The Hematologic System

 

Red Blood Cells

 

Essential substances are required to produce normal RBCs:

 

Iron

     For ages 22-65, you get all the iron you need from recycling of RBCs by the spleen

     It is almost impossible to get iron-deficiency anemia in the US for ages 22-65; if it occurs, it is probably actually due to losing blood.

     Need to increase dietary iron in those < 22 years old and during pregnancy (to be able to grow)

·         Mild anemia may be asymptomatic and should be suspected in infants, women of menstruation age, and pregnant women.

 

B12

     get from diet (animal protein) and Brewer’s yeast

     at risk:  vegetarians, elderly

     Vitamin B12 deficiency - similar to ALS, MS

     stored in liver - would take 5-7 years to become deficient (use in micrograms, store in milligram).  Thus, it is not an acute type of anemia.

 

Folic acid

     stored in bone marrow

     we store 40 days worth

     folic acid anemia is an acute type of anemia

     needed for development of neural tube (defects such as spina bifida due to lack of folic acid occurring in the first 28 days of gestation)

 

Amino acids - recycled by spleen

 

Erythropoietin (Epogen)

     hormone produced by kidneys

     patients with chronic infection, chronic inflammatory conditions, or cancer produce interleukin-I (IL-I), a potent suppressor of erythropoietin, resulting in “anemia of chronic disease”

     athletes get Epogen in black market ($25,000/year); use to pump up RBC’s ---> 75-80 Hct ---> sludge in cerebral arteries ---> stroke at age 20

 

Thyroid

     screen TSH at age 35

     hypothyroidism:  female:male 8:1 (18:1 in elderly female)

     hypothyroidism ---> anemia   (Sx:  increased cholesterol)

     hypothyroidism ---> decreased BMR ---> decreased RBC production

     Most common cause of high cholesterol in women = low TSH

Normal RBC count:   4.5-6.0 million (women closer to 4.5, men closer to 6.0)

            This is due to men being composed of more muscle, which needs RBCs for oxygenation.  Women, on the other hand, have more fat and less muscle, so do not            require as many RBCs.

 

Hgb (14) and Hct (42) should NOT be different in elderly

 

Erythropoiesis: formation and maturation of RBCs

            Stage of RBC maturation include:

·         erythroblast (in bone marrow, has nucleus)

·         pronormoblast

·         normoblast

·         reticulocyte (no nucleus; in peripheral blood)

·         erythrocyte (mature RBC)

 

Should not have any nucleated RBCs in peripheral blood (on lab report, “Nucleated RBCs” should be “0” because they are removed by the spleen).  If no spleen, may have a few nucleated RBCs.

 

Nucleated RBCs are seen in:

     sickle cell anemia

     thalassemia

     bone marrow problems

     leukemias

 

retic count = rate at which bone marrow produces RBCs

            reticulocytes are immature precursors; 2% of total RBCs

 

Case examples:

·         14 year old female with anemia:  retic count = 0.1% with anemia

            Diagnosis?  underproduction anemia (Iron-deficiency anemia from diet)

 

(Note:  For iron-deficient patients:  check retic count at least 7 days after starting iron)

 

·         22 year old female with anemia:  retic count = 16%       

            Diagnosis?   breakdown of RBC? (hemolytic)

                                 overdestruction of RBC 

                                 ? bleeding                            

 

When estrogen levels are highest, body cells look foreign ---> autoimmune disease (RA, SLE, sickle cell, thalassemia)

 

In sickle cell trait, RBC’s last 40 days compared to the normal of 109 days.  It is OK for sickle cell patients to have a high retic count.

 

RBC Morphology (RBC indices)

 

** MCV (mean cell volume) = 80-96

            (MCH, mean cell hemoglobin, does corresponding changes = 28-33)

 

(MCHC is usually a useless index)

 

RBC indices used to diagnose anemia, a condition characterized by a reduction in RBCs, which in turn decreases the oxygen-carrying capacity of the blood.  Hypoxia produces fatigue, among other problems.

 

Anemia is not in itself a disease, but a principal manifestation of many abnormal conditions, such as:

·         deficiency states caused by a dietary lack of iron, vitamin B12, and folic acid

·         hereditary disorders of RBCs

·         disorders involving the hematopoietic tissues (bone marrow damage or a hyperactive spleen)

·         bleeding from the GI tract or any organ secondary to cancer or trauma

·         a family history of anemia is common, especial in certain racial and ethnic groups

 

Etiology:  Major causes of anemia are:

·         excessive blood loss

·         deficiencies and abnormalities of RBC production

·         excessive destruction of RBCs

 

Remember the normals of MCV (80-96) and MCH (28-33) for the following case studies:

 

a.   RBC of 3 million, MCV of 65 and MCH of 22

     MCV is decreased, or small, so microcytic

     MCH is decreased, so pale, or hypochromic

     with microcytic, hypochromic anemia, S/S include being tired, palpitations, SOB

     9 out of 10 are iron-deficient:  assess for vaginal and GI bleeding, use of NSAIDs, exercising too much (marathon runners and weight lifters get muscle tears), drinking a lot of tea (can’t absorb iron)

     1 out of 10 are due to other causes such as thalassemia (Mediterranean background), lead poisoning (on smear will see basophilia and stippling, or blue dots on the RBCs).

     Those at risk for lead poisoning include children, plumbers, painters, policemen, those drinking moonshine (made in radiators), elderly

     Lead compounds with calcium and is stored in the bone.  Many current elderly ate lead as children, which was stored in their bones.  The lead is now coming out of the bone as they lose their calcium

 

b.   RBC of 3 million, MCV of 120 and MCH of 40

     MCV is increased, so large, or macrocytic

     MCH is increased, so “hyperpigmented”, or polychromic

     Note:  alcoholics have MCV of 100-110

·         ** Alcohol abuse can be associated with direct marrow toxicity, vitamin deficiency, and upper GI blood loss and is a common cause of a mixed microcytic and macrocytic anemia.

     With MCV > or = to 120, cause is Vitamin B12 or Folic acid deficiency.

     The deficiency may be due to being a “tea and toaster”, having a gastrectomy > 6 years ago, malabsorption in the small bowel due to Crohn’s or celiac sprue, tapeworm, or being on a drug for at least 40 days which inhibits folic acid. 

     Drugs which inhibit folic acid include methotrexate for rheumatoid arthritis, Septra/Bactrim, phenytoin (Dilantin).

 

c.   RBC of 3 million, MCV of 88 and MCH of 30

     MCV and MCH are both normal, so normocytic, normochromic

     But the RBC’s are low

     Causes:  anemia of chronic disease, hypothyroidism, cancer

 

Anemia of chronic disease – caused by chronic infection/inflammation or malignancy (decreased RBC lifespan, failure of compensatory erythropoiesis, disturbance of the iron cycle); RBCs more vulnerable to phagocytosis.

 

 

Other important lab tests:  serum iron, iron binding capacity, ferritin.

 

serum ferritin:  best measure of iron stores (2X higher in males)

 

Too high of iron level --> oxidation --> damaged artery wall --> increased risk of placing cholesterol in walls --> increased risk of coronary artery disease

(ASA after MI --> small amount of bleeding --> decreased iron level --> decreased CAD risk)

 

 

Hemoglobin A1C (glycosylated hemoglobin)

     Normal is < 6

     Indicates blood sugar level over a 3 month period (RBC lifespan is 109 days.  Glucose attaches and cannot unattach)

     Urine sugars are useless:  urine output today is what the blood sugar was 4 days ago.  (only good for ketone check in Type 1 diabetic)

     5-8 weeks after treatment change, should start to see improvement (decrease) in Hgb A1C

 


Polycythemia – an increase in both the number of circulating erythrocytes (many number as high as 8-12 million/mm3) and the concentration of Hgb within the blood (rises to 18-25 g/100 ml)

 

Three types of polycythemia:

1.                  Polycythemia vera – classified as a myeloproliferative disorder (meaning overgrowth of bone marrow).  Usually develops in middle age, particularly among Jewish men.

a.                   Possibly a form of malignancy similar to leukemia and is often considered a premalignant condition.

b.                  3 major hallmarks of the condition

                                                              i.      relentless, unrestrained production of erythrocytes

                                                            ii.      production of excessive myelocytes (WBCs within the bone marrow)

                                                          iii.      overproduction of platelets

c.                   Clinical manifestations:  increase in blood viscosity, increase in total blood volume (may be 2-3 times normal), severe blood congestion of all tissues and organs, resulting in many symptoms

d.                  Diagnosis:  RBC count, Hgb, Hct, and platelet count are increased, ABGs are normal, hyperplastic bone marrow, serum uric acid 3-4X normal

e.                   Management (goals are to reduce blood volume and viscosity and reduce bone marrow activity)

                                                              i.      Phlebotomy (500-2000 ml blood until Hct reaches 45%)

                                                            ii.      Myelosuppressive agents (radioactive phosphorus, chlorambucil, busulfan, hydroxyurea)

                                                          iii.      Radiation therapy (to decrease production of RBCs in the marrow)

f.                   Mortality (prognosis depends on age at diagnosis, treatment used, and complications)

                                                              i.      30% die of thrombotic complications

                                                            ii.      10-15% die from hemorrhage

                                                          iii.      15% die from either myelogenous leukemia or myelogibrosis accompanied by pancytopenia

 

2.                  Secondary polycythemia:  When the body’s demand for oxygen increases for any reason, the bone marrow must produce more RBCs in order to prevent tissue hypoxia.  This compensatory response to tissue hypoxia is called secondary polycythemia

a.                   Causes:  chronic lung disease (particularly emphysema), congenital heart disease, and prolonged exposure to altitudes of 10,000 feet or more

b.                  Symptoms and lab findings same as those with polycythemia vera, except the WBC and platelet counts are normal and splenic enlargement is absent

c.                   Management:  treat the underlying disease or condition causing hypoxia

 

3.                  Relative polycythemia:  Whenever the body loses plasma without losing RBCs, the concentration of RBCs increases relative to the amount of plasma remaining in the vascular system.

a.                   Causes are fluid loss and dehydration as a result of not enough fluid intake, diarrhea, vomiting, burns, and excessive administration of diuretics.

b.                  Management:  reestablishment of fluid and electrolyte balance

 

Hemolytic disease of newborn (HDN)

·         Can occur only if antigens on fetal erythrocytes differ from antigens on maternal erythrocytes

·         Maternal-fetal incompatibility exists if mother and fetus differ in ABO blood type or if the fetus is Rh-positive and the mother is Rh-negative

·         Incidence:  ABO incompatibility occurs in 20-25% of all pregnancies, but only one in 10 cases results in HDN.  Rh incompatibility occurs in <10% of pregnancies and rarely causes HDN in the first incompatible fetus (usually erythrocytes from the first incompatible fetus released when the placenta detaches at birth, cause the mother’s immune system to produce antibodies that affect the fetuses of subsequent incompatible pregnancies).

·         Pathophysiology:  HDN results if:

o   the mother’s blood contains pre-formed antibodies against fetal erythrocytes or produces them on exposure to fetal erythrocytes

o   sufficient amounts of antibody (usually IgG) cross the placenta and enter fetal blood, and

o   IgG binds with sufficient numbers of fetal erythrocytes to cause widespread antibody-mediated hemolysis and splenic removal

·         Clinical manifestations:  If mild HDN:  appear healthy or slightly pale with slight enlargement of the liver or spleen.

·         Pronounced pallor, splenomegaly, and hepatomegaly indicate severe anemia, which predisposes the neonate to cardiovascular failure and shock.

·         Because the maternal antibodies remain in the neonate’s circulatory system after birth, erythrocyte destruction can continue.  This causes hyperbilirubinemia and icterus neonatorum (neonatal jaundice) shortly after birth.

·         Without replacement transfusions, in which the child receives Rh-negative erythrocytes, the bilirubin is deposited in the brain, a condition termed kerniterus.  This produces cerebral damage and usually causes death.  Infants who do not die may have mental retardation, cerebral palsy, or high-frequency deafness.

·         Prevention/Diagnosis:  Coombs’ test

o   indirect Coombs measures antibody in the mother’s circulation and indicates if the fetus is at risk for HDN

o   direct Coombs measures antibody already bound to the surfaces of fetal erythrocytes and is used primarily to confirm the diagnosis of antibody-mediated HDN.

·         Treatment:  prevention through immunoprophylaxis

o   If an Rh-negative woman is given Rh immune globulin (RhoGAM) within 72 hours of exposure to Rh-positive erythrocytes, she will not produce antibody against the D antigen and the next Rh-positive baby will be protected.  (The mother must be given Rh immune globulin injections after the birth of each Rh-positive baby and with any abortion.)

·         Treatment:  exchange transfusions in which the neonate’s blood is replaced with new Rh-positive blood that is not contaminated with anti-Rh antibodies (done within 24 hours after birth).  Phototherapy also is used to reduce the toxic effect of unconjugated bilirubin.

 

 

Sickle cell disease:  a group of disorders characterized by the presence of an abnormal form of hemoglobin:  hemoglobin S (Hb S) within the erythrocytes.

·         Hb S is formed by a genetic mutation in which one amino acid (valine) replaces another (glutamic acid)

·         Hb S reacts to deoxygenation and dehydration by solidifying and stretching the erythrocyte into an elongated sickle shape.

·         Sickle cell disease is an inherited, autosomal recessive disorder that is expressed as sickle cell anemia, sickle cell-thalassemia disease, or sickle cell-hemoglobin C disease, depending on the mode of inheritance.

·         Sickle cell anemia, a homozygous form (inherits HB S from both parents), is the most severe.

·         Sickle cell-thalassemia and sickle cell-Hb C disease are heterozygous forms in which the child simultaneously inherits another type of abnormal hemoglobin from one parent (and Hb S from the other parent).

·         Sickle cell trait, in which the child inherits Hb S from one parent and normal hemoglobin (Hb A) from the other, is a heterozygous carrier state that rarely have clinical manifestations. (Because the child has 50% Hb S, clinical manifestations occur only with extreme stress.).

·         Incidence:  in persons with origins in equatorial countries, particularly central Africa, the Near East, the Mediterranean area, and parts of India.

·         Incidence:  In US, most common in African Americans, 1:4000-1:5000 live births.  In the general population, the risk of two African-American parents having a child with sickle cell anemia is 0.7%.  Sickle cell trait occurs in 7-13% of African Americans.

·         Clinical Manifestations:  When sickling occurs, the general manifestations of hemolytic anemia—pallor, fatigue, jaundice, and irritability—sometimes are accompanied by acute manifestations called crises.

·         4 types of crises:

o   vaso-occlusive (thrombotic) crisis (vasospasm causes “log-jam” of blood)

o   aplastic crisis (In sickle cell anemia, erythrocyte survival is only 10-20 days.  Despite the increased need for new erythrocytes, erythropoiesis is diminished, resulting in profound anemia)

o   sequestration crisis (large amounts of blood become acutely pooled in the liver and spleen (seen only in the young child)

o   hyperhemolytic crisis

·         Treatment:  supportive care aimed at preventing consequences of anemia and avoiding crises.  Avoid fever, infection, acidosis, dehydration, constricting clothes, and exposure to cold.  Bone marrow transplant.

 

 

Leukocytes/Lymphocytes

 

Infectious Mononucleosis

·         Acute infection of B-lymphocytes (B-cells)

·         Most common virus is the Epstein-Barr virus (EBV).  Others = CMV and other viruses and the bacterium Toxoplasma gondii)

·         Can be thought of as “it would have been a lymphoma, but it stopped short”

·         Usually affects young adults between the ages of 15 and 30 (“kissing disease”)

·         Unaffected B cells produce antibodies against the virus, and T cells assist the B cell response of attacking the virus directly.  The proliferation of clones of B and T cells and removal of dead and damaged leukocytes are largely responsible for the swelling of lymphoid tissues (lymph nodes, spleen, tonsils, and occasionally, liver).

·         Sore throat and fever, two of the earlier manifestations of infectious mono, are caused by inflammation at the site of viral entry (and initial infection), the mouth and throat.

·         Incubation period: 30-50 days

·         S/S:  headache, malaise, and fatigue during the first 3-5 days that continue for 7-20 days.  Temperature for 7-10 days, sore throat, enlargement and tenderness of the cervical lymph nodes.

·         Sore throat is the most common symptom.  It appears in the first week and is usually accompanied by hyperplasia of the pharyngeal lymphoid tissue with inflammation and edema, as well as exudative tonsillitis.

·         Lymph node enlargement is the predominant clinical manifestation of infectious mono.  Enlargement occurs gradually, most commonly in the anterior and posterior cervical chains, and generalized lymph node enlargement also may develop.  Splenomegaly occurs in about ˝ of affected individuals.

·         Usually self-limiting, and recovery occurs in a few weeks.  Treatment consists of rest and alleviation of symptoms and analgesics.

·         Lab findings:  increase in lymphocyte and monocyte counts, with 10-20% atypical forms.  During the first week of infection, the WBC count may be normal or slightly low, secondary to mild neutropenia.  After the first week, the WBC count (predominantly lymphocytes and monocytes) increases to 10,000 to 20,000 and persists at this level from 4-8 weeks.  Platelet counts are low (<140,000)

·         Serologic tests are necessary to diagnose infection by EBV.  A Monospot agglutination test is performed to show the presence of heterophilic antibodies associated with infection by EBV.  (Heterophilic antibodies are a heterogeneous group of antibodies that are agglutinins against sheep RBCs).

 

 

 

Leukemia:  a malignant disease of the blood and blood-forming organs causing an accumulation of dysfunctional cells and a loss of cell division regulation

·         Two major forms:  acute and chronic:  classified by predominant cell type and the rate at which the affected individual develops clinical symptoms

·         Acute leukemia is characterized by undifferentiated or immature cells, usually a blast cell.  The onset of disease is abrupt and rapid.  Disease progression results in a short survival time.

·         In chronic leukemia the predominant cell is mature in appearance but does not function normally.  The onset of disease is gradual and the prolonged clinical course results sin a relatively longer survival time.

·         Acute types:  ANLL (acute nonlymphoblastic leukemia) founds in adults, ALL (acute lymphoblastic leukemia) most common in children, and AEL (acute erythroleukemia)

·         Chronic types:  CML (chronic myelocytic leukemia) and CLL (chronic lymphocytic leukemia) seen in adults

 

Acute Leukemias

·         Mortality rate for the US is about 7 per 100,000 individuals (high in Japan due to atomic bombs)

·         Strikes both sexes and all ages.  Incidence rises steeply, however, beyond age 50

·         5-year survival rate is 35%, partly because of the poor survival of those with ANLL

·         Chemotherapy has helped increase the 4-year survival rate for people with ALL from 4% in the 1960s to 73% in the 1990s

·         S/S:  fatigue caused by anemia, bleeding resulting from thrombocytopenia, fever caused by infection, weight loss, bone pain, elevated uric acid, liver, spleen, and lymph node enlargement

·         Dx:  blood tests, bone marrow biopsy

·         Treatment:  chemotherapy is treatment of choice

·         Controversial are immunotherapy agents that induce differentiation of immature granulocytes, and marrow transplants.

 

Chronic Leukemias

·         Chronic leukemia cells are well differentiated and can be readily identified.

·         S/S:  Advances slowly and surreptitiously, without warning.  Symptoms, when they do appear, include splenomegaly, extreme fatigue, weight loss, night sweats, and low-grade fever.

·         Incidence:  of CML is approximately 1 per 100,000 people

·         CML = a group of diseases called myeloproliferative disorders, along with polycythemia vera, primary thrombocytosis, and idiopathic myelofibrosis

·         Presence of the Philadelphia chromosome is a diagnostic marker of CML

·         The acute effects of CML resemble those of acute leukemia, but with more prominent and painful splenomegaly.

·         CLL predominately involves malignant transformation of B-cells (although T cell CLL occurs less commonly).  The B cells fail to mature into plasma cells that synthesize immunoglobulin.

·         A possible mechanism responsible for CLL of B cells is a deficiency of normal helper T cells with an increase in suppressor T cells.

·         Treatment for CML:  bone marrow transplantation, biologic response modifiers, and combination chemotherapy (but does not prolong the average survival time)

 

Malignant Lymphomas:  tumors of primary lymphoid tissue (thymus and bone marrow) or secondary tissue (lymph nodes, spleen, tonsils, and intestinal lymphoid tissue).

·         The major subdivisions of malignant lymphomas are Hodgkin disease and non-Hodgkin lymphoma.

 

Hodgkin disease

·         Hodgkin disease is a clonal disorder:  distinctive abnormal chromosomes are present

·         An inhibitor in the serum and spleen of individuals with Hodgkin disease selectively binds to T cells and interferes with their function.

·         More popular theory of how the disease spreads is the contiguity theory, which suggests that Hodgkin disease spreads by the movement of existing tumor cells through lymphatic routes.

·         Role of EBV:  Higher-than-normal antibody titers to the Epstein-Barr virus are reported in individuals with Hodgkin disease.

·         S/S:  initial sign is usually an enlarged painless mass, lump, or swelling in the neck.  May have intermittent evening fever, weakness, malaise, weight loss, anemia (especially in the elderly)

·         Most commonly affected lymph nodes in Hodgkin disease:  cervical, axillary, inguinal, and retroperitoneal (mesenteric, epitrochlear, bronchial, and popliteal nodes are rarely involved).

·         Dx:  CXR, lymphangiography, and biopsy; presence of Sternberg-Reed cells

·         Treatment:  radiotherapy and chemotherapy

 

Non-Hodgkin Lymphoma

·         Cause of lymph node enlargement and cancerous transformation in non-Hodgkin lymphoma is unknown.

·         Immunosuppressed persons have a greater incidence of non-Hodgkin lymphoma, suggesting an immune mechanism.  In contrast to Hodgkin disease, T cell function is minimally affected.  B cell abnormalities, such as impaired and decreased antibody production, are more common in non-Hodgkin lymphoma than in Hodgkin disease.

·         Increasing frequency in persons with AIDS with extremely poor prognosis

·         S/S:  The cervical, axillary, inguinal, and femoral chains are the most frequent sites of lymph nodes enlargement (painless).  CBC initially is normal…abnormalities not seen until the disease is advanced.  Pancytopenia (decreased RBC, WBC, and platelet counts), when present, indicates hypersplenism or extensive replacement of bone marrow by lymphoma.

·         Treatment:  chemotherapy, radiation, bone marrow transplantation


Platelet Aggregation and the Coagulation Process

 

Platelets have no nucleus.

 

For cancer patients receiving platelets, the HLA antigens on the platelet surface membrane need to be matched.

 

Normal platelet count:  150,000 - 450,000 (depends on lab)

            thrombocytopenia:   platelet count < 100,000

            Symptoms when < 50,000

 

3 types of problems:

 

1.   Underproduction (bone marrow suppression) due to:

     chemotherapy

     alcohol

     Tagamet

     viruses

 

2.   Overdestruction due to:

     ITP

     autoimmune problem

     viruses

     HELLP syndrome (Hemolysis (--> anemia), Elevated Liver enzymes, Low Platelets); seen with preeclampsia --> DIC

     heparin-induced thrombocytopenia

 

3.   Overutilization due to DIC caused by:

     complication of pregnancy (#1 abruptio placentae, #2 amniotic fluid embolism)

     sepsis

     multiple trauma

     snakebites

     cancer

 

Heparin-induced thrombocytopenia (“white clot syndrome”):

     Increased risk with high molecular weight (MW) heparin

     Can even occur with heparin locks

     If on beef or pork heparin, check platelets daily. 

     Heparin should be discontinued within 5 days of starting

     After 7 days, it stimulates the immune system to form heparin antibodies (autoimmune problem) ---> “white clots” in arteries which can lead to multiple amputations

     Problems occur when platelets < 100,000

     Does not occur with Coumadin

 

Symptoms of low platelet count:

     superficial bleeding (mucous membranes, petechiae, purpura, oozing around tubes)

     prolonged menstrual period

     coffee ground emesis

     nosebleeds (bilateral if not due to nose picking)

 

It takes 7-10 days to reverse the effects of ASA.  It also takes time to reverse the effects of NSAIDs (for long-acting preparations, takes at least 7 days).

 

Coagulation cascade:

 

Left side (extrinsic pathway) is initiated by damage outside the blood vessel (in the tissue)

            * factor VII is: - dependent on Vitamin K

                                    - inhibited by Coumadin

                                    - measured by PT/INR

 

INR (International Normalized Ratio)

     standardizes tests among hospitals, labs, and offices

     normal is 2-3 when on Coumadin (= 1.3-1.6 PT)

     Exceptions:  normal needs to be higher for those with artificial heart valves

 

Coumadin is very sensitive to drugs (>77 drug interactions on Epocrates!):

            - Decreases Coumadin level:  phenobarbital, black licorice

            - Increases Coumadin level:  Septra

 

Major increase in intake of Vitamin K-containing foods (green, leafy vegetables) can mess up PT/INR (test is also affected by Questran, enteral feedings, and Metamucil)

 

Right side of cascade (intrinsic pathway) is initiated by damage within the blood vessel

 

Heparin blocks the intrinsic cascade

                        - monitor with PTT/aPTT

 

Fibrinogen:

     increased by estrogen, birth control pills, and smoking

     BUT estrogen-replacement therapy (p.o.) DOESN’T increase fibrinogen (unless the patient had had clots while on estrogen-containing BCPs)

 

DON’T put female smoker on estrogen BCPs (use Norplant, Depo-Provera, progestin)

OR If smoker < 35 years old, give < 35 mg. estrogen pills.