Polychyemia

Introduction
• Polycythemia is the production and presence of increased numbers of
RBCs.
• The increase in RBCs can be so great that blood circulation is impaired
because of the increased blood viscosity (hyperviscosity) and volume
(hypervolemia).
• The 2 types of polycythemia

Primary
Secondary
polycythemia, or
polycythemia
polycythemia vera
Primary polycythemia/Polycythemia vera
• Polycythemia vera is a chronic myeloproliferative disorder.
• It involves not only RBCs, but also WBCs and platelets, with an
increased production of all these blood cells.
• This leads to enhanced blood viscosity and blood volume and
congestion of organs and tissues with blood.
• Splenomegaly and hepatomegaly are common.
• Patients have hypercoagulopathies that predispose them to clotting.
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• Polycythemia vera is associated with
• mutations in the Janus kinase-2 (JAK2) gene.

• The JAK2 gene provides instructions for making a protein that

promotes proliferation of cells, especially blood cells from
hematopoietic stem cells.
• Polycythemia vera begins with 1 or more DNA mutations of a single
hematopoietic stem cell.
Secondary polycythemia
• Secondary polycythemia can be either hypoxia driven or hypoxia
independent.
• In hypoxia-driven secondary polycythemia, hypoxia stimulates
erythropoietin (EPO) production in the kidneys, which in turn
stimulates RBC production.
• The need for O2 may result from high altitude, lung disease,
cardiovascular disease, alveolar hypoventilation, defective O2
transport, or tissue hypoxia.
• In hypoxia-independent secondary polycythemia, cancer or benign
tumor tissue makes EPO.
Clinical Manifestations
• Headache, vertigo, dizziness, tinnitus,
and visual changes
• Generalized pruritus (often
exacerbated by a hot bath) may be a
striking symptom and is related to
histamine release from an increased
number of basophils.
• The patient may have angina, HF,
intermittent claudication
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• Paresthesias and
• erythromelalgia
• (painful burning and redness of the hands
• and feet) may be present.
• The patient may have pain from peptic ulcer caused either by
increased gastric secretions or liver and spleen engorgement.
• Plethora (ruddy complexion) may be present.
Complications
• The most common serious complication is stroke from a thrombosis.
• Hemorrhagic phenomena caused by either vessel rupture from
overdistention or inadequate platelet function may result in
petechiae, bruising, nosebleeds, or GI bleeding.
• Hepatomegaly and splenomegaly
• Hyperuricemia and gout.
• (Uric acid is one of the products of RBC destruction.
• As RBC destruction increases, uric acid production increases )
Diagnostic Studies
• The laboratory manifestations seen in a patient with polycythemia vera include
• (1) high hemoglobin and RBC count with microcytosis;
• (2) low to normal EPO level (secondary polycythemia has a high level);
• (3) high WBC count with basophilia and neutrophilia;
• (4) high platelet count (thrombocytosis) and platelet dysfunction;
• (5) high leukocyte alkaline phosphatase, uric acid, and cobalamin levels; and
• (6) high histamine levels.
• Bone marrow examination in polycythemia vera shows hypercellularity of RBCs,
WBCs, and platelets
Management

• Phlebotomy is the mainstay of treatment.

• The aim is to reduce the hematocrit and keep it less than 45%.
• Phlebotomy then may be needed every 2 to 3 months, reducing the
blood volume by about 500 mL each time.
• A person managed with repeated phlebotomies eventually becomes
deficient in iron, although this effect is rarely symptomatic.
• Avoid iron supplementation. Hydration therapy can reduce the
blood’s viscosity
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• Myelosuppressive agents, such as hydroxyurea, busulfan (Myleran),
and chlorambucil (Leukeran), may be given.
• Ruxolitinib (Jakafi), which inhibits the expression of the JAK2
mutation, is given to patients who have not responded to
hydroxyurea.
• Other therapies include anagrelide (Agrylin), which can reduce the
platelet count and inhibit platelet aggregation.
• Low-dose aspirin is used to prevent clotting.
• α-Interferon (α-IFN) and pegylated interferon are given to women of
childbearing age or those with intractable pruritus.
• Allopurinol (Zyloprim) may reduce the number of acute gouty attacks
Nursing care
• Assess fluid intake and output during hydration therapy to avoid fluid
overload (which worsens circulatory congestion) or fluid deficit (which
makes the blood even more viscous).
• If myelosuppressive agents are used, give the drugs as ordered,
observe the patient, and teach the patient about the side effects of
the drugs.
• Assess the patient’s nutritional status, since inadequate food intake
can result from GI symptoms of fullness, pain, and dyspepsia.
• Begin activities and/or medications to decrease thrombus formation.
Start active or passive leg exercises and ambulation when possible.
• Because of its chronic nature, polycythemia vera requires ongoing
evaluation. Assess the patient for complications
THROMBOCYTOPENIA
Definition
• Thrombocytopenia is a reduction of platelets below 150,000/µL (150
× 10 9 /L).
• Acute, severe, or prolonged decreases from this normal range can
result in abnormal hemostasis that presents as prolonged bleeding
from minor trauma or spontaneous bleeding without injury.
• Types
Immune Thrombocytopenia
• The most common acquired thrombocytopenia is a syndrome of
abnormal destruction of circulating platelets termed immune
thrombocytopenia (ITP).
• ITP is an acquired immune disorder in which the thrombocytopenia
results from antiplatelet antibodies, impaired platelet production, and
T-cell–mediated destruction of platelets.
• Platelets are coated with antibodies.
• These platelets function normally; however, when they reach the
spleen, these antibody coated platelets are mistaken as foreign and
destroyed by macrophage
• Platelets normally survive 8 to 10 days. However, in ITP, platelet
survival is shortened
Thrombotic Thrombocytopenic Purpura
• Thrombotic thrombocytopenic purpura (TTP) is an uncommon
syndrome characterized by hemolytic anemia, thrombocytopenia,
neurologic abnormalities, fever (in the absence of infection), and
renal abnormalities.
• Not all features are present in all patients.
• Because it is almost always associated with hemolytic-uremic
syndrome (HUS), TTP is often referred to as TTP-HUS
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• TTP is caused by the deficiency of a plasma enzyme (ADAMTS13) that
breaks down the von Willebrand clotting factor (vWF) into normal
size.
• vWF is the most important protein-mediating platelet adhesion to
damaged endothelial cells.
• Without the enzyme, unusually large amounts of vWF attach to
activated platelets, promoting platelet aggregation.
• TTP is a medical emergency because bleeding and clotting occur at
the same time.
Heparin-Induced Thrombocytopenia
• One of the risks associated with the use of heparin is the
development of heparin-induced thrombocytopenia (HIT), also called
heparin-induced thrombocytopenia and thrombosis syndrome
(HITTS).
• patients develop thrombocytopenia 5 to 10 days after the onset of
heparin therapy.
• HIT should be suspected if the platelet count falls by more than 50%
or falls to below 150,000/µL.
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• Although the major problem of HIT is venous thrombosis, arterial

thrombosis can occur.
• DVT and pulmonary emboli most often result as a complication of the
thromboses.
Causes
Impaired Platelet Production
• Cancers and other disorders
• Aplastic anemia
• Leukemia, lymphoma, myeloma, myelodysplastic disorders
• Marrow metastases by solid tumors
• Drugs (chemotherapy, others)
• Immune thrombocytopenia (ITP)
• Infections, bacterial, fungal, or viral (hepatitis C virus, HIV,
cytomegalovirus)
• Nutritional deficiencies, alcoholism
• Radiation
Causes continued..
• Increased Platelet Destruction

• Artificial surfaces (e.g., cardiopulmonary bypass, hemodialysis)

Disseminated intravascular coagulation (DIC)
• Heparin-induced thrombocytopenia (HIT)
• Pregnancy-related
• Thrombotic microangiopathy
• Thrombotic thrombocytopenic purpura (TTP)
• Atypical Hemolytic Uremic Syndromes (aHUS)
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• Abnormal Platelet Distribution

• Dilution (massive blood transfusion, fluids)
 Clinical Manifestations
• The most common symptom is bleeding, usually mucosal or cutaneous
• Manifest as nosebleeds and gingival bleeding
• Petechiae are small, flat, red or reddish brown microhemorrhages
• When the platelet count is low, RBCs may leak out of the blood vessels
and into the skin to cause petechiae.
• When there are many petechiae, resulting reddish skin purpura
• Purplish lesions caused by hemorrhage are termed ecchymoses.
Complications

• Hemorrhage.
• Cerebral hemorrhage
• Vascular thromboses
Diagnostic findings
• Platelets count decrease (less than 150,000/µL)
• PT, aPTT
• Bone marrow examination(Leukemia, aplastic anemia,
myeloproliferative disease)
• Specific assays( ITP Positive antigen specific C- serotonin release )
Management
• Immune Thrombocytopenic Purpura
• Corticosteroids : (e.g., prednisone, methylprednisolone) are used
initially to treat ITP because of their ability to suppress the phagocytic
response of splenic macrophages. This alters the spleen’s recognition of
platelets and increases the life span of the platelets.
• IV immunoglobulin (IVIG) & Anti-Rho (D) : These agents work is by
competing with the antiplatelet antibodies for macrophage receptors in
the spleen
•
• Rituximab (Rituxan) : May be used for its ability to lyse activated B
cells, thus reducing the immune recognition of platelets
• Eltrombopag (Promacta): Thrombopoietin receptor agonists, thus
increasing platelet production.
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• Splenectomy : a). the spleen has an abundance of the macrophages
that sequester and destroy platelets. B). structural features of the
spleen enhance the interaction between antibody-coated platelets
and macrophages. C). some antibody synthesis occurs in the spleen,
so antiplatelet antibodies decrease after splenectomy. D). the spleen
normally sequesters around one third of the platelets, so its removal
increases the number of platelets in circulation
• Immunosuppressives (e.g., cyclosporine)
• Platelet transfusions : Platelet transfusions may increase platelet
counts in cases of life-threatening hemorrhage.
• Epsilon-aminocaproic acid: Antifibrinolytic agent, may treat severe
bleeding
Thrombotic Thrombocytopenic Purpura
• Identification and treatment of cause
• Plasmapheresis (plasma exchange) :can aggressively reverse platelet
consumption by supplying the appropriate vWF and enzyme
(ADAMTS13) and removing the large vWF molecules that bind with
platelets.
• Treatment should be continued daily until the patient’s platelet
counts normalize and hemolysis has ceased.
• Corticosteroids may be given with this treatment.
• Rituximab (Rituxan)
• Immunosuppressives (e.g., cyclophosphamide)
• Splenectomy
Heparin-Induced Thrombocytopenia
• All forms of heparin must be stopped when HIT is first recognized.
(This includes heparin flushes for vascular catheter)
• To maintain anticoagulation , direct thrombin inhibitor, such as
argatroban.
• Fondaparinux (Arixtra), a factor Xa inhibitor (indirect thrombin
inhibitor), or bivalirudin (a synthetic thrombin inhibitor) may be used.
• Warfarin should be started only when the platelet count has reached
150,000/µL
• Plasmapheresis (plasma exchange)
• Warfarin (Coumadin)
• Thrombolytic agents
Decreased Platelet Production
• Identification and treatment or removal of cause
• Corticosteroids
• Platelet transfusions
Patient & Caregiver Teaching Thrombocytopenia
Notify HCP of any symptoms of bleeding. These include:
• Black, tarry, or bloody bowel movements
• Black or bloody vomit, sputum, or urine
• Bleeding from the mouth or anywhere in the body
• Bruising or small red or purple spots on the skin
• Difficulty talking, sudden weakness of an arm or leg, confusion
• Headache or changes in how well you can see
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• Ask HCP about restrictions in your normal activities, such as vigorous
exercise or lifting weights.
• Wear sturdy shoes or slippers.
• If are weak and at risk for falling, get help or supervision when
getting out of bed or chair.
• Do not blow nose forcefully; gently pat it with a tissue if needed.
• For a nosebleed, keep your head up and apply firm pressure to the
nostrils and bridge of your nose
• Do not bend down with your head lower than your waist.
• Prevent constipation by drinking plenty of fluids.
• Do not strain when having a bowel movement.
 Continued..
• May ne prescribed a stool softener.
• Do not use a suppository, an enema, or a rectal thermometer without the
permission of your HCP.
• Shave only with an electric razor, Do not use blades.
• Do not tweeze your eyebrows or other body hair.
• Do not puncture your skin, such as getting tattoos or body piercing.
• Do not use any medication that can prolong bleeding, such as aspirin.
• Other medications and herbs can have similar effects
• Use a soft-bristle toothbrush to prevent injuring the gums.
• Flossing is usually safe if done gently using the thin tape floss.
• Do not use alcohol-based mouthwashes since they can dry your gums and increase
bleeding.
• Ask your HCP before you have any invasive procedures done, such as a dental
cleaning, manicure, or pedicure.