rash
• On hands or abdomen
• Liver spleen enlargement

43
Disorders of the Lymphatic System

• Infectious Mononucleosis (cont)
• Diagnostic Findings
• Lymphocytosis
• A positive slide agglutination test
• Is presumptive evidence that the Epstein-Barr
  virus is causing the symptoms
• Epstein-Barr titer
• Increased - conclusive
• a Hererophil agglutination test
• 1224 or greater - conclusive

44
Disorders of the Lymphatic System

• Infectious Mononucleosis (cont)
• Medical management
• Bed rest
• Analgesia
• Antipyretic therapy
• Increased fluid intake
• Nursing management
• Rest
• Listen
• Provide forms of coping
• Instruct pt from donating any blood for at least
  6 months after recovering

45
Disorders of the Lymphatic System

• Lymphomas
• A group of cancers that affect the lymphatic
  system
• Two of the most common forms of lymphoma
• Hodgkins disease
• Non-Hodgkins lymphoma

46
Disorders of the Lymphatic System

• Lymphomas (cont)
• Hodgkins disease
• A malignancy that produces enlargement of
  lymphoid tissue, the spleen, the liver with
  invasion of other tissues such as the bone marrow
  lungs
• It appears in several forms
• Acute
• Localized
• Latent with relapsing pyrexia
• Splenomegaly
• Lymphogranulomitosis (mult granular tumors
  composed of lymphoid cells)

47
Disorders of the Lymphatic System

• Lymphomas (cont)
• Hodgkins disease (cont)
• Cause unknown
• Appears that a virus, particularly the
  Epstein-Barr virus (Infective Mononucleosis)
  causes mutations in some lymphocytes, creating a
  malignant cell type known as Reed-Sternberg cells
• Nearly immortal cells, continuing to reproduce
  prolifically, and perhaps due to their altered
  form, are somehow shielded from being destroyed
  by killer T-cells.
• The virus is believed to inactivated the immune
  systems ability to suppress tumor growth
• The malignant cells release chemicals known as
  cytokines
• Inflammatory response pain fever

48
Disorders of the Lymphatic System

• Lymphomas (cont)
• Hodgkins disease (cont)
• More common in men than in women
• Occurs most often in late adolescence young
  adulthood
• Some pts. Survive 10 or more years
• Cure is possible when the disease is localized to
  one section of the body
• Pts. Who receive treatment usually have
  remissions that last for months or even years
• Others die in 4 5 years
• Results from respiratory obstruction, cachexia,
  infections

49
Disorders of the Lymphatic System

• Lymphomas (cont)
• Hodgkins disease (cont)
• Assessment
• Painless enlargement of one or more lymph nodes
• As the nodes enlarge, they press on adjacent
  structures
• Marked weight loss, anorexia, fatigue weakness
• Low-grade fever, pruritis, night sweats
• Sometimes marked anemia thrombocytopenia
  develop
• Tendency to bleed
• Resistance to infection is poor
• Staphylococcal skin infections respiratory
  tract infections

50
Disorders of the Lymphatic System

• Lymphomas (cont)
• Hodgkins disease (cont)
• Diagnostic findings
• CBCLow RBCs,
• Elevated leukocytes a paradoxical decrease in
  lymphocytes
• Lymph node biopsy
• The Reed-Sternberg cells, characterized as giant
  multinuclear B lymphocytes
• Erythrocyte Sedimentation Rate (ESR)
• Suggestive of inflammatory process
• Liver enzymes
• Such as alkaline phosphatase is elevated

51
Disorders of the Lymphatic System

• Lymphomas (cont)
• Hodgkins disease (cont)
• Diagnostic findings
• Lymphangiography
• Chest X-Ray
• CT scan
• MRI
• Laparotomy
• Biopsy determines size of lymph nodes and spread
  of the disease in the thorax, abdomen or pelvis

52
Disorders of the Lymphatic System

• Lymphomas (cont)
• Hodgkins disease (cont)
• Staging (See Table 40-2)
• I IV, based on the number of positive lymph
  nodes and the involvement of other organs
• Determines treatment
• Categories
• B for those with defined general symptoms
• A - for those without B symptoms

53
Disorders of the Lymphatic System

• Lymphomas (cont)
• Hodgkins disease (cont)
• Categories
• The B category is for those demonstrating
• Unexplained loss of more than 10 of body weight
  in the 6 months before diagnosis
• Unexplained fever with temperatures above 38 C.
• Drenching night sweats

54
Disorders of the Lymphatic System

• Lymphomas (cont)
• Hodgkins disease (cont)
• Medical Management
• Localized radiation
• Chemotherapy
• Antibiotics
• Transfusions
• For resistance to treatment
• Autologous bone marrow or periperal stem cell
  transplant

55
Disorders of the Lymphatic System

• Lymphomas (cont)
• Hodgkins disease (cont)
• Assessment
• Look for a history of previous infectious
  mononucleosis or symptoms resembling it
• Check enlarged lymph nodes, how long the pt has
  noticed them, and note tenderness
• Check about fever, chills, night sweats
• Check out weight loss, enlargement of spleen,
  level of energy appetite
• See Teaching 40-1