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67 Cards in this Set

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This is the measure of effective erythropoiesis, representing the bone marrow's response to anemia.
Reticulocyte Index
What would cause a decreased RI?
Any disease that prevents the production of red blood cells such as iron deficiency or thalassemia.
Ineffective erythropoiesis is a hallmark of which disease?
Name 4 diseases that cause a decreased RI:
1. Iron Deficiency, vitamin B12/folate
2. Thalassemia
3. Anemia of chronic disease
4. Aplastic Anemia
5. Myelofibrosis
Name two causes of increased RI:
1. Hemolytic Anemia (HS, Sickle cell, G6PD)
2. Massive blood loss
Name three microcytic anemias:
1. Thalassemia
2. Anemia of chronic disease
3. Iron Deficiency
Immunohemolytic anemia, hereditary sphereocytosis, G6PD anemia, sickle cell disease, chronic renal disease, aplastic anemia (pure red cell aplasia) and myelofibrosis all cause which kind of anemia?
What is the role of haptoglobin in hemolytic anemia?
Haptoglobin is a plasma protein in the blood that binds Hb released from lysed RBCs.
What happens to haptoglobin levels if there is hemolytic anemia occuring?
The levels of haptoglobin drop- moreso in intravascular hemolysis because it binds free Hb but if EV is severe it will also decrease dramatically.
Lysis of the RBCs in the splenic macrophages results in an efflux of _________.
In hemolytic anemia (intravascular or extravascular) what happpens to the RI?
It increases- there is nothing wrong with the bone marrow and so it works to replace the degraded RBCs.
Which type of hemolytic anemia shows schistocytes on blood smears, has low haptoglobin, and hemoglobinuria (hemosiderinurea, urobilinurea)?
Intravascular Hemolysis
Which type of hemolytic anemia has spherocytes present in peripheral smears and has an efflux of unconjugated bilirubin?
Hereditary spherocytosis --> Extravascular hemolysis.
The principle place for removing most RBCs and is typically enlarged in most forms of hemolytic anemia.
Splenomegaly indicates which type of hemolysis? In which conditions would this occur?
Splenomegaly occurs in extravascular hemolysis- this occurs in spherocytosis, thalassemia, sickle cell, G6PD, etc.
Does aplastic anemia cause splenomegaly?
No - disease of production in the bone marrow.
Mutation of ankrin is the most common cause of this disease;
Hereditary Spherocytosis (HS)
Cells in this condition are abnormally sensitive to osmotic lysis, and show increased mean cell hemoglobin due to dehydration. After splenectomy the spherocytes persist but the anemia is corrected.
Hereditary Spherocytosis (HS)
This is the most common enzyme deficiency causing hemolytic anemia:
G6PD deficiency
This disease causes anemia because it reduces the ability of red cells to protect themselves against oxidative damage and leads to hemolysis:
G6PD deficiency
The blood smear of this condition shows dark appearing spherocytes with no central pallor and Howell-Jolly bodies:
Hereditary Spherocytosis
The peripheral smear in this condition shows red cells with precipitates of denatured globin (heinz bodies) and bite cells whrere splenic macrophages have plucked out these inclusions:
G6PD deficiency
This disease is caused by a somatic mutation in the PIG-A gene:
Paroxysmal Nocturnal Hemoglobinuria
This disease is caused by an acquired membrane defect in myeloid stem cells which prevent the membrane anchoring of cell surface proteins that regulate complement fixation:
The only hemolytic anemia caused by an acquired genetic defect:
This disease is characterized by the tendency for RBCs to lyse at night due to a slight decrease in blood pH during sleep, which increases the activity of complement:
What is the leading cause of death related to PNH?
Venous thrombosis- caused by dysfunction of platelets due to the absence of certain GPI linked proteins
A screen for antibodies against red blood cells;
Coombs test
This is the most common form of immunohemolytic anemia: What is the most causitive antibody?
Warm antibody type - most antibodies are the IgG class
This condition is most commonly mediated by IgG antibodies (less commonly IgA) that coat RBCs and bind with the Fc receptor on phagocytes which partially digest the red cell membrane, forming spherocytes which causes extravascular hemolysis.
Warm Antibody Type of immunohemolytic anemia.
What disease is Warm Antibody Type of immunohemolytic anemia associated with?
SLE- autoimmune disorders
This is a less common form of immunohemolytic anemia that coats RBCs in IgM- sometimes occurs transiently following infection (pneumonia, EBV, CMV, flu, and HIV)
Cold agglutinin type immunohemolytic anemia
This is an uncommon form of immunohemolytic anemia that coats RBCs in IgG antibodies and activates below 37 degrees- occurs mainly in children following viral infection.
Cold Hemolysin type
In this disease all three cell lines are affected and bone marrow biopsy is negative for malignancy or infection, normocytic, normochromic anemia.
Aplastic Anemia
This disease is characterized by weakness, recurrent infections, or bleeding - bone marrow biopsy shows depletion of blood cell precursors with marrow replaced by fat or fibrous tissue:
Aplastic Anemia
Rare autosomal recessive disorder caused by defects in a multiprotein complex that is required for DNA repair- it is an inherited defect that would be present since birth (primary)
Fanconi's Anemia
Has the same bone marrow findings as aplatic anemia but is usually accompanied by multiple congenital abnormalities such as hypopasia of the kidneys or spleen and bone anomalies.
Fanconi's Anemia
Primary marrow disorder where only erythroid progenitors are suppressed- in severe cases RBC progenitors may be absent from marrow.
Pure red cell aplasia
This disease has an autoimmune basis with a lack of RBC precursors in bone marrow- other cell lines aren't affected.
Pure red cell aplasia
This disease is a specialized form of red cell aplasia that preferentially infects and destroys red cell progenitors- usually causes transient red cell aplasia
Parvoviral bone marrow suppression
This usually transient anemia is caused by a virus and may cause chronic red cell aplasia and anemia in immunoupressed patients
Parvoviral bone marrow suppression
This disease is caused by anti-platelet antibody that coats platelets that become phagocytosed by spleen macrophages
Idiopathic Thrombocytopenic Purpura (ITP)
This disease has a blood smear that shows v. low platelets- may see some megathrombocytes. Pts are well appearing with bruising and petechiae - usually seen in children and the elderly
Idiopathic Thrombocytopenic Purpura (ITP)
What is the treatment for ITP?
If serious, IVIg and steroids. If chronic, do splenectomy.
This disease is caused by abnormal von Willebrand factor multimers that diffusely activate platelets and cause intravascular hemolysis.
Thrombotic thrombocytopenic purpura (TTP)
Patients with this condition are very sick, usually have underlying cancer or sepsis - have abnormal vWf mutlimers.
The Philadelphia chromosome is a specific chromosomal abnormality that is associated with which condition?
Chronic Myelogenous Leukemia (CML)
This chromosomal abnormality is caused by a reciprocal translocation between chromosome 9 and 22
Philadelphia chromosome
This disease is associated with a chromosomal translocation of the c-myc gene
Burkitt's Lymphoma
This is a cancer causing virus in the Herpes family:
Epstein Barr virus
Infections with this virus cause glandular fever or infectious mononucleosis - in some cases changes B cells into cancer cells leading to the development of lymphoma
EBV causes which type of cancer?
Burkitt's Lymphoma
This translocation produces the oncogenic BCR-ABL gene fusion which activates a number of cell cycle controlling proteins and enzymes speeding up cell division:
Philadelpha chromosome translocation - translocation between 9 and 22.
This gene mutation causes rapid cell division and inhibits DNA repair, causing genomic instability and potentially causing the feared blast crisis in CML
The oncogenic BCR-ABL fusion- philadelphia chromosome
What is the difference in total iron binding capacity (TIBC) between iron deficiency and anemia of chronic disease? Why?
In chronic disease TIBC is low because the problem is not lack of iron but lack of access- in iron deficiency anemia TIBC is high because iron is needed.
What is the difference in ferritin levels in anemia of chronic disease and iron deficiency anemia? Why?
Ferritin is high in chronic disease b/c it is an acute phase reactant. Ferritin is low in iron deficiency anemia because there is no iron in storage,
In this disease, increased HbF for age is the only abnormality on electrophoresis:
Beta thalassemia
Autoantibodies responsible for paroxysmal cold hemoglobinurea- this rare disorder causes substantial, sometimes fatal intravascular hemolysis and hemoglobinurea.
Cold Hemolysin Type
The common theme in these types of anemia is impairment of DNA synthesis that leads to distinctive morphologic changes, including abnormally large erythroid precursors and red cells.
Megaloblastic Anemias
In alpha thalassemia, excess unpaired gamma-globin chains form ______.
Hemoglobin Barts - Seen in newborns with alpha-thal
In older children with alpha-thalassemia, excess beta chains form ______.
Hemoglobin H
This disease is caused by a deletion of three alpha globin genes and is most common in Asian populations.
Hemoglobin H disease
This disease causes patients to be very sick - causes bleeding and clotting at the same time due to diffuse activation of the clotting cascade and consumption of clotting factors due to underlying illness- has a prolonged PTT
Disseminated Intravascular Coagulation (DIC)
The chronic form of this condition is caused by autoantibodies to platelets and is associated with SLE:
Immune Thrombocytopenic Purpura (ITP)
What class are the overwhelming majority of antibodies in ITP?
In ITP what does the peripheral smear usually show?
Often it shows abnormally large platelets, which are a sign of accelerated thrombopoiesis
This condition is often seen in women under the age of 40, presents with bleeding into the skin and mucosal surfaces and petechiae which may give rise to ecchymosis: