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Hematological Problems
Diseases and Disorders of the Hematological System
47
Nursing
Not Applicable
09/18/2010

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Term
Clinical Manifestations of Mild Anemia
Definition
Palpitations & exertional dsypnea
Term
Clinical Manifestations of Moderate Anemia
Definition
"Bounding pulse," dyspnea, "roaring in the ears" & fatigue
Term
Clinical Manifestations of Severe Anemia
Definition
Pallor,jaundice, pruritis, retinal hemorrhage, blurred vision, GLOSSITIS, tachycardia, increased pulse pressure, systolic murmurs, angina, HF, MI, tachypnea, orthopnea, dyspnea at rest, HA, vertigo, irritability, depression, impaired thought processes, anorexia, hepatomegaly, splenomegaly, difficulty swallowing, sore mouth, bone pain, sensitivity to cold, weight loss & lethargy
Term
Normocytic, Normochromic Types of Anemia
Definition
Acute blood loss, hemolysis, chronic kidney disease, chronic disease, cancers, sideroblastic anemia, refractory anemia, endocrine dysfunction, aplastic anemia, sickle cell disease & pregnancy
Term
Macrocytic, Normochromic Types of Anemia
Definition
Cobalmin (B12) deficiency, folic acid deficiency, liver disease (including effects of alcohol abuse) & S/P splenectomy
Term
Microcytic, Hypochromic Types of Anemia
Definition
Iron-Deficiency Anemia, Thalassemia & lead poisoning
Term
Iron Deficiency Anemia
Definition
Microcytic, hypochromic anemia caused by inadequate supply of Iron needed to synthesize hemoglobin. Characterized by pallor, fatigue and weakness. Those mose susceptible are very young, those on poor diets & women in productive years. Glossitis & cheilitis are common findings.
Term
Thalassemia
Definition
Autosomal recessive genetic disorder of inadequate production of normal hemoglobin. Heterozygous and homozygous types. Life-threatening disease that causes both physical and mental retardation. Signs and symptoms are similar to general anemia, but also include splenomegaly, hepatomegaly and bone marrow hyperplasia.
Term
Pernicious Anemia
Definition
Progressive megaloblastic macrocytic anemia resulting from inadequate gastric secretion of intrinsic factor necessary for cobalmin absorption. GI symptoms (sore tongue, nausea & vomiting)& neuromuscular symptoms (weakeness, parathesias of hands & feet, impaired thought processes)most common. NM symptoms help distinguish this from Folic Acid Deficiency.
Term
Folic Acid Deficiency
Definition
One cause of megaloblastic anemia. Causes include poor nutrition, malabsorption syndromes, antiseizure drugs, alcohol abuse, anorexia and hemodialysis. Manifestations are similar to those of B-12 Deficiency w/ exception to the lack of neuromuscular symptoms. Other symptoms not noted w/ Cobalmin Deficiency include Dyspepsia and the presence of a smooth, beefy red tongue.
Term
Anemia of Chronic Disease
Definition
Symptom of chronic Inflammatory, Autoimmune or Malignant diseases. Usually anemia is mild form. Iron is diverted to macrophages and therefore storage sites. the individual causative disease plays a key role in its etiology. Increased serum Ferritin & Iron stores help distinguish from Iron-deficiency type.
Term
Aplastic Anemia
Definition
AKA Pancytopenia. Deficiency of all formed elements of the blood, representing a failure of the cell-generating capacity of the bone marrow. Fatigue, dyspnea as well as cardiovascular and cerebral response may be seen. Neutropenia makes patient susceptible to infection while thrombocytopenia makes patient susceptible to bleeding. Causes include congenital diseases as well as chemical agents, pregnancy, radiation and bacterial infection.
Term
Clinical Manifestations of Acute Blood Loss
Definition
10%- None 20%- No detectable signs at rest. Tachycardia w/ exercise and slight postural hypotension 30%- Normal supine BP & pulse at rest, postural hypotension & tachycardia w/ exercise 40%- BP, Central venous pressure & CO lower than normal at rest; rapid, thready pulse & cold, clammy skin 50%- shock and potential death
Term
Chronic Blood Loss
Definition
Sources are similar to those of Iron Deficiency, including bleeding ulcer, hemorrhoids, menstrual loss, etc. Generally considered Iron-Deficiency Anemia
Term
Hemolytic Anemia
Definition
Caused by destruction of RBCs at a rate that exceeds production. Intrinsic causes include defects in RBCs, enzyme deficiencies or membrane abnormalities. Extrinsic causes include trapped cells in liver sinuses and spleen, antibody mediated destruction, toxins and mechanical injury as with prosthetic heart valves. Jaundice is likely b/c RBC destruction increases Bilirubin levels.
Term
Sickle Cell Disease
Definition
Group of inherited autosomal recessive disorders characterized by the presence of an abnormal form of hemoglobin. Sickling occurs most commonly in response to decrease O2 levels. Often fatal by middle age from renal or pulmonary failure. Chronic pain, fatigue, mucus membrane pallor, jaundice, gallstones, fever, nausea & vomiting are all common manifestations during sickling crises. Infection is a major cause of morbidity b/c spleen is unable to phagocytize foreign substances.
Term
Acquired Hemolytic Anemia
Definition
Physical factors, immune reactions & infectious agents/toxins are extrinsic causes of hemolysis. Automimmune reactions occur when patients develop antibodies against their own RBCs, this may be idiopathic or the result of autoimmune disease, such as Lupus, Leukemia, Lymphoma & the use of drugs such as Indocin, Quinine & methyldopa.
Term
How Infectious Agents Cause Hemolysis in Acquired Hemolytic Anemia
Definition
1) Invading RBC & destroying contents (parastites, malaria) 2) Releasing hemolytic substances (Clostridium perfringens) 3) Generating an antibody-antigen reaction 4) by contributing to splenomegaly as a means of increasing removal of damaged RBCs and causing hemolysis
Term
Hemochromatosis
Definition
Autosomal recessive disease characterized by increased intestinal iron absorption and, as a result, increased tissue iron deposition. Early symptoms are nonspecific and include fatigue, arthralgia, impotence, weight loss and abdominal pain. Later, excess iron accumulates in liver and leads to cirrhosis which leads to DM, "Bronzing," cardiomyopathy, arthritis & testicular atrophy.
Term
Polycythemia Vera
Definition
Chronic myeloproliferative disorder arising from a chromosomal mutation of a single stem cell. Effects RBCs, WBCs and platelets. Median diagnosis is 60+ and slightly predominantly male. Increased blood viscosity & volume and congestion of organs and tissues with blood. Hypercoagulopathies, splenomegaly and hepatomegaly are common. Dizziness, headache and itching made worse with a hot bath are common symptoms.
Term
Ranges of Abnormal Hemoglobin in Anemic Patients
Definition
Mild- 10-14g/dl Moderate- 6-10g/dl Severe- <6g/dl
Term
Secondary Polycythemia
Definition
Can be hypoxia-driven: hypoxia stimulates erythopoiten production in kidneys leading to increased erythropoises. Triggered by any disease or stimulus that leads to defective O2 transport or tissue hypoxia. Or Hypoxia independent: produced by a malignant or benign tumor.
Term
Immune Thrombocytopenic Purpura
Definition
Thrombocytopenia in which the platelets are covered in antibodies which leads to splenomegaly.
Term
Thrombotic Thrombocytopenia Purpura
Definition
Drug-induced with oral contraceptive or due to pregnancy. Medical emergency because blood is bleeding and clotting simultaneously.
Term
Heparin-Induced Thrombocytopenia
Definition
5-20% of people who take Heparin develop this type of thrombocytopenia. Leads to stroke. Patients with diagnosis should never be given Heparin.
Term
Thrombocytopenia
Definition
Platelet count <100,000/mcl. <20,000 is life-threatening. Many causes, including excessive Tylenol use, Dilantin and Lupus. Bleeding most common manifestation. Watch for neuro symptoms because of risk for Intracranial bleeding.
Term
Hemophilia Type A
Definition
Classic type cause by factor VIII deficiency. Most common type; makes up 80% of all cases. Hemophilia is passed on by mother to son, and carrier gene is passed on to daughters.
Term
Hemophilia Type B
Definition
Christmas Disease. Caused by factor IX deficiency.
Term
vonWildebrand Deficiency
Definition
Deficiency in vonWildebrand Coagulation Protein.
Term
Clinical Manifestations of Hemophilias & vonWildebrand Disease
Definition
1) slow, persistent, prolonged bleeding w/ minor trauma 2) Delayed bleeding, after minor injuries (hours to days) 3) Uncontrollable hemorrhage after dental extractions of gingiva irritations 4) Epistaxix, especially after a blow to the face 5) GI bleeding from ulcers or gastritis 6) Hematuria 7) Ecchymoses & subcut hematomas 8) Pain, parathesias and paralysis because hematomas compress nerves
Term
Neutropenia
Definition
Neutrophil count <1000/mcl. Symptoms are similar to those of infection (sore throat, non-productive cough, mouth sores), also no pus with wounds. Fever is life-threatening, even if low-grade.
Term
Myelodysplastic Syndrome
Definition
Decreased peripheral blood cells; increased cellular activity in bone marrow. Progression and maturation of some cells help differentiate from Acute Myelogenous Leukemia. Infection and bleeding are common.
Term
Leukemia
Definition
Broad term given to a group of malignant diseases characterized by diffuse replacement of bone marrow w/ proliferating leukocyte precursors. Adult to child ratio is 10:1. Follows progressive course that is fatal, if untreated. Oncogenes, chemical agents, viruses, radiation and immunologic deficiencies are all associated. WBC involvement and acute or chronic nature determines type.
Term
Acute Myelogenous Leukemia
Definition
Represents 1/4 of all leukemias, but 85% of acute leukemias in adults. Onset is abrupt and dramatic. Characterized by uncontrolled proliferation of myleoblasts; granulocyte precursors. Manifests as hyperplasia of bone marrow, fatigue & weakness, HA, mouth sores, anemia, bleeding, fever, infection, sternal tenderness, gingival hyperplasia, minimal hepatosplenomegaly and lymphadenopathy.
Term
Acute Lymphotic Leukemia
Definition
Most common leukemia type in children. 5 year event-free survival rate is 80% in children and 40% in adults. Immature lymphocytes proliferate in bone marrow, most are B-cell. Fever is present in most at time of diagnosis, bleeding, progressive weakness, fatigue and bone and/or joint pain may be evident. There is a tendency towards bleeding. CNS symptoms are especially common and meningitis d/t Arachnoid infiltration occurs in many.
Term
Chronic Myelogenous Leukemia
Definition
Excessive development of mature neoplastic granulocytes in the bone marrow that move into the blood stream in large numbers and infiltrate the liver and spleen. Cells contain dystinctive abnormality called the Philadelphia chromosome that results from translocation of genetic material. Chronic stable phase may last several years before progressing to acute, agressive phase referred to as the blastic phase. Chronic always leads to acute and once in acute, patient may only live for a few months. Excessive sweating, sternal tenderness and massive splenomegaly are key symptoms.
Term
Chronic Lymphotic Leukemia
Definition
Most common leukemia in adults (usually older adults). Characterized by production and accumulation of functionally inactive, long-living, small, mature-appearing lymphocytes, usually B-cell. Infiltration of bone marrow, spleen and liver occur. Lymphadenopathy is present throughout the body which can lead to Richter's syndrome (transformation to nonHodgkins Lymphoma) in 5-8% of cases. Pain and paralysis from pressure of nodes on nerves is a common symptom.
Term
Hairy Cell Leukemia
Definition
2% of adult leukemias. Usually seen in males 40+. Chronic disease of lymphoproliferation (B-cell) that infiltrates the bone marrow and spleen. Cells have a hairy appearance. Splenomegaly is common. Symptoms include those of splenomegaly, pancytopenia, infection and vasculitis. Many asymptomatic patients are diagnosed through routine CBC. Usually therapy is not indicated for 10 or so years.
Term
Unclassified Leukemia
Definition
Subtype of Leukemia cannot be identified. Malignant leukocytes may be lymphoid, myeloid or of mixed quality. These patients do not respond well to treatment so prognosis is poor.
Term
Lymphoma
Definition
Malignant neoplasms originating in the bone marrow and lymphatic structures resulting in the proliferation of lymphocytes. 5th most common type of cancer in US.
Term
Hodgkin's Disease
Definition
Makes up 12% of all lymphomas. Caused by the proliferation of abnormal, giant, multi-nucleated cells called Reed-sternberg cells, which are located in the lymph nodes. Occurs most frequently in 15-35 and 50+. In adults, 2x more prevalent in men. Epstein Barr infection, genetic predisposition and exposure to occupational toxins play a role in development. Increased incidence in HIV patients. In 2/3 of patients, cervical nodes are first to be infiltrated and generally nodes effected are localized.90% survival rate if found early, but can lead to problems with sterility. Fever, night sweats and weight loss together correlate with the worst prognosis.
Term
Non-Hodgkin's Lymphoma
Definition
Heterogenous group of malignant neoplasms involving lymphoid tissue, primarly B (90%) or T cells. Affects all ages. Classified according to cellular or lymph node characteristics. Most common occuring hematologic cancer and fifth leading cause of cancer death. Low-grade (indolent), Intermediate (agressive) and High-Grade (very agressive) types. EBV associated with Burkitt's, but not all EBV patients develop lymphoma. Painless lymphadenopathy and symptoms related to structures near affected lymph nodes most prevalent symptoms.
Term
Multiple Myeloma
Definition
Condition in which malignant neoplastic plasma cells infiltrate the bone marrow and destroy bone. Patient lives for approximately 2 years after diagnosis if untreated. 2x more common in men, usually 40+ with average age being 65. Occurs more commonly in African Americans. Exposure to toxins and radiation plays a role. Malignant plasma cells infiltrate bone marrow and produce excessive immunoglobulins (usually IgG). In some, Bence-Jones protein present in urine. Skeletal pain is likely and it intensifies or is triggered with movement.
Term
Acute Hemolytic Reaction
Definition
Infusion of ABO incompatible whole blood, RBC or component containing at least 10mL of RBC. Chills fever, lower back pain, flushing, tachycardia, dyspnea, tachypnea, hypotension, acute jaundice, shock, cardiac arrest and death are all manifestations.
Term
Febrile Non-hemolytic Reaction
Definition
Most common reaction. Sensitization to donor WBCs, platelets or plasma proteins. Sudden chills and fever (increase in temp >1 degree celsius), HA, flushing, anxiety, vomiting and muscle pain are all manifestations.
Term
Anaphylactic and Severe Allergic Reaction
Definition
Sensitivity to donor plasma proteins and/or infusion of IgA proteins to a IgA deficient patient who has developed antibodies. Anxiety, urticaria, dyspnea, wheezing, cyanosis, bronchospasm, hypotension, shock and cardiac arrest are all manifestations.
Term
Cultural and Ethnic Disparities w/ Hematological Problems
Definition
Sickle Cell- African Americans
Thalassemia- African Americans and Mediterraneans
Tay-Sachs- Eastern European Jews
Pernicious Anemia- African Americans and Scandinavians
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