exam 1-hematologic disorders

Question 1

Blood is comprised of (simple terms)

Answer 1

-Cells and proteins in plasma
-RBCs
-WBCs
-Platelets

Question 2

Components of human blood and their function

Answer 2

-RBC/erythrocytes: transport oxygen from the lungs to tissues and return carbon dioxide to the lungs for exhalation
-WBCs/leukocytes: defend the body against infections by recognizing and attacking pathogens such as bacteria, viruses, and foreign invaders
-platelets/thrombocytes: aid in blood clotting by clumping and forming plugs in blood vessel injuries to prevent excessive bleeding
-plasma: liquid component of blood that transports nutrients, hormones, and waste products. also helps to regulate body temperature and maintain blood pressure (huge part!)

Question 3

neutrophils

Answer 3

WBC
-first responder to bacterial infections; they engulf and destroy pathogens through a process called phagocytosis

Question 4

lymphocytes

Answer 4

WBC
-key players in the immune response divided into:
-B cells: produce antibodies to neutralize pathogens
-T cells: destroy infected or cancerous cells and regular immune responses

Question 5

monocytes

Answer 5

WBC
-develop into macrophages once they enter tissues; they engulf and digest dead cells, bacteria, and other debris through phagocytosis.

Question 6

eosinophils

Answer 6

WBC
-fight parasitic infections and play a role in allergic reactions by releasing enzymes that combat large invaders.

Question 7

basophils

Answer 7

WBC
-release histamine and other chemicals during allergic reactions and inflammation, helping to mediate the body’s response.

Question 8

plasma

Answer 8

the liquid portion of blood that serves as the medium that carries all these essential components throughout the body
-90% water: acts as solvent and helps regular body temperature and transport substances
-7% proteins including albumin, globulins, fibrinogen: albumin maintains osmotic pressure and helps transport hormones, vitamins, and drugs. globulins are involved in immune responses and transport of ions, hormones, and lipids. fibrinogen are essential for blood clotting.
-electrolytes: help maintain pH balance, proper nerve and muscle function, and fluid balance
-nutrients: glucose, amino acids, fatty acids. transported to cells for energy and growth
-waste products: carbon dioxide, urea, and lactic acid are transported to organs for excretion- during sepsis or infection check lactic acid to watch muscle breakdown.
-hormones and enzymes: regulate various physiological processes in the body.

Question 9

anemia

Answer 9

lack of healthy RBCs or hemoglobin

most common causes: acute blood loss, decreased or faulty production, or destruction

Question 10

hypoproliferative anemia

Answer 10

defect in production of RBCs

causes: iron, vitamin B12, folate deficiency, decreased erythropoietin production, cancer, bone marrow damage

Question 11

hemolytic anemia

Answer 11

excess destruction of RBCs

causes: altered erythropoiesis, or direct injury to the erythrocyte

Question 12

types of hypoproliferative anemias

Answer 12

-iron deficiency anemia
-aplastic anemia
-folic acid B9/folate deficiency
-vitamin b12 deficiency
-pernicious anemia

Question 13

iron deficiency anemia

Answer 13

iron stores are depleted leading to decreased iron supply for the manufacture of hemoglobin in RBCs, causes blood loss, increased metabolic demands, syndromes of gastrointestinal malabsorption, dietary inadequacy.

Question 14

aplastic anemia

Answer 14

deficiency of circulating erythrocytes and all other formed elements of blood, resulting from the arrested development of cells within the bone marrow (pancytopenia).

Question 15

folic acid B9/folate deficiency

Answer 15

RBCs are larger than normal, are oval-shaped rather than round-shaped due to lack of inadequate intake of folate
-folic acid is required for RBC formation and maturation
-causes: dietary deficiency, malabsorption, medications that decrease the absorption of folic acid, chronic alcoholism, hemodialysis, genetic

Question 16

vitamin b12 deficiency

Answer 16

anemia from inadequate intake of vitamin B12 or lack of absorption of ingested vitamin B12 from the intestinal tract

Question 17

pernicious anemia

Answer 17

autoimmune deficiency of intrinsic factor necessary for intestinal absorption of vitamin B12, gastric disease or surgery

Question 18

symptoms of hypoproliferative anemias

Answer 18

-fatigue, weakness
-pale skin
-shortness of breath, tachypnea
-dizziness or lightheadedness, change in LOC
-enlarged spleen or liver (hepatosplenomegaly) in bone marrow disorders
-petechiae or bruising suggesting bone marrow failure
-glossitis
-iron deficient anemia= PICA
-B12 deficiency= neurologic s/s nerve degeneration, neuropathy, altered MS

Question 19

diagnosis and treatment of hypoproliferative anemias

Answer 19

dx: patient history and symptoms- CKD, liver disease, dietary habits, blood loss, family hx, physical exam, lab tests to identify underlying cause of reduced RBC production= CBC, iron studies, vitamin levels, erythropoietin levels, bone marrow biopsy, BUN/Cr, LFTs, coagulation studies, stool guaiac

tx: depends on underlying cause, often including diet changes, iron supplements, vitamin B12 or folate supplementation, or treating the underlying disease. transfuse blood at H+H <7.

Question 20

assessment of hypoproliferative anemias

Answer 20

-VS, assess for tachycardia, hypotension, or hypoxia due to low oxygen carrying capacity
-assess for symptoms of anemia: fatigue, pallor, SOB, dizziness, activity intolerance
-monitor H+H
-assess neuro, especially in patients with vitamin B12 deficiency as it may cause numbness, tingling, or balance problems-fall risk!
-monitor for bleeding or bruising, especially in patients with concurrent platelet disorders or bone marrow failure- bleeding precautions!

Question 21

nursing management of hypoproliferative anemia

Answer 21

-administer iv therapy as needed
-elevate HOB
-encourage rest and limit activity
-administer iron supplements PO or IV
-administer vitamin B12 and folate injection or PO
-administer EPO for patients with anemia due to CKD to stimulate RBC production
-blood transfusion for severe anemia
-encourage nutrient rich diet with foods high in iron (red meat, beans, leafy greens), vitamin C to enhance iron absorption , avoid excess calcium intake during iron rich meals, animal products such as meat, eggs, and dairy or B12 fortified foods for B12, and leafy greens, legumes, and fortified cereals for folate.
-avoid antacids and calcium rich foods when taking vitamin C
-energy conservation and adequate rest
-educate self care strategies
-educate on worsening symptoms of anemia
-educate on preventing infection, especially with low white blood cell counts

Question 22

hemolytic anemia-sickle cell anemia

Answer 22

intrinsic/inherited: defects or abnormalities within the RBCs, inherited from parents
-chronic hemolysis= ongoing destruction of RBCs
-mild anemia to severe life-threatening conditions

sickle cell anemia: inherited blood disorder where RBCs become crescent or sickle shaped, leads to increased breakdown of RBCs (anemia) and blockage of blood vessels (vascular occlusion)

Question 23

symptoms of hemolytic anemia and sickle cell anemia

Answer 23

-fatigue and weakness
-pale or yellowish skin/jaundice
-dark urine due to excess breakdown of hemoglobin
-SOB
-enlarged spleen=splenomegaly leads to frequent infections
-sickle cell crisis: episode of pain in the chest, abdomen, joints, or bones
-acute chest syndrome: serious complication caused by sickled RBCs blocking blood vessels in the lungs, leading to chest pain, fever, and difficulty breathing
-dactylitis= hand-foot syndrome: swelling of the hands and feet
-vision problems due to blocked blood flow to the eyes

Question 24

diagnosis of hemolytic anemia

Answer 24

-family hx, genetic testing
-physical exam
-CBC, peripheral blood smear
-newborn screening (hemoglobin electrophoresis): critical in managing sickle cell anemia and reducing complications like pain crises, organ damage, and infections
-sickle cell test (sickling test)

early interventions such as vaccinations, abx, and monitoring can greatly improve outcomes for children with the disease

Question 25

medical management of hemolytic anemia

Answer 25

-no widespread cure: manage symptoms, prevent complications, improve quality of life
-only cure= bone marrow transplant
-medications: hydroxyurea, L-glutamine, folic acid supplements, pain management, antibiotics
-blood transfusions
-bone marrow transplant
-during a crisis: pain management (may have high tolerance!), aggressive hydration, oxygen if sat levels become critical (may halt formation of new RBCs)

HOPIA= hydration (watch BUN/Cr and Is and Os), oxygenation, pain control, infection prevention, avoid high elevations

Question 26

complication of sickle cell

Answer 26

sickle cell crisis
-occurs when sickle-shaped RBCs clump together, obstructing blood flow through small blood vessels (vaso-occlusion)
-episodes of severe pain (sickle cell crisis) and potential organ damage
-triggers increase the likelihood of RBCs sickling or reducing blood flow include: dehydration, infection, extreme temperatures (cold=vasoconstriction, hot=dehydration), hypoxia (high altitudes, vigorous exercise, respiratory issues), stress (stress hormone causes vasoconstriction), smoking (vasoconstriction)
-complications: infections, stroke, URIs, leg ulcers, splenomegaly

Question 27

nursing management of sickle cell anemia

Answer 27

-assess: intensity and location of pain, understand patient’s pain hx and triggers, VS closely for signs of infection or complications (fever, tachycardia), hydration status (dehydration, dry mucous membranes, low urine output)
-pain management: prescribed analgesics and adjunct therapies, use heat, relaxation techniques, positioning to help alleviate pain
-hydration: encourage fluid intake, IV fluids as needed, monitor electrolytes and correct imbalances
-preventing complications: infection control, vaccines as recommended, prophylactic abx for kids, monitor for s/sx of acute chest syndrome like cough, fever, or difficulty breathing.
-educate on disease, triggers, hydration, recognizing s/sx, lifestyle modifications for reduced stress, extreme temps, follow ups
-interdisciplinary collaboration: hematologists, social workers, nutritionists
-transition planning into adulthood
-monitor for long-term complications: signs of stroke, Hgb, organ function, potential complications
-emergency preparedness: crisis plan-develop individualized plan for managing pain crises and when to seek emergency care
-medication management: ensure patients have access to pain meds and understand their use

Question 28

polycythemia

Answer 28

an overproduction of RBCs, which lead to increased blood viscosity and a higher risk of clotting (monitor D-Dimer for presence of clots!)

-primary vera: increased volume of RBCs= disorder of bone marrow
-secondary polycythemia: excessive production of RBCs from hypoxia (high altitudes, heavy smoke-firefighters!)

management: treatment not needed if condition is mild, but treat underlying cause or use therapeutic phlebotomy which is the removal of blood to reduce RBC and Hct levels, with a goal of a Hct less than 45%

Question 29

symptoms of polycythemia

Answer 29

-ruddy complexion or flushed appearance
-HA
-SOB
-orthopnea
-dizziness or lightheadedness
-fatigue
-pruritus
-bone pain
-splenomegaly
-bleeding

diagnosis: CBC, JAK2 genetic mutation testing, bone marrow biopsy, erythropoietic levels, uric acid levels

complications: thrombosis- stroke, MI, PE, hemorrhage

Question 30

treatment and meds for polycythemia

Answer 30

-therapeutic phlebotomy: removal of blood to reduce RBC and Hct levels with a goal Hct below 45%
-medications: hydroxyurea, aspirin, GOUT- allopurinol, pruritus- antihistamines

Question 31

nursing management for polycythemia

Answer 31

-monitor blood count, Hgb, Hct, symptoms of thrombotic events, SOB, dyspnea
-patient education: importance of hydration, avoiding situations that may lead to thrombosis like immobility, recognizing s/sx of complications
-address s/sx of headaches, bone pain, or gout and manage pain
-psychological support as a chronic illness
-lifestyle modifications: increase fluid intake, encourage a heart-healthy diet, regular exercise, smoking cessation, avoid restrictive clothing and extreme temperatures
-work with multidisciplinary team including hematologists, dietitians, and mental health professionals

Question 32

thrombocytopenia

Answer 32

reduced number of platelets below the typical range of 150-400,000/mm3 as a result of:
1. decreased production of platelets by the bone marrow
2. increased platelet utilization (large clot formation that reduces number of circulating platelets)
3. increased platelet destruction

causes: malignancy, infection, medications (sulfa), autoimmune conditions (lupus), disseminated intravascular coagulation (DIC)

Question 33

types of thrombocytopenia

Answer 33

-immune/idiopathic thrombocytopenia purpura (ITP): most common autoimmune platelet consumption disorder
-hemophilia: group of bleeding disorders in which clotting factors are inadequate
-disseminated intravascular coagulation (DIC): occurs secondary to severe trauma to the body or following the administration of certain medication classes like chemotherapy
-heparin-induced thrombocytopenia (HIT): may occur in patients receiving heparin for an existing thrombus or clot

Question 34

symptoms of thrombocytopenia

Answer 34

-ecchymosis
-petechia
-prolonged bleeding
-menorrhagia
-hematuria
-epistaxis
-melena or hematochezia
-fatigue
-splenomegaly

Question 34

diagnosis and treatments of thrombocytopenia

Answer 34

dx: medical hx, family hx, physical exam, labs-CBC, peripheral blood smear, coagulation studies, LFTs, bone marrow bx

complications: spontaneous bleeding, shock (hypovolemic)

tx: treat underlying cause, meds-corticosteroids, IVIG, platelet transfusion, splenectomy, thrombopoietin receptor agonists, HIT-stop heparin infusion, avoid activities that increase the risk of bleeding
-always hold pressure when removing IVs as the patient will bleed!

Question 35

nursing management for thrombocytopenia

Answer 35

-monitor VS, s/sx of bleeding (urine, stool, skin), labs
-educate on signs of bleeding and when to get help, avoid activities that may lead to injury or bleeding like sports or heavy lifting, inform providers about condition and meds taken that could affect platelet function
-safety: fall precaution, bleeding precaution, gentle care to prevent trauma to skin and mucous membranes
-manage pain
-emotional support: address anxiety or fears, encourage open communication
-work with multidisciplinary team

Question 36

leukemia

Answer 36

type of cancer that affects the blood and bone marrow
-uncontrolled production of abnormal WBCs, which crowd out healthy blood cells and impair the body’s ability to fight infections, produce RBCs, and form platelets.
-affects bone marrow: causing anemia, leukopenia, thrombocytopenia, and decline in immunity

risk factors: genetic, viral, immunological, environment factors, exposure to radiation, chemicals, medications, chemotherapy

Question 37

ALL= acute lymphoblastic leukemia

Answer 37

rapidly progressing and primarily affects children, but also occurs in adults.
-uncontrolled proliferation of lymphoblasts present in bone marrow
-pain from enlarged liver/spleen, bone, CNS HA and vomiting
tx: chemotherapy, HSCT, monoclonal antibody therapy, corticosteroids

Question 38

CLL= chronic lymphocytic leukemia

Answer 38

progresses slowly and typically affects older adults
-mostly lymphocytes present in bone marrow
-“b symptoms”: a constellation of symptoms including fevers, drenching sweats especially at night, and unintentional weight loss
tx: early stage watch and wait, chemotherapy, monoclonal antibody therapy, IVIG for recurrent infections, HSCT

Question 39

AML= acute myeloid leukemia

Answer 39

affects both children and adults and progresses quickly
-mostly myeloblasts present in bone marrow
-fever and infection
-weakness and fatigue
-bleeding tendencies
-pain from enlarged liver or spleen
-hyperplasia of gums
-bone pain
tx: aggressive chemotherapy, hematopoietic stem cell transplantation (HSCT)
supportive care may be the only option, antimicrobial therapy and transfusions, death occurs within months.

Question 40

CML= chronic myeloid leukemia

Answer 40

slow-growing, but may eventually become more aggressive
-mutation in myeloid stem cell with uncontrolled proliferation of cells-mostly granulocytes present in bone marrow
-initially may be asymptomatic
-malaise
-anorexia
-weight loss
-confusion
-SOB
-enlarged spleen or liver
tx: chemotherapy, HSCT, imatinib mesylate (gleeve)

Question 41

assessment of leukemia

Answer 41

health hx: recurrent infections or illness

symptoms:
-anemia
-thrombocytopenia
-fatigue and weakness-anemia
-frequent infections-lack of healthy WBC
-bone pain- long bones, overcrowded bone marrow
-swollen lymph nodes, liver, or spleen

labs: CBC, coagulation studies, LFTs, bone marrow biopsy, cultures

Question 42

planning and goals for the patient with leukemia

Answer 42

-absence of complications and pain
-attainment and maintenance of adequate nutrition
-activity tolerance
-ability to provide self-care and to cope with the diagnosis and prognosis
-positive body image
-understanding of the disease process and its treatment

Question 43

mucositis

Answer 43

-frequent, gentle oral hygiene
-soft toothbrush or if counts are low, sponge-tipped applicators
-rinse only with NS, NS and baking soda, or prescribed solutions
-perineal and rectal care

Question 44

interventions for leukemia

Answer 44

-improve nutritional intake: oral care before and after meals, analgesics before meals, little chewing, tx of nausea, small frequent feeds, soft foods, high calorie diet with proteins and carbs, low-microbial diet
-easing pain and discomfort: acetaminophen for fever and myalgias, cool water sponging, frequent bedding changes, gentle massage, relaxation techniques
-decreasing fatigue and activity intolerance: assist with self care and mobility, allow adequate rest, only perform essential activities, assist in activities during periods of highest energy
-chemo nurses must be certified and follow special orders!

Question 45

complications of leukemia

Answer 45

-infection: major cause of death- initiate protective isolation procedures (neutropenic/reverse isolation/precautions), ensure frequent and thorough handwashing, reduce exposure to environmental organisms
-bleeding/disseminated intravascular coagulation: risk for bleeding and platelet count below 50,000, spontaneous bleeding with platelet count lower than 20,000 need platelet transfusion (petechiae, examine all body fluids and excrement for blood), anemia and fatigue-PRBC transfusion, allow adequate rest periods during care

Question 46

lymphoma

Answer 46

cancer that originates in the lymphatic system, lymph nodes, the spleen, thymus, and bone marrow
-lymphocytes grow uncontrollable
-two categories: hodgkin’s and non-hodgkin’s

Question 47

hodgkin lymphoma

Answer 47

-characterized by the presence of Reed-Sternberg cells, a specific type of abnormal cell
-typically begins in lymph nodes and spreads in an orderly fashion

Question 48

non-hodgkin lymphoma

Answer 48

-a diverse group of lymphomas that do not have reed-sternberg cells
-can arise in various parts of the body and spread more randomly

Question 49

symptoms of lymphoma

Answer 49

-swollen lymph nodes and enlarged spleen or liver
-fatigue
-fever and night sweats
-weight loss
-itching and skin rashes
-chills
-tachycardia
-loss of appetite
-anemia and thrombocytopenia

dx: biopsy lymph node, CT scans, PET scans, MRIs to identify extent of disease spread, or CBC

tx: chemotherapy, radiation therapy (may do radiation early when there is no node involvement, and chemo for more extensive cases), immunotherapy or targeted therapy, stem cell transplant

Question 50

multiple myeloma

Answer 50

type of cancer that affects plasma cells, a type of WBC found in bone marrow
-abnormal plasma cells multiply uncontrollably, producing large amounts of abnormal antibodies that can damage bones, impair the immune system, hypercalcemia, and cause kidney problems

Question 51

symptoms of multiple myeloma

Answer 51

-bone pain, especially in the spine, ribs, or pelvis
-fatigue
-frequent infections
-anemia
-kidney dysfunction
-weight loss
-weakness
-Bence Jones proteinuria and elevated total serum protein level
-osteoporosis
-thrombocytopenia and leukopenia
-abnormal number of immature plasma cells in bone marrow aspiration

dx: measure abnormal proteins (M-proteins) in blood, Ca levels, BUN/Cr, urine presence of Bence Jones proteins. bone marrow biopsy presence of abnormal plasma cells, imaging x-rays, MRI, or CT to detect bone damage

tx: chemotherapy, stem cell transplant, targeted therapy and immunotherapy, biphosphonates, neutropenic and bleeding precautions as necessary