Hematology, Anemia, and Coagulation

• The following notes cover information pertaining to hematology, coagulation, anemia, thrombocytopenia, aplastic anemia, sickle cell anemia, hemophilia, lymphoma and disseminated intravascular coagulation

Key Terms and Definitions

• Anemia is a deficiency of red blood cells (RBCs) or hemoglobin, which leads to reduced oxygen transport
• A condition where the bone marrow fails to produce sufficient blood cells is referred to as aplastic
• Disseminated Intravascular Coagulation (DIC) is a serious disorder with overactive blood clotting proteins, resulting in excessive clotting and bleeding
• Hemophilia is a genetic disorder impairs the body's ability to form blood clots
• Thrombocytopenia is a condition of abnormally low levels of platelets in the blood, which elevates the risk of bleeding

Importance of Terminology in Nursing

• Terminology is crucial for effective communication, accurate hematologic disorder diagnosis and treatment, and patient education

Overview of Hematologic Disorders

• Hematologic disorders involve abnormalities in red blood cells, white blood cells, or platelets, impairing oxygen transport, infection response, and causing bleeding
• Anemia can stem from impaired production, increased destruction, or blood loss
• Leukemia and lymphoma involve abnormal white blood cell proliferation, thus impacting the immune function
• Fatigue, pallor, tachycardia, and shortness of breath are symptoms of anemia due to reduced oxygen delivery
• Hemophilia patients may experience excessive bleeding and bruising
• Leukemia patients may experience recurrent infections and fatigue
• Glossitis and changes in nail shape can indicate specific types of anemia

Diagnostic Tests for Hematologic Disorders

• CBC (complete blood count) assesses RBC, WBC, and platelet levels
• Microscopic blood examination can reveal cell size and shape abnormalities
• Serum iron, ferritin, and vitamin levels help determine the cause of anemia

Disorders of Red Blood Cells

• Microcytic Anemia consists of smaller than normal RBCs, often due to iron deficiency; common in women of childbearing age and children
• Macrocytic Anemia consists of larger than normal RBCs, typically due to vitamin B12 or folate deficiency; untreated, it can lead to neurological issues

Nutritional Deficiencies and Anemia

• Iron, vitamin B12, and folate are critical for RBC production, and their deficiencies can lead to anemia forms
• Pernicious anemia results from a lack of intrinsic factor, thereby affecting vitamin B12 absorption
• Meats, dairy, leafy greens, and fortified grains are dietary sources

Signs and Symptoms of Anemia

• Fatigue, pallor, tachycardia, and shortness of breath are common symptoms
• Glossitis and neurological symptoms like numbness are specific symptoms of pernicious anemia
• Iron deficiency may present with spoon-shaped nails and angular cheilitis

Nursing Care and Management

• Monitor vital signs and oxygen saturation to assess the severity of anemia
• Dietary sources of iron, vitamin B12, and folate can improve nutritional intake
• Iron supplements are administered as prescribed with monitoring for side effects and effectiveness
• Prevent bleeding in thrombocytopenia or hemophilia patients with safety precautions
• Educate patients on signs of infection and the importance of prompt symptom reporting
• For patients undergoing splenectomy, give information about preventing infection and the need for vaccinations
• Patient response to interventions is assessed through labs and symptom relief
• Regularly evaluate the effectiveness of dietary changes and adherence to supplements
• Any changes in patient condition are communicated to the healthcare team

Overview of Anemia

• Anemia is classified based on etiology like iron deficiency, pernicious, and aplastic anemia
• Iron deficiency anemia is caused by insufficient iron, thus decreasing hemoglobin production
• Pernicious anemia is due to vitamin B12 deficiency, often linked to intrinsic factor antibodies that impair absorption
• Aplastic anemia is caused by the failure of bone marrow to produce adequate blood cells, leading to pancytopenia

Diagnostic Measures

• Serum folate and B12 levels are crucial for diagnosing specific anemia types, particularly pernicious anemia
• A bone marrow biopsy is a definitive test for aplastic anemia, identifying fatty, non-functional marrow
• Elevated homocysteine levels may indicate vitamin B12 deficiency in pernicious anemia
• Additional tests may be required to identify sources of bleeding if blood loss is suspected

Clinical Manifestations

• Anemia symptoms can range from fatigue and pallor to severe tachycardia and dyspnea
• Clinical manifestations of anemia vary based on hemoglobin levels

Therapeutic Measures for Anemia

• Treatment begins with identifying and eliminating underlying causes of anemia like dietary deficiencies
• Nutritional interventions increase intake of iron, folate, and vitamin B12 through diet or supplements
• Lifestyle modifications that can benefit nutrient absorption is reducing alcohol and managing chronic diarrhea
• Blood transfusions may be necessary to restore hemoglobin levels in acute cases
• Data collection involves monitoring hemoglobin (Hgb) and hematocrit (Hct) levels, as well as patient fatigue and activity tolerance
• Nursing diagnoses may include decreased activity tolerance and imbalanced nutrition, with specific expected outcomes for each
• Interventions for activity tolerance include monitoring vitals, planning care to conserve energy, and assisting with ADLs
• Consulting a dietitian and educating patients on dietary choices and supplement use is essential for nutritional imbalances

Aplastic Anemia

• Aplastic anemia is characterized by the failure of bone marrow to produce enough blood cells, leading to pancytopenia
• Aplastic anemia can be congenital or acquired due to exposure to toxic substances, infections, or autoimmune diseases
• The term 'aplastic' indicates a lack of development, while 'pancytopenia' refers to reduction in all blood cell types
• Early symptoms include fatigue, pallor, and shortness of breath, similar to other anemias
• The progression patients may experience tachycardia, heart failure, and bleeding due to low platelet counts
• Bleeding under the skin is shown by skin manifestations including ecchymoses and petechiae
• Diagnosis typically starts with a complete blood count (CBC), showing low levels of all blood components
• A bone marrow biopsy is definitive, often resulting in a 'dry tap' due to fatty bone marrow
• Elevated serum iron levels and total iron-binding capacity (TIBC) are additional tests
• Aplastic anemia is characterized by the failure of the bone marrow to produce sufficient blood cells, leading to a "dry tap" during aspiration, where fatty, yellow marrow is extracted versus normal red marrow
• The condition results in decreased red blood cell (RBC) production, leading to anemia, and often presents with elevated total iron-binding capacity (TIBC) and serum iron levels
• Diagnostic tests include elevated TIBC and serum iron levels
• Essential to A complete blood count (CBC assessment of the levels of RBCs, white blood cells (WBCs), and platelets
• Early identification and correction of the underlying cause of aplastic anemia is vital to promote survival, however, damage to the bone marrow is often irreversible
• Supportive measures focus on preventing infections and bleeding, including blood transfusions and the use of steroids
• Stem cell transplantation is the most effective treatment; colony-stimulating factors like epoetin alfa and filgrastim may also be used
• Nursing care includes monitoring for signs of bleeding and infection, especially in patients with low platelet counts (less than 20,000) or low WBC counts
• Bleeding precautions include using electric razors, soft toothbrushes, and avoiding invasive procedures to minimize bleeding risk
• Private rooms, ensuring hand hygiene, and avoiding contact ensure infection control
• A personal account from a parent of a child diagnosed with aplastic anemia highlights the decision making and emotional turmoil
• Support systems and the psychological impact of the illness are essential for family members

Sickle Cell Anemia

• Sickle cell anemia is an inherited disorder where RBCs undergo a mutation, making hemoglobin sensitive to oxygen changes
• Sickled cells are rigide and sticky, causing blockages in blood vessels, which can lead to pain and organ damage
• The lifespan of sickled RBCs reduced to 10-20 days versus the normal 120 days, leading to chronic anemia
• Sickle cell disease (SCD) is an autosomal recessive disorder, meaning both parents must pass on the sickle hemoglobin (HbS) for the child to have the disease
• If only one parent passes on abnormal hemoglobin, the child will have, sickle cell trait, which is generally asymptomatic but can be passed to future generations
• Symptoms of sickle cell anemia typically do not appear until after 4-5 months of age when fetal hemoglobin is replaced by adult hemoglobin.
• Common symptoms include episodes of pain (sickle cell crises), fatigue, jaundice due to increased bilirubin levels, and potential complications like gallstones and splenic sequestration
• Vaso-occlusive crises and acute chest syndrome are complications of sickle cell anemia, plus increased risk of infections due to spleen dysfunction
• Management strategies:
  • Pain control
  • Hydration
  • Blood transfusions
  • Preventative antibiotics
• SCD and Sickle cell trait, is an autosomal recessive disorder
• Symptoms typically do not manifest in infants until after 4-5 months of age due to fetal Hb
• The sickling process can lead to various complications, including vaso-occlusive crises
• Sickle cell anemia is prevalent among Black Americans and individuals from malaria-endemic regions, providing a survival advantage against malaria
• 1 in 13 Black babies are born with sickle cell trait, while 1 in 365 are born with sickle cell disease
• Environmental factors such dehydration, infections, and low O2 can trigger crises
• Symptoms during a crisis include severe pain, swelling in joints, abdominal pain, and hypoxia
• Priapism, or painful prolonged erection, can occur due to impaired circulation in males
• Chronic complications from repeated crises : Hand-foot syndrome (unequal growth of fingers and toes)
• Tissue necrosis can occur due to infarction from sluggish blood flow, leading to pain and swelling.
• Increased clotting in cerebral blood vessels elevates stroke risk, necessitating close monitoring
• The Sickledex test detects sickling of red blood cells under low oxygen tension
• Hemoglobin electrophoresis identifies the presence of HbS and assesses overall hemoglobin levels and red blood cell counts
• Treatment management varies based on severity need for crisis intervention education
• During crises, patients may require pain management, blood transfusions and oxygen therapy
• Hydroxyurea can reduce crisis frequency by increasing fetal hemoglobin levels but has potential side effects
• Diagnosis is confirmed through a complete blood count (CBC) and bone marrow aspiration, revealing elevated hemoglobin and hematocrit levels
• A low erythropoietin level indicates negative feedback from the kidneys, and genetic mutations like JAK2 or TET2 may be present
• Check circulation and neurological status to detect changes every 2 hrs during sickle cell
• Manage pain with analgesics and hydrate the pt to avoid complications.
• Apply warm compresses to painful areas and avoid cold exposure to reduce vasoconstriction
• Educate patients on avoiding triggers such as cold temperatures, strenuous exercise, and smoking
• Discuss the importance of getting regular vaccinations and medical follow-ups as well as adherence to prescribed therapies
• Advise patients to avoid tight-fitting clothing to prevent circulation issues, which can exacerbate symptoms
• Instruct patients to avoid cold temperatures and smoking, as both can cause vasoconstriction and trigger crises
• Recommend avoiding alcoholic beverages, which can also precipitate a crisis
• Emphasize the importance of avoiding high-altitude activities to prevent hypoxia
• Encourage vaccinations, such as pneumococcal and yearly flu vaccines, to prevent infections
• Encourage hydration to maintain by drinking plenty of fluids to reduce blood viscosity
• Refer patients to support groups for emotional and psychological support
• Discuss the importance of genetic counseling to understand the risks of passing the disease to offspring

Polycythemia

• Polycythemia: Increased red blood cell (RBC) count which can lead to an increased viscosity of the blood decreasing circulation
• Polycythemia vera (PV) is a primary form of polycythemia, often linked to a genetic mutation, typically presenting in adults over 50
• Secondary polycythemia arises from long-term hypoxia due to conditions like COPD, heart failure, or living at high altitudes
• Classic symptoms include headache, dizziness, tinnitus due to viscosity, HTN and vision changes
• Lab results usually show elevated RBC, WBC, and platelets
• Due to increased risk of bleeding, assess for bleeds from the gums and nose

Hemorrhagic Disorders: Disseminated Intravascular Coagulation (DIC)

• DIC involves widespread clotting and subsequent bleeding due to exhausted clotting factors and platelets
• It can lead to organ and limb necrosis, therefore affecting vital organs
• The mortality rate can be quite high, if not rapidly treated
• DIC can occur following major trauma, severe infections, obstetric complications, or cancer related issues
• Clinical manifestations include abnormal bleeding, often with no prior history of this
• DIC is the widespread activation of the clotting cascade, leading to the formation of blood clots throughout the small blood vessels, which can result in organ dysfunction
• Symptoms include joint pain, swelling, and signs of bleeding due to the consumption of clotting factors and platelets
• Massive bleeding can lead to severe complications such as shock, organ failure, and acute respiratory distress syndrome (ARDS)
• Early recognition and treatment are critical for improving patient outcomes
• Initial laboratory findings include prolonged prothrombin time (PT) and partial thromboplastin time (PTT), decreased platelet count, and increased fibrin degradation products
• The D-dimer test is essential for identifying the presence of blood clots
• A decrease in hemoglobin (Hgb) levels may occur due to hemolysis due to an increased numbers of broken red blood cells (RBCs)
• Blood urea nitrogen (BUN) levels may rise if related to gastrointestinal bleeding
• Treatment focuses on correcting the underlying cause of DIC, such as managing sepsis or trauma
• Oxygen therapy, hydration, as well as administration of blood products are supportive interventions needed
• Cryoprecipitate may be infused to provide clotting factors necessary for coagulation
• IV heparin may be used in some to prevent further clot formation, particularly in chronic DIC
• Nurses must monitor vital signs frequently and be vigilant for signs of bleeding, reporting any changes immediately
• Nursing care includes critical thinking scenarios and the importance of interdisciplinary collaboration

Immune Thrombocytopenic Purpura (ITP)

• ITP is characterized by increased destruction of platelets due to immune system dysfunction, leading to a higher risk of bleeding
• Acute ITP often follows viral infections, while chronic ITP is more common in adults over 60 years old
• Common symptoms include petechiae, ecchymoses, and bleeding from mucosal surfaces such as the mouth and nose
• A platelet count of less than 20,000/mm3 is indicative of ITP, along with a prolonged bleeding time
• The primary goal of treatment is to achieve a safe platelet count and prevent bleeding episodes
• Most cases resolve spontaneously, but steroids may be administered to prolong platelet life by reducing immune activity
• Medications like romiplostim (Nplate) can stimulate platelet, production, while immunoglobulin may be used in acute situations
• Splenectomy may be considered for patients who do not respond to other treatments
• Blood transfusions and vitamin K may be necessary during acute bleeding episodes

Hemophilia

• Caused by lack of clotting factors, specifically factors VIII and IX
• Recessive X trait that presents mostly in men
• Dependent on the level of clotting factor deficiency
• Hemophilia A accounts for 80% of cases, while B takes 15% of all cases
• Platelet plug still occurs but lack of clotting factor
• Joint bleeding (hemarthrosis) results in deformities
• Prolonged bleeding is observed and symptoms monitored closely
• Treatment involves replacing the deficient factor either VIII or IX
• Genetic counseling is important for patients and their families
• Reduce the number of individuals with severe hemophilia to less than 13.3% by 2030
• Diagnostic tests will show prolonged partial thromboplastin time (PTT)
• Treatment options include desmopressin -mild A, factors VIII and IX in severe cases
• Assess pt knowledge about hemophilia an monitor for bleeding
• Risk for falls or bleeding due to lack of clotting factors
• Education is imperative for patients in factor replacement therapy and lifestyle modifications to reduce injury risk

Disorders of White Blood Cells: Leukemia

• Is defined as white blood and was identified by excess transparent cells from a blood sample(1845)
• Leukemia is an overproduction of immature blasts in the body
• Accumulation of abnormal cells in the bone and periphery, decreasing the bodies ability to fight infection and produce RBC and platelets decreasing the O2 carrying and increasing risk of bleeding
• Pt present with severe infections fatigue and anorexia, and untreated the disease is fatal
• Classified as acute or chronic and as lymphoid or myeloid
• Lymphoid leukemias affect lymphocytes, while myeloid leukemias affect other blood cell types
• Acute Lymphocytic Leukemia (ALL) is the most common childhood cancer (lymphocyte precursors),
• Acute Myelogenous Leukemia (AML) typically affects older adults
• Symptoms of acute leukemia include high fever, abnormal bleeding, and easy bruising.
• CLL is the most common leukemia affecting B and T cells
• CML is marked by the Philadelphia chromosome and commonly occurs in older adults
• Smoking predispositions one to leukemia, family, genetic , radiation also increase risk
• Radiologists have a higher incidence of leukemia, and patients treated for cancers are at risk with radiation
• Pt show fever, pallor, weak,shortness of breath, malaise-related anemia, can find bleeding in the gums or nose
• The bone may be involved in discomfort
• CBC showing a decrease in the platelets, RBCs, and white blood cells.
• The bone marrow aspiration, which determines if their malignant reproduction of white blood cells
• Lumbar puncture may be done to see if the central nervous involved or Genetic Analysis CML
• Aims to induction normal production, aggressive treatment, consolation (keeping the patient in remission)
• Depression and severe side effects are a risk factor due to the intensity of Chemo
• Targets area for elimination, stem transplant by dosing chemo high and reintroducing cells of the patient or donor.
• Assess pt for bleeding, fatigue, and complications by understanding regimen
• Assess pt goals to prevent injury/and symptoms

Understanding Multiple Myeloma

• Plasma cells overproduce in multiple myeloma, making antibodies that dont function well
• There is an increase in osteoclast activity and significant born loss
• Anemia, thromb, leuko and can also involve organ systems
• Failure of bone marrow an dysfunction sepsis
• Pt may say skeletal pain which worsens with movement
• May cause neurologically issues
• Hypercalcemia related,anemia,,fractures,or weight loss as the disease develops
• Assess calcium, white blood cells (WBCs), red blood cells (RBCs), and kidney function Urine may have jones protein, lung change and osteoperpisis needs bone marrow biopsy

Lymphatic Disorders

• The lymphatic system is cancerous because of the Reed-Sternberg cells of hodgkins lymphoma
• Commonly affects men who are 20s-30s
• Spread through lymphatic pathways but begin in the cervical region
• Hard to know reason for what may cause
• AIDS and immunosuppressed have a high risk
• With enlarged Lymph notes, pain, fever, malaise
• Common symptoms of advanced Hodgkin lymphoma and shortness of breath, persistent low-grade fever, night sweats, fatigue
• Lymph node biopsy, Bone marrow biopsy and liver scan to diagnose the stage
• Stage 1 is single lymph node or organ,4 stages that can be determined
• Tx depends on the stage like radiation of chemo-therapy and complications may occur, cardio or lung
• Nurses are there to manage the pt, and address their concerns
• Address survivors and provide support to help there struggles Differ from Hodgkins with degree of malignancy NHL more than 60, the other around 20-30 and 55