Blood Disorders

Question 1

What cell types are found in the buffy coat?

Answer 1

White blood cells

Platelets

Question 2

Hemoglobin

Answer 2

Complex molecule consisting of 4 heme groups and 4 globins
Always 2 alpha chains, adult has beta, fetus has gamma
Purpose is to transport oxygen
Need iron!
Gets broken down to bilirubin
Live in circulation for 120 days

Question 3

What 4 things are needed for hemoglobin synthesis?

Answer 3

Iron
Vitamin B12
Vitamin B6
Folic acid

Question 4

How is hemoglobin degraded?

Answer 4

Split into heme and globin
Globin is degraded further into amino acids which are reutilized
Heme is broken into iron and bilirubin (iron reused, bilirubin to liver and turned into bile)

Question 5

What is anemia?

Answer 5

Reduction of hemoglobin in blood to below normal levels
May be associated with appearance of abnormal hemoglobin, reduced number of RBCs, or structural abnormalities of RBCs
Decreased hematopoiesis, abnormal hematopoiesis, or increased loss/destruction of RBCs

Question 6

2 causes of decreased hematopoiesis

Answer 6

Bone marrow failure (aplastic anemia or myelophthisic anemia)
Deficiencies of nutrients (low B12 and folic acid, or protein deficiency)

Question 7

Myelophthistic anemia

Answer 7

Bone marrow stem cells are damaged or replaced by infiltrates of metastatic tumors cells

Question 8

Cause of abnormal hematopoiesis

Answer 8

Usually a consequence of genetic abnormalities

Ex: sickle cell anemia

Question 9

4 causes of anemia from increased loss and destruction of RBCs

Answer 9

Bleeding
Intrasplenic sequestration
Immune hemolysis
Infections (malaria)

Question 10

Aplastic anemia

Answer 10

Idiopathic, secondary
Bone marrow depleted of hematopoetic cells - only fibroblasts, fat cells, and scattered lymphocytes
Causes anemia, leukopenia, and thrombocytopenia
Uncontrollable infections, bleeding tendency, chronic fatigue, sleepiness, weakness
Stem cells disappear from the bone marrow with consequent pancytopenia (lack of all blood cells) in the periphery

Question 11

Iron deficiency anemia

Answer 11

Most common form of anemia
Hypochromic, microcytic anemia
Etiology: increased loss of iron, inadequate iron intake or absorption, increased iron requirements

Question 12

Megaloblastic anemia
(caused by, morphology, what are the deficiencies from, effects on bone marrow/peripheral blood/neutrophils)

Answer 12

Caused by deficiency of vitamin B12 or folic acid
Opposite morphology from iron deficiency anemia (large red cells)
Lack of B12 from: pernicious anemia, lack of GIF, atrophic gastritis
Lack of folic acid from: inadequate intake in diet or malabsorption caused by intestinal disease
Bone marrow gets hypercellular with numerous megaloblasts
Macrocytic anemia in peripheral blood
Hypersegmentation of neutrophils

Question 13

Definitions

1. Hypochromic
2. Microcytic
3. Macrocytic

Answer 13

1. Pale red cells
2. Small red cells
3. Large red cells

Question 14

Hemolytic Anemia

Answer 14

Increased red blood cell destruction (hemolysis)
Intra and extra cellular defects
Compensatory erythroid hyperplasia of bone marrow
Hyperbilirubinemia, jaundice

Question 15

Intracorpuscular defects for hemolytic anemia

Answer 15

Structural abnormalities
Sickle cell anemia, thalassemia, hereditary spherocytosis
Infection (malaria)

Question 16

Extracorpuscular defects for hemolytic anemia

Answer 16

Antibodies, infectious agents, mechanical factors
Autoimmune hemolytic anemia, hemolytic disease of the newborn, transfusion reactions, hemolytic anemia caused by cardiac valve prosthesis, DIC

Question 17

Sickle cell anemia

Answer 17

Substitution of glutamic acid by valine
Synthesis of abnormal beta chain of globin
Multiple infarcts in various organs (neurologic defects, sharp pain in bones, spleen, extremities, retinal infarcts)
Hyperbilirubinemia, jaundice

Question 18

Long term complications of sickle cell anemia

Answer 18

Delayed intellectual development, neurologic deficits (largely avoided)
Cardiopulmonary insufficiency
Recurrent infections

Question 19

Thalassemia

Answer 19

Genetic defect in synthesis of hemoglobin A, reduced rate of globin chain synthesis
No abnormal hemoglobin produced
Beta and alpha types depending on what chain has reduced synthesis
Calvarium, hyperbilirubinemia, jaundice
Chronic anemia that slows children’s growth
Impairment of intellectual development
Cardiorespiratory insufficiency

Question 20

Thalassemia minor/trait vs thalassemia major

Answer 20

Minor: heterozygotes, mild non specific symptoms
Major: homozygotes, severe serious disease

Question 21

Calvarium

Answer 21

“Crew cut” hair on readiographic study
There is a lot of hematopoiesis happening in the skull which is abnormal
In thalassemia

Question 22

Definitions:

1. Autoantigens
2. Alloantigens
3. Neoantigens

Answer 22

1. Antibodies you make against yourself
2. Against someone else’s RBCs
3. When 2 things come together. Ex: penicillin gets absorbed into red cell membranes and forms something that causes antibodies to form

Question 23

Polycythemia (difference between primary and secondary)

Answer 23

Increased numbers of RBCs
Primary (polycythemia vera): clonal proliferation of hematopoietic stem cells resulting in an uncontrolled production of RBCs and an increased total red blood cell mass - erythropoietin INdependent
Secondary: increased RBC volume because of erythroid bone marrow hyperplasia caused by erythropoietin - caused by hypoxia

Question 24

Symptoms of Polycythemia

Answer 24

Hypertension
Dark red or flushed face
Headaches, visual problems, neurologic symptoms
Splenomegaly
Hypercellular bone marrow

Question 25

4 leukocytic disorders

Answer 25

Leukopenia Leukocytosis Leukemia Lymphoma

Question 26

Leukopenia

Answer 26

Reduction in WBC count Neutropenia due to bacterial infections or drugs Lymphopenia due to bacterial, viral, fungal, or parasitic infections

Question 27

Leukocytosis

Answer 27

Increased number of WBCs in peripheral blood Neutrophilia from bacterial infection Eosinophilia: allergies, some skin diseases, or parasitic infections Lymphocytosis: viral infections, chronic infections, some autoimmune disorders Splenomegaly, lymphadenopathy

Question 28

Leukemia

Answer 28

Malignant disease involving WBCs or their precursors in bone marrow, peripheral blood (acute and chronic) Can be myeloid or lymphoid Bone marrow overrun with leukemia cells Increased number of immature blood cells in peripheral blood Complications: anemia, recurrent infections, uncontrollable bleeding Doesn't make red/white cells or platelets normally

Question 29

Lymphomas

Answer 29

Lymphoid cell malignant diseases predominantly involving lymph nodes Non-Hodgkins or Hodgkins

Question 30

Plasma cell myeloma

Answer 30

Malignant disease of plasma cells

Question 31

Non-Hodgkin's vs Hodgkin's lymphoma

Answer 31

NH: B, T, or NK cells H: B cell only

Question 32

Translocations: 1. 8,14 2. 9,22 3. 15, 17

Answer 32

1. Burkitt's lymphoma 2. Chronic myelogenous leukemia (Philadelphia chromosome) 3. Acute promyelocytic leukemia

Question 33

Acute lymphoblastic leukemia

Answer 33

Most common form of leukemia in children Almost always B cell origin Massive infiltration of bone marrow, peripheral blood with immature lymphoid cells Symptoms: recurrent infections, weakness, bleeding into skin and major internal organs Treatment: chemo - remission possible in nearly all patients

Question 34

Acute Myelogenous Leukemia

Answer 34

Most common form of acute leukemia in adults Clonal proliferation of myeloid precursors in bone marrow 20% abnormal cells, or cytogenetic abnormalities Without treatment, most patients die within 6 months after symptom onset

Question 35

Chronic Myelogenous Leukemia

Answer 35

Bone marrow, peripheral blood overrun with neutrophils and their precursors 3 phases: chronic, accelerated, blast crisis Treatment: tyrosine kinase inhibitors Philadelphia chromosome, with BCR-ABL gene rearrangment t(9;22)

Question 36

Chronic lymphocytic leukemia

Answer 36

Malignant disease involving lymphocytes (almost all B cells) Most patients over 50 Should be suspected if lymphocytes greater than 5000/mL Median survival: 7-9 years from diagnosis

Question 37

Non-Hodgkin's Lymphomas

Answer 37

No benign lymphomas Most have B-cell phenotype All age groups affected (more common in adults than children) Can spill over into blood; present as leukemia Involve lymph nodes, bone marrow, spleen, thymus, but can be of extranodal origin

Question 38

Follicular lymphoma

Answer 38

Most common form of lymphoma in US Mostly older people Slow growing Presents with long-standing enlargement of lymph nodes, mild constitutional symptoms Median survival: 7-9 years after diagnosis

Question 39

Diffuse Large-Cell Lymphomas

Answer 39

Most common aggressive form of NHL Tissue in filtrated by large lymphoid cells with irregular nuclear outlines, prominent nucleoli Complete remission induced in 75% of patients with chemotherapy

Question 40

Is it easier to treat fast or slow growing cancers? Why?

Answer 40

Fast growing | If they grow more rapidly they are more sensitive to the chemos

Question 41

Burkitt's Lymphoma

Answer 41

Highly malignant tumor composed of small B-cells Extranodal masses: more prominent than enlarged lymph nodes Endemic and sporadic variant Most children and young adults can be cured

Question 42

Hodgkin’s Lymphoma

Answer 42

Bimodal age distribution curve: peaks at 25 and 55 years Five types Reed-Sternberg cells present Clinically: enlarged lymph nodes, rarely extranodal involvement or leukemic spread

Question 43

5 types of HL

Answer 43

``` Nodular sclerosing Lymphocyte rich Mixed cellularity Lymphocyte depleted Lymphocyte predominant (non classical) ```

Question 44

Classical vs Nonclassical HL

Answer 44

Reed-Sternberg cells present in classical only

Question 45

Reed-Sternberg cells

Answer 45

Bilobed or multilobed nucleus, prominent nucleoli surrounded by clear halo

Question 46

Plasma Cell Myeloma

Answer 46

``` Malignant disease of plasma cells Most patients older than 45 Proliferate in bone marrow Punched out holes in calvaria vertebrae Hypercalcemia, renal failure, anemia, leukopenia ```