Blood.txt

• Autosomal recessive inherited disease
• Two forms: minor and major
• Two types: alpha and beta

• defective gene inherited from only one parent
• affected person becomes a carrier with no symptoms

• defect gene inherited from both parents
• abnormal form of hemoglobin results in excessive destruction of RBCs and anemia

• occurs when gene(s) related to alpha globin proteins are missing or mutated
• ~14% African Americans are silent carriers

• occurs when gene defects affect production of beta globin protein
• ~10-15% Mediterranean and SE Asian descent have

• found in early childhood - present with symptoms of severe anemia
• physical exam shows enlarged spleen
• blood sample shows small, abnormally shaped RBCs; anemia
• Electrophoresis reveals abnormal forms of hemoglobin

• blood transfusion
• folate supplements

• abnormal increase in # of blood cells (primarily RBC) produced by bone marrow
• cause unknown, may be linked to abnormalities of chromosomes 8, 9, 13 & 20
• two types; relative and absolute
• most commonly seen in men 40-65

• increase in RBC mass due to loss of plasma volume w/out corresponding decrease in RBC
• due to dehydration or excessive use of diuretics

increase in mass of RBC, WBC and platelets in the absence of recognizable physical stimulus

• neurological; dizziness, headaches, visual disturbances, itching/pain in fingers and toes
• hypertension
• tromboembolism

all aimed at decreasing thickness of blood - phlebotomy, chemotherapy, pain meds, histamine 1 receptor antagonists (H1)

the arrest of bleeding from an injured blood vessl requiring the combined activity of vascular responses, platelets and clotting factors

• vasoconstriction
• compression of wounded blood vessl

• platelets adhere to damaged tissue to form aggregates
• mobilize clotting factors

• interact to produce thrombin which fibrinogen to fibrin for strengthening the clot
• (I don't think that "fibrinogen" is a verb. That sentence makes no sense)
• something here is formed in the liver
• Vitamin K is required to synthesize prothrombin and thrombin

• insufficient platelet levels associated with abnormal bleeding
• results from one of three conditions; reduced platelet production, platelet trapping in spleen, increased platelet destruction

• impaired platelet production in bone marrow
• causes include diseases (leukemia, anemia), viral infections (HIV) or toxic chemicals (chemo drugs, heavy alcohol consumption)

enlarged spleen may harbor too many platelets leading to decreased platelet numbers in circulation

conditions; pregnancy, idiopathic thrombocytopenia purpura (ITP - immune system identifies platelets as a threat and forms antibodies), autoimmune (lupus, RA), bacteremia (bacteria in the blood), thromboti thrombocytopenic purpura (TTP - small blood clots suddenly form throughout body destroying large numbers of platelets), hemolytic uremic syndrome (associated with E. coli; causes sharp drop in platelets, destroys RBC, impairs kidney functions), certain medications (trigger immune system to destroy platelets)

• microscopic exam of blood
• bone marrow biopsy
• physical exam

• determine, then treat underlying cause (ya think?)
• preventative measures
• platelet transfusions

• coagulation is a complex process involving different plasma proteins or factors to control bleeding
• involves forming a blood clot (thrombus) to prevent further blood loss from damanged tissues, blood vessels and/or organs
• coagulation factors are synthesized by the liver and require Vitamin K for normal activity

• Factor II (prothrombin)
• Factor VII
• Factor IX
• Factor X

inherited X-linked recessive diseases most often affecting males

• inherited; caused by deficiency in blood clotting factor VIII
• mild to moderate symptoms - bleeding does not occur unless there is localized trauma (dental procedure, surgery)
• severe - spontaneous bleeding may occur several times per month during childhood (GI bleeding, bleeding into joints); chronic bleeding and inflammation causes joint fibrosis, intracranial hemorrhage can lead to death

• replace missing clotting factor VIII
• use of synthetic hormone desmopressin in mild cases
• blood transfusion
• genetically engineered clotting factor VIII

• caused by deficiency in two blood clotting proteins - von Willebrand factor and blood clotting factor VIII
• most common hereditary bleeding disorder, most cases are mild
• usually diagnosed in adulthood
• affects both men and women
• bleeding may occur after surgery or tooth extraction
• asprin and other NSAIDs may worsen condition
• bleeding may decrease during pregnancy

• prolonged bleeding time
• platelet aggregation test (count may be normal)
• ristocetin cofactor test (von Willebrand factor level is reduced)
• symptoms may develop suddenly (excessive or uncontrollable bleeding following surgery or childbirth at site of intravenous injection) or slowly (clots in veins are more common than bleeding, as in people with cancer

• lifestyle changes to minimize trauma
• synthetic hormone desamino-8-arginine vasopressin
• antihemophilic factor alphanate
• blood plasma or clotting factor VIII

• inherited; condition where small blood clots develop throughout the bloodstream, blocking small vessels
• increased clotting depleted platelets and clotting factors needed to control bleeding, causing excessive bleeding
• occurs in different disorders; sepsis, vascular endothelial injury, shock, obstetrical complications, neoplasms

• involves release of thrombin into systemic circulation
• systemic coagulation occurs
• suppression of normal physiological anticoagulation processes
• extensive clotting produces diffuse tissue damage, organ damage, consumptive coagulopathy (extensive bleeding due to depleted platelets and clotting factors)

blood tests (decreased platelet numbers, prolonged clotting time)

• ID and treat underlying cause (obstetrical cause, infection, cancer)
• supportive medications (platelet transfusions, coagulation inhibitors, intravenous anticoagulants)

• WBC - abnormally low number of neutrophils in blood
• increases risk for bacterial and fungal infections
• complication of medication that suppress cellular functions within bone marrow such as chemo, radiation therapy and immunosuppressant therapy

• bacterial infections
• viral infections
• allergic disorders
• medications
• autoimmune disorders
• spleen disorders
• cancer
• toxins

• colony-stimulating growth factors to stimulate production of WBC
• corticosteroids may help if the neutropenia is caused by an autoimmune reaction
• antithymocyte globulin to suppress activity of the immune system in disorders such as aplastic anemia
• removal of enlarged spleen may cure the neutropenia resulting from hypersplenism

• WBC - characterized by peripheral blood eosinophilia causing organ system dysfunction
• directly related to eosinophilia in absence of parasitic, allergic or other causes of eosinophilia
• occurs predominantly in men 20-50
• organ dysfunction can develop - can be any organ but usually heart, lungs, spleen, skin, nervous system
• cardiac involvement often causes morbidity and mortality

• recognized in 1989 in NM in 3 patients who had an illness with a unique array of symptoms such as peripheral blood eosinophilia and myalgia
• multi-organ disease characterized by elevated circulating eosinophils
• associated with ingestion of OTC supplements containing L-tryptophan
• symptoms may rand from self-limited myalgias and fatigue to potentially fatal illness characterized by skin changes, nervous system abnormalities and pulmonary arterial hypertension