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110 Cards in this Set
- Front
- Back
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Development of hematapoiesis
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- Begins by the 3rd week gestation
- In the second month of gestation, the primary site is the liver - By the 5th to 6th month gestation, the process shifts to bone marrow - During infancy, all marrow is actively hematapoietic. - In childhood, the process moves to the central bones - By age 18, the production is limited to the end of the long bones - In adulthood, the marrow is replaces with fatty tissue and blood cell production is limited to sternum, ribs and iliac crest |
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Hematapoiesis in infancy
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All marrow is actively hematapoietic
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Hematapoiesis in childhood
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The process moves to the central bones
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Hematapoiesis by age 18
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The production is limited to the end of the long bones
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Hematapoiesis in adulthood
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The marrow is replaced with fatty tissue and blood cell production is limited to sternum, ribs and the ilia crest
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What are diagnostic hematological studies?
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They are an essential component of any patient evaluation, peds or adult
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Disease of the blood can be one of two types. What are they?
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Congenital
Acquired |
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Essential functions of blood include:
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- Transfer of gases
- Hemostasis - Phagocytosis - Provision of cellular and humoral components to fight infection |
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Erythrocytes
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- The production of erythrocytes is regulated by the hormone erythropoieten
- In response to a decrease of PaO2 in arterial blood, the kidneys release erythropoieten to stimulate RBC production - While the kidney produces most of the erythropoietin, the liver and other tissues also produce some. - In fetal life, the liver produced all of the erythropoietin- as the final kidney had not yet completed development |
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What hormone regulates erythrocyte production?
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Erythropoietin
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What organ releases erythropoietin and under what conditions?
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The kidneys when arterial PaO2 is decreased
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What kind of patients have difficulty with erythrocyte production?
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Renal patients
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Anatomy of Blood
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- Blood is composed of cellular components with a specialized function, and a fluid component called PLASMA
- The cellular components of blood consist of RBCs (erytrocytes), WBCs (leudocytes), and platelets (thrombocytes) |
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Factors necessary for erythropoiesis
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- Erythopoietin
- Iron - Vitamin B12 - Folic Acid (folate) - Ascorbic acid (Vitamin C) - Pyridoxine (Vitamin B6) - Amino acids |
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Life span of circulating blood cells
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- Erythrocyte: 120 days
- Platelets: 7-8 days - Neutrophil: 7-12 days - Eosinophils: 3-8 hours - Basophils/Mast Cells: 7-12 hours Monocyte/macrophages: 3 days |
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Abuse of Erythropoietin
What is Blood doping |
- Athletes used erythopoietin to enhance their athletic prowess
- Increases the concentration of RBCs and with it an increased chance for spontaneous coagulation - Several athletes have died as a result - It is not allowed by international sports associations |
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Hemoglobin
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- The oxygen carrying protein in the BRC
- At birth approximately 70% of hgb is made of fetal hemoglobin - By 12 months of age, 95% of the normal adult hemoglobin - The levelof hgb in a newborn ranges from 15-22 g/dl and drops to lowest point at 3-6 months - This is a physiological anemia caused by shortened survival of fetal RBC's and the rapid expansion of blood volume during this period. |
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Corpuscular Indices:
These labs indicate that there's an anemia going on with the patient. |
- MCV: mean corpuscular volume
- MCH: hgb/# RBCs - Increased Macrocytic Cells: possible anemia - Decreased Microcytic Cells: possible Iron deficiency |
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Maturation of Erythrocytes
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* RBCs mature through stages
* A mature RBC lasts 120 days before it is destroyed through phagocytosis in the: - Spleen: if not in good health, the spleen filters them out - Liver - Bone marrow |
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Antigenic properties of RBC's
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- Classified according to presence of antigens in the cell membrane
- The most common A, B, and Rh - A person inherits either A or B antigen, both antigens or neither which is type O - Of the 6 types of Rh factors, the most common is D, which accounts for Rh+ designation |
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What blood type is the universal donor?
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Type O-
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Percentages of people who are Rh+ in the US
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- 85% Caucasians
- 95% AA |
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Normal Hct levels
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- males: 42-50%
- females: 39-48% - Males have more b/c women lose RBCs through menstration every month - Any black person will also usually have a lower Hct |
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Special Circumstances
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* Emergency release
- when delaying transfusion poses risk of death - insufficient time to perform type screen and crossmatch - requires MD signature * Conditional Release - blood may be crossmatch compatible, however, blood band tensting is incomplete or cannot completely resolve antibody testing - requires MD signature |
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Granular Leukocytes
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- In children, granulocytes make up 30-60% WBCs
- these cells nature in BONE MARROW - Neutophils: phagocytosis - Basophils: inflammation and allergies - Eosinophils: allergic reactions |
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Never Let Mom Eat Beans
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60% Neutraphils
30% Leukocytes 6% Monocytes 3% Eosinophils 1% Basophils |
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Platelets
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- Smallest cellular components in blood
- Essential for hemostasis and clot formation - Normal platelet count = 150,000 to 300,000 |
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Anti-platelet Drugs
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* ASA
- inactivates COX-1, decreasing TSA2 (a platelet agonist) - Prevent stroke, MI, CAD, peripheral arterial occlusion * Thinopridine Derivatives - blocks ADP * GPIIb/IIIa inhibitors |
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Agranulocytes
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- T-cells part of cell mediated immune response
- B-cells precursors of humoral immune response which release antibodies - Monocytes function as macrophages and act as 1st line of defense against microbe invasaion |
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Anemias
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* Anemia is a decrease in the number of red blood cells (RBCs) as measured
- per mm3 on a slide or - by volume of packed RBC per 100 ml of blood. This is obtained by doing a Hct * Morphologic Classification - Hypochromic - Microcytic - Macrocytic - Normochromic - Normocytic |
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Hypochromic
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not enough iron
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Microcytic
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RBCs too small
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Macrocytic
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RBCs too large
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Normochromic
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RBCs the normal color
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Normocytic
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RBCs normal size
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What does it mean if a person is normochromic and normocytic?
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The person is probably loosing blood from somewhere?
Don't know if the question is worded correctly. I think it should be normochromic and microcytic. |
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General principles of anemia
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- Anemia is a sign, not a disease.
- Anemias are a dynamic process - It is never normal to be anemic - The diagnosis of iron deficiency anemia mandates further work-up |
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What usually causes hemolytic anemia?
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- the body rejecting the mother's blood during child birth
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Reticulocyte Count
What does a high reticulocyte count mean? |
- R. count is a blood test that measures how rapidly immature red blood cells called reticulocytes are made by the bone marrow and then released in to the blood stream
- if high, it means that the body is producing and releasing immature RBCs and there's probably something wrong going on |
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Reticulocytes
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- Circulate in the blood stream for about 2 days before developing into mature RBCs
- Normally, about 1% to 2% of the RBCs in the bloodstream are reticulocytes |
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Causes of Anemia
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* blood loss
* decreased production of RBCs (marrow failure) * increased destruction of RBCs (hemolysis) * distinguished by reticulocyte count - Decreased in states of decreased production - increased in destruction of RBC |
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Does chemotherapy cause bone marrow suppression?
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Yes
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SIC (Serum Iron Concentration)
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- measures the amount of circulating iron
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TIBC (Total Iron Binding Capacity)
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- measures the amount of tranferring or iron binding globulin which is necessary for transport of iron in blood stream
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TIBC and SIC
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- TIBC is typically measured with SIC to evaluate people suspected of having either iron deficiency or iron overload.
- SIC divided by TIBC gives the transferin saturation, which is a more useful indicator or iron status than SIC or TIBC alone - In healthy people, about 20-50% of available sites in trnasferrin are used to transport iron - In iron deficiency, iron is low, but TIBC is increased, and tranferrin saturation becomes very low. - In iron overload states such as hemochromatosis, iron will be high and TIBC will be low or normal, causing the transferrin saturation to increase |
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Specifics to children
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- Newborn: blood loss, isoimmunization, congenital hemolytic anemia
- Toddlers & young children: 90% are iron deficient, lead poisoning, infections and hemoglobinopathy - Also by chronic inflamamtion, renal and liver disease, endocrine disease and malignant neoplastic disease |
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What is the most common form of anemia
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Iron dificient anemia
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Remember: increased heart rate is often seen with anemia
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- RBCs are meant to oxygenate.
- If there aren't enough of them the blood will try to flow faster to get O2 to where it needs to go |
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Iron
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- Functions as electron transporter; vital for life
- Must be in ferrous state for activity - In anaerobic conditions, easy to maintain ferrous state - Iron readily donates electrons to oxygen, superoxide radicals, H2O2, OH radicals - Ferric ions cannot transport electrons or O2 |
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Causes of iron deficiency
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* Blood Loss
- GI tract - Menstrual blood loss - Urinary blood loss (rare) - Blood in sputum (rare) * Increased Iron Utilization - Pregnancy - Infancy - Adolescence - Polycythemia Vera * Malabsorption - Tropical sprue - Gastrectomy - Chronic atrophic gatritis * Dietary Inadequacy (almost never sole cause) * Combinations of above |
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Iron Absorption
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- Absorbed from the duodenum (where most drugs are absorbed) and upper jejunum
-!!!!! Vitamin C taken with the iron increases absorption by reducing dietary ferric to ferrous iron - Caffeine and other xanthines decrease absorption |
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Iron Preparations available
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* Only absorbed well in the ferrous ("us" is for us) form. Ferrous sulphate, ferrous gluconate and ferrous fumarate are three that are available.
* Ferric from only 10% absorbed (-ick!). Ferric ammonium citrate is in Geritol Tonic - However, patients feel good - could it be because of the 12% alcohol content? |
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Iron Therapy side effects of oral preparations
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- Nausea
- Gastric discomfort - Constipation - Diarrhea - Good idea to start patients on small doses and increase gradually. Less side effects that way |
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Iron parenteral solution
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* Should be used cautiously because .2% to 3% of patients develop hypersensitivity including anaphylactic shock
* Does not appear to provide a faster response than oral preparations * Reasons for use: - Renal failure - Short bowel - Celiac disease (sprue) * Shouldn't give Iron IV!!! |
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Intravenous Iron Therapy
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Ferric gluconate has been available in Europe for more than 20 years and was approved for IV use in the US in 1999 in patients on renal dialysis
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Vitamin B12 or Folic Acid
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* Lack of either prevents formation of DNA so RBC production does not occur or occurs abnormally
- Macrolytic cells (large cells) which may have enough Hb, but are not concave and are fewer in number - Therefore, cannot take up or transport oxygen normally - The cells are more easily damaged - also contributing to the anemia |
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What is the most common cause for hypochronic microlytic anemia?
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Iron deficiency
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What is the most common nutritional deficiency of anemia
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lack of dietary iron
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Vitamin B12 continued
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- Also called the extrinsic factor
- Must combine with intrinsic factor produced by the parietal glands of the stomach - The combination enables binding to receptor and phagocytosis of the complex by the distal ileum cells |
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What is the lack of vitamin B12 called?
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- Pernicious anemia
- It is usually a result of an autoimmune disease that destroys the parietal cells of the stomach - The patients also have achlorhydria which also results in halitosis - refers to a state of lower than normal levels or absence of gastric acid in the stomach |
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Vitamin B12
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- Must be ingested
- Is not synthesized in the body - Sources: meat, eggs, dairy products - Vegans are at a disadvantage - Is also present in multivitamin preparations - Can also be administered by injection in pernicious anemia as cyanocobalamin |
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What body process is Vitamin B12 important for?
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Metabolism
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Folate Insufficiency
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* Inadequate folate intake
- Alcoholics - Teenagers - Some infants * Malabsorption may be due to barbiturates, phenytoin, and oral contraceptives - Impaired metabolism may be due to methotrexate (chemo and demards) or rare enzyme deficiencies |
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Folic acid therapy
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* Recommended natural folate sources:
- Green vegetables - Nuts - Cereal - Fruit - yeast - Liver * Folate Terapeutics - Usually in multivitamin preparations - Also, as folic acid, 5 mg tablets |
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Selected Pharmacology of anemias: RBC loss
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* Therapy: packed RBCs and not whole blood
- Whole blood contains too much potassium from dead cells which lyse. Cardiac arrythmias ensue |
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RBCs from sickle cell anemia
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- Deoxygenation of SS erythrocytes leads to intracellular hemoglobin polymerization, loss of deformability and changes in cell morphology
- Circulation begins to slow - Person's with this disease do not form the normal Hgb A molecule, but rather synthesize Hgb S which carries amino acid valine instead of glutamic acid - Hbg S polemerizes at low PaO2 levels, low pH, low termp and low osmolarity - This process causes sickling of RBC's and hemolytic anemia with occlusive problems |
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Sickle Cell
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- Autosomal recessive pattern
- Found most often in persons of African descent - Gene is believed to confer some advantage against malaria infection - Sickle cell occurs in about 1 in every 400 infants of African descent - Children with sickle cell trait with only 30 to 40% HgbS have a benign clinical course |
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Sickle Cell diagnosis
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- Should be done in the newborn period by careful family history and newborn screening.
- Sickledex test confirms disease |
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When do sickle cell symptoms begin?
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- in babies around 6 months when Hgb F decreases and Hgb S increases
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Sickle Cell physical examination
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- Pale and jaundice with splenomegaly
- Painful swelling of hands and feet - Low grade fever - Leukocytosis - Painful involvement of lower extremities - Diffuse abdominal pain - Chest pain - Sequestration crisis with large amounts of blood in abdominal organs and spleen |
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Sickle Cell management
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- Baseline labs then CBC's, reticulocyte count monitored every few months
- Pneumococcal vaccine - Folic acid supplements - Aggressive treatment for infections - Analgesic for pain - Healthy lifestyle with good hydration - ANY KIND OF FEVER: SEEK HELP!!! - DRINK, DRINK, DRINK! |
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Sickle Cell Emergent Care
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- fever
- pneumonia - aplastic crisis - severe headaches |
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Sickle Cell Education
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- Advise about disease process and preventive health measures
- Genetic counseling - Advised about early signs of illness and when to seek health care |
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Thalassemias definition
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* Genetic defect in hemoglobin synthesis
- decrease synthesis of one of the 2 globin chains - Imbalance of globin chain synthesis leads to depression of hemoglobin production and precipitation of excess globin (toxic) - "ineffective erythopoiesis" - ranges in severity from asymptomatic to incompatible with life (hydrops fetalis) |
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What kind of RBCs present in Thalassemia Major"
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Poikilocytic Red Cells
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Thalassemias diagnosis
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- Smear: microcytic/hypochromic, misshapen RBCs
- Fe stores are usually elevated |
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Thalassemias treatment
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- Mild: none
- Severe: RBC transfusions with Fe chelation and stem cell transplants |
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Packed RBC transfusion
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* 1 unit = 1g/dL Hb; 3% hct
* decide with clinical judgment NOT lob values * transfuse slowly, so you can catch adverse reactions * RBCs should be infused alone or with .9% NaCl through a 170 clot-screen filter * Never mix with: - Ca containing solutions: may cause clumping or clots - Dextrose: hypotonic, may cause hemolysis or clumping - Medications - Hypertonic solutions * AVOID infusing with lactated ringers |
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ABO blood group population frequency
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- O = 45%
- A = 41% - B = 10% - AB = 4% |
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Suspected Transfusion Reaction
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* Hemolytic reaction symptoms are not specific and include:
- Fever chills - Hypotension - Oozing from IV site - Back pain - Hemoglobinuia - red urine * If any of these occur STOP transfusion, provide appropriate supportive care, notify blood bank * Send repeat samples for blood bank evaluation - DO NOT restart the unit - If there's only something like jugular vein distension and there's only fluid overload happening, simply slow the infusion. Don't stop it. |
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Hemolytic Anemias: hereditary spherocytosis
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* The RBCs are like balls b/c they've lost their membrane
* Autosomal dominate trait in Caucasians * Destroyed in spleen * G6PD: used to think it was sickle cell anemia, but this shows up more in white people and it's a different disease process * Inability to neutralize oxidation stress * Produces unstable hemoglobin molecule |
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Bleeding Disorders
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- Bleeding disorders in children should be considered in a child with petechiae, frequent nosebleeds, mucous membrane bleeding, or excessive bleeding from minor trauma
- Initial labs: CBC, platelet count, PT, PTT |
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Thrombocytopenia
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- Platelets below 150,000
- Mucocutaneous bleeding hallmark - Critical to rule out ALL (Acute Lymphocytive Leukemia) - If in children and febrile, consider meningitis - Can be secondary to: drug hypersensitivity, viral infections, and autoimmune disorders |
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Immune Thrombocytopenic Purpura
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- Most common thrombocytopenia in children
- Believed to be autoimmune - Circulating platelets are destroyed - Post viral - Spleen destroys platelets |
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Immune Thrombocytopenic Purpura findings
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- Acute onset of petechiae, purpura and bleeding
- Viral illness 1-4 weeks before. - Hemorrhage of mucous membranes - Liver, spleen, lymph nodes are not generally enlarged |
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Immune Thrombocytopenic Purpura Testing
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- Low platelet count (< 150,000)
- Normal Pt and PTT - Megothrombocytes on peripheral smear |
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Immune Thrombocytopenic management and education
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- Excellent prognosis - spontaneous recover of 75% in 1st 3 months
- Severe cases: corticosteroid therapy - Adults: Imuran (to suppress system synthesis of antiplatelet antibodies) - Most severe issue is intracranial bleeding in less than 1% of cases |
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Hemophilia
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- Deficiency of factor VIII
- Prolonged bleeding - Genes are linked to X chrom - recessive, affecting males - In US 1 in 10,000 males affected with Hemophilia A - Bleeding usually occurs in the joints (hemoarthrosis) |
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What is hemoarthrosis
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When bleeding in the joint happens in a person with hemophilia
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how is hemophilia passed on?
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Usually from mother to son
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Hemophilia Clinical Findings
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- Positive Family History
- Excessive bruising - Prolonged bleeding from mucous membranes and minor lacerations - Hemarthroses: pain and swelling in elbows, knees and ankles - Prolonges PTT - Specify Assay for VIII |
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Hemophilia Complications
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- Bleeding persists in closed spaces like joints
- Severe anemia to shock - Transfusion risks - Development of an acquired circulating antibody to factor VIII |
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Hemophilia management and education
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- Prevention of trauma and replacement therapy to increase factor VIII
- Mild: may respond to desmopressin acetate a vasopressin that triples production of factor VIII - Immunizations should be given SQ - Fe replacement may be necessary - Medical alert bracelet - Safety and exercises - Good prognosis if care coordinated through a hemophilia center where attention is given to emotional, physical and social support |
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Von Willebrand Disease
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* Common Disorder caused by deficiency of vWF an adhesive protein that:
- Bridges subendothelial collagen and platelets - Protect factor VIII from rapid clearance * DDAVP treatment of choice for bleeding episodes * When high levels of vWF needed an attenuated concentrate (Humate P) may be necessary |
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What is the treatment of choice for Von Willebrand Disease bleeding episodes?
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- DDAVP
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Thrombocytosis
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* Elevated platelet count
* Hereditary predisposition to thrombus caused by: - Deficiency in an anticoagulant protein - Abnormality of procoagulant protein resistant to proteolysis by its respective inhibitor (factor V Leiden) - These disorders can lead to: DVT or PE |
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Neutropenia
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- absolute neutrophil count (ANC) less than 1500 for Caucasian children over 1 yr of age
- black children normally have a lower count - susceptibility to infection unaffected until the ANC is less than 1000 - Patients do well until ANC is less than 500 - Congenital or acquired |
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Mechanisms of Neutropenia
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* Marrow Injury
- Drugs (chemo) - Radiation - Hereditary - Infections (HIV and hepatitis) - Infiltration process * Maturation defects - Folic deficiency - B-12 deficiency - Cyclic Neutropenia |
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Neoplastic Disorders: Pancytopenia
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* Decrease in all formed elements of the blood
- Erythrocytes - Leukocytes - Platelets * This condition may be result of failure in production, sequestration, or increased peripheral destruction |
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Pancytopenia: features that suggest bone marrow failure
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- tear drop formed RBCs
- abnormal shaped leukocytes - myleoid elements less mature than bands - Small platelets |
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Pancytopenia: Features that suggest increased destruction
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- Reticulocytosis
- Jaundice - Large Platelets - Immature myeloid elements - Increased Serum Bilirubin |
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Aplastic Anemia
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- Pancytopenia evolves as the hematopoietic elements of the bone marrow disappear are replaced with fat
- May be idiopathic or induced by drugs, toxins or infections - In severe cases BMT is treatment of choice |
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Leukemias
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- Cancer of the blood or bone marrow characterized by an abnormal proliferation of blood cells, usually leukocytes
- The cells are large, but they're not effective |
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Lymphomas
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- In Hodgkin's Disease, the abnormal lymphocyte involved is the Reed-Sternber Cell (B lymphocyte)
- This particular lymphocyte isn't found other types of lymphomas, so they are called non-Hodgkin's lymphoma - There are more than 30 different kinds of non-Hodgkin's lymphoma |
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Acute Leukemia
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- characterized by the rapid growth of immature blood cells
- it is a more common cause of death for children in the US than any other type of malignant disease - immediate treatment is required in acute leukemias due to the rapid progression and accumulation of the malignant cells, which then spill over into the bloodstream and spread to other organs of the body - If left untreated, the patient will die within months or even weeks |
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Chronic Leukemia
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- distinguished by the excessive buildup of relatively mature, but still abnormal, blood cells
0 Typically taking months to years to progress, the cells are produced at a much higher rate than normal cells, resulting in many abnormal white blood cells in the blood - Chronic leukemia mostly occurs in older people, but can theoretically occur in any age group - Chronic forms are sometimes monitored for some time before treatment to ensure maximum effectiveness of therapy |
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Prevalence of the 4 major types of leukemia
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* Acute lymphocytic Leukemia (ALL)
- the most common type of leukemia in young children - Also affects adults, especially those age 65 and older * Acute myelogenous leukemia (AML) - occurs more commonly in adults than in children. - was previously called acute nonlymphocytic leukemia * Chronic Lymphocytic Leukemia (CLL) - Most often affects adults over the age of 55 - Sometimes occurs in younger adults, but almost never affects children * Chronic Myelogenous Leukemia (CML) - occurs mainly in adults - a very small number of chilfren also develop this disease * The most common forms in adults are AML and CLL, whereas in children ALL is more prevalent |
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Treatment of leukemia
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- Chemotherapy to kill leukemia cells using strong anti-cancer drugs
- Interferon therapy to slow the reproduction of leukemia cells and promote the immune system's anti-leukemia activity - radiation therapy to kill cancer cells by exposure to high-energy radiation - Stem cell transplantation (SCT) to enable treatment with high doses of chemotherapy and radiation therapy - Surgery to remove an enlarged spleen or to install a venous access device to give medications and withdraw blood samples |
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Chemotherapy and leukemia
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- When beginning chemo there will be a large "explosion" of lysed cells. This is not something that can be debulked.
- The person will become hyperkalemic and hyperphosphortemic which causes hypocalcemia. - Renal failure can occur because of the increase in casts |
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Symptoms of Hodgkin's
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* The main symptom of both Hodgkin's and non-Hodgkin's lymphomas is swelling of lymph nodes in the neck, under the arms, or in the groin.
* Other symptoms include: - Fever - Night sweats - Fatigue - Abdominal pain - Unexplained weight loss - Itchy skin * Treatment varies by type and presenting issues including the age of the pt |
