HAEMATOLOGICAL DISORDERS 19.12.2023 L.SUNEETHA (1).pdf

Anemias
Polycythemias &
Bleeding Disorders
By
L. SUNEETHA
M. Sc Nursing 1st year
SPGCON, Tirupathi

Definition
• Hematological disorders can be defined as,
“the diseases of the blood and blood
forming tissues.”
“A disorder marked by abberations in
structure or functions of the blood cells
are the blood clotting mechanism”

• It is a condition in which the hemoglobin
concentration is lower than the normal. As
a result, the amount of oxygen carrying
capacity to the body tissues is diminished.

man
13.5-16.5gm/dl
woman
12-15gm/dl
New born
17-22gm/dl

1. On the basis of cause
• Bleeding (blood loss)
• Hypoproliferative anemia (inadequate
production of normal blood cells)
• Hemolytic (destruction of blood cells)

2. On the basis of morphology
• Microcytic – If the cells are smaller than normal,
e.g. iron deficiency anemia, anemia of chronic
disease, thalassemia.
• Normocytic – if cells are in normal size, e.g.-
acute blood loss, anemia of chronic disease,
hemolytic anemia, Aplastic anemia.
• Macrocytic - if they are larger than normal,
e.g.-Megaloblastic anemia

1. Iron deficiency anemia – it is caused due to lack
of iron. It develops when body stores of iron
drops is too low to support normal RBCs
production.
2. Anemia of chronic disease – it is caused by
inflammation, infection and malignancy.
3. Thalassemia – it is a genetic disorder that is
characterized by abnormal formation of hemoglobin

4. Aplastic anemia – it is a rare disease in which
the bone marrow and hematopoietic stem cells
that are damaged leads to pancytopenia.
(deficiency of all three cellular components of
the blood such as red cells, white cells, and
platelets).
4. Megaloblastic or folic acid deficiency anemia -
in this condition the bone marrow usually
produce large, abnormal and immature RBCs. It
results from inhibition of DNA synthesis during
RBCs production so it leads to continuing cell
growth without division.

6. Pernicious anemia - it occurs when the intestine
can’t properly absorb vitamin B12.
7. Sickle cell anemia – it is characterized by RBC
that assume the abnormal, rigid, sickle shape.
It results from the presence of mutated form
of Hb.

• Decrease in RBCs, Hb, HCT levels
• Diminished oxygen carrying capacity
• Hypoxia and their affects on organ functions
• Signs and symptoms of anemia

• Easy fatigue and loss of energy
• Hypotension, rapid heart rate when exercise
• Shortness of breath and headache in
exercise.
• Difficulty in concentration.
• Dizziness, insomnia, leg cramps
• Pale skin, changes in stool color.
• Spleenomegaly

 History collection
 Physical examination
 Complete blood count
 Iron,Folate, Vitamin b12, bilirubin.
 Reticulocyte count
 Bone marrow biopsy

1. Blood transfusion – specially RBCs transfusion.
2. Iron supplements – oral ferrous sulphate,
parenteral iron therapy.
3. Nutritional therapy and dietary consideration
– Vitamin B12 rich diet, iron rich diet,
and folic acid rich diet.
4. Spleenectomy – removal of spleen.
5. Bone marrow and stem cell transplantation –
to enhance the production of RBCs, WBC,
platelets.

6. Activity restriction – minimize the
activities which induce symptoms of
anemia

• Hypoxia
• Severe fatigue
• Physical & mental growth retardation
• Neurologic damage
• Heart problem – irregular heart rate, heart
enlargement, heart failure
• Pregnancy complication – premature birth
• Death

1. Nursing diagnosis - Imbalanced nutrition less then
body requirement related to inadequate intake of
essential nutrients as evidenced by skin integrity,
color and body weight.
• Goals: Improve nutrition level
• Intervention:
• A healthy diet should be encouraged.
• Avoid alcoholic beverages.
• Dietary teaching sessions should be
individualized including related to food
preference and food culture aspect preparation.
NURSING MANAGEMENT

2. Nursing diagnosis – activity intolerance related
to low level of Hb in body as evidenced by the
weakness, fatigue and malaise.
• Goal: Improve the activity intolerance
• Intervention:
• assist the patient to prioritize the Activities and
establish a balance between the activity and
rest that is realistic and feasible from the
patient‘s perspective.
• Patient with chronic anemia need to maintain some
physical activity and exercise to prevent the
deconditioning that results from the inactivity.

3. Nursing diagnosis – ineffective tissue perfusion
related to less blood volume as evidenced by skin
color (pallor).
• Goal: Improve tissue perfusion.
• Intervention:
• The nurse monitor the vital sign closely.
• lost volume replaced with blood transfusion or IV
fluids.
• Supplemental oxygen may be necessary but it is
rarely needed on a long term basis.
• Other medication such as antihypertensive agent
may be needed to be adjusted.

Introduction :
Polycythemia is a blood disorder in which the body
produces too many blood cells as a result of a problem
with the bone marrow or an increased production of
the hormone erythropoietin(EPO).
• Often patients with polycythemia are detected
through an incidental finding of elevated hemoglobin
or hematocrit levels.
• Concern that the hemoglobin level may be abnormally
high is usually triggered at 170 g/L (17 g/dL) for men
and 150 g/L (15 g/dL)for women. Hematocrit levels
>50% in men or
>45% in women may be abnormal.
Definition :
• Polycythemia is defined as an increase in the
hemoglobin above normal.

POLYCYTHEMIA
RELATIVE ABSOLUTE
SECONDARY
APPROPRIATE INAPPROPRIATE
PRIMARY

• Dehydration
• Burns
• Prolonged vomitings
• Diarrhea
• Excessive diuretics
• Stress
RELATIVE POLYCYTHEMIA due to :
Low volume states :

• Polycythemia vera (PV) is a slow-growing type of
blood cancer in which the bone marrow makes too
many red blood cells. Polycythemia vera may also
result in production of too many of the other types
of blood cells — white blood cells and platelets.
• PV is a clonal stem cell disorder in which there is an
alteration in the pluripotent precursor cells leading
to excessive proliferation of erythroid, myeloid and
megakaryocytic progenitor cells. Over 95% of
patients with PV have acquired mutations of the
gene Janus Kinase 2 (JAK2) (kumar and clark’s clinical
medicine).

• polycythemia vera is caused by a change, or mutation, to DNA in a
single cell in the bone marrow.
RISK FACTORS
• Some factors may increase your risk of developing
polycythemia vera :
• Age. Polycythemia vera is more common in adults and older
than 60. It's rare in people younger than 20.
• Sex. Polycythemia vera affects more in men than in women
• Family history. gene JAK2 may contribute to the disease.
ETIOLOGY

• Headache
• Dizziness
• Itchiness
• Redness of the skin
• Breathing difficulty when you lie down
• Numbness, tingling, burning or weakness in
extremities.
• Chest pain
• A feeling of fullness or bloating abdomen
• Fatigue
CLINICAL MANIFESTATIONS

Blood tests Shows :
An increase in the number of red blood cells
Elevated hematocrit measurement,
Elevated levels of hemoglobin
Lower than normal levels of oxygen
Very low levels of erythropoietin (EPO)
Bone marrow aspiration or biopsy: Examination of bone
marrow shows that it's producing higher than normal
amounts of blood cells
DIAGNOSTIC EVALUATION

Minor criteria
 Bone marrow biopsy, showing hypercellularity for age with trilineage
growth (panmyelosis) with prominent erythroid, granulocytic and
megakaryocytic proliferation.
 Serum erythropoietin level below the reference range for normal
 Endogenous erythroid colony (EEC) formation in vitroa
 Diagnosis requires the presence of both major criteria and one minor
criterion or the presence of the first major criterion together with two
minor criteria.
Major criteria
•
 Haemoglobin >185 g/L in men, 165 g/L in women or other evidence of
increased red cell volume.
 Presence of JAK2 tyrosine kinase V617F or otherfunctionally similar
mutation such as JAK2 exon 12mutation.

Treatment may include :
 Phlebotomy.
 Medication to decrease blood cells.
 Low-dose aspirin.
 Therapy to reduce itching.

 I) Coagulation Factor
Deficiency:
 Congenital:
• Hemophilia A and B
• von Willebrand Disease
• Other factor deficiencies
 II) Fibrinolytic Defects:
• Streptokinase therapy
• DIC
Acquired
• Liver disease
• Vitamin K deficiency
• DIC

III) Vascular Disorders:
• Scurvy
• Purpura
• Hereditary hemorrhagic telangiectasia
• Cushing syndrome
• Ehlers Danlos syndrome

IV) Platelet Disorders:
1] Quantitative Disorders (Thrombocytopenia):
Acquired:
• Liver disease
• Drug-induced
• Alcoholism
Congenital:
• Glanzmann thrombasthenia
• von Willebrand disease
2] Qualitative Disorders:
Immune Mediated:
• Idiopathic
• Collagen vascular disease
• Sarcoidosis
Non-Immune Mediated:
• Microangiopathic hemolytic anemia
• Leukemia
• Myelofibrosis

Terminologies:
• Petechiae: small, pinpoint bleeding spot, measures 1-2 mm.
• Purpura: red, non-blanching maculo-paular lesions caused by
intradermal capillary bleeding. Measures ≥ 3mm.
• Ecchymosis: A discoloration of skin resulting from bleeding
underneath, typically caused by bruising. Measures ≥ 1cm.
• Hematoma: A pool of partially clotted blood in an organ, tissue or
body space, usually caused by a broken blood vessel.

• It’s important to have relevant clinical information before
interpreting results.
• From the history, it’s important to differentiate hereditary
disorders from acquired.
• Hereditary disorders generally start from an early age or
recurrent in nature and may have family history.
• Acquired disorders on the other hand occur at any age and have an
underlying predisposing cause.
• Depending on type of bleeding, it’s important to differentiate
between platelet and factor bleeds.

• History of medication should include replacement therapy, use of
anticoagulants like heparin besides intake of analgesics and
antibiotics etc.
• In general, symptoms of:
1. Platelet bleeds: petechial hemorrhage, mucosal bleed.
2. Coagulation factor disorders: deep abdominal bleeds, muscle
bleeds, hemostasis, ecchymotic patches.

• Bleeding time
• Clotting time
• Platelet count
• Activated partial thromboplastin time (aPTT)
• Prothrombin time (PT)
• Thrombin time (TT)
• D-dimer
Bleeding Time:
• It’s the time from puncture of skin and stoppage of blood oozing out.
• Normal Range: 2 – 5 mins
• Prolonged in platelet disorders: thrombocytopenia, thrombocytopenic
purpura.

Clotting Time:
• It’s the time required for blood to form a clot.
• Normal Range: 4 - 10 mins
• Prolonged in: Hemophilias, vitamin K deficiency.
aPTT (Activated Partial Thromboplastin Time):
• It’s performed by adding a surface activator (like kaolin/ellagic
acid), phospholipid and calcium to patient’s plasma.
• Normal Range: 26 – 37 sec
• Prolonged aPTT: Hemophilia A and B, von Willebrand disease, DIC,
heparin therapy.

Prothrombin Time:
• It’s measured by adding tissue factor (thromboplastin) and calcium to
the patient’s plasma.
• Normal Range: 12 – 16 sec
• Prolonged in: liver diseases, DIC, vitamin K deficiency,
D-dimer:
• It’s a fibrin degradation product, a small protein fragment present in
the blood after a blood clot is degraded by fibrinolysis.
• It contains 2 cross-linked D fragments of the fibrinogen protein.
• D-dimer concentration helps to diagnose thrombosis.
• While negative result practically rules out thrombosis, positive result
can indicate thrombosis but does not rule out other potential causes.

HAEMATOLOGICAL DISORDERS 19.12.2023 L.SUNEETHA (1).pdf

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