G6PD/Polycythemia Vera/ITP/Hemophilia/DIC PHINMA-UPANG

Prof: Leonardo R. Sanchez IV, RN CHS Batch 2024

Adapted from: PowerPoint/Lecture NUR 155 (MS LEC)
Transcribed by: Julia Rae Delos Santos (3BSN-12) September 13, 2022

an offending medication, they may develop pallor,

The abnormality in this disorder is in the G-6-PD gene;
this gene produces an enzyme within the RBC that is
essential for membrane stability.
A few patients have inherited an enzyme so defective
that they have a chronic hemolytic anemia; however, the
most common type of defect results in hemolysis is only
when the RBCs are stressed by certain situations, such as
fever or the use of certain medications.
Heinz Bodies
The type of deficiency found in the Mediterranean
population is more severe than that in the African
Caribbean population, resulting in greater hemodialysis
and sometimes in life-threatening anemia.
jaundice, and hemoglobinuria (hemoglobin in
urine). The reticulocyte count rises, and symptoms
of hemolysis develop.
❖ Special stains of the peripheral blood may then
disclose Heinz bodies – (degraded hemoglobin)
within the RBCs.
❖ Hemolysis is often mild and self-limited. However,
in the more severe Mediterranean type of G-6-PD
deficiency, spontaneous recovery may not occur
and transfusions may be necessary.

All types of G-6-PD deficiency are inherited as X-linked

defects; therefore, many more men are at risk than
women.

Medications that have hemolytic effects

for people with G6PD:
Assessment and Diagnostic Finding
▪ antimalarial drugs (chloroquine [Aralen])
❖ Diagnosis through Newborn Screening
▪ Sulfonamides(trimethoprim and
sulfomexazole)
▪ nitrofurantoin (Macrodantin)
▪ Analgesics (including aspirin in high doses)
▪ thiazide diuretics (hydrochlorothiazide,
chlorothiazide)
▪ Oral hypoglycemic agents (glyburide,
metformin)
▪ Chloramphenicol (Chlomycetin)
▪Vitamin K (phytonadione)
▪ In affected people, a severe hemolytic
episode can result from ingestion of fava beans
Medical Management

❖ The treatment is to stop the offending

Clinical Manifestations medication. Transfusion is necessary only in
❖ Patients are asymptomatic and have normal severe hemolytic state, which is more commonly
hemoglobin levels and reticulocyte counts most of seen in the Mediterranean variety of G-6-PD
the time. However, several days after exposure to deficiency.

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 G6PD/Polycythemia Vera/ITP/Hemophilia/DIC
Prof: Leonardo R. Sanchez IV, RN
Adapted from: PowerPoint/Lecture
Transcribed by: Julia Rae Delos Santos (3BSN-12)
Polycythemia vera, or primary polycythemia, is a
proliferative disorder in which the myeloid stem
cells seem to have escaped normal control
mechanisms.
The bone marrow is hypercellular, and the RBC, WBC,
and platelet counts in the peripheral blood are elevated.
However, the RBC elevation is predominant; the
hematocrit can exceed 60%.
This phase can last for an extended period {10 years or
longer}. The spleen resumes its embryonic function of
hematopoiesis and enlarges. Over time, the bone
marrow may become fibrotic, with a resultant inability to
produce as many cells {“burnt out” or spent phase}.
The disease evolves into myeloid metaplasia with
myelofibrosis and or AML in a significant proportion of
patients; this form of AML is usually refractory to
standard treatment.
The median survival time exceeds 15 years.
Medical Management
Clinical Manifestations
❖ Patients typically have a ruddy complexion
and splenomegaly {enlarged spleen}.
❖ The symptoms result from the increased
blood volume {headache, dizziness, tinnitus,
fatigue, paresthesias, and blurred vision} or
form increased blood viscosity {angina,
claudication, dyspnea, and thrombophlebitis},
particularly if the patient has atherosclerotic
blood vessels.
❖ Another common and bothersome problem is
generalized pruritus, which may be caused by
histamine release due to the increased
number of basophils.
❖ Erythromelalgia, a burning sensation in the
finger and toes, may be reported and is only
partially relieved by cooling.
PHINMA-UPANG
CHS Batch 2024
NUR 155 (MS LEC)
September 13, 2022
Assessment and Diagnostic Findings
❖ Diagnosis is made by finding an elevated RBC
mass (a nuclear medicine procedure), a
normal oxygen saturation level, and an
enlarged spleen.
❖ Other factors useful in establishing the
diagnosis include elevated WBC and platelet
counts.
❖ The erythropoietin level is not as low as would
be expected with an elevated hematocrit; it is
normal or only slightly low.
Complications
❖ Patients with polycythemia vera are at
increased risk for thromboses resulting in a
CVA (brain attack, stroke) or heart attack (MI);
thrombotic complications are the most
frequent cause of death.
❖ Bleeding is also a complication, possibly due to
the fact that the platelets {often very large}
are somewhat dysfunctional.
❖ The bleeding can be significant and can occur
in the form of nosebleeds, ulcers, and frank
gastrointestinal bleeding.
❖ Phlebotomy is an important part of therapy and
can be performed repeatedly to keep the
hematocrit within normal range. This is achieved
by removing enough blood {initially 500 mL once
or twice weekly} to deplete the patient’s iron
stores, thereby rendering the patient iron
deficient and consequently unable to continue
to manufacture RBCs excessively.
❖ Patients need to be instructed to avoid iron
supplements, including those within
multivitamin supplements.
❖ If the patient has an elevated uric acid
concentration, allopurinol [Zyloprim]
❖ If the patient develops ischemic symptoms,
dipyridamole [eg, Persantine] is sometimes
used.
❖ Radioactive phosphorus {32P] or
chemotherapeutic agents [eg, hydroxyurea
[Hydrea] can be used to suppress marrow
function, but they may increase the risk for
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 G6PD/Polycythemia Vera/ITP/Hemophilia/DIC PHINMA-UPANG
Prof: Leonardo R. Sanchez IV, RN CHS Batch 2024
Adapted from: PowerPoint/Lecture NUR 155 (MS LEC)
Transcribed by: Julia Rae Delos Santos (3BSN-12) September 13, 2022

leukemia. Patients receiving hydroxyurea

appear.
❖ Aspirin is also useful in diminishing pain
associated with erythromelalgia.
❖ Aagrelide (Agrylin) inhibits platelet aggregation What is ITP?
and can also be useful in controlling the
thrombocytosis associated with polycythemia ITP is a disease that affects people at all ages, but it is
vera. more common in children and young women.

Nursing Management Other names for the disorder are idiopathic

❖ The nurse’s role is primarily that of educator.
Risk factors for thrombotic complications
should be assessed, and patients should be
instructed regarding the signs and symptoms
of thrombosis.
❖ Patients with a history of bleeding are usually
advised to avoid aspirin and aspirin-containing
medications, because these medications alter
platelet function.
❖ Minimizing alcohol intake should also be
emphasized to further diminish any risk for
bleeding.
❖ For pruritus, the nurse may recommend
bathing in tepid or cool water, along with
applications of cocoa butter-based lotions and
bath products.
thrombocytopenic purpura and immune
thrombocytopenia.
Primary ITP occurs in isolation; secondary ITP often
results from autoimmune disease, viral infections
(hepatitis C and HIV), and various drugs (sulfa drugs).
Primary ITP is defined as a platelet count less than
100 × 10⁹/L with an inexplicable absence of a cause for
thrombocytopenia.
PATHOPHYSIOLOGY
ITP is an autoimmune disorder characterized by a
destruction of normal platelets by an unknown stimulus.
Antiplatelet antibodies develop in the blood
and bind to the patient’s platelets
↓
These antibody-bound platelets are then
ingested and destroyed by the reticuendothelial
system (RES) or tissue macrophages
↓
The body attempts to compensate for this
destruction by increasing platelet production
within the marrow
↓
However, platelet may also be impaired as the
antibodies may also induce cell death (via
apoptosis) of the megakaryocytes and thus
inhibit platelet production in the bone marrow

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 G6PD/Polycythemia Vera/ITP/Hemophilia/DIC
Prof: Leonardo R. Sanchez IV, RN
Adapted from: PowerPoint/Lecture
Transcribed by: Julia Rae Delos Santos (3BSN-12)
Clinical Manifestations
❖ Platelet count of less than 30,000/𝑚𝑚 3 is not
uncommon.
❖ Easy bruising, heavy menses in females, and
petechiae on the extremities or trunk.
❖ Patients with simple bruising or petechiae
(“dry purpura”) tend to have fewer
complications from bleeding than those with
bleeding from mucosal surfaces, such as the GI
tract (including the mouth) and pulmonary
system (e.g., hemoptysis), which is termed
wet purpura.
❖ Patients with wet purpura have a greater risk
of life-threatening bleeding than those with
dry purpura.
Assessment and Diagnostic Findings
❖ Patients should be tested for hepatitis C and
HIV to rule out potential causes.
❖ Bone marrow aspiration may reveal an
increase in megakaryocytes.
❖ Platelet count less than 20,000/𝑚𝑚 3 is a
common finding.
❖ Some patients may be infected with
helicobacter pylori, and eradicating the
infection may improve the platelet count. It is
unclear why H. pylori and ITP are correlated.
Medical Management
❖ The primary goal of treatment is a “safe”
platelet count. Because the risk of bleeding
typically does not increase until the platelet
count is less than 30,000/𝑚𝑚 3.
❖ Tell patients to stop taking sulfa-containing
medications if they have been taking any.
❖ Transfusions are ineffective because the
patient’s antiplatelet antibodies bind with the
transfused platelets, causing them to be
destroyed.
❖ Aminocaproic acid may be useful for patients
with significant mucosal bleeding since it slows
the dissolution of clots.
❖ IVIG is commonly used to treat ITP.
PHINMA-UPANG
CHS Batch 2024
NUR 155 (MS LEC)
September 13, 2022
❖ Monoclonal antibodies (e.g., rituximab) since it
may increase platelet counts for up to 1 year in
20% to 35% of those treated.
❖ Thrombopoietin receptor agonists:
Romiplostim (Nplate) is given weekly as
a subcutaneous injection.
Eltrombopag (Promacta) is given orally
on an empty stomach since food may
alter drug metabolism.
Surgical Management
❖ Splenectomy is an alternative
treatment and results in a sustained
normal platelet count approximately
50% of the time.
❖ Patients who have undergone
splenectomy are permanently at risk
for sepsis and shoud receive
pneumococcal, haemophilus influenza
type B, and meningococcal vaccines
preferably 2-3 weeks before
splenectomy.
Nursing Management
❖ Assessment of patient’s lifestyle to
determine the risk of bleeding from activity
❖ A careful medication history is also obtained
and be alert for sulfa-containing
medications and aspirin.
❖ All injections and rectal medications and
rectal temperature measurements should
be avoided because they can stimulate
bleeding.
❖ Encourage high-fiber diet to prevent
constipation.
❖ Avoid vigorous flossing of teeth; electric
razors should be used for shaving; soft-
bristled toothbrush should replace stiff-
bristled ones.
❖ Patient is also counseled to refrain from
vigorous sexual intercourse when the
platelet count is less than 10,000/𝑚𝑚3 .
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 G6PD/Polycythemia Vera/ITP/Hemophilia/DIC PHINMA-UPANG
Prof: Leonardo R. Sanchez IV, RN CHS Batch 2024
Adapted from: PowerPoint/Lecture NUR 155 (MS LEC)
Transcribed by: Julia Rae Delos Santos (3BSN-12) September 13, 2022

Nursing Management
❖ Advise the patient to watch signs of
- defect in clotting mechanisms of blood bleeding.
❖ Patient teaching would include the ff:
Genetic disorder: X-linked recessive transmission ▪ Avoid using sharp objects
➢ Usually occur in males ▪ Wear shoes at all times
➢ Classification ▪ use electric razor
o Factor VIII deficiency (Classic hemophilia); ▪ Cut nails across
hemophilia A ▪ Use soft-bristled toothbrush
o Factor IX deficiency (Christmas disease);
hemophilia B

Clinical Findings

▪ prolonged bleeding from any wound

▪ bleeding into joints (hemarthrosis), resulting in pain,

deformity, and retarded growth

▪ Intracranial hemorrhage DIC is not an actual disease but a sign of an underlying

condition. It may be triggered by sepsis, trauma, cancer,
Severity of bleeding shock, abruptio placenta, toxins, allergic reactions, and
other conditions.
▪ Mild
● Factor VIII activity of 5% to 50% The vast majority of cases (two-thirds) of DIC are
● Bleeding with severe trauma or surgery
initiated by infection or malignancy.
▪ Moderate The severity of DIC is variable, but potentially life-
● Factor VIII activity of 1% to 5% threatening.
● Bleeding with trauma

▪ Severe
●Factor VIII activity of 1%
● Spontaneous bleeding without trauma

Treatment Adjunctive Measures

▪ Control of bleeding ▪ Aminocaproic acid (Amicar):
▪ Prevention of bleeding inhibits the enzyme that
with use of factor destroy formed fibrin and
replacement increases fibrinogen activity
o Factor VIII in clot formation
concentrate from ▪ Fibrinogen: maintain plasma
recombinant DNA fibrinogen levels required for
clotting materials
o Factor IX complex
▪ Thrombin: suppies
contains factor II,
physiologic levels of natural
VII, IX, X
material at superficial
(concentrated) bleeding site to control
bleeding.

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 G6PD/Polycythemia Vera/ITP/Hemophilia/DIC
Prof: Leonardo R. Sanchez IV, RN
Adapted from: PowerPoint/Lecture
Transcribed by: Julia Rae Delos Santos (3BSN-12)
Medical Management Nursing Management
❖ Treat the underlying cause
❖ Improve oxygenation, replacing fluids, correcting
electrolyte imbalances, and administering
vasopressor medications is also important.
❖ Blood transfusion of platelet concentrate for
serious hemorrhage.
❖ Cryoprecipitate is given to replace fibrinogen
and factors V and VII.
❖ Heparin is used for patients with
thrombocytopenic manifestations.
PHINMA-UPANG
CHS Batch 2024
NUR 155 (MS LEC)
September 13, 2022
❖ Avoid procedures/activities that can increase
intracranial pressure.
❖ Monitor V/S closely including neurologic checks.
❖ Avoid medications that interfere with platelet
function (e.g., aspirin, beta-lactam antibiotics).
❖ Avoid rectal probes, and rectal medications
❖ Avoid injections
❖ Monitor amount of bleeding
❖ Perform oral hygiene carefully by using sponge-
tipped swabs, salt/baking soda mouth rinses.