Pruritus may or may not be present, depending on the disorder; it frequently accompanies Hodgkin and non-Hodgkin lymphoma, although the exact cause of the pruritus is unknown. Patients tend to regard pruritus as a nuisance and not to associate it with the blood disorder. Questions about pruritus should focus on the approximate time the patient first noticed pruritus, what aggravates it, and what home remedies seem to relieve the itching. Ask also about the condition of the skin of the lower extremities. The lower extremities seem to be especially vulnerable to blood abnormalities. Adult patients with sickle cell disease frequently have leg ulcers [5,6].

Pruritus may or may not be present, depending on the disorder; it frequently accompanies Hodgkin and non-Hodgkin lymphoma, although the exact cause of the pruritus is unknown. Patients tend to regard pruritus as a nuisance and not to associate it with the blood disorder. Questions about pruritus should focus on the approximate time the patient first noticed pruritus, what aggravates it, and what home remedies seem to relieve the itching. Ask also about the condition of the skin of the lower extremities. The lower extremities seem to be especially vulnerable to blood abnormalities. Adult patients with sickle cell disease frequently have leg ulcers [5,6].

The use of aspirin is so common that both the patients and the nurse can overlook or disregard its significance. Aspirin reduces platelet aggregation (the ability of platelets to be called to an injury site), increasing the potential for bleeding, especially in patients with compromised hematologic functioning. Thus, determining the patient's use of aspirin and noting when the patient last used aspirin are especially important. Alcohol consumption must also be assessed. Alcohol abuse damages the liver: Liver damage alters the production of clotting factors [12].

Exposure to chemicals increases the incidence of hematologic disorders. Patients may come into contact with chemicals through their jobs or avocations, by living in a contaminated area, or by using certain cosmetics. At increased risk for developing hematologic disorders are patients who are continuously exposed to asbestos, asphalt, industrial dyes, lead, and dry-cleaning fluid. Dyes used in fabrics and even on the patient's own hair may cause hematologic injury. Patients living close to industrial plants are also at increased risk [12].

Ask about the patient's bleeding tendencies after injury, surgery, or dental extraction: Has the patient had any problems with abnormal bleeding or bruising? The patient's history of receiving blood transfusions – the number of transfusions, why they were administered, and whether there were any complications during or following the transfusion — should be documented. Ask about the patient's surgical history. In particular, the fact that a patient has undergone a splenectomy or surgical resection of the duodenum may give valuable information about blood dyscrasias. For example, knowing that a patient has a past history of splenectomy might also reveal the reason for splenectomy (usually trauma or spherocytosis). Removal of the stomach and duodenum interferes with the patient's ability to properly absorb vitamin B and sets the stage for a surgically induced pernicious anemia. Information about the patient's postoperative healing may give clues to the patient's bone marrow integrity and competence of immune response [1].

Ask about the patient's bleeding tendencies after injury, surgery, or dental extraction: Has the patient had any problems with abnormal bleeding or bruising? The patient's history of receiving blood transfusions – the number of transfusions, why they were administered, and whether there were any complications during or following the transfusion — should be documented. Ask about the patient's surgical history. In particular, the fact that a patient has undergone a splenectomy or surgical resection of the duodenum may give valuable information about blood dyscrasias. For example, knowing that a patient has a past history of splenectomy might also reveal the reason for splenectomy (usually trauma or spherocytosis). Removal of the stomach and duodenum interferes with the patient's ability to properly absorb vitamin B and sets the stage for a surgically induced pernicious anemia. Information about the patient's postoperative healing may give clues to the patient's bone marrow integrity and competence of immune response [1].

Color of the patient's skin depends not only on the level of the patient's hemoglobin but also on the amount of pigmentation in the skin. If patients have darkly pigmented skin, disorder-induced color changes may be obscured by the patient's natural skin tones or hues. Hence, skin color cannot be used as the sole indicator of overall oxygenation of body tissues. To assess skin color properly in these situations, closely inspect the patient's oral mucosa, conjunctivae, and nail beds. Another good way to assess oxygenation to tissues without interference from skin pigmentation or hemoglobin level is to inspect the color of palmar creases. Have the patient open the hand with fingers fully extended. If oxygenation to the body tissues is adequate, the palmar creases should be pink [14,15].

Patients having problems with platelets and those with clotting-mechanism dysfunction have alterations in skin integrity that can be observed on physical examination. Frequently, the nurse observes purpura (redness), ecchymosis, or petechiae. Ecchymosis (hemorrhagic spots larger than petechiae) may be dark purplish, brown-yellow, or greenish, depending on the age of the lesion, and they may or may not be precipitated by a bump or injury. Lesions can be flat or elevated and may be painful or tender to palpation. Petechiae are more common when patients have disorders of platelet functions, life span, or decreased platelet numbers. Petechiae can occur over any part of the skin but are most common when pressure has been applied to a body part (after application of a tourniquet or blood pressure cuff). Petechiae are usually flat, nontender lesions that do not blanch with pressure. Ecchymoses are also seen [14,15].

Patients having problems with platelets and those with clotting-mechanism dysfunction have alterations in skin integrity that can be observed on physical examination. Frequently, the nurse observes purpura (redness), ecchymosis, or petechiae. Ecchymosis (hemorrhagic spots larger than petechiae) may be dark purplish, brown-yellow, or greenish, depending on the age of the lesion, and they may or may not be precipitated by a bump or injury. Lesions can be flat or elevated and may be painful or tender to palpation. Petechiae are more common when patients have disorders of platelet functions, life span, or decreased platelet numbers. Petechiae can occur over any part of the skin but are most common when pressure has been applied to a body part (after application of a tourniquet or blood pressure cuff). Petechiae are usually flat, nontender lesions that do not blanch with pressure. Ecchymoses are also seen [14,15].

Jaundice of the sclera may accompany jaundice of the skin. Retinal or scleral hemorrhages may be observed with thrombocytopenia. Conjunctival pallor can be seen in many of the anemias [16].

The complete blood count (CBC) with white blood cell (WBC) differential is one of the most important tests for evaluating the status of the patient's hematologic system; the CBC with differential provides a measure of the blood's ability to carry and transport oxygen (erythrocyte measurement) and to resist invading infectious organisms (leukocyte measurement). The CBC with differential usually requires approximately 7 mL of venous blood [20].

The erythrocyte sedimentation rate (ESR) is the rate at which erythrocytes in anticoagulated whole blood settle to the bottom of a tube. The ESR great value is to indicate the presence of an active inflammatory disease process. The ESR is elevated in leukemia, malignant lymphoma, Hodgkin disease, severe anemia, and agranulocytosis but may be within the normal range in polycythemia and sickle cell disease. Hyperchromic is a term used to describe erythrocytes with color indicating a high hemoglobin content [20].

The reticulocyte count is considered the most useful test in the evaluation of anemia and is a good index of effective erythropoiesis and bone marrow response to anemia. Reticulocytes are uninucleated, immature RBCs capable of oxygen transport. They remain in the bone for 24 to 48 hours, after which they are mature. An elevated reticulocyte count, or reticulocyte, occurs in disease states or conditions that cause a relative anoxic or hypoxic state in the patient. Elevated reticulocyte counts are seen in acquired autoimmune hemolytic anemia, acutely posthemorrhagic anemia, suckle cell anemia, thalassemia major, treatment of iron-deficiency anemia, and treatment of vitamin B12 and folic acid deficiency [20].

The bleeding time, a test that records the duration of active bleeding, provides information on vascular response to injury and helps to evaluate platelet function. The two principal methods of performing the bleeding time are Duke and IVY methods. In the Duke method, bleeding time is measured by observing active bleeding after a puncture on one of the patient's ear lobes. The IVY method, preferred because it is less liable to variation among testers, measures the bleeding time after two small punctures on the patient's forearm. Pressure is applied by inflating a blood pressure cuff up to 40 mm Hg. The patient's bleeding time may be prolonged in any of the leukemias, aplastic anemia, disseminated intravascular coagulation (DIC), idiopathic thrombocytopenic purpura, infectious mononucleosis, multiple myeloma, and pernicious anemia. Bleeding time is also used in the diagnosis of von Willebrand disease [20].

Prothrombin time (PT) evaluates stages II and III in the coagulation cascade. An increased PT may be present in the acute leukemias, DIC, factor VII or X deficiency, polycythemia vera, and multiple myeloma. This test is also used to regulate warfarin therapy [20].

The partial thromboplastin time (PTT) is a general evaluation of the entire coagulation system except for factors VII, XIII, and platelets and can also be used to regulate heparin therapy. A prolonged PTT can occur in DIC and in deficiencies of factors V, VIII, IX, X, XI or XII [20].

Factor VIII_AHF (antihemophilic factor) activity is the test for classic hemophilia or hemophilia A. Patients with little or no factor VIII are classified as severe hemophiliacs; those with 1% to 5% of normal values are called moderate hemophiliacs; and those with values between 6% and 30% of the normal are termed mild hemophiliacs. Patients with von Willebrand disease have deficiencies in both factor VIII_AHF and factor VIII_VWF (von Willebrand factor). Deficiency of factor VIIIVFW may range anywhere from 0% to 50% of normal [20].

Immunoglobulin studies evaluate the amount and types of immunoglobulin (e.g., IgG, IgA, IgM, IgD, IgE) present in the patient. A sample of blood is collected and examined under immunoelectrophoresis. Each immunoglobulin has its own unique pattern. Abnormal levels occur in many hemiplegic disorders. Any immunizations or vaccinations that the patient may have received within the previous 6 months may affect the results. These should be documented and reported to the laboratory; testing may need to be delayed [21].

Total iron-binding capacity (TIBC) measures the amount of available transferrin (a protein that binds with iron the transports it throughout the body) in the blood. TIBC increases as iron levels and iron stores decrease. Elevated TIBC is found in iron deficiency states, infancy, pregnancy, and blood loss. Decreased TIBC is found in iron overload states such as hemochromatosis, chronic hemolytic anemias (e.g., pernicious anemias), and blood transfusing overload [20].

Oxygen therapy may be administered to further promote gas exchange. Elevating the head of the patient's bed facilitates downward movement of the diaphragm, allows greater lung expansion, and increases the volume of air taken into the lungs, promoting a better gas exchange [2].

Patients are often predisposed to oral sores and bleeding. The mucous membranes should be evaluated each day and every eight hours in patients who are neutropenic or thrombocytopenic. The use of soft-bristled toothbrushes or cotton swabs, as well as the use of soothing nonalcoholic mouthwashes every two to four hours, should be recommended for mouth care. These patients should be advised not to use toothpicks or dental floss [26].

The incidence of infection and the absolute neutrophil count (ANC) are inversely related: as ANC falls, the incidence of infection rises. The patient having an ANC less than 500 cells/mcL is at increased risk for developing an infection. Institute infection prevention measures, such as good hand washing technique and isolation and aseptic technique with any invasive procedure. It is important that both nurse and patient practice good hand washing techniques. Isolation of the patient is controversial. The patient should be educated to avoid contact with individuals with known infections, with recently immunized individuals, and with large crowds [26].

The Rh system includes more than 50 red cell antigens, but the five main antigens are D, C, c, E, and e [31,32,33]. The D antigen is the most significant antigen in the Rh typing system. If the D antigen is present, the individual is considered Rh+; if it is absent, the individual is Rh-. It is particularly important to avoid transfusing Rh+ red blood cells into Rh- girls and women during pregnancy or childbirth because anti-D-related hemolytic disease of fetus and newborn can result in mild-to-severe anemia and even death of the Rh+ fetus and/or newborn [33,34].

Major crossmatch is the most important of the crossmatching process, allowing for safe blood transfusion. The minor crossmatch is less important as the donor antibodies will be swiftly diluted within the recipient of the blood product. However, should the minor test be incompatible, it is strongly suggested that transfusion be avoided [38,39].

Today, whole blood transfusion is rarely used; instead, component therapy is the preferred approach. The components of blood include plasma, platelets, RBCs, and WBCs [40]. The transfusable components of blood include red blood cells, plasma, platelets, cryoprecipitated AHF (cryoprecipitate), and granulocytes. The white blood cells are removed from donated blood before transfusions [41].

Platelet transfusions can be administered to individuals to prevent bleeding with platelet counts less than reference range, prior to medical procedures with shared decision making, and to treat bleeding associated with thrombocytopenia or platelet dysfunction [50].

Cryoprecipitate is derived from plasma and contains fibrinogen, factor VIII, von Willebrand factor, factor XIII, and fibronectin. It is used for immediate blood loss or can be directly prior to procedures in which patients have hypofibrinogenemia [54]. Conditions that are potential indications for cryoprecipitate transfusion include [51,52,53]:

During infusion of blood or blood products, no medications can be administered through the same infusion line. It is generally in the best interest of the patient to have two access sites: one for the blood product and the other for any potential medications.

Obtain the blood products from the laboratory, blood bank, or transfusion services department. The process to obtain blood products is organization-specific, and the facilities procedures should be followed. After the blood is obtained, the nurse should inspect the product for the correct product ordered, expiration date, ABO group and Rh type of both the donor and recipient, and date/time the blood was issued. There is a four-hour window from the time the blood is released from the blood bank to completion of transfusion, which is why it is important to only obtain the blood products to be transfused once all equipment is available and ready to be used. The blood product should be handled with gloves and should be examined for any leaks, abnormal color, clots, excessive air, or unusual odor. Any expired or abnormal appearing/smelling blood should be returned to the blood bank as soon as possible.

At the bedside, two nurses qualified to administer the blood products should be present to provide another verification process to match the patient to the blood ordered by the provider and to the blood product received. Information to verify includes:

The blood product is now ready to be infused. The infusion can be completed by drip (gtt) counts or by utilizing an infusion pump specifically labeled for blood transfusion. Using the gtt method, open the common tubing clamp and the clamp to blood bag, the regulate blood infusion to allow only 2 mL/minute to infuse in the initial 15 minutes. The nurse must remain with the patient during this period to monitor and assess the patient. Initial flow rate during this time should be 1–2 mL/minute or 10–20 gtt/minute. For administration using the electronic infusion device (EID), insert the primed infusion tubing into chamber of the control mechanism of the EID, with a roller clamp on the common IV tubing between the EID and the patient. The tubing through an "air in line" alarm system (equipped on most EIDs). Finally, close the door, turn on the power, and select the required blood administration rate or blood transfusion program.

These reactions may be mild enough to go unnoticed, or they may be severe enough to result in anaphylaxis. In general, the greater the number of transfusions the patient receives, the greater the risk for developing antibodies against blood or blood products. Because of the large number of transfusions received by patients with sickle cell disease or cancer, be especially attuned to their increased risk for developing a reaction. With any reaction during a transfusion [59]:

In patients with sickle cell disease experiencing pain crises, the lower back, knee/shin area, and hips are the sites most often affected. A higher number of pain sites are found in patients with depression and in those 45 years of age and older [69].

For prevention of acute pain episodes, hydroxyurea is most often used [66]. This agent acts by ribonucleotide inhibition and induction of fetal hemoglobin, which possesses superior affinity for oxygen binding. It is FDA-approved for use in adults and children 2 years of age and older and is the only treatment for sickle cell disease that modifies the disease process. Hydroxyurea is effective in reducing pain crises, painful symptoms, need for blood transfusion, and mortality. As such, it represents the backbone of sickle cell disease management. The usual daily oral dose is 15–35 mg/kg [66,73]. Inconsistent adherence reduces its efficacy, and patient adherence can be challenged by the three- to six-month delay between treatment initiation and the onset of clinical response. More frequent follow-up contact with support and encouragement may be needed in some patients.

Because most episodes of hemolysis are self-limiting, no intervention is usually necessary. No specific drug therapy is indicated for G-6-PD deficiency, but patients should avoid certain drugs, including analgesics, ascorbic acid (vitamin C), naphthalene, methylene blue, and phenylhydrazine. Patients should avoid ingestion of fava beans. If hemolysis is severe, transfusion therapy with packed RBCs may be administered [85,86].

Hemophilia is diagnosed by laboratory serum determinations. Tests include serum calcium, prothrombin time (PT)), partial thromboplastin time (PTT), quantitative platelets, coagulation factors assay, and antibody directed against factor VIII (for hemophilia A). Serum calcium level, PT, and platelet count are normal in hemophilia, and PTT is prolonged.

Nursing management during an acute bleeding episode involves the IV administration of the deficient factor—either factor VIII_AHF human or factor IX human. With joint pain and hemarthrosis, splinting or casting the involved joint may be indicated. Whenever a patient with hemophilia is admitted to the hospital, nursing assessments are directed toward the prompt recognition of hemorrhage [3].

After the diagnosis of pernicious anemia and the initiation of vitamin B12 therapy, the patient's hemogram quickly returns to normal. Results of the peripheral blood smear may begin to improve as early as 4 days after the initiation of vitamin therapy. In addition, a dramatically increased sense of well-being accompanies the hematology improvement. The patient with pernicious anemia is committed to a lifetime of vitamin B12 therapy. Noncompliance causes the return of symptoms and possibly more dramatic consequences, such as neurologic involvement. Thus, these patients must understand the rationale for vitamin B12 therapy and comply completely with the chemotherapeutic regimen. Vitamin B12 therapy administered before the onset of neurologic signs and symptoms is ideal. When these signs and symptoms have already appeared, prompt and accurate diagnosis and treatment is imperative. Physical therapy may help restore some use and function, but residual neurologic damage may persist for life [3].

Leukemia occurs in acute forms (involving proliferation of immature cells) and chronic forms (involving proliferation of mature cells) and can affect the erythrocyte or any of the white cell precursors. The four most common types of leukemia are: acute lymphocytic leukemia (ALL), chronic lymphocytic leukemia (CLL), acute myeloid leukemia (AML), and chronic granulocytic leukemia (CGL). The principles guiding nursing management are similar for all the leukemias [108,109].