Pathology Tbl 3: Hematopathology Block I

Front 1

This is the measure of effective erythropoiesis, representing the bone marrow's response to anemia.

Back 1

Reticulocyte Index

Front 2

What would cause a decreased RI?

Back 2

Any disease that prevents the production of red blood cells such as iron deficiency or thalassemia.

Front 3

Ineffective erythropoiesis is a hallmark of which disease?

Back 3

Thalassemia

Front 4

Name 4 diseases that cause a decreased RI:

Back 4

1. Iron Deficiency, vitamin B12/folate
2. Thalassemia
3. Anemia of chronic disease
4. Aplastic Anemia
5. Myelofibrosis

Front 5

Name two causes of increased RI:

Back 5

1. Hemolytic Anemia (HS, Sickle cell, G6PD)
2. Massive blood loss

Front 6

Name three microcytic anemias:

Back 6

1. Thalassemia
2. Anemia of chronic disease
3. Iron Deficiency

Front 7

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?

Back 7

Normocytic

Front 8

What is the role of haptoglobin in hemolytic anemia?

Back 8

Haptoglobin is a plasma protein in the blood that binds Hb released from lysed RBCs.

Front 9

What happens to haptoglobin levels if there is hemolytic anemia occuring?

Back 9

The levels of haptoglobin drop- moreso in intravascular hemolysis because it binds free Hb but if EV is severe it will also decrease dramatically.

Front 10

Lysis of the RBCs in the splenic macrophages results in an efflux of _________.

Back 10

Bilirubin

Front 11

In hemolytic anemia (intravascular or extravascular) what happpens to the RI?

Back 11

It increases- there is nothing wrong with the bone marrow and so it works to replace the degraded RBCs.

Front 12

Which type of hemolytic anemia shows schistocytes on blood smears, has low haptoglobin, and hemoglobinuria (hemosiderinurea, urobilinurea)?

Back 12

Intravascular Hemolysis

Front 13

Which type of hemolytic anemia has spherocytes present in peripheral smears and has an efflux of unconjugated bilirubin?

Back 13

Hereditary spherocytosis --> Extravascular hemolysis.

Front 14

The principle place for removing most RBCs and is typically enlarged in most forms of hemolytic anemia.

Back 14

Spleen

Front 15

Splenomegaly indicates which type of hemolysis? In which conditions would this occur?

Back 15

Splenomegaly occurs in extravascular hemolysis- this occurs in spherocytosis, thalassemia, sickle cell, G6PD, etc.

Front 16

Does aplastic anemia cause splenomegaly?

Back 16

No - disease of production in the bone marrow.

Front 17

Mutation of ankrin is the most common cause of this disease;

Back 17

Hereditary Spherocytosis (HS)

Front 18

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.

Back 18

Hereditary Spherocytosis (HS)

Front 19

This is the most common enzyme deficiency causing hemolytic anemia:

Back 19

G6PD deficiency

Front 20

This disease causes anemia because it reduces the ability of red cells to protect themselves against oxidative damage and leads to hemolysis:

Back 20

G6PD deficiency

Front 21

The blood smear of this condition shows dark appearing spherocytes with no central pallor and Howell-Jolly bodies:

Back 21

Hereditary Spherocytosis

Front 22

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:

Back 22

G6PD deficiency

Front 23

This disease is caused by a somatic mutation in the PIG-A gene:

Back 23

Paroxysmal Nocturnal Hemoglobinuria

Front 24

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:

Back 24

PNH

Front 25

The only hemolytic anemia caused by an acquired genetic defect:

Back 25

PNH

Front 26

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:

Back 26

PNH

Front 27

What is the leading cause of death related to PNH?

Back 27

Venous thrombosis- caused by dysfunction of platelets due to the absence of certain GPI linked proteins

Front 28

A screen for antibodies against red blood cells;

Back 28

Coombs test

Front 29

This is the most common form of immunohemolytic anemia: What is the most causitive antibody?

Back 29

Warm antibody type - most antibodies are the IgG class

Front 30

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.

Back 30

Warm Antibody Type of immunohemolytic anemia.

Front 31

What disease is Warm Antibody Type of immunohemolytic anemia associated with?

Back 31

SLE- autoimmune disorders

Front 32

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)

Back 32

Cold agglutinin type immunohemolytic anemia

Front 33

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.

Back 33

Cold Hemolysin type

Front 34

In this disease all three cell lines are affected and bone marrow biopsy is negative for malignancy or infection, normocytic, normochromic anemia.

Back 34

Aplastic Anemia

Front 35

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:

Back 35

Aplastic Anemia

Front 36

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)

Back 36

Fanconi's Anemia

Front 37

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.

Back 37

Fanconi's Anemia

Front 38

Primary marrow disorder where only erythroid progenitors are suppressed- in severe cases RBC progenitors may be absent from marrow.

Back 38

Pure red cell aplasia

Front 39

This disease has an autoimmune basis with a lack of RBC precursors in bone marrow- other cell lines aren't affected.

Back 39

Pure red cell aplasia

Front 40

This disease is a specialized form of red cell aplasia that preferentially infects and destroys red cell progenitors- usually causes transient red cell aplasia

Back 40

Parvoviral bone marrow suppression

Front 41

This usually transient anemia is caused by a virus and may cause chronic red cell aplasia and anemia in immunoupressed patients

Back 41

Parvoviral bone marrow suppression

Front 42

This disease is caused by anti-platelet antibody that coats platelets that become phagocytosed by spleen macrophages

Back 42

Idiopathic Thrombocytopenic Purpura (ITP)

Front 43

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

Back 43

Idiopathic Thrombocytopenic Purpura (ITP)

Front 44

What is the treatment for ITP?

Back 44

If serious, IVIg and steroids. If chronic, do splenectomy.

Front 45

This disease is caused by abnormal von Willebrand factor multimers that diffusely activate platelets and cause intravascular hemolysis.

Back 45

Thrombotic thrombocytopenic purpura (TTP)

Front 46

Patients with this condition are very sick, usually have underlying cancer or sepsis - have abnormal vWf mutlimers.

Back 46

TTP

Front 47

The Philadelphia chromosome is a specific chromosomal abnormality that is associated with which condition?

Back 47

Chronic Myelogenous Leukemia (CML)

Front 48

This chromosomal abnormality is caused by a reciprocal translocation between chromosome 9 and 22

Back 48

Philadelphia chromosome

Front 49

This disease is associated with a chromosomal translocation of the c-myc gene

Back 49

Burkitt's Lymphoma

Front 50

This is a cancer causing virus in the Herpes family:

Back 50

Epstein Barr virus

Front 51

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

Back 51

EBV

Front 52

EBV causes which type of cancer?

Back 52

Burkitt's Lymphoma

Front 53

This translocation produces the oncogenic BCR-ABL gene fusion which activates a number of cell cycle controlling proteins and enzymes speeding up cell division:

Back 53

Philadelpha chromosome translocation - translocation between 9 and 22.

Front 54

This gene mutation causes rapid cell division and inhibits DNA repair, causing genomic instability and potentially causing the feared blast crisis in CML

Back 54

The oncogenic BCR-ABL fusion- philadelphia chromosome

Front 55

What is the difference in total iron binding capacity (TIBC) between iron deficiency and anemia of chronic disease? Why?

Back 55

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.

Front 56

What is the difference in ferritin levels in anemia of chronic disease and iron deficiency anemia? Why?

Back 56

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,

Front 57

In this disease, increased HbF for age is the only abnormality on electrophoresis:

Back 57

Beta thalassemia

Front 58

Autoantibodies responsible for paroxysmal cold hemoglobinurea- this rare disorder causes substantial, sometimes fatal intravascular hemolysis and hemoglobinurea.

Back 58

Cold Hemolysin Type

Front 59

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.

Back 59

Megaloblastic Anemias

Front 60

In alpha thalassemia, excess unpaired gamma-globin chains form ______.

Back 60

Hemoglobin Barts - Seen in newborns with alpha-thal

Front 61

In older children with alpha-thalassemia, excess beta chains form ______.

Back 61

Hemoglobin H

Front 62

This disease is caused by a deletion of three alpha globin genes and is most common in Asian populations.

Back 62

Hemoglobin H disease

Front 63

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

Back 63

Disseminated Intravascular Coagulation (DIC)

Front 64

The chronic form of this condition is caused by autoantibodies to platelets and is associated with SLE:

Back 64

Immune Thrombocytopenic Purpura (ITP)

Front 65

What class are the overwhelming majority of antibodies in ITP?

Back 65

IgG

Front 66

In ITP what does the peripheral smear usually show?

Back 66

Often it shows abnormally large platelets, which are a sign of accelerated thrombopoiesis

Front 67

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:

Back 67

ITP