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UA Pathophysiology (Lachel Story) Ch. 3 - Hematopoietic Function

Terms in this set (38)

How much blood is in our bodies
~5L
Hematocrit
Amount of blood volume occupied by erythrocytes (RBCs)
Thrombocytes
platelets
Hemostasis
Stoppage of blood flow. Stages 1: Vessel spasm/vasoconstriction, 2. Formation of platelet plug, 3. Blood coagulation/clotting to form scab, 4. Clot retraction, 5. Clot dissolution to resume blood flow.
Leukocytes
WBCs; Normal number range is 5,000 - 10,000 mm3; key in inflammatory response and fighting infections.
Leukopenia
decreased number of WBCs
Leukocytosis
increased number of WBCs
Neutrophils
Type of leukocyte; usually first to arrive at infection site. Normal number range is 2,000 - 7,500 cells/microL. Increased number means it's at the beginning of an infection.
Neutropenia
Less than 1,500. WBCs could be under attack, or are all used up. Causes: increased usage, drug suppression, radiation therapy, congenital conditions, bone marrow cancers, spleen destruction, vitamin deficiency. Must protect patient from US. ClinMan: s/s of bacterial or fungal infections such as malaise, chills, fever. Respiratory tract, skin, vag, GI tract are most common sites of infection. Diag: Serum neutrophil levels and bone marrow biopsy. Treatment: treat infections as they develop. Hematopoietic growth factors to stimulate maturation of neutrophils.
Spleen's role in leukocytes
gets rid of old RBCs/WBCs and stores WBCs and immature WBCs. If w/o spleen, may end up with neutropenia/be careful of infections. Difficult to fight a 2nd infection after another one bc the 1st one uses all the neutrophils. Immature WBCs aren't as useful.
Infectious Mononucleosis
Most prevalent in adolescents and young adults. Caused by Epstein-Barr virus in Herpes fam that infects the B cells by killing it or incorporating into its genome. The B cells then produce heterophile antibodies. Few cells remain after eradication, so it can come back when under stress. ClinMan: Insidious onset, 4-8 wk incubation, anorexia, malaise, chills which intensify to leukocytosis, fever, chills, sore throat, lymphopathy, enlarged spleen bc it's producing WBCs. Acute stage lasts 2-3 wks, but may not fully recover for 2-3 mo. Treatment: symptomatic and supportive.
Lymphoma
cancers affecting lymphatic system. Most common hematologic cancer in the USA. Two main types: Hodgkin's and Non-Hodgkins.
Hodgkin's Lymphoma
Least common of two lymphoma types. Solid tumor usually in lymph nodes of upper body. Reed-Sternberg cells. ClinMan: painless large nodes above waist, weight loss, fever, night sweats, pruritis/itch, coughing, difficulty breathing, chest pain, recurrent infections, splenomegaly. stage 1- lymph node involvement above the waist in one area, 2- more than one node, 3- mult nodes above waist, and some nodes below waist, 4- rampant. Recurrent- the disease returns after treatment. Diag: presence of Reed-Sternberg cells in node biopsy, CBC, chest x-ray, CT scan, MRI, PET scan, marrow biopsy. Treatment: chemo, radiation, surgery
Non-Hodgkin's Lymphoma
More common but has poor prognosis and many diff types. Similar ClinMan, staging, and treatment as Hodgkin's, but diff in spread and diagnosis. Can originate in the T or B cells and don't have Reed-Sternberg cells. Metastasis happens fast and the patient becomes sicker with more than just lymphoma.
Leukemia
cancer of the leukocytes where the cells abnormally proliferate and crowd normal blood cells. ClinMan: leukopenia, anemia, thrombocytopenia (low platelets), lymphadenopathy (joints hurt), joint swelling, bone pain, weight loss, anorexia, hepatomegaly, splenomegaly, and central nervous system dysfunction. Diag: peripheral blood smears, CBC, and bone marrow biopsy. Treatment: chemotherapy and bone marrow transplant
Acute Lymphoblastic Leukemia / ALL
affects children primarily, responds better to chemotherapy than chronic leukemias and has good prognosis
Acute Myeloid Leukemia / AML
affects adults primarily, responds better to chemotherapy than chronic leukemias, but prognosis is somewhat worse than ALL.
Chronic Lymphoid Leukemia / CLL
affects adults primarily, responds poorly to therapy yet most patients life many years after diag.
Chronic Myeloid Leukemia / CML
affects adults primarily and responds poorly to chemo, but bone marrow transplant improves prognosis.
Multiple Myeloma
plasma cell cancer with excessive numbers of abnormal plasma cells in bone marrow that crowd the blood-forming cells and cause Bence Jones proteins to be excreted in urine. Bone destruction leads to hypercalcemia and pathologic fractures. ClinMan: insidious onset, anemia, thrombocytopenia, leukopenia, decreased bone density, bone pain, hypercalcemia, renal impairment. Diag: Blood and urine proteins, calcium level, renal function tests, CBC, biopsy, x-rays, CT and MRI scans. Treatment: chemo and complication mngmt. Not curable.
Erythropoiesis
Production of erythrocytes, regulated b erythropoietin and occurs in bone marrow. Disorders typically result from deficit or defect of the erythrocytes.
Anemia
decreased number of erythrocytes, reduction in hemoglobin or abnormal hemoglobin. Decreases Oxygen carrying capacity leading to tissue hypoxia. General ClinMans: weakness, fatigue, pallor, syncope, dyspnea, and tachycardia. The male hematocrit level will be <41 and female will be <37 and are more prone.
Iron-Deficiency Anemia
Very common. Fe is necessary for hemoglobin production. Causes: decreased Fe consumption, or absorption, and increased bleeding. ClinMan: cyanosis of eye sclera, brittle nails, decreased appetite, headache, irritability, stomatitis, pica, and delayed wound healing. Diag: CBC (low hemoglobin, hematocrit, MCV, MCHC), serum ferritin and Fe, and transferring saturation. Treatment: identify and treat cause, increase dietary Fe intake (green leafy veggies, liver/red meat) and supplements (may lead to constipation tho).
Pernicious Anemia
Vit B12 deficiency usually caused by lack of intrinsic factor protein needed to absorb B12 in stomach. Causes are autoimmune. Vit B12 required for DNA synth. Leads to decreased maturation of RBCs and cell division. May see myelin breakdown and neuro complications. ClinMan: bleeding gums, diarrhea, impaired smell, loss of deep tendon reflexes, anorexia, personality/memory changes, + Babinski's sign, stomatitis, paresthesia, unsteady gait. Diag: serum B12 levels, Schilling's test (ability to absorb B12), CBC, gastric analysis, bone marrow biopsy. Treatment: inject B12.
Aplastic Anemia
bone marrow depression of all blood cells (pancytopenia). Common with Lupus patients. Causes: insidious & severe, autoimmune, meds, medical treatments, virus, genes. ClinMan: Anemia, leukocytopenia (recurrent infections), thrombocytopenia (bleeding). Diag: CBC and bone marrow biopsy. Treatment: ID and manage underlying cause, oxygen therapy, infection control and treatment, bleeding precautions, transfusions, marrow transplant.
Hemolytic Anemia
excessive erythrocyte destruction. Causes: unknown/idiopathic, autoimmune, genes, infections, transfusion rxn, blood incompatibility in newborn/neonate. Types: Sickle Cell, Thalassemia, Erythroblastosis fetalis.
Sickle Cell Anemia
Hemoglobin S causes RBCs to be sickle shaped, carry less oxygen and clog vessels causing hypoxia and tissue ischemia. Sickle cell Trait: heterozygous/ <½ of RBCs are sickled. Sickle cell Disease: homozygous, most sever, almost all RBCs are sickled. ClinMan: abt 4 mo of age. Abdominal and bone pain, delayed growth, dyspnea, fatigue, fever, jaundice, pallor, tachycardia, skin ulcers, angina (heart pain), excessive thirst, freq urination, priapism, and vision impairment. Diag: hemoglobin electrophoresis, CBC, blirubin test. Treatment: Palliative/give IV pain meds during "crisis", avoid triggers like dehydration, stress, high alt, fever.
Thalassemia
AutoDom genetic inheritance. Abnormal hemoglobin from lack of one of two proteins that make up hemoglobin (alpha and beta globin). Effects heart and liver and is most common in Mediterraneans. ClinMan: abortion, delayed growth and dev, fatigue, dyspnea, heart failure, hepatomegaly, splenomegaly, bone deformities, jaundice. Diag: CBC (low MCV/MCHC) and Fe levels. Treatment: transfusions, chelation therapy and splenectomy.
Polycythemia
abnormally high erythrocytes increasing blood volume and viscosity, usually in men. Rare and considered a neoplastic disease leading to tissue ischemia and necrosis. Complications: thrombosis, hypertension, heart failure, hemorrhage, splenomegaly, hepatomegaly, and acute myeloblastic leukemia. ClinMan: cyanotic or plethoric skin, high BP, tachycardia, dyspnea, headaches, visual abnormalities. Diag: CBC, marrow biopsy, uric acid levels. Treatment: chemo, radiation, phlebotomy and managing clotting disorders.
Platelets
normal range from 150,000 to 350,000 mm3. Disorders include issues w/ quantity and quality of platelets.
Thrombocytosis
increased platelet levels
Thrombocytopenia
decreased platelet levels
Hemophilia A
X-linked recessive bleeding disorder. Deficiency or abnormal clotting factor 8. Varies in severity. ClinMan: bleeding or indications of bleeding (bruising, petechia). Diag: clotting studies and serum factor 8 levels. Treatment: clotting factor transfusions, recombinant clotting factors, desmopressin (DDAVP), and bleed precautions.
Von Willebrand's Disease
most common hereditary bleeding disorder. Decreased platelet adhesion and aggregation. ClinMan: bleeding or indications of bleeding (bruising, petechia). Diag: bleeding studies and factor 8 levels. Treatment: mild cases usually don't require. Cryoprecipitate infusions, Desmopressin (DDAVP), bleed precautions, measures to control bleeding.
Von Willebrand's Disease types
Type 1: most common and mildest. AutoDom, reduced Von Willebrand's factor levels. Can cause signif bleeding and trauma or surgery. Type 2: either AutoDom or AutoRecessive. Five subtypes. Von Willebrand's factor building blocks are smaller than usual or break down easily. Type 3: AutoRecessive. No measurable VW factor or factor 8. Causes sever bleeding problems. Acquired type: occurs with Wilms' tumor, congenital disease, systemic lupus erythematosus and hypothyroidism.
Disseminated Intravascular Coagulation / DIC
Complication of multiple conditions and inappropriate immune response. Widespread coagulation followed by massive bleeding bc of depletion of clotting factors. ClinMan: tissue ischemia and bleeding. Complications: shock and multisystem organ failure. Diag: CBC and bleeding studies. Treatment: replace clotting components and prevent activation of clotting mechanism.
Idiopathic Thrombocytopenia Purpora / ITP
lack of coagulation due to destruction of platelets. Acute form: more common in children, sudden onset, self-limiting. Chronic form: more common age 20-50 and in women. Causes: idiopathic, autoimmune diseases, immunizations w/ a live vaccine, immunodeficiency disorders, viral infections. ClinMan: bleeding or indictions of bleeding. Diag: CBC (platelet count <20,000) and bleeding studies. Treatment: Acute ITP: glucocorticoid steroids for anti-inflammatory response, immunoglobulins, plasmapheresis, and platelet pheresis.
Thrombotic Thrombocytopenic Purpora / TTP
Deficiency of enzyme necessary for cleaving VW factor leading to hypercoagulation which depletes platelet levels. Causes: idiopathic, heredity, bone marrow transplants, cancer, meds, pregnancy, and HIV. ClinMan: purpora, changes in consciousness, confusion, fatigue, fever, headache, tachycardia, pallor, dyspnea on exertion, speech changes, weakness, jaundice, thromboses, thrombocytopenia, and bleeding. Diag: CBC, blood smears, lactate dehydrogenase levels. Treatment: plasmapheresis, splenectomy, and glucocorticoid steroids.
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