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Haematological Disorders by RZgA9gV

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									Haematological Disorders


               B. Pimentel, M.D.
            University Of Makati
             College of Nursing
The Red Blood Cell

• Transports oxygen, called oxyhemoglobin, when it gives
  up its oxygen it is deoxyhemoglobin.

• Also binds and transports carbon dioxide,
  carbaminohemoglobin.

• Makes up ± 97 % of RBC  ± 250 million Hb molecules
  per RBC

• Men: 14-18 mg/dl blood

• Women: 12-16 mg/dl blood
The Red Blood Cell

Types of Hemoglobin (Hb)
• Hb A
  – 96% of adult Hb (α2 β2)
• Hb A2
  – 3% of adult Hb (α2 σ2)
• Hb F
  – 1 % of adult Hb (α2 γ2)
The Red Blood Cell
The Red Blood Cell
The Red Blood Cell
Transfusion Therapy

Blood     Antigen     Antibody     Donor to      Recipient
group                                            from
A         A           Anti-B       A             A, O

B         B           Anti-A       B             B, O

AB        AB          Neither      AB            A, B, AB, O

O         Neither     Anti-A/Anti- O, A, B, AB   O
                      B

Universal donor "O"
Universal recipient "AB"
Adverse Transfusion Reactions

Acute Hemolytic Reaction
• Symptoms
  – Fever, chills and fever, the feeling of heat along the
    vein in which the blood is being transfused
  – Pain in the lumbar region
  – Constricting pain in the chest, tachycardia, hypo-
    tension
  – Hemoglobinemia with subsequent hemoglo-binuria
    and hyperbilirubin-emia.
     • "feeling of impending doom"
Adverse Transfusion Reactions

Acute Hemolytic Reaction
• Causes
  – Human error!
     • Transfused red cells react with circulating antibody in the
       recipient with resultant intravascular hemolysis
Adverse Transfusion Reactions

Acute Hemolytic Reaction
• Frequency
  – Rare

• Prevention
  – Proper identification of patients, pretransfusion blood
    samples and blood components at the time of
    transfusion
Adverse Transfusion Reactions

Delayed Hemolytic Reaction
• Falling hematocrit
  – due to extravascular destruction of the transfused red
    blood cells)

• Positive direct antiglobulin (Coombs) test (DAT)

• Occurs about 4-8 days after blood transfusion

• Patients may manifest fever and leukocytosis
  – Appearing to have an occult infection.
Adverse Transfusion Reactions

Febrile Transfusion Reaction
• Fever or chill fever
  – temperature rise of 1.5 F or 1.0 C from the baseline
• Cytokines and antibodies to leukocyte antigens
  reacting with leukocytes or leukocyte fragments
• 1 in 8 transfusions
Adverse Transfusion Reactions

Allergic – urticaria
• Laryngeal edema and bronchospasm
• 1% of recipients
  – If coupled with another sign, such as fever,
    evaluation for a hemolytic reaction may be indicated.
Adverse Transfusion Reactions

Allergic – Anaphylaxis
• Anaphylactic or anaphylactoid
  – Respiratory involvement with dyspnea or stridor


• Cardiovascular instability
  – hypotension, tachycardia, loss of consciousness,
    cardiac arrhythmia, shock and cardiac arrest
Adverse Transfusion Reactions

Volume Overload
• Transfusion-related volume overload
• Infuse smaller volumes more slowly
Adverse Transfusion Reactions

Bacterial Contamination
• Hypotension, shock, fever and chills, nausea and
  vomiting, and respiratory distress
• Gram stain and blood culture
  IF A TRANSFUSION REACTION IS
           SUSPECTED


Follow protocol for transfusion
 reactions implemented by the
 institution
   IF A TRANSFUSION REACTION IS
            SUSPECTED
• Stop the transfusion immediately!

• Disconnect the intravenous line from the
  needle.

• Seek medical attention immediately. If the
  patient is suffering cardiopulmonary collapse,
  and medical attention is not immediately
  available, press for “Code"
   IF A TRANSFUSION REACTION IS
            SUSPECTED
• Check to ensure that the patient name and
  registration number on the blood bag label
  exactly with information on the patient's
  identification

• Do not discard the unit of blood that has been
  discontinued because it may be necessary for the
  investigation of the transfusion reaction.
Treatment for Transfusion Reactions
Reaction Type   Treatment - Adult                         Pediatric               Follow-up

Acute           Diuretic therapy: Initially, give 40-80   Pediatric dose: 1-2     Treat shock and disseminated
Hemolytic            mg Furosemide (Lasix)                     mg/kg/dose.              intravascular coagulation with
Reactions            intravenously. This dose can be           May repeat once          appropriate measures if and when
                     repeated once. Lack of response           at 2-4 mg/kg.            they appear.
                     to furosemide in 2-3 hours
                     indicates the presence of acute
                     renal failure.
                Water loading: The patient should be      Pediatric patients
                     hydrated to maintain urinary              should receive a
                     output of at least 100 mL/hr until        smaller loading
                     urine is free of hemoglobin.              volume of fluid
                Infuse a loading dose of 0.9% sodium           in proportion to
                     chloride or 5% dextrose in 0.45%          their body
                     sodium chloride. Chart hourly             surface area.
                     urine output. Maintain the urine
                     output by administering
                     intravenous fluid at 100 mL/hour
                     until the urine is free of
                     hemoglobin. If the patient's
                     urinary output does not increase,
                     with this hydration any additional
                     fluids should be infused with
                     caution.
Treatment for Transfusion Reactions

Reaction Type   Treatment - Adult                     Pediatric   Follow-up



Delayed         Specific treatment generally is not               Supplemental transfusion of blood lacking
Hemolytic            necessary                                         the antigen corresponding to the
Transfusion                                                            offending antibody may be necessary
Reactions                                                              to compensate for the transfused
                                                                       cells that have been removed from
                                                                       the circulation.
Treatment for Transfusion Reactions
Reaction Type   Treatment - Adult                         Pediatric               Follow-up


Allergic        Antihistamines(e.g., Benadryl). Give      Pediatric dose: 1-2     Routine use of Benadryl as premedication
Transfusion     50-100 mg orally or intravenously. If     mg/kg                   for all transfusions, regardless of a history
Reactions       urticaria develops slowly,                intramuscularly or      of allergic reactions, is discouraged.
                antihistamines may be given orally.       intravenously for 25-
                                                          50 mg per average
                                                          dose.


                Aminophylline for wheezing, at a dose     Pediatric dose: 3
                of 125-250 mg intravenously slowly        mg/kg/dose in
                over a period of about five minutes       intravenous drip over
                                                          of 20 minutes.

                Epinephrine for severe, acute reactions   Pediatric dose: 0.03
                including laryngeal edema or              mL/M2 (0.03 mg/M2
                bronchospasm Give 0.1-0.5 mg (0.1-0.5     of a 1:1000 solution)
                mL of a 1:1000 solution)                  given subcutaneously.
                subcutaneously. Subcutaneous dose         A single pediatric
                may be repeated at 10-15 minute           dose should not
                intervals. The total subcutaneous dose    exceed 0.3 mg.
                in a 24-hour period, with rare
                exception, should not exceed 5 mg.
Treatment for Transfusion Reactions
Reaction Type    Treatment - Adult                        Pediatric   Follow-up


Febrile          Premedicate the patient with                         Aspirin will adversely affect the patient's
Transfusion          acetaminophen or other                                platelet function, so non-aspirin
Reactions            antipyretic agents when previous                      antipyretic agents are preferable.
                     reactions have been extremely
                     bothersome. Pediatric dose: 10
                     mg/kg to a maximum of 600 mg.


Severe shaking   (rigors) can be controlled by the                    Note: Demerol may cause acute
Chills                 sedative effect of Benadryl or                      respiratory arrest. An opiate
                       Demerol (25-50 mg given                             antagonist (Narcan) should be
                       intramuscularly or intravenously                    immediately available.

Sepsis Due to    Treatment of septic shock includes:
Bacterial             terminating the suspected
Contamination         transfusion immediately, cardio-
of Donor Blood        vascular and respiratory support,
                      blood culture of the patient, and
                      administration of broad spectrum
                      antibiotics including anti-
                      pseudomonas coverage if the
                      blood component involved is Red
                      Blood Cells.
Anemia

• Abnormally low number of RBC or Hb levels
• Reduced oxygen carrying capacity
Causes
• Blood loss
• Increased rate of red cell destruction
  – Hemolytic anemia
• Deficient or impaired red cell production
Anemia

Risk factors
• Poor diet
• Intestinal disorders
• Menstruation
• Pregnancy
• Chronic conditions
• Family history
Anemia

• “NOT A DISEASE” but a symptom
  – Dependent on severity, speed of development, age,
    health status and compensatory mechanisms
  – Associated with impaired O2 transport, alteration in
    RBC structure or with chronic illness
  – Not expressed until 50% of RBC mass is lost
Anemia

Signs and symptoms
• The main symptom of most types of anemia is
  fatigue
  –   Weakness
  –   Pale skin
  –   Tachycardia
  –   Shortness of breath
  –   Chest pain
  –   Dizziness
  –   Cognitive problems
  –   Numbness or coldness in your extremities
  –   Headache
Anemia

Iron Deficiency Anemia
• Most common form of anemia
  – Affects about one in five women
  – Half of pregnant women and 3 percent of men in the
    United States.
• The cause is a shortage of the element iron
  – Nutritional imbalance
  – Slow, chronic bleeding disorders
  – Inability to recycle plasma iron
Anemia

Vitamin Deficiency Anemias
• Folate and vitamin B-12 deficiency
• Intestinal disorder that affects the absorption of
  nutrients
• Fall into a group of anemias called megaloblastic
  anemias, in which the bone marrow produces
  large, abnormal red blood cells.
Anemia

Anemia of Chronic Disease
• Interfere with the production of red blood cells,
  resulting in chronic anemia
• Kidney failure also can be a cause of anemia
  – The kidneys produce a hormone called
    erythropoietin, which stimulates your bone marrow
    to produce red blood cells.
     • A shortage of erythropoietin, which can result from kidney
       failure or be a side effect of chemotherapy, can result in a
       shortage of red blood cells.
Anemia

Aplastic Anemia
• Life-threatening anemia caused by a decrease in
  the bone marrow's ability to produce all three
  types of blood cells — red blood cells, white
  blood cells and platelets
• Cause of aplastic anemia is unknown
  –   autoimmune disease
  –   Chemotherapy
  –   Radiation therapy
  –   Environmental toxins
Anemia

Anemias associated with bone marrow disease
• Leukemia and myelodysplasia, can cause
  anemia by affecting blood production in the
  bone marrow
• Effects vary from a mild alteration in blood
  production to a complete, life-threatening
  shutdown of the blood-making process
   – Myelodysplasia is a pre-leukemic condition
     that can cause anemia.
     • Other cancers of the blood or bone marrow, such
       as multiple myeloma, myeloproliferative disorders
       or lymphoma, can cause anemia.
Anemia

Hemolytic Anemias
• Red blood cells are destroyed faster than bone
  marrow can replace them.
• Autoimmune disorders can produce antibodies
  to red blood cells, destroying them prematurely
  – Hemolytic anemias may cause yellowing of the skin
    (jaundice) and an enlarged spleen.
Anemia

Hereditary Spherocytosis
• Mutations in the ankyrin molecule with a
  secondary deficiency of spectrin along the cell
  membrane
  – Reduced red cell stability
     • Does not affect oxygen carrying capacity
     • Splenic sequestration
Anemia

Sickle cell anemia
• Defective form of hemoglobin that forces red
  blood cells to assume an abnormal crescent
  (sickle) shape.
  – Mutation for the gene coding for the β-globulin chain
     • Valine is substituted for glutamic acid   HbS


• Red cells die prematurely, resulting in a chronic
  shortage of red blood cells.
  – Block blood flow through small blood vessels in the
    body, producing other, often painful, symptoms.
Anemia

α- Thalassemia
• Common in Asians
• Deletion of glubulin chain loci
• 4 possible degrees of α thalassemia:
  –   Silent carrier, loss of a single α globulin gene
  –   α thalassemia trait, loss of a pair of globulin gene
  –   HbH disease, only a single gene is present
  –   Hydrops fetalis, deletion of all α globulin
Polycythemia Vera

• An acquired disorder of the bone marrow that
  causes the overproduction of all three blood cell
  lines
   – white blood cells, red blood cells, and platelets
• It is a rare disease that occurs more frequently in
  men than women, and rarely in patients under
  40 years old.
• causes is unknown
Polycythemia Vera

• Usually develops slowly, and most patients are
  asymtomatic
   – abnormal bone marrow cells proliferate
     uncontrollably leading to acute myelogenous
     leukemia
• Patients have an increased tendency to form
  blood clots that can result in strokes or heart
  attacks
   – Some patients may experience abnormal bleeding
     because their platelets are abnormal
Polycythemia Vera

Symptoms
•   Headache
•   Dizziness
•   Pruritus
•   Fullness in the left upper abdomen
•   Erythema (face)
•   Shortness of breath
•   Orthopnea
•   Symptoms of phlebitis
White Blood Cells

• Collectively known as White Blood Cells (WBC)

• Formed elements of the blood with organelles
  and a nucleus but lack hemoglobin

• Protect the body against microorganisms and
  remove dead cells and debris from the body
White Blood Cells
White Blood Cells

                  Per µl blood   Per µl of blood

Total WBC count                  5,000 – 10,000
Neutrophils       50 - 70%       2,000 – 7,000
Lymphocytes       20 - 40%       1,000 – 4,000
Monocytes         1 – 6%         50 – 600
Eosinophils       1 – 5%         50 – 500
Basophils         0 – 2%         0 - 100
WBC Disorders

• Leukopenia
  – Decreased peripheral white cell count due to decrease
    numbers of any specific types of leukocytes
• Leukocytosis
  – Non–neoplastic elevation of WBC count
WBC Disorders

Neutropenia
• Reduction in the number of granulocytes
  (<1500/µl)
• Increased risk of infection
  – Reduced phagocytosis response
WBC Disorders

Neutropenia
• Decreased or defective granulopoiesis
  – Aplastic anemia
  – Anti-neoplastic agents
  – Other drugs: chloramphenicol, sulfonamides,
    chlorpromazine
• Accelerated removal or destruction
  – Aggressive and chronic infections
WBC Disorders

Manifestation of Neutropenia
• Infections

Signs and Symptoms
• Malaise, chills, fever
• Ulcerative necrotizing lesions of the mouth, skin
  vagina and GI tract
WBC Disorders

Reactive Leukocytosis
• Increase number of WBC
• Common reaction due to a variety of
  inflammatory states caused by microbial or non-
  microbial stimuli
• Usually non-specific
WBC Disorders

                                 Causes of Leukocytosis

Polymorphonuclear leukocytosis   Acute bacterial infections
Eosinophilic leukocytosis        Allergic disorders

Monocytosis                      Chronic infections
Lymphocytosis                    Chronic immunologic disease
Neoplastic Proliferation of White Cells

1.   Leukemia – neoiplasms of the hematopoietic
     stem cells
2.   Malignant lymphomas – cohesive tumor
     lesions; neoplastic lymphocytes
3.   Plasma cell dyscrasias – arising from the
     bones; localized disseminated proliferation of
     antibody forming cells
4.   Histocytoses – proliferative lesions of
     histiocytes
Neoplastic Proliferation of White Cells

Leukemia
• Malignant neoplasm of the hematopietic stem
  cells
• BM replaced by unregulated, proliferating,
  immature neoplastic cells  blood  leukemia
   enter spleen, lymph nodes
• Most common cancer in the paediatric age
• Leading cause of death in children between 3
  and 14 years old
Neoplastic Proliferation of White Cells

Classification of Leukemia
A. According to cell type and state of cell
    maturity
     •     Lymphocytic – immature lymphocytes and their
           progenators
     •     Myelocytic – pluripotent myeloid stem cells and
           interferes with maturation of all granulocytes, RBC
           and platelets
B.       Acute or Chronic
     •     Acute – immature cells (blast)
     •     Chronic – well differentiated leukocytes
Neoplastic Proliferation of White Cells
Acute Leukemia (Cell Kinetic Studies)

• Block in the differentiation of leukemic cells
  with prolonged genration time  clonal
  expansion of the transformed stem cells + failure
  of maturation  accumulation of leukemic
  blast  suppress normal hematopoietic stem
  cells
Acute Leukemia

Features
• Sudden onset (3 months)
• Depressed marrow function
• Bone pain and tenderness
• Generalized lymphadenophaty
• Splenomegaly, hepatomegaly
• CNS: headache, vomiting
Acute Lymphocytic Leukemia (ALL)

•        Most common leukemia in children (80%)
•        Treatable and potentially curable
•        Classified according to lymphocytes and state
         of maturation
    1.     Early B cell
    2.     Pre-B cell
    3.     Mature B cell
    4.     Early T cell
    5.     Mature T cell
Acute Myleocytic Leukemia (AML)

• Acute Non-lymphocytic Leukemia (ANLL)
• Most common in adults; >50% 60years old
• 70% of adults will enter remission with
  induction chemo
  – 25-35% of those in remission will have a 5 year
    survival rate
• BM transplant
Acute Myleocytic Leukemia (AML)

Treatment
• Selective radiation
• Chemotherapy
    1.     Induction
    2.     Intensification
    3.     Maintenance and consolidation
•        Bone marrow transplant
Chronic Leukemia

• Insidious onset
• Incidental findings during routine exam
Chronic Lymphocytic Leukemia

• Proliferation and accumulation of mature
  lymphocytes which are immunologically
  incompetent
  – B cell line (US)
  – T cell line (Asia)
• Hairy cell leukemia
Chronic Myelocytic Leukemia

• 15% of all leukemias
• Chromosomal abnormality (Ph1)
• Mostly B cell disease
  –   Leukocytosis
  –   Splenomegaly
  –   Hepatomegaly
  –   Lympadenopathy
• Bone marrow transplant  5 year survival for
  50-75% of patients
Chronic Myelocytic Leukemia

Two distinct phases
• Chronic
  – Last about 3-4 years
  – Near end  accelerated phase: fever, night sweats,
    malaise
• Acute
  – 2-4 months
  – Poor prognosis, palliative management
Malignant Lymphomas

• Primary solid tumors of the lymphoid system
• Cancers involving lymphocytes during
  maturation or storage in the bone marrow
• Third most common malignacy in children
Malignant Lymphomas

Hodgkin’s Lymphoma
• Disorders primarily involving the lymphoid
  tissues
• Anatomical spread
• Morphological presence of Reed-Sternberg cells
• 60-90% cure rate
Malignant Lymphomas

Manifestations of Hodgkin’s
• A symptoms
  – Painless progressive enlargement of a single or group
    of nodes (neck)
  – May spread continuously through out the lymphatic
    system
• B symptoms
  – Fever, night sweat, weight loss
  – Fatigue, anemia
Malignant Lymphomas

Treatment for Hodgkin’s
• Radiation
• Chemotherapy
Malignant Lymphomas

Non-Hodgkin’s Lymphoma
• Involves lymphoid tissue and may spread to
  various tissues
• Mostly B cell (80%)
• Cause may be viral or genetic
  – EBV
  – Immunosuppresed patients
     • AIDS
     • After organ transplant
Malignant Lymphomas

Treatment
• Early stage  radiation
• Late stage  chemo and radiation
• BM transplant

								
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