Hematologic 2023 with solution questions and answers

What percentage of body weight is blood? 8% What are the two main parts of blood and what are their percents? plasma: 55% formed elements: 45% What are the parts of plasma? protein: 7% water: 91% solute: 2% What are the parts of formed elements? -platelets: 250-400 thousand -WBCs: 5-9 thousand -RBCs: 4.2-6.2 million What is anemia? a symptom of an underlying disorder in which there is an O2 supply deficit What are the types of anemia? relative and absolute What is relative anemia? an increase in plasma liquid volume that gives an appearance of anemia (hemodiluted) What is absolute anemia? a decrease in RBC numbers Moderate anemia Hgb 7-10 Severe anemia Hgb 7 What are the classifications of absolute anemia? -hemolytic -hypoproliferative -loss What is hemolytic anemia? an increase in RBC destruction Give examples of hemolytic anemia -sickle cell anemia -babies attacking "momma blood" after birth which is normal What is hypoproliferative anemia? a decrease in RBC production What can cause hypoproliferative anemia? -defective hemoglobin synthesis (Fe, B12, and B9 deficiency) -impaired kidney, bone marrow, or stem cell function) Which type of anemia is caused by a B12 deficiency and what are some causes of it? Pernicious anemia is a result of B12 deficiency which can be caused by GI problems/sleeves and NGTs What is loss anemia? an overall decrease in volume due to bleeding How do the kidneys contribute to blood formation? they secrete erythropoietin which stimulates the production of RBCs MCV mean corpuscular volume (average volume of RBCs) What are the types of hypoproliferative anemias? -pernicious/megaloblastic -folate deficiency -iron deficiency -decrease if erythropoietin -anemia of chronic dx -aplastic anemia RETIC count count of reticulocytes (immature RBCs) What is the outcome of megaloblastic anemias? the shape of the cells is abnormal and large due to impaired DNA synthesis How does ETOH affect RBC production? ETOH increased the need for folate; decreased folate leads to poor RBC production Lab values for pernicious/megaloblastic anemia -decreased B12 level -increased MCV -elevated homocysteine levels -low RETIC count What is the MCV increased and the RETIC count decreased in pernicious anemia? -MCV increased because the new RBCS are bigger -RETIC count is low because the body is unable to make more RBCs because of the deficiency Tx for pernicious/megaloblastic anemia -replace B12 -cyanocobalamin 1000 ug IM monthly Unique manifestation of megaloblastic anemias? neurological effects-monitor gait, coordination, sensation, and safety Foods high in B12 -organ meats -shellfish -meat -poultry -eggs -milk What causes a folate deficiency r/t anemia? -poor intake -ETOH (impairs folate metabolism in the liver) -leads to increased erythroblast cell death Lab values for folate deficiency anemia -decreased folate levels -increased MCV Tx of folate deficiency anemia oral folic acid 1-5 mg/day What causes a iron deficiency r/t anemia? -chronic blood loss -demand exceeds intake -lower Hgb production due to low iron stores Lab values for iron deficiency anemia -decreased reticulocytes -decreased Fe and ferritin -decreased iron saturation -decreased MCV -increased TBC Tx of iron deficiency anemia -iron replacement therapy -ferrous sulfate 325 mg TID What causes a decrease in erythropoietin r/t anemia? renal disease leads to decreased production which leads to a decrease in RBCs Lab values for erythropoietin deficiency anemia -decreased erythropoietin -normal MCV and MCH -increased creatinine What is MCH? mean corpuscular hemoglobin Tx of erythropoietin deficiency anemia erythropoietin replacement (SQ injections) Anemia of chronic dx marked is marked by what 3 defects? -shortened erythrocyte lifespan -bone marrow poor to respond to erythropoietin -altered iron metabolism Lab values of anemia of chronic dx -normal MCV and MCHC -low serum Fe -low/normal TIBC -normal/high ferritin Tx of anemia of chronic dx -treat dz -does not respond to Fe therapy What causes aplastic anemia? an immune response damages cells in bone marrow--replacement of the marrow with fat--decrease in the production of all blood cells Specific manifestation of aplastic anemia Normal anemia symptoms plus bruising (decreased clotting) and possibly decreased ability to fight infection Lab values for aplastic anemia -decreased RETIC count -decreased WBCs, Hgb, Hct, and PLTs Tx for aplastic anemia -immunosuppressive therapy -stem cell transplant Management of aplastic anemia same as general plus infection control and bleeding precautions What are the types of hemolytic and loss anemias? -bleeding (loss) -autoimmune hemolytic anemia -thalassemias What are thalassemias? a group of inherited forms of autoimmune hemolytic anemia in which there is a decrease in the hemoglobin content of RBCs (hypochromia) Who is most likely to have thalassemia? Mediterraneans, Africans, and Southeast Asians Lab values for bleeding anemia -increased RETIC count -normal Hgb, Hct, MCV (normal then drop) -decreased ferritin and Fe (later) Tx for bleeding anemia -packed RBC transfusion -ID source of bleed and control it What is the cause of autoimmune hemolytic anemia? -either inherited or acquired leading to hemolysis within blood vessels or lymphoid tissue Lav values for autoimmune hemolytic anemia -presence of schistocytes -increased spherocyte level -increased reticulocytes Tx of autoimmune hemolytic anemia -if acquired: remove insult and tx the underlying disorder -if inherited: immunosuppressive therapy What causes thalassemias? cellular abnormalities of the Hgb structure, marked by an imbalance between the beta and alpha chains of hemoglobin which leads to RBC membrane damages, ineffective RBC production, and hemolysis Lab values for thalassemias -decreased MCV -fragmented RBCs -increased RETIC count Tx of thalassemias transfusion therapy with PRBCs Tx of hemolytic anemias high dose of corticosteroids until hemolysis decreases; splenectomy if necessary General manifestations of anemia -initially asymptomatic -weakness, significant fatigue -pallor -brittled, ridged, spoon shaped nails -slow healing, loss of skin elasticity, dryness, early thinning/graying hair -tachycardia, palpitations, peripheral edema, hypotension -dyspnea, tachypnea, orthopnea -anorexia, dysphagia, abd pain, hepatomegaly, splenomegaly, pica What is pica? craving of unusual items including ice, starch, or dirt Which assessment finding is specific to hemolytic, megaloblastic, and iron deficiency anemias? -hemolytic: jaundice -megaloblastic: beefy red sore tongue -iron deficiency: smooth and red tongue What is angular cheilosis? ulcerated corners of the mouth What are 3 important things to manage in anemia patients? -fatigue -nutrition -perfusion Most common anemia Iron deficiency anemia What foods are high in iron? -liver -red meats -beans -leafy greens -raisins -VIT-C helps absorption How can iron be given and are there considerations for these? -PO (give with vitamin C like OJ) -IM (ztrack in gluteus maximus) -IV (slow and monitor for infiltration/nausea) Patient education regarding iron deficiency anemia -Constipation may occur (increase fiber, water, or take stool softner) -Increase Vit C intake (citrus, strawberries, tomatoes, broccoli) -Stool may change color and become dark -Take liquid iron via straw to prevent teeth staining Patients on dialysis have deficiencies in what substances? Fe and folate When do you begin to see anemia in renal dz? when creatinine is 3/mg 100 mL What is given for renal dz and anemia? epoetin alpha and oral Fe What are leukemias? a group of hematological malignancies involving the abnormal overproduction of leukocytes Which cells are involved in leukemia? granulocytes, monocytes, and lymphocytes Leukemias are a defect in what? the lymphoid or myeloid stem cells What are common features of leukemia? unregulated proliferation of leukocytes in the bone marrow--bone marrow gets FULL of these cells--release of premature cells into circulation--leukocytosis--reduced ability of other cells to proliferate--pancytopenia What is pancytopenia? decrease in all blood cells and increase in immature WBCs What is acute myeloid leukemia? defect in the hematopoietic stem cells that differentiate into ally myeloid cells What are the myeloid cells? -monocytes -granulocytes (neutrophils, basophils, eosinophils) -erythrocytes -platelets Incidence of AML rises with age, peak at age 60; most common nonlymphocytic leukemia Physiologic alterations in AML -neutropenia -anemia -thrombocytopenia -proliferation of the WBCs in other tissues leads to swelling and pain (gums, liver, spleen, bone) Manifestations of AML -fever, infection, weakness, fatigue, bleeding tendencies, pain -major cause of death: bleeding and infection Management of AML -induction therapy (chemo); kill off the leukemic cells --healthy cells die too--inability to fight infection -stem cell transplant to help manufacturing plant -infection precautions, admin RBCs, bleed precautions, promote comfort, support pt What is neutropenia? -absolute neutrophil count 500 cells/ul Calculation of neutropenia? [(%neutrophils + %bands) x WBC]/100 What happens in the ANC fall below 500? -the body cannot mount a sufficient immune response Neutropenia requires what special intervention? revere isolation (protects the patient) What are reverse isolation precautions? gown, gloves, and mask What is chronic myeloid leukemia? a mutation in the myeloid stem cells (chromosome translocation from 22 to 9); normal myeloid cells continue but there is an increase in the production of blast cells What are the phases of CML? chronic accelerated blast crisis Incidence of CML uncommon 20 yo; median age varies by race (white=75, black=40 and 70) What happens during the chronic phase of CML? there is an adequate number of healthy cells along with leukocytosis What happens during the accelerated phase of CML? abnormal cells are being produced at a faster rate What happens during blast crisis of CML? more immature cells than healthy cells are present Manifestations of CML -chronic phase: rarely symptomatic; leukocytosis on a CBC -fatigue, bleeding, wt loss -crisis: SOB, confusion (dt decreased capillary perfusion secondary to increased number of leukocytes clogging flow) -tender spleen and liver dt enlargement -malaise, anorexia, and wt loss Management of CML -TKI inhibitors (block signal to express the BCR-ABL protein which prevents the effected cell from growing and dividing) -chemo -leukapheresis (removal of leukocytes) -stem cells transplant -supportive therapy What is acute lymphocytic leukemia? uncontrolled proliferation of immature cells (lymphoblasts) derived from the lymphoid stem cell Incidence of ALL most often in young children (boysgirls); peak incidence is 4 yo; after 15 yo is more uncommon Alterations that occur with ALL -lymphocytes proliferate out of control leading to impaired development of normal myeloid cells leading to reduction in RBCs, WBCs, and platelets -leukocyte counts may be low or high but always immature Manifestations of ALL -manifestations of leukocytes infiltration into other organs more common with ALL (bone pain, enlarged end organs, CNS disturbances, testicular swelling) -headaches, visual changes, vomiting, neurologic deficits Management of ALL -Complicated specialized chemo protocols -Viral infections are common leading to infection prevention is big -Prophylactic antibiotics to prevent infections -Bleeding precautions -Symptom management What does ALL lead to? anemia, increased risk for infection, and clotting problems What is chronic lymphocytic leukemia? malignant clone of B-lymphocytes; the cells are fully mature; escape apoptosis (cell death) leading to a build up of cells in the marrow and circulation Incidence of CLL common in older adults; 81% are 60 yo; most common form of leukemia in US and Europe Alterations in CLL -Increase in B-Lymphocytes in circulation and marrow -Lymphadenopathy -Enlarged spleen and liver -Anemia, thrombocytopenia -Autoimmune disorders occur as well Manifestations of CLL -Many are asymptomatic 15% present with "B Symptoms": fevers, fatigue, drenching sweats and unintentional weight loss -Abnormal humoral and cell-mediated immune response: infection rates are higher -Decrease response to skin sensitivity testing = anergy -Fatigue, weakness, bleeding Management of CLL -Possibly no treatment in early stages -Chemo as the disease progresses -Monoclonal antibody therapy (responds to the CD52 antigen on the surface) -Symptom management -Monitor labs: Leukocyte count, ANC, Hematocrit, PLT, CREAT, Electrolytes, LFTs, -Monitor for subtle changes in assessment indicative of infection -Monitor temp for changes warranting blood cultures What is lymphoma? neoplasms of lymphoid tissue What are they types of lymphoma? -hodgkins -non-hodgkins What are reed-sternberg cells? cells that indicate malignancy and hodgkin's lymphoma What is Hodgkin's lymphoma? malignancy of lymph nodes that originates in a single node (unicentric) that spreads by contiguous extension along the lymphatic system What is the cause of Hodgkin's lymphoma? -unknown but suspect viral in nature (EBV), agent orange, wood workers, or immunosuppression therapy What characterizes Hodgkin's lymphoma? generalized painless lymphadenopathy Incidence of Hodgkin's lymphoma higher in males and 20s or 50s Prognosis of Hodgkin's lymphoma good 5 year survival rate 90%; recurrences are not uncommon Diagnosis of Hodgkin's lymphoma biopsy of node indicating Reed-Sternberg cells (stages include 1-1 node, 2-2+ nodes same side of diaphragm, 3-multiple nodes and one non lymph organ, 4-diffuse involvement Treatment of Hodgkin's lymphoma -radiation -chemo -splenectomy Assessment finding of Hodgkin's lymphoma -lymph nodes (non painful, enlarged) -labs: CBC (anemia, thrombocytopenia, increased leukocytes) -fever (increased infection risk) -anorexia, wt loss -malaise, bone pain -night sweats Nursing plans and interventions for Hodgkin's lymphoma -infection prevention, monitoring, and tx -anemia monitoring, tx, and support -encourage rest, and HIGH NUTRIENT DIET -post op care -emotional support What is non-hodkin's lymphoma? malignant disease of the lymph system with various cell types Cause of non-hodgkin's lymphoma unknown but -immunodeficiency or autoimmune disorders (higher risk) -prior cancer tx -prior organ transplant -EBV and HIV -exposure to pesticides, solvents, dyes, or agent orange Which lymphoma is more deadly? Non-Hodgkins Diagnosis of non-hodgkin's lymphoma usually once it is the later stages Manifestations of Non-Hodgkin's lymphoma -lymphadenopathy, B-symptoms B-symptoms fever fatigue wt loss night sweats Tx of Non-Hodgkin's lymphoma -radiation -chemo alone and with prednisone Nursing management of Non-Hodgkin's lymphoma -management of tx side effects (mouth sores, GI upset/disturbance) -infection prevention, control, and tx What is multiple myeloma? malignant disease of the plasma cells (not a lymphoma) What happens during multiple myeloma? plasma cells secrete immunoglobulin which are needed to fight infection which causes the inability to fight infection and ultimately causes infection Incidence of multiple myeloma? most common in people over 70 What is the prognosis of multiple myeloma? 3 years with standard therapy and 5 years with intense therapy; death usually from infection What does multiple myeloma cause? bone lesions 90% of the time (causes intense bone pain that increased with movement and decreases with rest) What is the process of multiple myeloma? the malignant plasma cells secrete osteoclast activating factors causing bone breakdown and lytic lesions (sections of bone being broken down) and osteoporosis Diagnosis of multiple myeloma bone marrow biopsy; more than 10% plasma cells=positive test Tx of multiple myeloma -no cure -chemo, marrow transplant, immunosuppressive therapy, corticosteroids -radiation therapy (strengthening bone, reduce cell size, reduce pain) -monitoring and treatment of VTE due to increased risk Management of multiple myeloma -PAIN MANAGEMENT (NSAIDs and opioids) -educate about activity restriction -monitor for peripheral neuropathy (a common SE of tx) -assist with ADLs -educate about s/s of hypercalcemia (a common complication) -monitor for, prevent, and tx infection -vaccinations -monitor for SE of corticosteroid tx SE of corticosteroid tx -hyperglycemia -insomnia -osteopenia, osteoporosis -cataracts -diabetes What is hemostasis? the stoppage of blood flow What is coagulation? the formation of an insoluble clot regulated by two naturally occurring substances (procoagulants and anticoagulants) What are thromboembolic disorders? when the body forms undesirable clots; too much clotting; arterial or venous What are the arterial and venous thromboembolic disorders? -arterial: PE -venous: DVT Risk factors for DVT/PE -extended immobility -major trauma -major surgery -hypercoagulability states -drug therapy What are coagulation disorders? result from too little clotting Types of coagulation disorders -thrombocytopenia -hemophilia What is thrombocytopenia? -platelet count below 150,000 mm3 Causes of thrombocytopenia -decrease in platelet production -increase in platelet destruction Hemophilia A not enough factor 8 Hemophilia B not enough factor 9 Hemophilia C not enough factor 11 Causes of coagulation disorders -condition or dz that suppresses bone marrow -folic acid or B12 deficiencies -hepatic failure -drug therapy What are the type of coagulation modifiers? -prevention of clot formation -removal of an existing clot -promotion of clot formation What drugs prevent clot formation? -anticoagulants -antiplatelet agnets