Hematology Review

Hematologic System

• Blood and blood-forming sites include bone marrow and the reticuloendothelial system (RES).
• Blood consists of plasma (the fluid portion) and blood cells (erythrocytes, leukocytes, thrombocytes).
• Hematopoiesis refers to the formation of blood cells.

Bone Marrow

• Bone marrow contains stem cells, which differentiate into myeloid and lymphoid lineages.
• The Myeloid lineage produces erythrocytes (RBCs), leukocytes (WBCs), and platelets.
• The Lymphoid lineage produces lymphocytes (T cells, B cells & plasma cells).
• Stroma is the part of a tissue or organ with a structural or connective role.

Hematopoiesis

• Hematopoiesis is the complex process of blood cell formation and maturation that occurs in bone marrow.
• Stem cells differentiate into myeloid or lymphoid stem cells.
• Myeloid stem cells differentiate erythrocytes, leukocytes, and platelets.
• Lymphoid stem cells differentiate into either T or B lymphocytes.

Question #2

• Neutrophils increase in number when a patient encounters a bacterial infection.
• Neutrophils are mature, circulating white blood cells.
• Neutrophils increase to phagocytize bacteria during a bacterial infection.

Reticuloendothelial System

• The RES is composed of special tissue macrophages.
• Monocytes released from marrow circulate briefly (about 24 hours) and then enter body tissues.
• Within the tissues, monocytes differentiate into macrophages, which can survive for months or years.
• Macrophages defend the body against bacteria and pathogens via phagocytosis.
• Macrophages remove old or damaged cells, stimulate inflammation, and present antigens to the immune system.
• Macrophages give rise to tissue histiocytes, which are phagocytic cells in loose connective tissue.
• Histiocytes include Kupffer cells of the liver, peritoneal macrophages, and alveolar macrophages.
• The spleen also is part of the RES.

Hemostasis

• Hemostasis occurs in three stages following blood vessel injury:
  • Vasoconstriction occurs first.
  • Platelet plug formation proceeds after vasoconstriction.
  • The final step is blood coagulation.

Assessment of Hematologic Health History

• Assess family history for blood disorders or abnormal bleeding.
• Evaluate history of bleeding: nosebleeds, tooth/gum bleeding, hematuria, menorrhea, hematochezia, GIB, or ulcers.
• Assess for extreme fatigue and for easy, deep bruising.
• Evaluate any abnormal bleeding or joint pain (potential sickle cell disease).
• Review blood cell count for abnormalities.
• Assess for any present illness despite low risk.
• Note any prior radiation/chemotherapy treatments.
• Ask about occupation, especially exposure to military Agent Orange.
• Assess alcohol consumption, diet history, herbal supplement use, and current medications.

Physical Assessment

• Skin: Note any gray, tan, brown or ruddy complexion, ecchymoses, petechiae, conjunctiva hemorrhage, pallor, or jaundice.
• Oral cavity: Check for petechiae in buccal mucosa, beefy red tongue, altered corner of mouth, or enlarged gums.
• Check for enlarged, firm, and fixed lymph nodes.
• Note increased respiratory rate, deep respiration, and adventitious sounds.
• Check for distended neck veins, edema, hypertension, murmur, or gallop.
• Assess for hematuria and proteinuria.
• Musculoskeletal: check for rib/sternal tenderness, back pain, loss of height, kyphosis, or pain/swelling in knees/wrists/hands.
• Abdominal: check for enlarged spleen and liver as well stool positive for occult blood.
• Neurologic: Assess for cranial nerve dysfunction, peripheral nerve dysfunction, visual changes, and headaches.
• Gynecologic: Check for menorrhagia.

Diagnostic Evaluation

• Hematologic studies, such as CBC and peripheral blood smear (RBC, WBC, H/H, and platelets) are important.
• Cellular morphology/shape and appearance are key.
• Bone marrow aspiration and biopsy will provide additional data.

Therapeutic Approaches

• Splenectomy is a surgical procedure in therapeutic approaches.
• Apheresis (Separation) is for platelet pheresis, leukapherisis, erythrocytapheresis, plasmapheresis, and stem cell harvest.
• Hematopoietic stem cell transplantation (HSCT) is a therapeutic approach.
• Phlebotomy and blood component therapy are treatment approches.
• Special preparations may also be used in therapeutic approches.

Blood Transfusion

• Pretransfusion assessment includes history of previous transfusion and reactions, number of pregnancies, and cardiac, pulmonary, and vascular disease.
• Informed consent must be obtained before the procedure.
• Patient education should include signs/symptoms of transfusion reaction.
• Ensure correct administration techniques based on agency policies and procedures.

Transfusion Complications

• Febrile nonhemolytic reaction, acute hemolytic reaction, allergic reaction, TACO, bacterial contamination
• TRALI, delayed hemolytic reaction, disease acquisition, long-term transfusion therapy

Transfusion Complications Explained

• A febrile nonhemolytic reaction is caused by antibodies to donor leukocytes, in patients with prior transfusions and in Rh-negative women with Rh-positive children.
• An acute Hemolytic Reaction is the most dangerous type of transfusion reaction and occurs when donor blood is incompatible with recipient blood.
• An allergic Reaction is due to sensitivity to a plasma protein causing urticaria, itching, and flushing.
• Allergic reactions are usually mild and respond to antihistamines.

Transfusion Complications Explained cont.

• Bacterial Contamination incidence is very low and can occur from procurement/processing.
• Platelets stored at room temperature are at greater risk of contamination.
• A Delayed Hemolytic Reaction usually occurs within 14 days after transfusion. Symptoms include fever, anemia, and jaundice. There may rarely be hemoglobinuria.

Transfusion-Associated Circulatory Overload (TACO)

• Results from too much blood infused too quickly, causing hypervolemia, and is aggravated in patients with CHF, renal dysfunction, advanced age, or acute MI.
• Assess carefully for circulatory overload or positive fluid status before transfusion.
• Pts at risk may be given diuretics before/between units of PRBCs.
• The infusion rate should be titrated to patient's tolerance of less than 100–120 mL/h.
• Signs of circulatory overload include dyspnea, orthopnea, tachycardia, increased blood pressure, sudden anxiety, jugular vein distention, crackles, and hypoxemia. Pulmonary edema can develop quickly, causing severe dyspnea and pink, frothy sputum.

Transfusion-Related Acute Lung Injury (TRALI)

• TRALI is the most common cause of transfusion-related death and is defined as acute lung injury within 6 hours of transfusion.
• This can occur after blood transfusion or blood components, including IVIG, cryoprecipitate, and stem cells.
• Symptoms include acute SOB, hypoxia [SaO2] less than 90%, hypotension, fever, and eventual pulmonary edema.
• A commonly used preventive strategy involves limiting the frequency and amount of blood products transfused.

Nursing Management of Transfusion Reactions

• Stop the transfusion, assess the patient, and notify the primary provider.
• Implement any treatments that are prescribed and continue to monitor.
• Return blood, obtain any necessary samples, and document.

Transfusion Alternatives

• Growth factors (hematopoietic).
• Erythropoietin.
• Granulocyte/macrophage colony-stimulating factor.
• Thrombopoietin to enhance platelet formation.

Chapter 29: Management of Patients with Non-Malignant Hematologic Disorder

• Beginning of second chapter.

Anemia Defined

• Lower than normal levels of hemoglobin and fewer than the normal circulating erythrocytes; it is a sign of an underlying disorder.
  • Hypoproliferative: defect in production of erythrocytes (RBCs); caused by deficiency in iron, vitamin B12, or folate erythropoietin.
  • Hemolytic: excess destruction of erythrocytes (RBCs); caused by altered erythropoiesis or direct injury to the erythrocyte.

Manifestations of Anemia

• Manifestations depend on the rapidity/duration of anemia, metabolic demands, concurrent problems, and concomitant features.
• General symptoms include fatigue, weakness, and malaise.
• Physical signs include pallor or jaundice.
• Patients may experience cardiac, GI, neurologic, and respiratory symptoms.
• Tongue and nail changes also may occur.
• Angular cheilitits and Pica are also potential signs of anemia.

Symptoms of Anemia

• Eyes - Yellowing
• Skin - Paleness, Coldness and Yellowing
• Respiratory - Shortness of breath
• Muscular - Weakness
• Intestinal - Changed stool color
• Central - Fatigue, Dizziness, Fainting
• Blood vessels - Low blood pressure
• Heart - Palpitations, Rapid heart rate, Chest pain, Angina, Heart attack
• Spleen - Enlargement

Diagnostic Testing for Anemia

• Hemoglobin and hematocrit.
• Reticulocyte count and RBC indices.
• Iron studies, vitamin B12 and Folate.
• Haptoglobin and erythropoietin levels.
• Bone marrow aspiration.

Medical Management of Anemias

• Correct or control the cause of the anemia.
• Provide Transfusion via packed RBCs.
• Treat the condition specific to the anemia.
• Dietary therapy.
• Iron/Vitamin supplementation with iron, folate, B12.
• Transfusions, immunosuppressive therapy, and other treatments as needed.

Hemolytic Anemias

• Sickle cell disease.
• Thalassemia.
• Glucose-6-phosphate dehydrogenase deficiency.
• Immune hemolytic anemia.
• Hereditary hemochromatosis.
• other anemias.

Hypoproliferative Anemias

• Iron deficiency anemia.
• Anemia in renal disease.
• Anemia of inflammation.
• Aplastic anemia.
• Megaloblastic anemia:
  • Folic acid deficiency.
  • Vitamin B12 deficiency.

Neutropenia

• Neutropenia is defined by decreased production/increased destruction of neutrophils (<2000/mm³).
• Neutropenia leads to increased risk for infection; monitor closely.
• Absolute neutrophil count (ANC).
• Medical management involves treatment dependent on the cause.
• Nursing management involves patient education, preventing/managing complications.

Lymphopenia

• Reduction of lymphocytes to below 1500/mm³.
• Causes:
  • Exposure to radiation.
  • Long-term use of corticosteroids.
  • Infections/Neoplasms/Alcohol abuse.

Polycythemia

• Increased volume of RBCs.
• Excessive production of erythropoietin is associated with reduced oxygen or cyanotic heart disease.
• Nonpathologic associations include smoking, obstructive sleep apnea, sever heart disease, high altitude exposure etc.
• Secondary polycythemia can be the result of neoplasms that stimulate erythropoietic production.
• Treatment not needed if condition is mild: -Treat underlying cause
  • Therapeutic phlebotomy.

Bleeding Disorders #1

• Failure of hemostatic mechanisms. Causes:
  • Trauma.
  • Platelet abnormality.
  • Coagulation factor abnormality.
• Medical management: the use of specific blood products.
  • Nursing management includes limiting injury, assessing for bleeding and bleeding precautions.

Bleeding Disorders #2

• Secondary thrombocytosis.
• Thrombocytopenia.
• Immune thrombocytopenic purpura (ITP).
• Platelet defects.
• Hemophilia.
• von Willebrand disease.

Assessment of the Patient with Anemia

• Includes health history, physical exam, and laboratory data.
• Note the presence of symptoms and their impact on life, such as fatigue, weakness, malaise, and pain.
• Conduct nutritional as well as medication assessments.
• Assess cardiac and GI systems; look for blood loss.
• Neurologic assessment is important.

Complications of Anemia

• Heart failure.
• Angina.
• Paresthesias.
• Confusion.
• Injury related to falls.
• Depressed mood.

Goals & Intervention for the Patient with Anemia

• Major goals include decreased fatigue.
• Maintenence of adequate nutrition and tissue perfusion.
• Promote balance of physical activity/exercise and rest.
• Patient education to promote compliance with medications and nutrition.
• Monitor VS and pulse oximetry; provide supplemental oxygen as needed.
• Monitor for potential complications.

Sickle Cell Disease

• SCD is an autosomal recessive disorder caused by inheriting the sickle hemoglobin (HbS) gene.
• Marked by severe hemolytic anemia.
• The HbS gene causes the erythrocyte to change shape when exposed to low oxygen.
• Erythrocytes are usually pliable/biconcave but become rigid and sickle shaped in this disease.
• Rigid cells adhere to the walls of blood vessels, causing decreased blood flow and ischemia/infarction, with severe pain, swelling, and fever or sickle cell crisis.
• Exposure to oxygen can reverse the process if cell membranes aren't too rigid.
• can be triggered by cold due to vasoconstriction that slows blood flow

Assessment for Sickle Cell Disease

• Collect a comprehensive health history, including a physical examination and pain assessment.
• Get lab data, focusing on S-shaped hemoglobin.
• Identify presence of symptoms; swelling, fever, pain.
• Conduct overall sickle cell crisis assessment.
• Monitor for blood loss along with cardiovascular /neurologic issues.

Complications of Sickle Cell Disease

• Include: hypoxia, ischemia, infection, dehydration and CVA.
• Anemia, acute/chronic kidney disease, and heart failure are potential complications.
• Impotence , along with poor compliance and substance abuse are also complications.

Interventions for the Patient with Sickle Cell Disease

• Pain management.
• Manage fatigue.
• Infection prevention.
• Promote coping and patient education regarding the disease.
• Monitor for complications.

Acquired Coagulation Disorders Acquired Coagulation Disorders

• Liver disease, Vitamin K deficiency, along with complications of anticoagulant therapy.
• Disseminated intravascular coagulation (DIC) and Thrombotic disorders.
• Hyperhomocysteinemia.
• Antithrombin deficiency, along with Protein C & S deficiency
• Activated factor for protein C resistance and Factor 5 Leiden malfunction.
• Also acquired can be Acquired thrombophilia and Malignancy.

Disseminated Intravascular Coagulation (DIC)

• DIC isn't a disease but a sign of an underlying disorder.
• Severity can vary and be fatal.
• Triggers include sepsis, other trauma, cancer and abruptio placenta. -Altered hemostasis leads to clotting in microcirculation. -Clotting factors and platelets are consumed, leading to bleeding.
• Treatment: target underlying cause; correct tissue ischemia.

Pathophysiology of DIC

• Significant illness leads to Activation of coagulation, resulting in activation of fibrinolysis (initially).
• Also forms thrombi, consuming platelets/clotting factors which can lead to bleeding .
• Tissue ischemia then organ failure then death.

Assessment of the Patient with DIC

• Be aware and access those who are at risk to be able to see symptoms and signs of the overall conditions.
• Be able to see signs, symptoms and progression of thrombi, and bleeding.

Complications of DIC

• Include: Kidney injury, Gangrene and Pulmonary embolism or hemorrhage.
• Acute respiratory distress syndrome.
• Stroke.

Goals and Interventions for the Patient with DIC

• hemodynamic state, and maintain intact skin,
• Assessment for signs of damage within in target areas is critical.
• Monitoring and trauma prevention are necessary.

Question #3

• The question statement is true.
• DIC is caused by an alteration of normal homeostasis mechanisms

Chapter 30: Management of Patients with Hematologic Neoplasms

• This chapter deals with hematologic neoplasms.

Leukemia

• Leukemia is hematolymphoid cancer with irregular proliferation.
• The types of leukemia- Acute myeloid, chronic myeloid, acute lymphocytic, chronic lymphocytic.
• Acute Myeloid (AML) Acute Lymphatic (ALL) Chronic Myeloid (CLM) Chronic Lymphatic (CLL)

Assessment of the Patient with Leukemia

• Requires health history.
• Assesses for sign of leukemia and complications related to Anemia.
• Look for weaknesses, fatigue, low leukocytes.
• Culture tests need to be done as needed.

Symptoms of Leukemia

• Nose bleeds, weight loss, swollen lymph nodes, fever and night sweats.
• Can also cause, swollen lymph nodes and easily bleeding.

Complications of the Patient with Leukemia

• Infection along with bleeding/DIC
• Tumor lysis syndrome
• Leukemia can lead to renal failure.
• Tumor lysis syndrome

Acute Myeloid Leukemia (AML)

• This is stem cell defect that separates into all myeloid cells with monocytes, platelets.
• It's the most not common type of leukemia and the rate can peak from 67 and onwards.
• AML has variable to poor prognosis and the manifestations include fever and infection.

Acute Myeloid Leukemia (AML) Treatment

• Treatment can be aggressive with using Chemotherapy and also involves hematopoietic stem cell implementation.
• Treatment can be Antimicrobial combined along with Transfusions in death cases as those are a primary factor.

Chronic Myeloid Leukemia (CML)

• CML involves mutation in myeloid stem cells from Philadelphia chromosomes.
• Stages of CML- chronic, transformational phase and blast crisis.
• Patients over the age of 20 are uncommon, the higher rates are with patients from age 64 upwards.
• Patients may be asymptomatic, but others include malaise, loss of weight can also be caused by leukostasis.

Lymphadenopathy

• Displayed.

Acute Lymphocytic Leukemia (ALL)

• Stem cells have uncontrolled growth from lymphoid cells.
• This it quite common in boys from age 4 forward.
• Its good to note the rate for 3 year is a near 85% chance, that will drop from a lower rate till 45 % as the person gets older.
• Symptoms displayed include severe pain from the liver and CNS.
• Chemotherapy and monoclonal antibody therapy can be used.

Chronic Lymphocytic Leukemia (CLL)

• CLL is a common malignancy of aged adults and a large scale of leukimia.
• The survival rates vary from 2-14 years depending on stages of the disease.
• The "B Cell" stage is defined as a stage when the patient has constellation.
• A stage will include- "watch and wait chemotherapy, IVIG,HSCT.

Planning and Goals for the Patient with Leukemia

• Major goals include the absence of problems and pains.
• Activity tolerance and attainment, in maintenance to care are also necessary.

Interventions for the Patient with Leukemia #1

• Interventions for those at risk with risk of infection.
• Mucositis has Frequent gentle, oral hygiene and frequent dental care are necessary.
• Soft tooth brush is required.

Interventions for the Patient with Leukemia #2

• Improve nutritional intake
• Oral care before and after meals
• Manage and administer analgesics are required before meals.

Interventions for the Patient with Leukemia #3

• Ease pain and discomfort,
• Tylenol for fever and other body aches.
• Decrease fatigue and maintain activity.

Interventions for the Patient with Leukemia #4

• Maintain fluid and electrolytes balance,
• track input and output volumes.
• laboratory study, asses electrolytes.
• Help with self care to help boost confidence.

Myelodysplastic Syndromes (MDS)

• Disorder of the myeloid stem cell that lead to fatigue.
• Those with CBC or bone marrow, or with the mean of 65-70.
• The only cure is with HSCT. Other wise treatment are stem cells.

Autologous Stem Cell Transplantation

• Displayed.

Myeloproliferative Neoplasms

• Polycythemia vera
• Essential thrombocythemia
• Primary myelofibrosis

Polycythemia Vera

• Proliferative disorder of myeloid stem cells.
• Typically diagnosed by the age of 60 and survival of 14-20 years.
• Symptoms include ruddy complexion, and some pressure issues
• Testing done for elevated hematocrit and a acquired mutation.

Polycythemia Vera Treatment

• Initially use phlebotomy and follow with suppression.
• use aggressive management of atherosclerosis.

Essential Thrombocythemia

• Also called primary thrombocythemia.
• Some type of vascular occlusion and the average median age is 65.
• Treatment is for thrombosis or the presence of hemorrhage.

Primary Myelofibrosis

• Those with older age rates are more common of males for more then 14 years.
• It is common for myelopribilosis.
• Treatment is based on reducing the bone density and improving bone count.

Question #1 for Neoplasms

• The statement is false and does not link to the cancer.
• Its due to the number of platelets.

Lymphoma

• Neoplasm of lymphoid origin
• Usually start in lymph nodes but can involve lymphoid tissue in the spleen, GI tract, liver, or bone marrow
• Classified according to degree of cell differentiation and origin of predominant malignant cell
• Two major categories: Hodgkin and Non-Hodgkin lymphoma

Hodgkin Disease

• Rare that the cure is high
• Due to the viral family passing down the generation along family tree then by the age of 50's it comes about. Reed stern berg cells are abnormal that may contain more then 1 nucleas and is fond in folks.

Manifestations Hodgkin Disease

• painless lymph node enlargement;
• Treatment is determined by the stage of the disease
• Radiation and chemo may be required for more serious cases.

Non-Hodgkin Lymphoma Treatment

• Lymphoid tissues become infiltrated with malignant cells
• Treatment for those that are effected, can come down to autoimmune and high exposures

Manifestations non-Hodgkin

• B symptoms/weight loss.
• Radiation may be needed depending on the stage.

Clinical Differences Between Hodgkin

• Shown .

Lymphoma

• Shown.

Question #2

• Is the following statement true or false?
• Hodgkin lymphoma is characterized by highly malignant cells that arise from a variety of tissues?
• Answer here is No.

Question #2 Rationale

• The lymphomas are neoplasms of cells of lymphoid origin; these tumors usually start in lymph nodes.

Multiple Myeloma

• cells that are affecting the bones along with ribs. In time that will fail if you fail to treat your issues.
• Chemotherapy may be required.

Multiple Myeloma Treatment

• The drug consist of a high number of immunomodulatory.

Question #3:

• What should any older adult patient be evaluated for
• If back pain is a constant issue, that is not a good sign and must seek medical attention so it does not make the pain worse.
• Cancer is the answer.

Questions covering key concepts in hematology, including blood cell types, their functions, and related clinical conditions. Topics include the role of the stroma in bone marrow, neutrophil counts, myeloid stem cell lineage, monocyte differentiation, macrophage defense mechanisms, platelet function, and the reticuloendothelial system.