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HEMATOLOGY HEMATOPOIESIS > RBC disorders part 2 > Flashcards

RBC disorders part 2 Flashcards

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
Q

Name the three (3) diagnostic tests for folate deficiency

A
  • Microbiological assays
  • Serum folate (<3 ug/L)
  • Red cell folate
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26
Q

“shift reticulocytes” especiallly in response to acute blood loss, hemolysis, and bone marrow infiltration

A

Non-megaloblastic anemia

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27
Q

what cell is seen in folic acid deficiency, Vitamin B12 deficiency, and pernicious anemia

A

Oval macrocyte

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28
Q

Seen in alcoholism, hypothyroidism and liver disease

A

Round hypochromic macrocyte

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29
Q

*Seen in neonate response to anemic stress, response to anemic stress
*Reticulocyte stain with supravital stain

A

Blue-tinged macrocyte

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30
Q

Associated with marrow replacement by involvement with abnormal cells or tissue components

A

Myelophthisic anemia

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31
Q

*Bone marrow does not produce any blood cells
*Pancytopenia
*Macrocytosis
*Increase RDW
*Chloramphenicol

A

Aplastic anemia

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32
Q

is a medication that is commonly can cause aplastic anemia

A

Chloramphenicol

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33
Q

*Inherited Aplastic Anemia

*Autosomal recessive *Normochromatic and may be macrocytic
* ncreased levels of Hb F and i antigen
*Pancytopenia

A

Fanconi’s syndrome

34
Q

*Parvovirus B19 infection
*Presence of scattered giant pronormoblasts in the bone marrow

A

Transient Aplastic Crises

35
Q

*Congenital Red Cell Aplasia
*Macrocytic, reticulocyte level is low
*Hb F is elevated, antigen i is often present

A

Diamond’s Blackfan Aplasia

36
Q

Blood is lost over a short time in amounts sufficient to cause anemia

A

Acute Posthemorrhagic Anemia

37
Q

*Blood is lost in small amounts over an extended period
*Iron deficiency anemia

A

Chronic Posthemorrhagic Anemia

38
Q

*Defect of RBC itself
*Membrane, metabolic, and hemoglobin defects

A

Intrinsic hemolytic anemia

39
Q

*Due to a factor of the RBC and acting upon it
*Acquired

A

Extrinsic hemolytic anemia

40
Q

*Increased hemolysis is also an increased in ________?

A

LDH

41
Q

This test is used to determine whether it is immune- mediated or not

A

Antiglobulin test

42
Q

Most red cell destruction about 80-90% is presumed to be ____________

A

Extravascular

43
Q

Intravascular : __________
Liver & spleen : _________
Activation of igG & IgM : _________
Cell mediated phagocytosis (IgM and IgG coated cells) : _________

A
  • Blood
  • Extravascular
  • Intravascular
  • Extravascular
44
Q

*Most common prevalent hereditary hemolytic anemia among people of Northern European descent
*Deficiency of the key membrane protein: spectrin

A

Hereditary Spherocytosis

45
Q

increased osmotic fragility

A

Hereditary Spherocytosis

46
Q

Spherocytes are hyperpermeable to ________ which causes osmotic fragility

A

Sodium

47
Q

*Horizontal defect
*deficiency in the proteins commonly associated with the ALPHA & BETA-spectrin regions.

A

Hereditary Elliptocytosis

48
Q

*Red cell fragments at 45°C to 46 °C
*50 to 75 fL MCV

A

Hereditary Pyropoikilocytosis

49
Q

*Melanesian and Malaysian populations
*Well-defined band 3 molecular deletion
*Increased resistance to malaria

A

South Asian Ovalocytes

50
Q

There is red cell dehydration due to loss of cations, predominantly K+ and water

A

Hereditary Stomatocytosis

51
Q

*Increased surface-to-volume ratio leading to moderate to severe anemia
*Decrease osmotic fragility, and high MCHC

A

Hereditary Xerocytosis

52
Q

Gene suppression or the present of a silent Rh gene

A

Rh null disease

53
Q

What RBC membrane defects can be associated with Rh null disease?

A

stomatocytes and spherocytes

54
Q

This form of acanthocyte-associated hemolytic anemia is seen in patients with established alcoholic cirrhosis

A

Spur Cell Hemolytic Anemia

55
Q

*Decay Accelerating Factor deficiency
*Intravascular hemolysis
*Intermittent (paroxysmal) sleep associated (nocturnal) blood in the urine (hemoglobinuria)

A

Paroxysmal Nocturnal Hemoglobinuria

56
Q

*A rare hemolytic anemia caused by anti-P
*Also called Donath Landsteiner antibody with anti-P specificity

A

Paroxysmal COld Hemoglobinuria

57
Q

*Disseminated Intravascular Coagulation
*March Anemia

A

Microangiopathic Hemolytic Anemia

58
Q

*Most common human enzyme deficiency in the world; sex linked; highest in young RBCs
*Presents with lots of Heinz bodies inclusions

A

Glucose-6-phosphate-dehydrogenase (G6PD)

59
Q

*Mature erythrocytes lack mitochondria and are exclusively dependent on anaerobic glycolysis for generation of ATP
*has been shown to be resistant to malaria

A

Pyruvate kinase deficiency

60
Q

medication that only blacks are affected

A

primaquine

61
Q

Most common human enzyme deficiency in the world; sex linked; highest in young RBCs

A

G6PD

62
Q

*Increase methemoglobin
*Hemoglobin M disease
*Cyanosis because the methemoglobin cannot carry oxygen to the tissues

A

Methemoglobin reductase deficiency

63
Q

*There is an impaired nucleotide metabolism, accumulation of pyrimidine; impairs degradation of RNA;

A

Pyrimidine 5 nucleotide deficiency

64
Q

*Third most common enzyme deficiency
*Autohemolysis is increase and is partially corrected by glucose

A

Glucose phosphate isomerase deficiency

65
Q

*Heterozygous state for Hb S
*most common hemoglobinopathy in the United States
*a benign condition without clinical symptoms or hematologic abnormalities

A

Sickle Cell trait (Hemoglobin AS)

66
Q

Confers protection against P. falcifarum

A

Sickle Cell trait (Hemoglobin AS)

67
Q

*glutamic acid in the sixth position on the β- chain is replaced by valine
*Hb S

A

Sickle Cell disease

68
Q

*Common in African Americans
* Anicytosis and poikilocytosis are mild to severe
*Target cells (85% of the erythrocytes)

A

Hemoglobin SC disease

69
Q

*No Hemoglobin A present
*Microcytic hypochromic
*Splenomegaly is usually present

A

Hemoglobin S-Beta thalassemia

70
Q

Presence of crystalline factors in the red cells that appears as blocks or bars of gold

A

Hemoglobin C disease

71
Q

*Second most common Hemoglobin variant worldwide
*Extremely high occurrence in individuals from southeast Asian countries

A

Hemoglobin E disease

72
Q

This constitutes the most common D variant in African Americans

A

Hb D Los Angeles (Punjab) (B121 glu–> gln)

73
Q

Most common alpha chain variant in black people

A

Hb G Philadelphia ( a 68asn–> lys)

74
Q

which hemoglobin is analogous to the Lepore hemoglobin which is associated with HPFH phenotype?

A

Hb Kenya

75
Q

The distribution of Hb F is uneven (heterocellular): Both F cells and erythrocytes lacking Hb F are present

A

Heterocellular or Swiss type HPFH

76
Q

Hb F is homogeneously or evenly distributed among the red cells

A

Pancellular

77
Q

*Diagnosed based on elevated hematocrit level above the normal range
*Hgb > 18.5 g/dL for men, > 16.5 g/dL for women (WHO)

A

Polycythemia (Erythrocytosis)

78
Q

*Apparent polycythemia, Gaisbock syndrome
*The red cell mass is often high normal and the plasma volume is low normal

A

Spurious Polycythemia

79
Q

increase in the total red cell mass in the body

A

Absolute

80
Q

the total red cell mass is normal, but the Hct is elevated because the plasma volume is decreased usually seen in dehydration

A

Relative

81
Q

Condition where there is an excessive proliferation of erythrocytes as well as other cells lines (panmyelosis)

A

Polycythemia Vera

HEMATOLOGY HEMATOPOIESIS (7 decks)

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