cardio vascular Hematologic disorders (2).pdf
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Jun 30, 2024
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About This Presentation
ppt
Slide Content
O
Hematologic disorders
Hematologic disorders
Anemia
e Anemia is not a specific disease state but a sign of an
underlying disorder
o It is by far the most common hematologic condition
* Anemia, a condition in which the hemoglobin concentration
is lower than normal, reflects the presence of fewer than
normal RBCs within the circulation
° Asa result, the amount of oxygen delivered to body tissues
is also diminished
e Anemia is not a specific disease state but a sign of an
underlying disorder
o It is by far the most common hematologic condition
* Anemia, a condition in which the hemoglobin concentration
is lower than normal, reflects the presence of fewer than
normal RBCs within the circulation
° Asa result, the amount of oxygen delivered to body tissues
is also diminished
Anemia
° There are many different kinds of anemia but all can be
classified into three broad etiologic categories
e Loss of RBCs
+ Decreased production of RBCs
e Increased destruction of RBCs
© )
° There are many different kinds of anemia but all can be
classified into three broad etiologic categories
e Loss of RBCs
+ Decreased production of RBCs
e Increased destruction of RBCs
© )
Clinical Manifestations...
Aside from the severity of the anemia itself, several factors
influence the development of anemia-associated
symptoms:
e The speed with which the anemia has developed
o The duration of the anemia (ie, its chronicity)
e The metabolic requirements of the individual
° Other concurrent disorders or disabilities (eg,
cardiopulmonary disease)
y
Aside from the severity of the anemia itself, several factors
influence the development of anemia-associated
symptoms:
e The speed with which the anemia has developed
o The duration of the anemia (ie, its chronicity)
e The metabolic requirements of the individual
° Other concurrent disorders or disabilities (eg,
cardiopulmonary disease)
y
e The more rapidly an anemia develops, the more severe its
symptoms
o Healthy person can often tolerate as much as a 50% gradual
reduction in hemoglobin without pronounced symptoms or
significant incapacity
symptoms
o Healthy person can often tolerate as much as a 50% gradual
reduction in hemoglobin without pronounced symptoms or
significant incapacity
Iron deficiency anemia
o Iron deficiency anemia typically results when the intake
of dietary iron is inadequate for hemoglobin synthesis
o The body can store about one fourth to one third of its
iron, and it is not until those stores are depleted that
iron deficiency anemia actually begins to develop
o Iron deficiency anemia is the most common type of
anemia in all age groups, and it is the most common
anemia in the world
o )
o Iron deficiency anemia typically results when the intake
of dietary iron is inadequate for hemoglobin synthesis
o The body can store about one fourth to one third of its
iron, and it is not until those stores are depleted that
iron deficiency anemia actually begins to develop
o Iron deficiency anemia is the most common type of
anemia in all age groups, and it is the most common
anemia in the world
o )
Causes
° In men and postmenopausal women is bleeding (from
ulcers, gastritis, inflammatory bowel disease, or
gastrointestinal tumors)
° In premenopausal women is menorrhagia (excessive
menstrual bleeding) and pregnancy with inadequate iron
supplementation
e Patients with chronic alcoholism often have chronic blood
loss from the gastrointestinal tract, which causes iron loss
and eventual anemia
° Iron malabsorption, as is seen after gastrectomy or with
celiac disease
® )
° In men and postmenopausal women is bleeding (from
ulcers, gastritis, inflammatory bowel disease, or
gastrointestinal tumors)
° In premenopausal women is menorrhagia (excessive
menstrual bleeding) and pregnancy with inadequate iron
supplementation
e Patients with chronic alcoholism often have chronic blood
loss from the gastrointestinal tract, which causes iron loss
and eventual anemia
° Iron malabsorption, as is seen after gastrectomy or with
celiac disease
® )
Clinical Manifestations
o Fatigue, dyspnea, palpitation, syncope
* Headache, lightheadedness, tinnitus, vertigo, difficulty
of concentration
o Anorexia, nausea, indigestion
o Pallor, tachycardia,
systolic murmur.
wide pulse
pressure / ejection
y
o Fatigue, dyspnea, palpitation, syncope
* Headache, lightheadedness, tinnitus, vertigo, difficulty
of concentration
o Anorexia, nausea, indigestion
o Pallor, tachycardia,
systolic murmur.
wide pulse
pressure / ejection
y
Clinical Manifestations (Typical)
o Pica: desire (craving) to eat unusual substances like soil,
ice.
o Koilonychia: Thin, brittle nail with depressed (concave
or spoon) distal half.
e Glossitis or angular stomatitis: the tongue and angel the
mouth inflamed and sore.
© )
o Pica: desire (craving) to eat unusual substances like soil,
ice.
o Koilonychia: Thin, brittle nail with depressed (concave
or spoon) distal half.
e Glossitis or angular stomatitis: the tongue and angel the
mouth inflamed and sore.
© )
Assessment and Diagnostic Findings
e CBC
e low Hgb and hematocrit,
elow MCV,
elow MCH,
e CBC
e low Hgb and hematocrit,
elow MCV,
elow MCH,
Medical Management
e Several oral iron preparations—ferrous sulfate, ferrous
fumerate and ferrous gluconate—are available for treating
iron deficiency anemia
+ In some cases, oral iron is poorly absorbed or poorly
tolerated, or iron supplementation is needed in large
amounts. In these situations, intravenous or intramuscular
administration of iron dextran may be needed
o Before parenteral administration of a full dose, a small test
dose should be administered to avoid the risk of anaphylaxis
with either intravenous or intramuscular injections
e Several oral iron preparations—ferrous sulfate, ferrous
fumerate and ferrous gluconate—are available for treating
iron deficiency anemia
+ In some cases, oral iron is poorly absorbed or poorly
tolerated, or iron supplementation is needed in large
amounts. In these situations, intravenous or intramuscular
administration of iron dextran may be needed
o Before parenteral administration of a full dose, a small test
dose should be administered to avoid the risk of anaphylaxis
with either intravenous or intramuscular injections
Medical Management
e Indications for IV iron therapy
* Intolerance to oral iron therapy.
* Anemia secondary to chronic kidney disease with a
requirement for erythropoietin.
* No improvement in hemoglobin after 4 weeks of oral iron.
e Existence of conditions that interfere with absorption of iron
from the GI tract e.g., atrophic gastritis, gastrectomy,
inflammatory bowel disease
* Blood loss difficult to cope with oral iron therapy e.g., heavy
menstrual bleeding, bleeding telangiectasia
© Severe anemia during late second or third trimester of
pregnancy
e Indications for IV iron therapy
* Intolerance to oral iron therapy.
* Anemia secondary to chronic kidney disease with a
requirement for erythropoietin.
* No improvement in hemoglobin after 4 weeks of oral iron.
e Existence of conditions that interfere with absorption of iron
from the GI tract e.g., atrophic gastritis, gastrectomy,
inflammatory bowel disease
* Blood loss difficult to cope with oral iron therapy e.g., heavy
menstrual bleeding, bleeding telangiectasia
© Severe anemia during late second or third trimester of
pregnancy
Medical Management
e IV iron administration
e Iron sucrose 200mg, IV, administer over 5 minutes,
every 3 days for a total of 5 doses (a total of 1000mg).
This dose is usually sufficient but if hemoglobin is not
corrected, additional doses can be given.
OR
e Iron sucrose 200mg diluted in 100ml NS; administer
over 30 minutes.
e IV iron administration
e Iron sucrose 200mg, IV, administer over 5 minutes,
every 3 days for a total of 5 doses (a total of 1000mg).
This dose is usually sufficient but if hemoglobin is not
corrected, additional doses can be given.
OR
e Iron sucrose 200mg diluted in 100ml NS; administer
over 30 minutes.
Medical Management
Iron dextran
° Intramuscular supplementation is used infrequently
° The volume of iron required may be excessive. The intramuscular
injection causes some local pain and can stain the skin
° These side effects are minimized by using the Z-track technique
for administering iron dextran deep into the gluteus maximus
muscle (buttock)
° Avoid vigorously rubbing the injection site
© )
Iron dextran
° Intramuscular supplementation is used infrequently
° The volume of iron required may be excessive. The intramuscular
injection causes some local pain and can stain the skin
° These side effects are minimized by using the Z-track technique
for administering iron dextran deep into the gluteus maximus
muscle (buttock)
° Avoid vigorously rubbing the injection site
© )
Nursing Management
o Preventive education is important, because iron deficiency
anemia is common in menstruating and pregnant women
Food sources high in iron include organ meats (beef or
calf’s liver, chicken liver), other meats, beans, leafy green
vegetables
o Taking iron-rich foods with a source of vitamin C
+ Because iron is best absorbed on an empty stomach, patients
should be advised to take the supplement 2 hours before
meals
o Preventive education is important, because iron deficiency
anemia is common in menstruating and pregnant women
Food sources high in iron include organ meats (beef or
calf’s liver, chicken liver), other meats, beans, leafy green
vegetables
o Taking iron-rich foods with a source of vitamin C
+ Because iron is best absorbed on an empty stomach, patients
should be advised to take the supplement 2 hours before
meals
o If taking iron on an empty stomach causes gastric distress,
the patient may need to take the iron supplement with meals
e Antacids or dairy products should not be taken with iron,
because they greatly diminish the absorption of iron
the patient may need to take the iron supplement with meals
e Antacids or dairy products should not be taken with iron,
because they greatly diminish the absorption of iron
Aplastic anemia
e Aplastic anemia is a rather rare disease caused by a decrease
in or damage to marrow stem cells, damage to the
microenvironment within the marrow, and replacement of
the marrow with fat
o It results in bone marrow aplasia (markedly reduced
hematopoiesis)
+ Therefore, in addition to severe anemia, significant
neutropenia and thrombocytopenia (a deficiency of
platelets) are also seen.
e Aplastic anemia is a rather rare disease caused by a decrease
in or damage to marrow stem cells, damage to the
microenvironment within the marrow, and replacement of
the marrow with fat
o It results in bone marrow aplasia (markedly reduced
hematopoiesis)
+ Therefore, in addition to severe anemia, significant
neutropenia and thrombocytopenia (a deficiency of
platelets) are also seen.
Pathophysiology
° Aplastic anemia can be congenital or acquired, but most
cases are idiopathic
e Infections can trigger it
o It may be caused by certain medications, chemicals, or
radiation damage
e Agents that regularly produce marrow aplasia include
benzene and benzene derivatives
o Certain toxic materials, such as inorganic arsenic and several
pesticides as potential causes
° Aplastic anemia can be congenital or acquired, but most
cases are idiopathic
e Infections can trigger it
o It may be caused by certain medications, chemicals, or
radiation damage
e Agents that regularly produce marrow aplasia include
benzene and benzene derivatives
o Certain toxic materials, such as inorganic arsenic and several
pesticides as potential causes
Clinical Manifestations
o The manifestations of aplastic anemia are often insidious
* Anemic features are similar to other types
+ Complications resulting from bone marrow failure may
occur before the diagnosis is established
° Typical complications are infection and symptoms of anemia
(eg, fatigue, pallor, dyspnea)
° Purpura (bruising) may develop later and should trigger a
© CBC and hematologic evaluation
o The manifestations of aplastic anemia are often insidious
* Anemic features are similar to other types
+ Complications resulting from bone marrow failure may
occur before the diagnosis is established
° Typical complications are infection and symptoms of anemia
(eg, fatigue, pallor, dyspnea)
° Purpura (bruising) may develop later and should trigger a
© CBC and hematologic evaluation
Management
+ A bone marrow transplantaton (BMT) may cure the disease
° Supportive therapy plays a major role in the management of
aplastic anemia
° Any offending agent is discontinued
° The patient is supported with transfusions of RBCs and
platelets as necessary
° Death usually is caused by hemorrhage or infection
+ A bone marrow transplantaton (BMT) may cure the disease
° Supportive therapy plays a major role in the management of
aplastic anemia
° Any offending agent is discontinued
° The patient is supported with transfusions of RBCs and
platelets as necessary
° Death usually is caused by hemorrhage or infection
Megaloblastic anemias
o In the anemias caused by deficiencies of vitamin B12 or folic
acid, identical bone marrow and peripheral blood changes
occur, because both vitamins are essential for normal DNA
synthesis
o In either anemia, the RBCs that are produced are abnormally
large and are called megaloblastic RBCs.
e Other cells derived from the myeloid stem cell (nonlymphoid
WBCSs, platelets) are also abnormal
e )
o In the anemias caused by deficiencies of vitamin B12 or folic
acid, identical bone marrow and peripheral blood changes
occur, because both vitamins are essential for normal DNA
synthesis
o In either anemia, the RBCs that are produced are abnormally
large and are called megaloblastic RBCs.
e Other cells derived from the myeloid stem cell (nonlymphoid
WBCSs, platelets) are also abnormal
e )
Pathophysiology
Folic acid deficiency
o Folic acid, a vitamin that is necessary for normal RBC
production, is stored in folate stores
o The folate stores in the body are much smaller than
those of vitamin B12, and they are quickly depleted
when the dietary intake of folate is deficient (within 4
months)
o Folate is found in green vegetables and liver
o Folate deficiency occurs in people who rarely eat
uncooked fruits and vegetables
e )
Folic acid deficiency
o Folic acid, a vitamin that is necessary for normal RBC
production, is stored in folate stores
o The folate stores in the body are much smaller than
those of vitamin B12, and they are quickly depleted
when the dietary intake of folate is deficient (within 4
months)
o Folate is found in green vegetables and liver
o Folate deficiency occurs in people who rarely eat
uncooked fruits and vegetables
e )
Vitamin B12 deficiency
o A deficiency of vitamin B12 can occur in several ways
Inadequate dietary intake is rare but can develop in strict
vegetarians who consume no meat or dairy products
o Faulty absorption from the gastrointestinal tract is more
common
e Another cause is the absence of intrinsic factor, as in
pernicious anemia
o A deficiency of vitamin B12 can occur in several ways
Inadequate dietary intake is rare but can develop in strict
vegetarians who consume no meat or dairy products
o Faulty absorption from the gastrointestinal tract is more
common
e Another cause is the absence of intrinsic factor, as in
pernicious anemia
Clinical manifestations
+ Symptoms of folic acid and vitamin B12 deficiencies are similar,
and the two anemias may coexist.
+ However, the neurologic manifestations of vitamin B12
deficiency do not occur with folic acid deficiency, and they
persist if B12 is not replaced.
e Careful distinction between the two anemias must be made.
+ Symptoms of folic acid and vitamin B12 deficiencies are similar,
and the two anemias may coexist.
+ However, the neurologic manifestations of vitamin B12
deficiency do not occur with folic acid deficiency, and they
persist if B12 is not replaced.
e Careful distinction between the two anemias must be made.
Clinical manifestations
° Gastrointestinal manifestations are common, including
e diarrhea,
e glossitis, and
e Anorexia
* Neurologic manifestations,
e peripheral neuropathy,
e gait instability,
9 paresthesias
° Gastrointestinal manifestations are common, including
e diarrhea,
e glossitis, and
e Anorexia
* Neurologic manifestations,
e peripheral neuropathy,
e gait instability,
9 paresthesias
Diagnostic Investigations
e CBC
* Anemia with high MCV (>100fl).
e Leukopenia and thrombocytopenia may also be found.
e Serum Cobalamin or folate level is decreased
e CBC
* Anemia with high MCV (>100fl).
e Leukopenia and thrombocytopenia may also be found.
e Serum Cobalamin or folate level is decreased
Medical Management
o Folic acid, 1 to 5mg P.O., daily for 1-4 months, or until
complete hematologic recovery.
° Vitamin B12 level should be checked before giving folic acid
alone; as treatment with folic acid alone might worsen
neurologic manifestation of vitamin B12 deficiency.
e If vitamin B12 can’t be checked, both Folic acid and vitamin
B12 should be started at the same time.
o Folic acid, 1 to 5mg P.O., daily for 1-4 months, or until
complete hematologic recovery.
° Vitamin B12 level should be checked before giving folic acid
alone; as treatment with folic acid alone might worsen
neurologic manifestation of vitamin B12 deficiency.
e If vitamin B12 can’t be checked, both Folic acid and vitamin
B12 should be started at the same time.
Medical Management
° Cyanocobalamin (Vitamin B12) 1000micrograms (1mg),
IM, to be given according to the following schedule
o Every day for one week
° Every week for four weeks. If hemoglobin has not
normalized, continue weekly until it gets normal.
o If the underlying disorder persists, Img every month for
the rest of the patient’s life
° Cyanocobalamin (Vitamin B12) 1000micrograms (1mg),
IM, to be given according to the following schedule
o Every day for one week
° Every week for four weeks. If hemoglobin has not
normalized, continue weekly until it gets normal.
o If the underlying disorder persists, Img every month for
the rest of the patient’s life
Iron - folate supplementation
+ WHO has recommended a 6-month regimen of a daily
supplement containing 60 mg of elemental iron along with
400 mcg of folic acid for all pregnant women.
° In areas with a higher prevalence of anemia, it is
recommended that supplementation continues for three
months postpartum.
° Similarly, the Ethiopian national guideline for control and
prevention of micronutrient deficiencies endorsed the above
prevention protocol
+ WHO has recommended a 6-month regimen of a daily
supplement containing 60 mg of elemental iron along with
400 mcg of folic acid for all pregnant women.
° In areas with a higher prevalence of anemia, it is
recommended that supplementation continues for three
months postpartum.
° Similarly, the Ethiopian national guideline for control and
prevention of micronutrient deficiencies endorsed the above
prevention protocol
SICKLE CELL ANEMIA
o Sickle cell anemia is a severe hemolytic anemia that results
from inheritance of the sickle hemoglobin gene
° This gene causes the hemoglobin molecule to be defective
° Consequently, the RBC containing (HbS) loses its round,
very pliable, biconcave disk shape and becomes deformed,
rigid, and sickle-shaped
o Sickle cell anemia is a severe hemolytic anemia that results
from inheritance of the sickle hemoglobin gene
° This gene causes the hemoglobin molecule to be defective
° Consequently, the RBC containing (HbS) loses its round,
very pliable, biconcave disk shape and becomes deformed,
rigid, and sickle-shaped
Etiology and pathophysiology
° In sickle cell anemia, the hemoglobin in RBC acquires an
elongated crescent or sickle shape
° The sickled cell are rigid and obstruct capillary blood flow
+ Microscopic obstructions lead to engorgement and tissue
ischemia
+ The local tissue hypoxia causes further sickling and
ultimately large infarction
+ Damaged tissue in organs throughout the body become
scarred, resulting impaired function
° In sickle cell anemia, the hemoglobin in RBC acquires an
elongated crescent or sickle shape
° The sickled cell are rigid and obstruct capillary blood flow
+ Microscopic obstructions lead to engorgement and tissue
ischemia
+ The local tissue hypoxia causes further sickling and
ultimately large infarction
+ Damaged tissue in organs throughout the body become
scarred, resulting impaired function
Clinical manifestations
° Symptoms of sickle cell anemia vary and are only somewhat
based on the amount of Hgb
e Symptoms and complications result from chronic hemolysis
or thrombosis
° The sickled RBCs have a shortened life span
o Patients are always anemic, usually with hemoglobin values
of 7 to 10 g/dL
o Jaundice is characteristic and is usually obvious in the sclera
+ The bone marrow expands in childhood in a compensatory
effort to offset the anemia, sometimes leading to
enlargement of the bones of the face and skull
° Symptoms of sickle cell anemia vary and are only somewhat
based on the amount of Hgb
e Symptoms and complications result from chronic hemolysis
or thrombosis
° The sickled RBCs have a shortened life span
o Patients are always anemic, usually with hemoglobin values
of 7 to 10 g/dL
o Jaundice is characteristic and is usually obvious in the sclera
+ The bone marrow expands in childhood in a compensatory
effort to offset the anemia, sometimes leading to
enlargement of the bones of the face and skull
Management
Currently there are only three primary treatment modalities for sickle
cell diseases:
BMT
Hydroxyurea (Chemotherapeutic agent)
wn =
Long term RBC transfusion
+ BMT offers the potential for cure for this disease. However, this
treatment modality is available to only a small subset of the patient
population, because of either the lack of a compatible donor or the
severe organ (eg, renal, liver, lung) damage already present in the
patient
Currently there are only three primary treatment modalities for sickle
cell diseases:
BMT
Hydroxyurea (Chemotherapeutic agent)
wn =
Long term RBC transfusion
+ BMT offers the potential for cure for this disease. However, this
treatment modality is available to only a small subset of the patient
population, because of either the lack of a compatible donor or the
severe organ (eg, renal, liver, lung) damage already present in the
patient
Hemophilia
° Hemophilia is usually an inherited condition and is
caused by the deficiency of clotting factors in the
blood.
o It is almost always due to a defect or mutation in the
gene for the clotting factor.
° Hemophilia A and B are the most common severe
hereditary hemorrhagic disorders.
° Hemophilia is usually an inherited condition and is
caused by the deficiency of clotting factors in the
blood.
o It is almost always due to a defect or mutation in the
gene for the clotting factor.
° Hemophilia A and B are the most common severe
hereditary hemorrhagic disorders.
Hemophilia
e Both hemophilia A and B are inherited via an X-
linked recessive pattern
+ Hemophilia A and B result from factor VII and factor
IX protein deficiency.
o Patients present with prolonged bleeding with or
without trauma
@ )
e Both hemophilia A and B are inherited via an X-
linked recessive pattern
+ Hemophilia A and B result from factor VII and factor
IX protein deficiency.
o Patients present with prolonged bleeding with or
without trauma
@ )
Contact activation Tissue factor
(intrinsic) pathway (extrinsic) pathway
Damaged surface
Trauma
TFPI
XII Xila wa
TT A Vila vil
XI Xla vu,
4 nO j Tissue factor «—— Trauma
IXa Villa |
Aah Ay - Antithrombin
x
Prothrombin (D > E Thrombin (lla) Common
x Va pathway
Whe
Be Fibrinogen (I) Fibrin (la),
i ae
Active Protein C alla Au
Protein S Cross-linked
pe fibrin clot
Protein C + Thrombomodulin
(intrinsic) pathway (extrinsic) pathway
Damaged surface
Trauma
TFPI
XII Xila wa
TT A Vila vil
XI Xla vu,
4 nO j Tissue factor «—— Trauma
IXa Villa |
Aah Ay - Antithrombin
x
Prothrombin (D > E Thrombin (lla) Common
x Va pathway
Whe
Be Fibrinogen (I) Fibrin (la),
i ae
Active Protein C alla Au
Protein S Cross-linked
pe fibrin clot
Protein C + Thrombomodulin
Hemophilia - Clinical features
+ Depending on the extent of deficiency, patients with
hemophilia may present with
easy bruising;
* easy bruising
* inadequate clotting of traumatic or even mild injury;
or,
* in the case of severe hemophilia, spontaneous
hemorrhage.
+ Depending on the extent of deficiency, patients with
hemophilia may present with
easy bruising;
* easy bruising
* inadequate clotting of traumatic or even mild injury;
or,
* in the case of severe hemophilia, spontaneous
hemorrhage.
Hemophilia - Clinical features
o Signs of hemorrhage include the following:
© General: Weakness, orthostasis, tachycardia, tachypnea
e Musculoskeletal (joints): Tingling, cracking, warmth,
pain, stiffness, and refusal to use joint (children)
e CNS: Headache, stiff neck, vomiting, lethargy,
irritability, and spinal cord syndromes
o Signs of hemorrhage include the following:
© General: Weakness, orthostasis, tachycardia, tachypnea
e Musculoskeletal (joints): Tingling, cracking, warmth,
pain, stiffness, and refusal to use joint (children)
e CNS: Headache, stiff neck, vomiting, lethargy,
irritability, and spinal cord syndromes
Hemophilia - Clinical features
o Signs of hemorrhage include the following:
e Gastrointestinal: Hematemesis, melena, frank red
blood per rectum, and abdominal pain
* Genitourinary: Hematuria, renal colic, and post-
circumcision bleeding
° Other: Epistaxis, oral mucosal hemorrhage,
hemoptysis, dyspnea (hematoma leading to airway
obstruction), compartment syndrome symptoms, and
contusions; excessive /prolonged bleeding with routine
dental or other procedures
o Signs of hemorrhage include the following:
e Gastrointestinal: Hematemesis, melena, frank red
blood per rectum, and abdominal pain
* Genitourinary: Hematuria, renal colic, and post-
circumcision bleeding
° Other: Epistaxis, oral mucosal hemorrhage,
hemoptysis, dyspnea (hematoma leading to airway
obstruction), compartment syndrome symptoms, and
contusions; excessive /prolonged bleeding with routine
dental or other procedures
Hemophilia - Diagnostic Investigations
° Complete blood cell count
° Screening coagulation studies (prothrombin time
[PT], activated partial thromboplastin time [aPTT])
e FVIII or FIX assay
° Complete blood cell count
° Screening coagulation studies (prothrombin time
[PT], activated partial thromboplastin time [aPTT])
e FVIII or FIX assay
Hemophilia - Diagnostic Investigations
° Imaging studies
e Head computed tomography scans
e MRI scans of the head, spinal column, and joints
e Ultrasonography
° Imaging studies
e Head computed tomography scans
e MRI scans of the head, spinal column, and joints
e Ultrasonography
Hemophilia - Medical & Nursing Interventions
° Control bleeding
e Local measures
» Direct pressure
Elevation
Ice application
e Epistaxis
Sit up and lean forward
° Control bleeding
e Local measures
» Direct pressure
Elevation
Ice application
e Epistaxis
Sit up and lean forward
Hemophilia - Medical & Nursing Interventions
e Avoid
© Joint degeneration — Progressive exercise when there
is no active bleeding
e Injections
e NSAIDs — especially Aspirin
e Activities which require intense contact
e Avoid
© Joint degeneration — Progressive exercise when there
is no active bleeding
e Injections
e NSAIDs — especially Aspirin
e Activities which require intense contact
Hemophilia - Medical & Nursing Interventions
e Replacement therapy
e Fresh whole blood
o Fresh frozen plasma
e Cryoprecipitate
e Factor VIII or IX concentrate
e Replacement therapy
e Fresh whole blood
o Fresh frozen plasma
e Cryoprecipitate
e Factor VIII or IX concentrate
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