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Hematology
Heme- bleeding disorders
77
Medical
Graduate
11/14/2009

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Term
Erythropoietin production is regulated by this.
Definition
Production is regulated by hypoxia (from peritubular fibroblast secreted hypoxia inducible factor)
Term
Fetal RBC's are made here from weeks 3-7 and are nucleated.
Definition
Extraembryonic yolk sack makes these
Term
HbF is made of these chains
Definition
Made of Alpha(2)Gamma(2). Also, resistant to 2,3-BPG causing a left shift on the O2 binding curve
Term
Site of bilirubin formation in the body
Definition
Bilirubin is formed in the mitochondria of macrophages
Term
Building blocks of heme
Definition
Glycine, succinyl-CoA and Fe make this, in the mitochondria of liver and BM cells
Term
Lead blocks these two enzymes of heme synthesis
Definition
Blocks dALA and Ferrochetalase
Term
Fetal RBC development during 2nd trimester
Definition
Fetal RBCs made in liver and spleen at this time
Term
Fetal RBCs made here at week 3
Definition
Embryonic visceral mesoderm gives rise to angioblasts intravascularly at this time
Term
Fetal yolk sack produces RBCs at this time
Definition
1st trimester RBC production happens here
Term
This mutation creates resistance to activated protein C, causing a hypercoaguable state
Definition
Factor V leiden mutation (Arg506Gln)
Term
This is a Vitamin K dependent serine protease that degrades factor Va and factor VIIIa
Definition
Protein C (depends on protein S for activation)
Term
Risk factors for this condition include: pregnancy, long air travel, obesity, smoking, oral contraceptives, surgery, trauma, cancer, COPD, antiphospholipid AB syndrome and can result in permanent damage to venous valves
Definition
Deep Vein Thrombosis
Term
Fibrin degradation product that is very sensitive (but not specific) for thrombis
Definition
D-Dimer
Term
Test that measures intrinsic arm of clotting cascade. Prolonged most easily when there are reduced levels of factor VIII or Factor IX. Used to measure effects of unfractionated Heparin
Definition
Activated Partial Thromboplastin Time (aPTT)
Term
May-Thurner Syndrome
Definition
It is easier to compress the left common iliac vein and cause a DVT due to course of vasculature.
Term
Platelet aggregation depends on these 3 factors
Definition
Depends on 1)adhesion to wall via vWF 2) release of ADP and 3)aggregation (partially due to TxA2)
Term
3 anti-aggregation factors
Definition
Factors include 1)PGI2 2)NO and 3) ADPase
Term
3 anti-coagulation factors
Definition
Factors include 1)Heparin sulfate 2) Protein C/S and 3)tPA
Term
Protein that carries factor VIII
Definition
von Willebrand's factor carries this clotting factor
Term
A dysfunction in this causes increased bleeding time, superficial and mucous membrane bleeds, petichiae and epistaxis
Definition
Effects of a dysfunction in platelet aggregation
Term
A dysfunction in this leads to late re-bleeds, hemarthrosis, menorrhagia and post-tooth extraction bleeding
Definition
Effects of a dysfunction in coagulation
Term
This thrombin induced enzyme activates Protein C
Definition
Thrombomodulin
Term
This condition causes 1) multi-organ failure from microthrombi 2) bleeding caused by consumption of platelets and fibrinogen and 3) secondary fibrinolysis
Definition
Effects of DIC
Term
What is the most common trigger for DIC?
Definition
The most common trigger for this is exposure of blood to tissue factor
Term
What is the light, blue-grey inclusion in periphery of PMN cytoplasm?
Definition
Dohle body
Term
What are the dark bodies in PMNs that suggest an inflammatory process?
Definition
Toxic granules
Term
What are the 7 causes of DIC?
Definition
The 7 causes of this condition are Sepsis, Trauma, Obstetric complications, Pancreatitis, Malignancy, Nephrotic syndrome and Transfusion. (Mnemonic: STOP Making New Thrombi)
Term
What is the general name for conditions that cause intravascular RBC fragmentation, resulting in schistocytes?
Definition
Description of Microangiopathic Hemolytic Anemias (including DIC, TTP and HUS)
Term
Caused by factor VIII deficiency
Definition
Hemophilia A (most common hemophilia. X-linked recessive)
Term
Caused by a factor IX deficiency
Definition
Hemophilia B (Christmas disease)
Term
Test that measures the extrinsic pathway: factor 7 and the common pathway. Use to measure Warfarin fx.
Definition
PT test measures this.
Term
Contact with collagen or other negatively charged substances (like ground glass) activate this arm of the clotting cascade
Definition
These activate the intrinsic arm of the clotting cascade
Term
This substance activates the extrinsic pathway of the clotting cascade (measured with PT test)
Definition
Tissue factor activates this arm of the clotting cascade
Term
Site of iron absorption
Definition
Duodenom absorbs this
Term
Cells store iron as:
Definition
Hemosiderin
Term
Iron absorption is regulated by:
Definition
Iron absorption is regulated by need, so ineffective erythropoiesis (ex. B-thalassemia) can cause iron overload
Term
Poikilocytosis (pencil shaped cells) are often seen in this anemia
Definition
These cells are often seen in iron deficiency anemia
Term
Anisocytosis is often seen on RDW in this micro-nutrient deficiency anemia
Definition
This feature is often seen on RDW in iron deficiency anemia
Term
Deficiency in these two micronutrients leads to pancytopenia and marrow hyperplasia
Definition
Signs of B12 and folate deficiency (megaloblastic anemias)
Term
These cells secrete Intrinsic Factor
Definition
Secreted by Parietal Cells in the stomach, binds to B12
Term
Signs of this condition include a 'beefy' tongue (atrophic glositis), "intestinalization" of the stomach, and spinal cord degeneration
Definition
Signs of Pernicious Anemia
Term
Ribosome precipitation results in this
Definition
Results in Basophilic Stipling (seen in lead poisoning)
Term
B12 - intrinsic factor complex is absorbed in this part of the intestines
Definition
This is absorbed in the Ileum (along with bile salts)
Term
This enzyme decreases Iron absorption
Definition
Hepcidin decreases absorption of this nutrient
Term
Elevated levels of serum Methyl-malonic acid indicate a deficiency in this nutrient
Definition
Blood levels indicate a B12 deficiency
Term
Hyperhomocysteinemia is caused by low levels of these nutrients, and cause an increased risk of DVT and vascular disease
Definition
High blood levels of this are caused by folate (B9) and B12 deficiency
Term
Vitamin B6 is a cofactor for production of this
Definition
Cofactor in the production of Heme
Term
Hyper-segmented PMNs are seen in this condition, along with increased erythropoietin and increased iron absorption
Definition
Features of megaloblastic anemia
Term
Two functions of vitamin B12
Definition
This nutrient converts Homocysteine to Methionine and converts methyl-THF to THF.
Term
This anemia results in a decreased TIBC and a normal serum ferritin, with increased Fe levels in the bone marrow
Definition
Anemia of chronic disease has these iron levels in the body
Term
What is the proximal cause of most RBC pathology in thalassemia?
Definition
RBC damage in thalassemia is caused by cell membrane damage (due to Heinz bodies and macrophages in the spleen)
Term
This anemia often results in an elevated RBC count and a decreased Hb level. Clumps of non-paired globin chains are seen.
Definition
Characteristics of thalassemias
Term
Characteristics of Thalassemia major
Definition
This disease results in splenomegaly, increased levels of HbF, bone distortions and hemosiderosis, and is fatal in childhood.
Term
Mutation of Sickle Cell disease
Definition
Glu6Val mutation (autosomal recessive)
Term
Factors that cause HbS precipitation in sickle cell anemia
Definition
Sickling caused by low O2 content, intracellular dehydration (increased MCHC) or a decrease in pH.
Term
Where are most sickled cells formed in Sickle Cell anemia?
Definition
Most of the pathologic cells are formed in the bone marrow or the spleen (slow vascular beds where pO2 is low)
Term
This test measures Ab's bound to RBC surface.
Definition
Direct Coomb's test
Term
This test measures unbound Ab's in the serum
Definition
Indirect Coomb's Test
Term
Autosomal disease causing an intrinsic defect in RBC membrane, due to loss of Ankyrin protein
Definition
Features of Hereditary Spherocytosis (a type of hemolytic anemia)
Term
Condition caused by an abnormality of the hexose monophosphate shunt
Definition
Cause of G-6-PD deficiency (inability to reduce glutathione)
Term
Acquired intrinsic defect in cell membrane that makes cells vulnerable to lysis by compliment and predisposes to DVT.
Definition
Paroxysmal Nocturnal Hemoglobinurea (PNH)
Term
In this condition IgG Ab's coat RBC's, and splenic macrophages remove chunks of RBC membrane. May be due to drugs acting as Haptens.
Definition
Warm Ab immunohemolytic anemia
Term
In this condition IgM Ab's bind and agglutinate RBC's, especially in the fingers, toes and ears. This leads to splenic destruction of RBCs.
Definition
Cold Agglutination Immunohemolytic anemia
Term
This condition is due to a failure of the bone marrow, leading to pancytopenia, and may be due to benzene, chloramphenicol, alkylating agents, radiation or vincristine (also caused by Parvovirus B19 in people with Sickle Cell anemia)
Definition
Causes of aplastic anemia
Term
Rare autosomal recessive disorder resulting in marrow hypoplasia and short stature
Definition
Fanconi Anemia
Term
This anemia is due to destruction of bone marrow, often from metastatic cancer from one of these 3 areas
Definition
Metastatic cancer from the breast, lung or prostate can do this
Term
A decrease in plasma volume (from dehydration) can cause this
Definition
Can show a relative polycythemia (showing an increase in hematocrit)
Term
What causes a febrile reaction following a blood transfusion?
Definition
You get a febrile reaction when the host has anti-HLA Ab's against donor leukocytes. Lysis releases pyogens.
Term
What causes a delayed hemolytic reaction to a blood transfusion?
Definition
Delayed hemolytic reactions to blood transfusions occur when the recipient has plasma cells that recognize donor RBC Ag's
Term
Blood type O mothers have what Ab's?
Definition
Type O mothers have anti-AB IgG's which can cross the placenta, causing a very mild anemia or jaundice in first 24 hours of life
Term
This situation can result in hydrops fetalis, kernicterus and severe anemia in babies
Definition
This occurs when the mother is Rh- and the fetus is Rh+ and the mother has been previously exposed to Rh+ blood. (Note that ABO incompatibility prevents Rh sensitization). Prevent sensitization with anti-D globulin.
Term
When would you give cryoprecipitate?
Definition
Give cryoprecipitate for coagulation disorders involving factor VIII or fibrinogen
Term
When would you give fresh frozen plasma?
Definition
Give this blood product to treat DIC or for Warfarin anti-coagulation
Term
When would you give platelets to someone?
Definition
Give this blood product for significant bleeding related to thrombocytopenia or a platelet defect (note that platelets lack Rh Ag).
Term
How do you treat a Heparin overdose?
Definition
Treat overdose with Protamine Sulfate
Term
This drug accelerates the activity of antithrombin
Definition
Mechanism of Heparin
Term
This drug blocks vitamin K dependent carboxylation of glutamate, takes several days to be effective, and can cross the placenta causing birth defects
Definition
Characteristics of Warfarin, which is measured using the INR (PT)
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