Endocrine/​Hematology/​Gastrointestinal/​Renal/​Integumentary Study Guide for the CCRN

Hematology/Immunology

Hematology and immunology focus on conditions affecting the blood and immune systems. Patients with alterations in normal lab values may experience an array of symptoms and complications. Early symptoms of these conditions should be recognized to prevent severe effects and even death.

General Tests and Values

There are numerous lab tests and values that are important in measuring and monitoring hematologic/immunologic factors. Nurses must understand expected ranges and identify changes among these that may indicate concerns in their patients.

Review the expected ranges for both males and females in these lab tests:

• red blood cell count
• hemoglobin
• hematocrit
• mean corpuscular volume (MCV)
• reticulocyte count
• C-reactive protein
• erythrocyte sedimentation rate (sed rate)
• white blood cell count
• white blood cell differential profile
• prothrombin time (PT)
• partial thromboplastin time (PTT)
• activated partial thromboplastin time (aPTT)
• thrombin clotting time (TCT) or Thrombin time (TT)
• bleeding time
• platelet count

Anemia

Anemia is defined as a condition where the body does not have enough red blood cells to appropriately deliver oxygen throughout the body. When this occurs, the body tries to correct the oxygenation throughout the body by increasing cardiac output and redirecting blood toward the vital organs such as the brain and heart. This reduces the perfusion of the “less essential” organs to survival, such as the kidneys and other abdominal organs. There are numerous classifications of anemia, including:

• Iron-deficiency anemia is the most common type of anemia and is characterized by anemia due to low iron levels. This could be caused by blood loss but also through not eating enough iron-rich foods or malabsorption.

• Pernicious anemia is anemia caused by a vitamin B12 deficiency, which is needed to create healthy red blood cells. We have proteins in our gastrointestinal system that allow for the absorption of this vitamin. This can be secondary to surgeries to the GI system, such as gastric bypass, or medical conditions, such as celiac disease or Crohn’s, can impair this normal process. This can also be entirely autoimmune.

• Hemolytic anemia occurs when red blood cells break down faster than they can be made. Potential causes are genetics, autoimmune reactions, infections, and mechanical heart valves. Two types of inherited hemolytic anemias are sickle cell anemia and thalassemia.

• Aplastic anemiais a rare condition where the bone marrow stops producing red blood cells. This can be a result of autoimmune reactions, radiation or chemotherapy, exposure to toxins, and viral infections such as HIV.

Symptoms

Patients in critical care units should be closely monitored for anemia, especially if they have recently undergone surgery or had a significant trauma. Patients with anemia may present with pallor, fatigue, hypotension, weakness, and, if severe, mental status changes and chest pain. Vital signs may show decreased oxygenation levels, tachycardia, and increased respiratory rate. Patients with concerns for hemolytic anemia should be monitored for jaundice and splenomegaly.

Diagnosis

Diagnosis of anemia is usually determined by lab work. A complete blood count or CBC will help to identify decreased red blood cell (RBC) values and hemoglobin/hematocrit. Reticulocyte count can show how fast new blood is being made by the bone marrow. Iron studies help to identify deficiencies that may be contributing to the patient’s anemic state. Serum B12 levels can be taken to assess for vitamin B12 deficiency.

Treatment

Anemia is often treated by addressing any underlying cause of the disease. Iron may be given to patients with iron deficiency anemia. Vitamin B12 can be supplemented via injection or orally. In patients with severe blood loss or hemolysis, blood transfusions may be indicated if hemoglobin is less than 7 g/dL or less than 8 g/dL in those with existing cardiovascular disease. Patients must have appropriate lab work, including a type and screen prior to the order and infusion of blood products. In some cases, patients may need additional support with erythropoietin to stimulate red blood cell production to reduce anemia.

Coagulopathies

Coagulopathies refer to conditions that impair the body’s ability to form or regulate blood clots. These disruptions can result in uncontrolled bleeding, inappropriate clot formation, or a combination of the two.

Immune Thrombocytopenia

Immune thrombocytopenia, once referred to as idiopathic thrombocytopenic purpura (ITP), is an autoimmune disorder that responds to platelets, destroying them and ultimately causing decreased platelet counts. Acute ITP occurs mostly in young children, while chronic ITP occurs more commonly in young women and adults. Platelet counts with this condition may be as low as 0 to 100,000 cu/mL, with 150,000 to 400,000 cu/mL being the expected range. This disorder may be triggered by viral illnesses, other autoimmune conditions, or when taking certain medications, such as sulfa drugs.

Care

While ITP is generally a very treatable and non-fatal condition, patients should be closely monitored for intracranial hemorrhage. This may occur when platelet levels fall below 30,000 cu/mL. Early symptoms of ITP include bruising, petechiae, hematoma, epistaxis, and increased bleeding, particularly during the menstrual cycle in females. ITP, if left untreated, can cause patients to continue to bleed internally, causing dysfunction of organs and neurologic status changes due to intracranial hemorrhage. Patients should be closely monitored for changes in level of consciousness, headache, increased lethargy, pupillary changes, and coma.

Treatment

Treatment for ITP begins with corticosteroids to depress the immune response. Corticosteroids can also help boost platelet counts. Intravenous immunoglobulin or IVIG can be given to temporarily stop the body from destroying platelets. This is helpful for a short-term boost of platelets for surgery or to allow time for other treatments to work. In chronic ITP, a splenectomy may be considered if the patient has recurrent episodes of platelet drops and is at high risk for complications. If the patient’s platelets are low enough, a platelet transfusion may be initiated to boost the amount of circulating platelets to prevent bleeding and intracranial hemorrhage. In all patients with ITP, aspirin and NSAIDs should be avoided to prevent triggering of platelet destruction.

Disseminated Intravascular Coagulation (DIC)

DIC is a blood disorder characterized by both coagulation and hemorrhage. It may also be called a consumption coagulopathy. DIC occurs secondary to other health events such as trauma, advanced heart disease, or acute cardiac events, sepsis, necrotizing enterocolitis, and severe infections, both viral and bacterial.

Causes

When the body’s regulation system is disrupted, such as in trauma, tissue factor (transmembrane glycoprotein) is released and binds with the circulating coagulation factors. This triggers the coagulation cascade, leading to widespread clot formation and consumption of platelets and clotting factors. The clots disseminate throughout the body, causing microvascular and sometimes macrovascular obstruction. In response to decreased perfusion and excessive clotting, the body activates fibrinolysis to break down the clots, which ultimately results in hemorrhage due to the depletion of platelets and clotting factors.

Symptoms

As these cascades occur together, the patient becomes very unstable and difficult to treat as it requires management of opposing symptoms of both clotting and bleeding. Symptoms of DIC include bleeding from multiple, unrelated sites, internal bleeding such as the GI/GU systems, hypotension, acute shock, petechiae, and purpura. Lab results may show prolonged prothrombin and partial prothrombin times (PT/PTT), decreased platelet counts, fragmented RBCs, and decreased fibrinogen. Specifically, elevated fibrin degradation products (FDPs), particularly in combination with a high D-dimer, are hallmark laboratory findings for DIC. Symptoms typically progress suddenly and lead to multiorgan dysfunction if not treated. Chronic DIC has a slower onset and is less common. It is usually associated with cancers.

Treatment

Since DIC is a secondary condition, treatment of the patient’s primary condition or underlying cause must be performed. Hemorrhages may be managed by initiating the massive blood transfusion protocol (MTP). This is an emergency process used when a patient is experiencing life-threatening blood loss, allowing blood products to be delivered quickly and in balanced ratios without delays. Heparin may be used selectively when thrombosis is the predominant issue to help prevent further clot formation, but in most cases will be avoided because it can significantly worsen bleeding. Cryoprecipitate can be given to increase fibrinogen levels. Other medication interventions include initiation of coagulation inhibitors and coagulation factors to stop the cascading clotting and bleeding processes. Supportive care such as IV fluids, vasopressors, mechanical ventilation, and renal replacement therapy may be indicated as well.

HIT

Patients receiving heparin for anticoagulation must be monitored closely for heparin-induced thrombocytopenia and thrombosis syndrome (HIT/HITTS). Two types of HIT exist: Type I and Type II.

• In Type I HIT, the body’s reaction to the heparin causes a mild depletion of platelets within a few days of heparin initiation. Laboratory results will likely show platelet counts less than 100,000 \(mm^3\). This usually resolves without intervention and heparin may be continued as long as platelet counts return to expected ranges.

• In Type II HIT, the body has an autoimmune reaction to the heparin, causing a severe decrease in platelet count, >50% of their baseline, usually below \(\bf{50,000 \,mm^3}\). Generally, the onset ranges from five to \(\bf{14}\) daysafter heparinization but can be sooner with prior exposure. Type II HIT has less than a 30% mortality rate, but must be diagnosed quickly and reversed to prevent continuing complications from the increase in heparin-antibody complexes. These antibiotics trigger the clotting cascade, promoting thrombus formation. Patients with this autoimmune reaction are at high risk for stroke, myocardial infarction, and limb ischemia.

Treatment

Treatment of HIT is usually only necessary in Type II. In Type I HIT, the patient may be monitored for a resolution in symptoms without treatment. If the patient continues to have decreased platelet counts, then discontinuation of heparin is likely indicated. In patients with Type II HIT, discontinuation of heparin should be the first line of action. Additional medications to inhibit thrombin production, such as lepirudin and argatroban, may be added to the patient’s therapy. Nurses should be responsible for monitoring for thrombus formation, especially presenting as DVT, myocardial infarction, and stroke. Review common signs and symptoms of these conditions to be familiar with the early indications of thrombus formation.

Immune Deficiencies

There are many conditions that fall under the term immune deficiency. Primary immune deficiencies are conditions, either genetic or inherited, that disrupt a person’s immune system. They are usually classified by a variety of lab results, including abnormal levels of antibodies, issues with the antibodies, or defects in immune cells like T-cells and B-cells.

Some immune deficiencies, like common variable immune deficiency, are not diagnosed until adulthood. In this condition, the patient will experience low serum immunoglobulin and decreased antibodies. Secondary immune deficiencies are acquired later in life and can be caused by infections such as HIV, medications such as chemotherapy or corticosteroids, and cancers.

Symptoms

Nurses should be aware of some of the signs and symptoms that may develop due to an immune deficiency. The primary symptom of immunodeficiency is recurrent infection. This may involve any of the many organ systems (e.g., respiratory, GI, skin) but often presents most frequently in the respiratory system. These infections are typically severe and do not respond to standard treatments. Patients may have a variety of other symptoms as well, including lymphadenopathy, splenomegaly, abdominal pain, nausea, vomiting, diarrhea, weight loss, polyarthritis, and granulomas (small, benign clusters of immune cells).

Diagnosis and Treatment

Family histories should be reviewed in all patients with suspected immune deficiency. Diagnosis may be based on serum lab levels, history, and assessment. Patients with this condition will likely be prescribed immunoglobulin replacement, or IVIG. IVIG is administered very similarly to a blood product, and specific guidelines must be followed to prevent an administration error and to reduce any adverse reactions. Treatment measures may be a long-term commitment for patients. Often, IVIG is given every three to four weeks, and patients must have access to the hospital to receive their infusions. Infusions may last up to three to four hours.

Another therapy for patients with immune deficiencies is the possibility of a prescription for long-term antibiotics. Be able to educate families on how to take these medications in addition to important hygiene measures to prevent infection. Patients should know to wash their hands frequently, avoid sick people as much as possible, cook food thoroughly, and decrease crowd exposure.

Leukopenia

Leukopenia and neutropenia are often used similarly in writing. Leukopenia is the decrease of white blood cells (WBC), whereas neutropenia is the decrease in neutrophils, a type of white blood cell. Normal WBC count is around 4,500 to 11,000 WBCs per microliter, and the classification of leukopenia would be less than this range. Alternatively, a normal neutrophil count is greater than 1,500 neutrophils per microliter, and neutropenia would be classified as a count less than this on a mild, moderate, and severe scale based on how low the count is. These conditions may occur due to a variety of issues. Some patients may have leukopenia or neutropenia due to the body’s inability to produce white blood cells or the destruction of the cells due to infection, cancer, autoimmune disorders, medication, or radiation. Patients with low white blood cell counts are at a significantly increased risk of infection and illness.

Symptoms

Signs of leukopenia and neutropenia develop due to the body’s inability to maintain enough of the fighter, white blood cells. Nurses should be aware that infections are more prevalent in people with insufficient white blood cell counts, including pneumonia, skin abscesses, urinary tract infections, viral illnesses, and gastrointestinal concerns. In addition to general infections, these patients are also at risk for opportunistic infections. These are illnesses that are rare in healthy individuals but occur due to a weakened immune system, such as fungal pneumonia and invasive Candida. Patients should be closely monitored for signs of sepsis. Signs and symptoms are dependent on the type of infection. These may include things such as malaise, fever, night sweats, chills, shortness of breath, cough, painful or burning urination, wounds that do not heal, and mouth ulcerations.

Treatment

Treatment for leukopenia and neutropenia includes supportive therapy, preventative measures, and administration of hematopoietic growth factors if white blood cell counts fall too low. Patients should have early identification of infections and be treated promptly to prevent serious complications and sepsis. Monitor patients closely for signs of fever, lethargy, and changes in wound appearance. Patients should be educated to avoid large crowds and limit their exposure to sick contacts. Nurses should take measures to prevent infection through isolation and using appropriate sanitation methods.

Oncologic Complications

Some patients diagnosed with cancer will require intensive care services. Patients may be admitted due to neutropenia, fever, sepsis, and the systemic effects of the cancer, and/or treatment reaction.

Tumor Lysis Syndrome

Tumor lysis syndrome is a critical condition when tumor cells release tumor contents and toxins into the bloodstream. This can occur most commonly during or after a treatment. It can rarely occur spontaneously. It occurs most commonly in hematologic cancers, especially non-Hodgkin lymphoma and acute leukemia. Tumor lysis syndrome is characterized by electrolyte imbalances including hyperuricemia, hyperkalemia, hyperphosphatemia, and hypocalcemia. This response creates a medical emergency that can quickly cause renal insufficiency, seizures, cardiac arrhythmias, multiorgan failure, and death.

Symptoms

Nurses should monitor patients closely for signs and symptoms of tumor lysis syndrome. These include neurologic changes, restlessness, irritability, nausea, vomiting, numbness, cramping, tingling, joint pain, and decreased urinary output. Late symptoms of tumor lysis syndrome include loss of muscle control, arrhythmia, delirium, hallucinations, and seizures. If left untreated, this condition can lead to multiorgan dysfunction or failure and death.

Treatment

Treatment of tumor lysis syndrome focuses on the preservation of kidney and other organ function. Patients may need increased IV fluids, diuretics, and medications to correct electrolyte imbalances. Allopurinol (Aloprim®) and rasburicase (Elitek®) can be used to remove excess uric acid. Dialysis can be used in kidney failure to help filter out excess minerals and toxins. Prevention and early identification are keys to reducing the negative impact of this condition.

Pericardial Effusion

Pericardial effusion can occur in patients who have metastatic cancer to the pericardium. This is a serious, potentially fatal complication. It occurs most commonly in metastasis from lung and breast cancers.

Symptoms

Symptoms of pericardial effusion include shortness of breath, cough, and chest pain. It can develop over time or rapidly and can present in patients as cardiac tamponade or cardiogenic shock. Diagnosis of pericardial effusion can be obtained via echocardiogram. On a chest X-ray, the heart can appear in a globular shape, often referred to as a “water bottle heart.”

Treatment

Treatment of pericardial effusion may include pericardiocentesis, pericardial window, and/or surgical decompression to alleviate pressure around the heart. If the effusion is small, medical management with medications such as NSAIDs and steroids may be used to decrease inflammation. Mortality rate is high for patients with recurrent pericardial effusion caused by invasive tumor metastasis.

Thrombocytopenia

Thrombocytopenia occurs due to decreased platelet counts. It may be related to decreased production or destruction of the platelets. A type of thrombocytopenia discussed above is HIT, heparin-induced thrombocytopenia. Thrombocytopenia may also occur due to DIC (also discussed above), bone marrow failure, cancer, infection, drug and alcohol abuse, nutritional deficiencies, vasculitis, sepsis, and autoimmune diseases.

Symptoms

Patients at risk for thrombocytopenia should be monitored for similar signs as mentioned above, including petechiae, ecchymosis, epistaxis, poor skin color, weakness, fatigue, splenomegaly, hematuria, melena, and jaundice. It can be diagnosed through a complete blood count (CBC). Further investigation of this condition may indicate the need for a bone marrow biopsy.

Treatment

Patients with platelet counts less than 50,000 or who are actively bleeding may be candidates for platelet transfusion. Platelet transfusions are not indicated in thrombotic thrombocytopenic purpura, as this is both a clotting and bleeding disorder. Underlying conditions causing thrombocytopenia must be addressed to reverse the effects on the platelets. Medications such as romiplostim (Nplate®) and eltrombopag (Promacta®) may be prescribed to help the bone marrow boost platelet production if a decrease in production is noted. Corticosteroids such as prednisone can be used to decrease the destruction of platelets as discussed under ITP. Splenectomy may be needed to remove the spleen if it is trapping large numbers of platelets.

Continued Care

Nurses should continue to monitor patients for acute bleeding risk factors. Reducing the risk of patient injury as well as providing supportive measures to reduce platelet consumption are critical to decreasing complications from this condition.

Transfusion Reactions

Many patients benefit from blood transfusions. There are several types of product that can be administered when needed. These include whole blood, packed red blood cells, platelets, fresh frozen plasma (FFP), and cryoprecipitate. Review the indications for these transfusions and when compatibility testing must be run to help reduce the risk of transfusion reaction.

Nursing Considerations

Nurses are the medical personnel responsible for administering blood products. Many institutions will have protocols in place to ensure appropriate blood products are administered to the correct patient and with the least amount of reaction risk. Transfusion complications include febrile reaction, infection, allergic or anaphylaxis response, hemolytic transfusion reaction, transfusion-related acute lung injury (TRALI), graft vs. host disease, post-transfusion purpura, transfusion-related immunosuppression, and hypothermia.