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Heme Disorders 5
SOPN Heme Disorders 5
Question | Answer |
---|---|
deficiency in number of erythrocytes or in hemoglobin | ANEMIA |
Name 3 causes of anemia | -Blood loss-impaired production of erythocytes-increased destruction of erythrocytes |
skin change related to decreased amount of HgB and blood flow to the skin | Pallor |
skin change related to hemolysis (destruction) of RBC's causing increased concentration of bilirubin | Jaundice |
skin change related to increased bilirubin causing increased bile salt concentration on the skin | pruritis |
jaundice of sclera of the eyes | icterus |
cardiopulmonary manifestations of anemia | SOB - increased heart rate - increased pulse - tachypnea (fast breathing) |
anemias caused by decreased erythrocyte production | hypoprolific anemias |
inadequate dietary intake of iron or malabsorption of iron (absorption occurs in the duodenum) | Iron deficiency anemia |
inflammation of the tongue | glossitis |
inflammation of the lips | chielitis |
TIBC | Total Iron Binding Capacity |
Diagnosis of iron deficiency also includes determination of root cause of blood loss - name a nasty test | Guiac test for occult blood |
With what would you take an oral iron agent to increase absorption | Orange Juice |
What drugs would you take when taking iron | STOOL SOFTENERS - colace or docusate sodium |
Foods high in iron......name em' Popeye! | spinich, liver, muscle meat, whole grains, eggs |
Another name for Thalassemia - this can cause retardation and affect physical or mental growth | Cooleys Anemia |
genetic disease involves inadequate production of normal HgB due to an absent or decreased globulin protein THEREFORE abnormal HgB synthesis | Thalassemia |
type of thalassemia that has "2" genes and is life threatening | Thalassemia Major |
type of thalassemia has "1" gene and is milder | Thalassemia Minor |
due to the presence of TARGET CELLS in the blood. Thin fragile RBC's also considered a what???? | Inherited Hemolytic Anemia |
chelating agents to reduce high blood iron levels which results from multiple transfusions | hemochromatosis |
anemia caused by impaired DNA systhesis causeing LARGE (macrocytic) DEFECTIVE RBC's | megaloblastic anemia |
a deficiency in what would cause a decreased or absent production of the INTRINSIC FACTOR, normally excreted by pariental cells of the GI tract | cobalamin (vitamin B-12) |
What is required for cobalamin (vitamin B-12) absorption | INTRINSIC FACTOR |
Disease where gastric mucosa does not secrete intrinsic factor due to antibodies acting against it | Pernicious anemia |
Name that procedure! Patient receives radioactive cobalamin, a 24 hr urine collection is preformed to determine the amount of cobalamin excreted | SHILLING TEST FOR pernicious anemia |
What would you need if we found the intrinsic factor lacking in you | vit B12 injections |
What is needed for DNA synthesis leading to RBC formation and maturity | FOLIC ACID |
decreased RBC's - decreased WBC's - AND decreased platelets generally a decreased level of all types of blood cells within the marrow and body | PANCYTOPENIA |
treatment for pancytopenia would be what? Hmmm | Immunosuppression and/or stem cell transplant |
If you have no platelets what side effect can you expect? | Longer bleeding time |
Sudden hemorrhage causing reduction in TOTAL BLOOD VOLUME (hypovolemic shock) | Anemia from ACUTE blood loss |
What am I dying of if I have: tachycardia, thready pulse, hypotension, decreased cardiac output, increased respiratory rate, and eventually shock??? Some party trick! | anemia from acute blood loss |
How would you save my sorry butt from anemia due to blood loss? | WHOLE BLOOD for acute loss Packed RBC's for slow bleed |
Name that condition! I am caused by increased erythrocyte destruction | Hemolytic anemia |
Name that condition! I am caused from RBC defects from abnormal HgB as seen in Sickle Cell Disease, or G-6-PD (glucose 6 phosphate dehydrogenase deficiency, and RBC membrane abnormalities!!! BLAME YOUR MOTHER! Hereiditary. It's all ***IN*** the family | intrinsic hemolytic anemia |
Name that condition. The patients RBC's are normal, BUT hemolysis (destruction) is caused by ***OUTSIDE*** forces. IE: | EXTRINSIC HEMOLYTIC ANEMIA |
Hemolytic anemias generally result in what Michael Jackson-esque condition? | Jaundice and yellow eyes....THRILLING! |
Name that disease! Genetic disorder characterized by an abnormal form of HgB in the erythrocyte called HEMOGLOBIN S (HB S) | Sickle Cell Disease (fatal) |
What causes the RBC to stiffen and elongate into a "sickle" shape in response the low O2 levels or increased O2 demand | Hemoglobin S (HG S) |
What condition is triggered by LOW O2 tension in the blood caused by decreased oxygenation of the RBC's ***due to viral or bacterial infections, high altitude, or dehydration*** | Sickling episodes |
priapism | prolonged erection |
severe PAINFUL exacerbation of condition with small vessels and capillary hypoxia occuring, thrombus formation resulting in hypoxia, ischemia, and eventual nacrosis, shock, organ failure | Sickle Cell Crisis |
What test would detect HgB S (hemoglobin S) the destructive factor that results in a Sickle cell anemia diagnosis | ELECTROPHORESIS |
PCA | patient controlled anthesthia |
The use of what would ***increase the production of fetal HgB*** which may be used to decrease hemolysis, SICKLING, with and increase in HgB! Treatment for sickle cell anemia | HYGROXYUREA (Hydrea) |
What would cause the PHYSICAL destruction of RBC's either from the trauma of hemodialysis, prothetic heart valves, bypass burns, autoimmune reactions ie lupus, leukemia, or immune reactions ie med like penicillin or infectious agents VENOM | ACQUIRED HEMOLYTIC ANEMIA |
condition caused by genetic disease. Due to increased intestinal absorption of iron = increased iron deposits in the tissues. Seen in thalassemia or multiple blood transfusions | HEMOCHROMATOSIS |
In a diagnosis of Hematochromatosis you would see and increased level of what | serum ferritin |
Name that condition! production and presence of increased RBC's in blood causing impaired blood circulation related to INCREASED BLOOD VISCOSITY. THICK BLOOD | polycythemia |
form of polycythemia GENETIC includes increased WBC's and platelets as well as RBC's | polycythemia vera |
form of polycythemia caused by an increased need for O2 physiologically OR from an increased production of erythropoiten from malignant or benign tumor, NON-GENETIC | secondary polycythemia |
causes increased blood viscosity, volume, and congestion of organs and tissues with blood causing HYPERCOAGUABILITY (clotting) .....traffic jam of cells leads to 20 car pile-up...nice. Blood pressure's up now! | Polycythemia |
What is the most common (yet acute) complication of polycythemia? | CVA from thrombosis, vessel rupture can cause bleeding, increased uric acid (due to cell breakdown....could lead to gout) IF YOU LIVE! Cause the thrombosis could kill ya! |
name the anti-gout medicine | allinpurinol |
Treatment if someone is hypoxic | Oxygenation |
treatment if someone who needs to reduce bloods viscosity | Hydration |
Medicine to suppress bone marrow activity | hydroxyurea |
condition due to decreased platelets (thrombocytees) below 150,000/ul. Manifests inself by ***BLEEDING*** | thrombocytopenia |
define the acronym *ITP* | immune thrombocytopenia |
an abnormal acquired AUTOIMMUNE syndrome of the abnormal destruction of the circulating platelets | PURPURA (pureed, chopped, minced, diced, it does it all!) |
Patients with ITP bless them when they sneeze then teach them to what? | WASH and for the love of god DAB DON'T BLOW!!!! You'll bleed to death and need that "Bless you," BIG TIME! |
Name the major complication of ITP. | ahhhh HEMORRHAGEDAB DON'T BLOW! |
Best lab test for immune thrombocytopenia? | ITP ANTIGEN |
What condition would require a platelet transfusion only if count BELOW 10,000 - 30,000/ul (if too many may produce more antibodies) NO ASPIRIN! EVER! NEVER EVER! | Immune Thrombocytopenia |
Condition characterized by HEMOLYTIC ANEMIA (kills RBC's) and THROMBOCYTOPENIA with clinical mainifestations of neurological and renal abnormalities. ALMOST ALWAYS ASSOC. WITH HEMOLYTIC UREMIC SYNDROME (HUS) | THROMBOTIC THROMBOCYTOPENIC PURPURA (TTP) |
Condition that causes enhanced agglutination of platelets causing microthrombi, bleeding as well. | Thrombitic thrombicytopenic purpura (TTP) |
What medicine would you use to treat herpes? NO VAL DOES NOT HAVE HERPES!!! | Valtrex |
Increased use of heparin can cause this condition that causes heparin to bind to platelets causing clots and destroying other platelets | Heparin induced thrombocytopenia and thrombosis ***HITT*** |
What is the antidote to heparin | protamine sulfate |
genetic disorder caused by defective or deficient coagulation factors | Hemophilia |
classic hemophilia characterized by a DEFICIENCY OF FACTOR VIII | hemophilia A |
CHRISTMAS DISEASE deficiency of FACTOR IX | hemophilia B |
deficiency of the *VON WILLIBRAND FACTOR* which is involved in carrying FACTOR VIII = usually not life threatening | von Willibrand's Disease |
bleeding into the joints will lead to joint injury / deformity | hemarthrosis |
How would you treat a patient with hemophilia | Blood transfusion if needed, NO ASPIRIN OR ANTI COAGULANTS, ice and rest for hemarthrosis as there will be pain, redness, and swelling |
condition characterized by serious bleeding and thrombotic (clotting). Always caused by an underlying disease or condition ie shock, septicemia, infections, obstetiric conditions, malignancies, severe trauma, snakebite, anorexia, burn, and MORE!!! | Disseminated Intravascular Coagulation (DIC) |
What lab test would you perform to determine DIC? Test looks for a specific breakdown of fibrin, prolonged PT, PTT, aPTT, decreased platelets and increased fibrin split products | D-Dimer Assay |
what is FFP | Fresh Frozen Plasma |
what is a decrease in the number of neutrophils resulting in a compromised immune system (ISOLATE THIS PATIENT) | neutropenia |
What is needed to phagocytize pathogenic microbes. If reduced would lead to an increased risk for infections (ISOLATE THIS PATIENT!) | neutrophils |
what drugs would you use to treat autoimmune diseases | immunosuppressants |
Many signs and symptoms of infection and inflammation are suppressed because normal phagocytosis are impaired, therefore even a LOW GRADE FEVER is significant and requires immediate attention due to patients inability to fight could lead to sepsis & shock | neutropenia |
how would you diagnose neutropenia | WBC with differential |
What medicine would you give to fight neutropenia | Neupogen |
filgrastim - stimulates production of neutrophils | NEUPOGEN |
a group of related hemotologic disorders characterized by a change in the quantitity and quality of BONE MARROW CELLS | MYELODYSPLASTIC SYNDROME (MDS) |
abnormal stem cells in bone marrow produce abnormal blood cells which can develop into neoplasms & eventually cause anemia, thrombocytopenia, and neutropenia | MYELODYSPLASTIC SYNDROME |
erythropoiten | epogen |
filgrastim | Neupogen |
what medicine will help restore normal growth control | azacitidine = Vidaza |
general term used to describe a group of malignant disorders affecting leukocytes (WBCs), it results in an accumulation of dysfunctional cells and if left untreated = fatal | Leukemia |
Which leukemia tends to involve more MATURE WBC'S and disease onset is more gradual | CHRONIC LEUKEMIA |
Uncontrolled proliferation of myeolblasts, these crowd and replace the normal cells in the bone marrow and eventually into other tissues and organs as well | ACUTE MYELOGENOUS LEUKEMIA (AML) |
Increase in mostly IMMATURE B LYMPHOCYTES in the bone marrow | ACUTE LYMPHOCYTIC LEUKEMIA (ALL) |
Excessive development of MATURE NEOPLASTIC GRANULOCYTES in the bone marrow, which move into the peripheral circulation in massive numbers, ulimately infiltrating the liver and spleen. *CONTAIN PHILEDELPHIA CHROMOSOME* | Chronic Myeolgenous leukemia |
excessive production and accumulation of mostly inactive SMALL "B" lymphocytes which infiltrate bone marrow, spleen, and liver | CHRONIC LYMPHOCYTIC LEUKEMIA |
What is the treatment for the leukemias. Helps to destroy leukemic cells in the tissues, bone marrow, and peripheral blood, Cause severe bone marrow depression causing the patient to become ill due to destruction of normal as well as cancer cells | chemotherapy |
Proliferation of *GIANT* multinucleated cells called REED_STEINBERG cells in the lymph nodes, and without treatment would spread to other organs especially lungs, spleen, & liver | Hodgkin's Lymphoma |
Name one infection know to cause Hodgkin's Lymphoma | Epstein-Barr virus |
How would you determine a diagnosis of Hodgkin's Lymphoma | Lymph node biopsy to detect presence of REED-STEINBERG CELL |
A large grouping of malignant cells of B or T cell origins in all stages of development. most common in hemotologic cancer. Symptom painless lymph node enlargement | Non-hodgkin's lymphoma |
malignant plasma cells which normally help protect the body by producing immunoglobins (antibodies) infiltrate bone marrow thus destroying the bone. Excessive amt of abnormal immunoglobins produced called M-protein | Multiple Myeloma |
How would you diagnose multiple myeloma | presence of M-Protein found in blood and urine |
In multiple myeloma what can be detected in blood and urine that confirms diagnosis | BENCE-JONES PROTEIN |
What simple intervention will help prevent hypercalcemia caused from bone destruction | FLUID |