Coagulation and Hematopathology Flashcards
Primary hemostasis is accomplished by ______ and secondary hemostasis by ______
- Platelets
- cascade fibrin formation
What forms platelets?
Megakaryocytes in bone marrow
What 4 things do platelets do to form a plug?
1-Adhere to exposed subendothelium/von willebrand factor
2-Secrete contents of granules
3-Aggregate with other platelets
4-Provide a procoagulant surface for coagulation cascade
when thrombin is formed, soluble fibrinogen turns into what?
Insoluble fibrin clot
Which vitamin is crucial for coagulation?
Vitamin K
*facilitates creation of calcium binding sites
Mediated by plasmin, what is the break down of fibrin clot resulting in fibrin degradation products such as d-dimer called?
Fibrinolysis
What three basic tests are used to evaluate hemostasis?
1-Platelets (count/morphology)
2-Coagulation (PT, PTT)
3-Fibrinolysis (d-dimer)
PT measures ____ pathways and PTT measures ____ pathways
extrinsic (12-15 sec)
intrinsic (24-35 sec)
A normal platelet count is between 150,000-450,000/uL, what is it called when it is decreased under 100,000?
Thrombocytopenia
*decreased bone marrow production or sequestration in spleen
_____ abnormalities in coagulation factors affect a single factor, ____ usually affects multiple
Inherited (von willebrand, hemophilia)
Acquired (liver disease, it K deficiency)
What protein mediates lately binding to sites of vascular injury by binding collagen and GP1b receptor?
Von Willebrand factor (vWF)
*carrier for coagulation factor VIII
What condition is autosomal dominant, and has mucocutaneous platelet like bleeding and often has normal PTT?
von Willebrand disease
*Type 1 (partial quantitative) most common
What condition is a factor VIII deficiency, has hematomas, hemorrhage in joints and easy bleeding?
Hemophilia A
*prolonged PTT, normal PT and platelet
What condition is a factor IX deficiency, has hematomas, hemorrhage in joints and easy bleeding?
Hemophilia B
*prolonged PTT, normal PT and platelet
Massive systemic intravascular activation of coagulation is called?
Disseminated intravascular coagulation (DIC)
What is elevated in severe acute DIC?
d-dimer (fibrin degradation product)
*schistocytes, decreased platelets, hematocrit, fibrinogen
What is the pathologic counterpart of hemostasis?
Thrombosis
What are the 3 elements of Virchows triad?
1-Endothelial injury
2-Abnormal blood flow
3-Hypercoagulability of blood
What is the most common inherited thrombophilia in caucasians?
Factor V leiden
*single nucleotide change in coagulation factor V
How does factor V leiden affect clotting?
Protein C and protein S cannot degrade altered factor V, favoring fibrin formation and clotting
What two things make up the majority of cells in a normal WBC?
1-Neutrophils (55%)
2-Lymphocytes (35%)
*eosinophils, basophils and monocytes are part of WBC as well
What are the two broad categories of WBC disorders?
1-Leukocytosis (too many)
2-Leukocytopenia (not enough)
At what level of neutropenia should the patient stay in the hospital?
Lower than 1500 cells/ul
Normal white blood cell counts are in what range?
3,500-10,000
What are 4 causes of leukocytosis?
1-Increased marrow production
2-Increased release of marrow stores
3-decreased margination
4-decreased extravasation into tissues
What are the 3 size categories of anemia?
1-Microcytic Anemia (under 80)
2-Normocytic Anemia (80-100)
3-Macrocytic anemia (above 100)
What is the most common reason for microcytic anemia?
Iron deficiency
*usually because of bleeding
What are 6 clinical features of iron deficiency anemia?
1-Pica (unusual craving) 2-Koilonychia (thin nails) 3-atrophic glossitis 4-esophageal web 5-pallor 6-weakness
What are the two types of macrocytic anemia?
1-Non-megaloblastic
2-Megaloblastic
What are the two likely causes of megaloblastic macrocytic anemia?
1-Folic acid deficiency (DNA synthesis)
2-B12 deficiency
What three things could cause folic acid deficiency and result in megaloblastic macrocytic anemia?
1-Poor diet
2-Decreased absorption (jejunal resection)
3-Increased utilization
What are 2 absorption requirements for Vitamin B12?
1-Intrinsic factor
2-Intact ileum
Systemically distributed neoplasms of white cells are called?
Leukemia
Solid tumors of hematopoietic system/neoplasms of lymphoid origin are called?
Lymphoma
Lymphomas and leukemias are clonal expansions of cells at certain ________
Developmental stages
If less than 20 % blasts in the periphery it is _____ vs more than it is _____
Chronic (mutation in peripheral maturation)
Acute (mutation in bone marrow maturation)
Leukemia is either ____ or ____ and _____ or _____
Acute or Chronic
Myeloid or Lymphoid
What 4 things go into go into making a diagnosis of leukemia?
1-Clinical Data
2-Phenotype
3-Genetic/molecular
4-Morphology
What are the 3 sub groups of AML that the WHO recognizes?
1-Recurrent genetic abnormalities
2-Multilineage dysplasi
3-Not otherwise categorized
Lymphomas are named based on what?
Where B cell development goes wrong. Whether in bone marrow or lymphoid tissue
Which type of lymphoma is the minority, curable in most and has variable month-years survival?
Hodgkin Lymphoma
Which type of lymphoma has chronic (years), aggressive (months) and very aggressive (weeks) types and is curable in some cases?
Non-hodgkin lymphoma
*indolent/chronic generally not curable
What are 3 clinical findings of lymphomas?
1-Enlarged, painless lymphadenopathy
2-B-symptoms (fever, weight loss)
3-Impingement or obstruction of other structures
What are the two most common types of Non hodgkin lymphoma?
1-Follicular lymphoma
2-Diffuse large B cell lymphoma
Which lymphoma is the most common nonhodgkins lymphoma, occurs in at older age, is indolent and involves chromosomal translocation (14:18)?
Follicular Lymphoma
*bone marrow involvement common
Which type of nonhodgkins lymphoma is derived from B-cells, and is more likely to have extra nodal sites?
Diffuse Large B Cell Lymphoma
*peripheral blood involvement rare
Which 4 factors are vitamin K dependent?
II, VII, IX, X
Which factor is associated with von Willebrand disease?
VIII
