Episode 8 Overview of Hematological Pathology Flashcards

1
Q

What is anemia?

A

A reduction one or more of the major RBC measurements - Hg concentration, hematocrit, and/or RBC count

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2
Q

What is the most common cause of anemia worldwide?

A

Iron deficiency

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3
Q

What is the result of defective Hb synthesis on RBC formation?

A

Microcytic (MCV is low) and Hypochromic (low MCH and MCHC)

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4
Q

Why do pencil cells appear in a blood smear?

A

Unbalanced surface membrane to cytoplasmic volume due to reduced Hb

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5
Q

What does a reduced rate of Hb synthesis result in?

A

More cell division and smaller cells

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6
Q

How do lower levels of Hb affect the appearance of RBCs?

A

They look pale

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7
Q

What are the major causes of iron deficiency?

A

Blood loss, decreased iron absorption (GI tract problem or drug induced), Dietary, Increased iron requirements (pregnancy and growth spurts)

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8
Q

What is the effect of vitamin B12 deficiency on RBCs?

A

RBCs become macrocytic (MCV is elevated)

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9
Q

What are enlarged RBCs sometimes referred to as?

A

Megaloblasts - “megalobalstic anemia”

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10
Q

Which is affected by vitamin B12 - RNA or DNA?

A

DNA, RNA is unaffected and protein synthesis continues and accumulates making the RBC larger

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11
Q

What are the main causes of Vitamin B12 deficient anemia?

A

Lack of intrinsic factor (IF), dietary, and gastrectomy or other stomach procedures

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12
Q

What does the lack of IF lead to?

A

Decreased absorption of vitamin B12

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13
Q

What is pernicious anemia?

A

Vitamin B12 deficiency specifically due to decreased IF

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14
Q

What are the causes in a lack of IF?

A

Lack of gene, autoimmune attack on IF or on the Parietal cells in the stomach

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15
Q

What does an attack on parietal cells produce, besides lack of IF?

A

Chronic atrophic gastritis

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16
Q

T/F Vitamin B12 deficiency and folate deficiency both produce very similar RBCs under the microscope

A

True, both produce increased MCV

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17
Q

Does folate absorption require IF?

A

No, only Vit. B12 does

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18
Q

What are the potential causes of folate deficiency?

A

Poor nutrition, increased requirements (pregnancy), malabsorption (inflammatory diseases of the intestines), Drugs interfering with absorption

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19
Q

What are the general features of anemia due to acute blood loss?

A

Increased HR and Low BP

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20
Q

Why would Hb concentration and a full blood count taken immediately after acute blood loss be normal?

A

B/c RBCs and plasma are lost together

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21
Q

When MCV remains normal during this is called what?

A

Normocytic

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22
Q

Where must the movement of fluid go to develop anemia after acute blood loss?

A

From the extravascular space to the intravascular

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23
Q

What is anemia due to chronic blood loss most often associated with in the developed world?

A

Iron deficient anemia

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24
Q

What are examples of anemia due to chronic blood loss?

A

Unrecognized loss from a bleed gastric ulcer or heavy menstrual flow

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25
Q

What is typical of anemia of chronic disease?

A

It is typically normocytic

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26
Q

Why would chronic renal failure cause anemia?

A

Kidney’s produce erythropoeitin, w/o which RBCs production cannot respond to falling oxygen levels in tissues

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27
Q

What is a hemolytic anemia?

A

Disorder in which the RBCs are destroyed faster than normal - bone marrow cannot keep pace with the rate of destruction

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28
Q

How few days may RBCs live when someone has hemolytic anemia?

A

As few as 20 days

29
Q

What is the autoimmune response directed against in Acquired Autoimmune Hemolytic Anemia?

A

RBCs own antigens

30
Q

Virtually all hereditary anemias are what?

A

Hemolytic

31
Q

Inherited anemias may be classified into what three major groups of disorders?

A

Those affecting:

1) RBC membrane - spherocytosis, elliptocytosis
2) RBC enzymes - G6PD deficiency
3) Hb molecules - sickle cell, thalassemia

32
Q

What defects cause hereditary spherocytosis?

A

Molecular defects in genes that code for Ankryn, Protein 4.2, Spectrin, Band 3 protein

33
Q

What is the most helpful RBC index in hereditary spherocytosis?

A

Mean Cell Hemoglobin Concentration (MCHC), which is routinely elevated

34
Q

What does an elevated MCHC indicate?

A

membrane loss and RBC dehydration

35
Q

What does an elevated RDW favor the diagnosis of?

A

hereditary spherocytosis

36
Q

What is the difference between Hereditary Elliptocytosis (HE) and pencil cells in iron deficient anemia?

A

Pencil cells in iron deficient anemia appear occasionally by with HE the vast majority of cells look elliptical

37
Q

When would an Osmotic Fragility Test (OFT) be performed?

A

When one is suspected of having spherocytosis or elliptocytosis

38
Q

What is the OFT?

A

Measure of RBC lysis in an NaCl concentration at which 50% lysis occurs - spherocytes and elliptocytes lyse more readily than normal RBCs

39
Q

G6PD is the only source of what in RBCs?

A

NADPH, which maintains glutathione in its reduced form

40
Q

The RBCs of G6PD often show what characteristics?

A

Heinz Bodies (a cluster of denatured Hb) and Bite cells (removal of HBs afterr processing thru the spleen)

41
Q

Where are HBs usually located on an RBC?

A

Periphery - look like olives with pits

42
Q

What is the sickle cell anemia a result of?

A

Point mutation in the Beta globin gene - resulting in the change of amino acid number 6 - Glutamate to Valine

43
Q

What is Thalassemia?

A

Unbalanced globin chain production

44
Q

Where is thalassemia prevalent?

A

Some of the same areas as sickle cell anemia - Africa, Mediterranean, Middle East, India, and Asia

45
Q

How is thalassemia classified?

A

Classified after the gene affected - alpha-thalassemia if the alpha-globin gene is altered, or beta-thalassemia etc

46
Q

What is the consequence of thalassemia?

A

RBCs that are microcytic and hypochromic

47
Q

What cells get cancer in Leukemia?

A

WBC precursors

48
Q

What are the two classic leukemia forms?

A

Acute and chronic

49
Q

What happens in leukemia?

A

progressive accumulation of abnormal WBCs in the bone marrow and other organs, which spill out into the peripheral blood; progressive bone marrow failure with a reduction in other normal cell types. Tend to have reduced number of RBCs and Megakaryocytes

50
Q

What is Acute Myeloid Leukemia (AML)?

A

Cancer of myeloid line of stem cells. Rapid growth of abnormal WBCs that accumulate in bone marrow an interfere with production of normal blood cells

51
Q

What is the most common acute leukemia?

A

AML

52
Q

What are the symptoms of AML?

A

Tiredness, infections, bruising, bone pain, respiratory and neurological symptoms

53
Q

What are the symptoms of AML caused by?

A

drop in RBCs, platelets, and normal WBCs

54
Q

What is acute lymphoblastic leukemia (ALL)?

A

cancer of lymphoid line of stem cells

55
Q

At what age is ALL most common? AML?

A

ALL is most common in ages 2-5. AML is most common in adults and increases with age

56
Q

What is the difference b/w acute (AML) and chronic (CML) myeloid leukemia?

A

CML has the presence of the Philadelphia chromosome

57
Q

What is the Philadelphia chromosome?

A

Translocation in which two parts of two chromosomes 9 and 22 are swapped

58
Q

What does the Philadelphia chromosome mutation lead to?

A

Transcription of proteins with high tyrosine kinase activity

59
Q

What does the drug Gleevec treat?

A

CML

60
Q

What are three phases of CML?

A

Chronic phase, Accelerated phase, and Blast Crisis

61
Q

Where is the Chronic Lymphoblastic Leukemia most common?

A

Western societies w/ a peak incidence in pts b/w 60-80 and a male:female ratio of 2:1

62
Q

CLL is a slow-growing disorder characterized by progressive accumulation of cancerous cells where?

A

Bone marrow, spleen, liver, and lymph nodes

63
Q

What is the difference between leukemia and lymphoma?

A

Leukemia is a cancer of WBC precursors that occurs in the bone marrow. Lymphoma is a solid mass tumor that can originate w/in many different lymphoid tissues - lymph nodes (most common), spleen, liver, GI tract, thymus or bone marrow

64
Q

What are the symptoms of Hodgkin’s lymphoma?

A

Itchy skin, night sweats, unexplained weight loss, enlarged lymph nodes, splenomegaly, hepatomegaly

65
Q

What type of cells are characteristically seen in biopsies of Hodgkin’s lymphoma?

A

Reed-Sternberg cells - AKA “popcorn cells”

66
Q

What are Reed-Sternberg cells - AKA “popcorn cells”?

A

B lymphocytes that have lost the ability to produce antibodies

67
Q

What is the prognosis for Hodgkin’s Lymphoma?

A

Good prognosis (unlike Non-Hodgkin’s Lymphoma)

68
Q

What is Non-Hodgkin’s Lymphoma (NHL)?

A

A group of lymphoma’s that includes everything Hodgkin’s Lymphoma. No good prognosis for treatment or cure