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HEMATOLOGY HEMATOPOIESIS > RBC disorders part 2 > Flashcards

RBC disorders part 2 Flashcards

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1
Q

*Hypercellular Bone Marrow
*Presence of megaloblast
*Ineffective erythropoiesis
*Active intramedullary hemolysis

A

Macrocytic anemia

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2
Q

Unimpaired DNA synthesis

A

Non-megaloblastic (macrocytic) anemia

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3
Q

How do you examine patients with non-megaloblastic, macrocytic anemia from those with megaloblastic anemia?

A
  • Patients with non-megaloblastic, macrocytic anemia lack hypersegmented neutrophils and;
  • oval macrocytes in the peripheral blood and;
  • megaloblasts in the bone marrow
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4
Q

characteristics of a megaloblast

A
  • Progenitor cell
  • finely stippled lacey nuclear chromatin pattern
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5
Q

Megaloblastic anemia has two (2) major division:

A
  • Vitamin b12 (cobalamin, Cbl) deficiency
  • Folic acid deficiency
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6
Q

key features of Megaloblastic Anemia

A
  • Diminished capacity of DNA synthesis
  • Manifest macro-ovalocytes and giant hypersegmented neutrophils - - Increase MCV
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7
Q

How does megaloblastic anemia develops?

A

Deficiency of vitamin B12 or folates —> Impaired DNA synthesis —> Slows down nuclear replication —> prolonged premitotic interval —> resulting large nucleus for megaloblasts

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8
Q

This refers to decrease amount of cells in all cell lines i.e. WBC, RBC, platelets)

A
  • Pancytopenia
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9
Q

M:E ratio for MA?

A

M:E ratio - 10:1

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10
Q

Cells found in the bone marrow of a patient with megaloblastic anemia

A

megaloblasts

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11
Q

Illeum: _____________
_____________: Liver, storage
Castle’s factor : ____________
_____________: Plasma
Transcobalamin : __________

A

Absorption
Adenosylcobalamin
Intrinsic factor
Methylcobalamin
Transporter

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12
Q

VItamin B12 is otherwise known as?

A

cyanocobalamin

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13
Q

Causes for Vitamin B12 deficiency

A
  • D. latum infection
  • Pernicious anemia
  • Malabsorption syndrome
  • Nutritional deficiency
  • Hypochlorhydria
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14
Q

What condition is associated decrease production of hydrochloric acid in the stomach

A

Hypochlorhydria
(often seen in pernicious anemia)

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15
Q

*Addison’s anemia
*Caused by failure of the gastric mucosa to secrete intrinsic factor

A

Pernicious Anemia

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16
Q

other term for pernicious anemia

A

atrophy gastritis

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17
Q

which condition is an autoimmune disease caused by two antibodies— anti- parietal cell antibodies
- anti-intrinsic factor antibodies.

A

Pernicious anemia

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18
Q

An autosomally recessive inherited defect in the intestinal absorption of cobalamin that occurs in the presence of normal intrinsic factor

A

Immerslund-Grasbeck Syndrome

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19
Q

*Ability of the patient to absorb an oral dose of radioactive cobalamin
*Considered as the reference procedure for the determination of pernicious anemia

A

Schilling’s Test

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20
Q

Microbiological assay which utilizes the organism called Euglena gracilis

A

Serum Cobalamin Assay

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21
Q

Both increase in megaloblastic anemia

A

Methylmalonic Acid & Homocysteine Assays

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22
Q

Measures the ability of the marrow cells in vitro to utilize the deoxyuridine in DNA synthesis

A

Deoxyuridine suppression test

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23
Q

if Vitamin B12 is absorbed within the ileum, where does Folate being absorbed?

A

Jejunum

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24
Q

Causes for Folate deficiency

A
  • Chronic alcoholics
  • Poor dietary habits
  • Pregnancy
  • Steatorrhea
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25
Name the three (3) diagnostic tests for folate deficiency
- Microbiological assays - Serum folate (<3 ug/L) - Red cell folate
26
“shift reticulocytes” especiallly in response to acute blood loss, hemolysis, and bone marrow infiltration
Non-megaloblastic anemia
27
what cell is seen in folic acid deficiency, Vitamin B12 deficiency, and pernicious anemia
Oval macrocyte
28
Seen in alcoholism, hypothyroidism and liver disease
Round hypochromic macrocyte
29
*Seen in neonate response to anemic stress, response to anemic stress *Reticulocyte stain with supravital stain
Blue-tinged macrocyte
30
Associated with marrow replacement by involvement with abnormal cells or tissue components
Myelophthisic anemia
31
*Bone marrow does not produce any blood cells *Pancytopenia *Macrocytosis *Increase RDW *Chloramphenicol
Aplastic anemia
32
is a medication that is commonly can cause aplastic anemia
Chloramphenicol
33
*Inherited Aplastic Anemia *Autosomal recessive *Normochromatic and may be macrocytic * ncreased levels of Hb F and i antigen *Pancytopenia
Fanconi's syndrome
34
*Parvovirus B19 infection *Presence of scattered giant pronormoblasts in the bone marrow
Transient Aplastic Crises
35
*Congenital Red Cell Aplasia *Macrocytic, reticulocyte level is low *Hb F is elevated, antigen i is often present
Diamond's Blackfan Aplasia
36
Blood is lost over a short time in amounts sufficient to cause anemia
Acute Posthemorrhagic Anemia
37
*Blood is lost in small amounts over an extended period *Iron deficiency anemia
Chronic Posthemorrhagic Anemia
38
*Defect of RBC itself *Membrane, metabolic, and hemoglobin defects
Intrinsic hemolytic anemia
39
*Due to a factor of the RBC and acting upon it *Acquired
Extrinsic hemolytic anemia
40
*Increased hemolysis is also an increased in ________?
LDH
41
This test is used to determine whether it is immune- mediated or not
Antiglobulin test
42
Most red cell destruction about 80-90% is presumed to be ____________
Extravascular
43
Intravascular : __________ Liver & spleen : _________ Activation of igG & IgM : _________ Cell mediated phagocytosis (IgM and IgG coated cells) : _________
- Blood - Extravascular - Intravascular - Extravascular
44
*Most common prevalent hereditary hemolytic anemia among people of Northern European descent *Deficiency of the key membrane protein: spectrin
Hereditary Spherocytosis
45
increased osmotic fragility
Hereditary Spherocytosis
46
Spherocytes are hyperpermeable to ________ which causes osmotic fragility
Sodium
47
*Horizontal defect *deficiency in the proteins commonly associated with the ALPHA & BETA-spectrin regions.
Hereditary Elliptocytosis
48
*Red cell fragments at 45°C to 46 °C *50 to 75 fL MCV
Hereditary Pyropoikilocytosis
49
*Melanesian and Malaysian populations *Well-defined band 3 molecular deletion *Increased resistance to malaria
South Asian Ovalocytes
50
There is red cell dehydration due to loss of cations, predominantly K+ and water
Hereditary Stomatocytosis
51
*Increased surface-to-volume ratio leading to moderate to severe anemia *Decrease osmotic fragility, and high MCHC
Hereditary Xerocytosis
52
Gene suppression or the present of a silent Rh gene
Rh null disease
53
What RBC membrane defects can be associated with Rh null disease?
stomatocytes and spherocytes
54
This form of acanthocyte-associated hemolytic anemia is seen in patients with established alcoholic cirrhosis
Spur Cell Hemolytic Anemia
55
*Decay Accelerating Factor deficiency *Intravascular hemolysis *Intermittent (paroxysmal) sleep associated (nocturnal) blood in the urine (hemoglobinuria)
Paroxysmal Nocturnal Hemoglobinuria
56
*A rare hemolytic anemia caused by anti-P *Also called Donath Landsteiner antibody with anti-P specificity
Paroxysmal COld Hemoglobinuria
57
*Disseminated Intravascular Coagulation *March Anemia
Microangiopathic Hemolytic Anemia
58
*Most common human enzyme deficiency in the world; sex linked; highest in young RBCs *Presents with lots of Heinz bodies inclusions
Glucose-6-phosphate-dehydrogenase (G6PD)
59
*Mature erythrocytes lack mitochondria and are exclusively dependent on anaerobic glycolysis for generation of ATP *has been shown to be resistant to malaria
Pyruvate kinase deficiency
60
medication that only blacks are affected
primaquine
61
Most common human enzyme deficiency in the world; sex linked; highest in young RBCs
G6PD
62
*Increase methemoglobin *Hemoglobin M disease *Cyanosis because the methemoglobin cannot carry oxygen to the tissues
Methemoglobin reductase deficiency
63
*There is an impaired nucleotide metabolism, accumulation of pyrimidine; impairs degradation of RNA;
Pyrimidine 5 nucleotide deficiency
64
*Third most common enzyme deficiency *Autohemolysis is increase and is partially corrected by glucose
Glucose phosphate isomerase deficiency
65
*Heterozygous state for Hb S *most common hemoglobinopathy in the United States *a benign condition without clinical symptoms or hematologic abnormalities
Sickle Cell trait (Hemoglobin AS)
66
Confers protection against P. falcifarum
Sickle Cell trait (Hemoglobin AS)
67
*glutamic acid in the sixth position on the β- chain is replaced by valine *Hb S
Sickle Cell disease
68
*Common in African Americans * Anicytosis and poikilocytosis are mild to severe *Target cells (85% of the erythrocytes)
Hemoglobin SC disease
69
*No Hemoglobin A present *Microcytic hypochromic *Splenomegaly is usually present
Hemoglobin S-Beta thalassemia
70
Presence of crystalline factors in the red cells that appears as blocks or bars of gold
Hemoglobin C disease
71
*Second most common Hemoglobin variant worldwide *Extremely high occurrence in individuals from southeast Asian countries
Hemoglobin E disease
72
This constitutes the most common D variant in African Americans
Hb D Los Angeles (Punjab) (B121 glu--> gln)
73
Most common alpha chain variant in black people
Hb G Philadelphia ( a 68asn--> lys)
74
which hemoglobin is analogous to the Lepore hemoglobin which is associated with HPFH phenotype?
Hb Kenya
75
The distribution of Hb F is uneven (heterocellular): Both F cells and erythrocytes lacking Hb F are present
Heterocellular or Swiss type HPFH
76
Hb F is homogeneously or evenly distributed among the red cells
Pancellular
77
*Diagnosed based on elevated hematocrit level above the normal range *Hgb > 18.5 g/dL for men, > 16.5 g/dL for women (WHO)
Polycythemia (Erythrocytosis)
78
*Apparent polycythemia, Gaisbock syndrome *The red cell mass is often high normal and the plasma volume is low normal
Spurious Polycythemia
79
increase in the total red cell mass in the body
Absolute
80
the total red cell mass is normal, but the Hct is elevated because the plasma volume is decreased usually seen in dehydration
Relative
81
Condition where there is an excessive proliferation of erythrocytes as well as other cells lines (panmyelosis)
Polycythemia Vera

HEMATOLOGY HEMATOPOIESIS (7 decks)

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