Blood Disorders Flashcards

1
Q

Erythropoiesis:

A

synthesis of RBC

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2
Q

What are the organs that contribute to erythropoiesis?

A

Second trimester:
- liver
- spleen
- lymph nodes

Late third trimester & after birth:
- exclusively in bone marrow

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3
Q

What are the stages of differentiation of RBC?

A
  • Proerythroblast
  • Basophil erythroblast
  • Polychromatophil
  • Orthochromatic erythroblast
  • Reticulocyte
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4
Q

Proerythroblast:

A
  • the first cell in RBC series
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5
Q

Basophil erythroblast:

A
  • stain with basic dyes
  • low level of hemoglobin
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6
Q

Polychromatophil erythroblast:

A
  • increase in hemoglobin
  • condensation of nucleus
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7
Q

Orthochromatic erythroblast:

A
  • increase in hemoglobin
  • condensation of nucleus
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8
Q

Reticulocyte:

A
  • small amount of cytoplasmic organelle
  • nucleus absorbed or extruded
  • maturation to erythrocyte: 1-2 days
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9
Q

Which of the stages of differentiation of RBCs are in the BONE MARROW?

A

Proerythroblast

Basophil erythroblast

Polychromatophil erythroblast

Orthochromatic erythroblast

Reticulocyte

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10
Q

Which of the stages of differentiation of RBC’s are in the BLOOD (<1%)?

A

Reticulocyte

Erythrocytes

(shouldn’t be in bloodstream esp. immature ones for the most part, but if they are then there is an issue)

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11
Q

What are the major functions of RBC?

A
  • Transport hemoglobin (and O2)
  • Contain carbonic anhydrase (CO2 transport)
    CO2 + H2O CA H2CO3
  • Hemoglobin is an acid-base buffer
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12
Q

What is the regulation of RBC production?

A
  • Tissue oxygenation
  • high altitude (less O2 at high altitudes)
  • cardiac failure (when HF happens, not enough BF therefore O2)
  • lung diseases (b/c of low tissue levels of O2)
  • Erythropoietin
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13
Q

Normal life span of RBC is about _____

A

120 days (~4 months)

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14
Q

RBCs are destroyed in the ____

A

spleen

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15
Q

Hemoglobin is released and phagocytized by the ______ (liver, spleen, bone marrow)

A

macrophages

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16
Q

Hemoglobin is converted to _____ and conjugated in the _____

A

bilirubin

liver (& excreted through body in feces & urine)

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17
Q

Iron is released and bound to ______ in blood or stored in form of _____ in liver

A

transferrin

ferritin

(free iron is toxic in body)

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18
Q

What are the different types of hemoglobin?

A
  • HbA
  • HbA2
  • HbF
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19
Q

HbA:

A
  • major form of ADULT Hb
  • 2 alpha and 2 BETA chains
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20
Q

HbA2:

A
  • 2 alpha and 2 DELTA chains
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21
Q

HbF:

A
  • major type of FETAL Hb
  • 2 alpha and 2 GAMMA chains
22
Q

What do all the 3 types of Hb have in common?

A

have alpha-chains, therefore if problem with it, it can cause big issues

23
Q

Describe the lab assessment for a complete blood cell count (CBC)

A

includes the following measurements:
* Hb: expressed as grams of Hb per 100 ml blood * Hct: percentage of RBC to total blood volume
males > females
* MCV: mean cell volume
* MCH: mean cell hemoglobin
* MCHC: average concentration of hemoglobin in a given volume of packed RBC
* WBC, RBC, platelet count

24
Q

What is Anemia?

A

Anemia is defined as reduced number of RBC (↓Hb, ↓Hct)

25
Q

What is the classification of anemia based on RBC morphology?

A

SIZE:
- normocytic
- microcytic
- macrocytic

Hb level:
- normochromic
- hypochromic

26
Q

What are the clinical symptoms of anemia?

A
  • weakness
  • paleness (if # of RBCs decrease, it leads to paleness)
  • malaise
  • fatigue
  • dyspnea (↓ O2 content)
  • spoon shape nail
  • myocardial hypoxia
  • CNS symptoms (may cause faint) - b/c not enough O2 to brain
  • kidney hypoxia
27
Q

What is the classification of anemia based on underlying mechanism?

A

1) Blood loss:
- acute (trauma)
- chronic (GI tract lesions, gynecologic disturbances)

2) Increased rate of destruction (hemolytic anemia) A) Hereditary
- membrane disorders
- enzyme deficiencies
- deficient globin synthesis (thalassemia)
- structurally abnormal globin (sickle cell anemia))

B) Acquired (means there’s a factor in the body)
- antibody mediated (blood transfusions)
- autoantibodies (Systemic Lupus Erythematosus) - infections (Malaria - parasite that lives in RBC & can cause anemia over time)

3) Impaired RBC production
A) ↓ Proliferation / differentiation of stem cells
- aplastic anemia
- renal failure (b/c no prod. of erythropoietin in kidney)
- endocrine disorders

B) ↓ Maturation of erythroblasts
● defective DNA synthesis
- Vitamin B12 deficiency
- Folic acid deficiency
● ↓ Hb synthesis:
- ↓ heme synthesis (iron deficiency)
- ↓ globin synthesis (thalassemia)

28
Q

Describe Anemia of acute blood loss

A
  • normocytic normochromic anemia
  • caused by external or internal bleeding
  • loss of intravascular volume
  • may lead to cardiovascular collapse, shock, and death
  • blood volume is rapidly restored by shift from interstitial fluid
  • renal hypoxia causes ↑erythropoietin
  • increase in reticulocytes after a few days
  • leukocytosis (mobilization of granulocytes)
29
Q

Describe Hemolytic anemia

A

share the following features:
- shortened RBC life span
- increase in erythropoietin
- increase in erythropoiesis in bone marrow
- accumulation of products of Hb catabolism

(rate of destruction is increased, therefore lifespan of RBC are shorter)

30
Q

What is Sickle cell anemia?

A
  • Is caused by production of a defective Hb (HbS)
  • A point mutation in beta globin chain
  • Higher incidence in black Americans
  • Protects against malaria
31
Q

What does Sickle cell anemia look like?

A
  • When deoxygenated, HbS molecules aggregate and forms polymers
  • Defective RBCs: phagocytosis in spleen
  • ↑ Bilirubin
  • Enlarged spleen (tries to make more RBC to compensate)
  • Bone marrow hypreplasia
  • Chronic hypoxia may cause organ damage (spleen, heart, kidney, lung)
  • Autosplenectomy (infarction, fibrosis) (spleen is enlarged & then becomes smaller & smaller)
32
Q

What is Thalassemia?

A
  • ↓ Synthesis of alpha or beta globin chains * Microcytic hypochromic anemia
  • ↑ RBC destruction
  • ↑ Erythropoietin (due to anemia)
  • Erythroid hyperplasia in bone marrow
  • Extramedullary hematopoiesis (liver, spleen)
  • ↑ Iron absorption (tries to make more RBCs - precipitates in diff tissues & damages)
  • ↑ HbF and HbA2 in thalassemia major
33
Q

What are the types of Thalassemia?

A

β-thalassemia

α-thalassemia

34
Q

β-thalassemia:

A

classified into 2 categories:
β0: total absence of β chain β+: ↓β chain synthesis

Surgically removed spleen of a thalassemic child (15 times larger than normal)

35
Q

α-thalassemia:

A
  • Reduced or absent synthesis of α-globin chains
  • α-thalassemia major is lethal (all Hbs contain α-chain)
36
Q

Describe Impaired RBC production

A

A) Megaloblastic anemia
* Vitamin B12 deficiency (pernicious anemia)
* Folic acid deficiency

37
Q

What is Megaloblastic anemia characterized by?

A
  • impaired DNA synthesis
  • normochromic macrocytic anemia
  • decrease in RBC, WBC, platelets
  • large hypersegmented neutrophils
  • bone marrow is hypercellular (b/c trying to make more RBCs)
  • ineffective hematopoiesis and ↑RBC destruction
38
Q

What are the causes of Vitamin B12 deficiency?

A
  • Decreased intake
  • inadequate diet
  • vitamin B12 cannot be produced by human body, must
    be obtained from diet
  • impaired absorption
  • Intrinsic factor deficiency
  • intrinsic factor is produced by
    parietal cells of gastric mucosa
  • pernicious anemia (antibodies
    to parietal cells & intrinsic factor
  • gastrectomy
39
Q

food
–> ____

A

Vit B12 - protein –>
released by proteases in small intestine –>
Vit B12 - intrinsic factor –> absorbed in ileum

40
Q

What is a Folic acid deficiency?

A
  • Humans are dependent on diet folic acid.
  • Causes of folic acid deficiency:
  • Decreased intake
  • inadequate diet
  • impaired absorption
  • Increased loss or need
  • hemodialysis (kidney disease) - increased need (pregnancy)
41
Q

What is Iron deficiency anemia?

A
  • Most common nutritional disorder (children, infants, females)
  • Microcytic hypochromic anemia
  • ↓Erythroid progenitors
  • Iron is absorbed in duodenum
  • Free iron is highly toxic
  • Iron is bound to ferritin or transferrin
  • Mimics the symptoms of anemia in
    chronic disease (e.g. infections, cancer)

Koilonychia (spoon- shaped nails)

42
Q

What is Aplastic anemia?

A
  • Bone marrow failure associated with ↓WBC, ↓RBC, and ↓platelet
  • Suppression of multi-potent myeloid stem cells
  • Caused by exposure to infections, chemicals,
    radiation or drugs
  • Could be reversible or irreversible
  • Hypocellular bone marrow
43
Q

What is Polycythemia?

A
  • Abnormally increased number of RBC, WBC, platelets
  • ↑ Hb and/or ↑ HCT level
  • Hypercellular bone marrow
  • Relative polycythemia:
    • results from any cause of dehydration
44
Q

What is Absolute polycythemia?

A
  • primary polycythemia:
    caused by intrinsic abnormality of myeloid stem cells
  • secondary polycythemia: caused by ↑erythropoietin
45
Q

What is leukemia?

A

Leukemia is cancer of blood-forming tissues, including bone marrow and lymphatic system.
* It usually involves WBCs

46
Q

What are factors that ↑ risk of leukemia?

A
  • chemotherapy/radiation therapy for other cancers
  • exposure to chemicals (e.g. benzene)
  • smoking cigarettes
  • genetic disorders (e.g. Down syndrome)
  • family history of leukemia
47
Q

Classification of leukemia based on PROGRESSION:

A
  • Acute leukemia
  • immature blood cells (blasts) replicate rapidly
  • disease worsens quickly
  • cells cannot carry out normal function
  • requires timely treatment
  • Chronic leukemia
  • involves more mature blood cells
  • replicate more slowly
  • typically can function normally
48
Q

Classification of leukemia based on type of affected WBC:

A
  • Lymphocytic leukemia
  • affects lymphoid cells (lymphocytes)
  • lymphocytes are part of immune system
  • Myelogenous leukemia
  • affects the myeloid cells
  • myeloid cells give rise to RBCs, WBCs, platelets
49
Q

The major types of leukemia are:

A
  • Acute lymphocytic leukemia (ALL):
  • most common type of leukemia in young children
  • Acute myelogenous leukemia (AML)
  • occurs in children and adults
  • most common type of acute leukemia in adults
  • Chronic lymphocytic leukemia (CLL)
  • most common chronic adult leukemia
  • patient may feel well for years without treatment
  • Chronic myelogenous leukemia (CML)
  • mainly affects adults
  • patient may have no symptoms for years
50
Q

What are the signs & symptoms of leukemia?

A
  • Symptoms depends on the type of leukemia
  • Common signs and symptoms include:
  • fever or chills, ↑sweating especially at night
  • persistent fatigue, weakness
  • losing weight without trying
  • swollen lymph nodes, enlarged liver or spleen
  • frequent or severe infections
  • easy bleeding or bruising
  • recurrent nosebleeds
  • petechiae (red spots in skin) * bone pain or tenderness
51
Q

What is Thrombocytopenia?

A

Low blood platelet count (<150,000 per microliter blood)

52
Q

What are the major causes of thrombocytopenia?

A

A) Trappingofplateletsinspleen:
- Enlarged spleen harbor platelets → ↓blood platelets

B) Decreased platelet production
- leukemia, other cancers
- some types of anemia (e.g. folic acid deficiency)
- viral infections (e.g. hepatitis C, HIV)
- chemotherapy, radiation therapy
- heavy alcohol consumption

C) Increased destruction of platelets
* pregnancy: usually mild and improves after childbirth
* immune related: autoimmune disease (e.g. lupus,
rheumatoid arthritis)
* bacteremia (bacteria in blood)
* thrombotic thrombocytopenic purpura (small blood clots form throughout body so platelets are used)
* hemolytic uremic syndrome (↓platelets, ↓RBC, impaired kidney function)
* medications (e.g. heparin)