Case Scenario:

A 25-year-old man of jew ethnicity was referred to a hematology clinic for LEGEND
erythrocytosis. He was seen in urgent care for flu-like symptoms, and routine complete
blood count revealed elevated hemoglobin and hematocrit levels. The patient noted a The 2016 WHO Classificationrevised the POLYCYTHEMIA Disease
history of discoloration in his hands, as well as chronic fatigue. He recalled being told
he had a high hemoglobin level when he was 12 years old but is not sure why. He has
previous diagnostic criteria for PV:
VERA Risk Factors
Major Criteria
symptoms of fever, night sweats, weight loss, pruritus, and burning/redness in his -Elevated hemoglobin (Hb) Diagnostic Test
extremities. He also has a history of venous thrombosis. -Hematocrit (Hct) levels
-Bone marrow panmyelosis Nursing Management
He is not a smoker and had no history of heart or lung disease, and no known family -AK2 mutations PREDISPOSING PRECIPITATING
Minor Criteria FACTORS FACTORS Pathophysiology
history of erythrocytosis or other blood disorders. He denied any history of snoring or
-Suppressed erythropoietin (EPO) level -Gender: Male -Over-exposure Clinical Manifestations
daytime sleepiness.
-Advanced Age to Mutagenic
-Race: Jews Agents Manifestations/Complications
The examination revealed an O2 saturation of 80 percent at rest and 60 percent with
moderate activity on pulse oximetry, and no evidence of lymphadenopathy or Medical Management
splenomegaly. His nail beds and lips showed a bluish discoloration. The rest of his Increased susceptibility Alteration/Damage Pharmacological Management
examination was normal. to gene mutation to the DNA

The patient?s lab results are as follows:

Hemoglobin : 16.1
Janus Kinase 2 Mutation
Hematocrit : 50.6
Leukocytes : 4.1 (normal differential)
Platelets :197 Expression of altered
Erythropoietin :25 gene product
Creatinine: 0.8

Cytokine-independent
signalling activity

Proliferation of Stem Cells

Hyperactivity of the Bone Excessive production of

Marrow defective blood cells
CBC
-Hg Level: ? 18/dL
-RBC: ? 6 million/mm
Bone Marrow Biopsy Myelofibrosis Bone Marrow Transplant
-Hct: ? 55%
-WBC: ? 10.000/mm
-Platelet: 500,000/uL

Extreme hypercellularity Massive production of Excessive production of Excessive production of

Erythropoietin Test Phlebotomy Shorter blood cell lifespan
of peripheral blood defective WBCs defective WBC's defective platelets
-EPO Level: 0-19
mU/mL
Hydroxyurea
Impaired oxygen-carrying Abnormal histamine Increased Impaired Impaired Rapid turnover of
Increased blood viscosity release susceptibility to clotting clotting circulating blood cells
capacity
infections capacity capacity

Increased Increased amount of cell Hepatomegaly

Slower
peripheral Occulusions of tiny debris Splenometogaly
blood flow Pruritus Peptic Ulcer Bleeding
resistance blood vessels in the
Risk for injury related to potential formation extremitites
of venous thrombi. Increased uric
Distended More sluggish
Independent: Vascular acid and
superficial Hypertension Impaired skin integrity related to abnormal blood flow
1. Tell patient to avoid constrictive or tight stasis potassium in
nerves histamine release secondary to Aspirin Erythromelalgia
clothing. the blood
Polycythemia Vera
2. Instruct to wear support hose or stockings
Dependent: Redness, burning
while awake and up. Aspirin Thrombosis
1. Communicate with a dietician as appropriate. sensation, and Gout and
3. Encourage to increase fluid intake. Aliopurindol
Independent: swelling of the Hyperkalemia
4. Advice to elevate feet when seated.
Pulse Oximetry 2. Encourage adequate nutrition and hydration extremities
5. Tell patient to avoid massage. Blood
-O2 saturation: 3. Instruct to avoid warm bath
vessel
97-100% 4. Encourage proper skin hygiene.
occlusions Ineffective protection related to abnormal blood
5. Instruct to keep fingernails short.
profiles (coagulation)
Dependent:
Ineffective tissue perfusion related to Risk for infection related to inadequate
Hypoxia 1. Administer Vitamin K and Protamine sulfate as
decreased oxygen concentration Risk for decreased cardiac output related to primary defenses
prescribed by the physician
secondary to impaired oxygen-carrying decreased circulation in the heart associated Independent:
Independent:
capacity of the blood. with myocardial infraction. 1. Promote nail care by keeping the client and
2. Educate patient to not take medications that
Dependent: Anoxia Dependent: the nurse?s fingernails short and clean.
contains warfarin and heparin
1. Administer oxygen therapy as prescribed. 1. Administer oxygen therapy as prescribed. 2. Promote and teach proper hand washing.
3. Instruct to use an electric shaver.
2. Administer medications to improve blood 2. Advice to exercise slowly and only on the advice 3. Encourage to observe personal hygiene.
4. Instruct to use soft-bristled toothbrush.
flow Infraction of the physician. 4. Instruct to avoid crowded places.
5. Advice to avoid any activities that may cause
Independent: Independent: 5. Instruct to avoid activities that may cause
injuries such as working with sharp objects.
3. Position patient in a semi or high fowlers 3. Instruct to stop activity at the first very signs of injuries.
position. Chest pain chest pains.
4. Educate patient about any prescribed and 4. Tell patient to take nitrate if chest pain occurs.
treatments such as Phlebotomy and abdominal 5. Identify an emergency plan, including the use of
chemotherapy and encourage compliance. pain CPR.
5. Monitor Vital signs and O2 Sat