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HEMATOLOGY 1 > ANEMIA, PORPHYRIA, HEMOGLOBINOPATHIES, THALASSEMIA > Flashcards

ANEMIA, PORPHYRIA, HEMOGLOBINOPATHIES, THALASSEMIA Flashcards

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1
Q

morphologic classification of anemia

A

normocytic normochromic anemia
microcytic hypochromic anemia
macrocytic normochromic anemia

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2
Q

normal or decreased reticulocyte count (3)

A

aplastic anemia
kidney diseases
acute blood loss

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3
Q

increased reticulocyte count

A

paroxysmal nocturnal hemoglobinuria
paroxysmal cold hemoglobinuria ( donath landsteiner hemolytic anemia)
sickle cell disease
enzyme deficiency (G6PD and PK deficiency)
other hemolytic anemia

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4
Q

described as a rare but potentially deadly bone marrow failure syndrome

A

aplastic anemia

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5
Q

markedly decreased in the number of RBCs, WBCs, and platelets in the blood

A

pancytopenia

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6
Q

markedly increased in the number of RBCs, WBCs, and platelets in the blood

A

polycythemia vera

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7
Q

pancytopenia, reticulocytopenia, bonemarrow hypocellularity, depletion of hematopoietic stem cells, these are characteristics of what type of anemia

A

aplastic anemia

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8
Q

type of aplastic anemia ; approximately 80-85% of plastic anemia cases

A

acquired aplastic anemia

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9
Q

type of acquired aplastic anemia with no known cause

A

idiopathic acquired aplastic anemia

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10
Q

type of acquired aplastic anemia associated with an identified cause

A

secondary acquired aplastic anemia

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11
Q

a drug most frequently implicated in acquired aplastic anemia

A

chlorampenicol

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12
Q

insecticide, benzene, epstein-barr virus, and chlorampenicol can cause what type of aplastic anemia

A

secondary acquired aplastic anemia

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13
Q

type of aplastic anemia approximately 15% to 20% cases

A

inherited aplastic anemia

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14
Q

Dyskeratosis congenita
Schwachman-Bodiam Diamomd syndrome
Fanconi Anemia

these are diseases associated with what type of aplastic anemia

A

inherited aplastic anemia

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15
Q

most common of the inherited aplastic anemia

A

fanconi anemia

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16
Q

a type of inherited aplastic anemia with a chromosome instability disorder

A

fanconi anemia

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17
Q

a type of inherited aplastic anemia characterized by aplastic anemia, cancer susceptibility, and physical abnormalities

A

fanconi anemia

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18
Q

paroxysmal nocturnal hemoglobinuria is also known as

A

Marchiafava- Micheli syndrome

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19
Q

examples of compplement regulatory proteins for PNH

A

DAF (Decay-accelerating factor of CD55)
MIRL (membrane inhibitor of reactive lysis, or CD59)

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20
Q

TESTS FOR PNH

A

HAM’S ACIDIFIED SERUM TEST
SUGAR WATER TEST (SUCROSE HEMOLYSIS TEST)
FLOW CYTOMETRY (using FLAER)

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21
Q

Confirmatory test for PNH

A

FLOW CYTOMETRY USING FLAER

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22
Q

With small cells that have increased central pallor on the smear

A

Microcytic hypochromic anemia

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23
Q

This type of anemia results from an iron level insufficient for maintaining normal erythropoiesis and is characterized by abnormal result of iron studies

A

Microcytic anemia

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24
Q

Associated conditions in microcytic hypochromic anemia (CTAILS)

A

CHRONIC BLOOD LOSS
THALASSEMIA
ANEMIA OF CHRONIC INFECTION
IRON DEFICIENCY
LEAD POISONING
SIDEROBLASTIC ANEMIA

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25
Most common anemia among hospitalized patients
Anemia of chronic infection
26
Central feature of Anemia of chronic inflammation :
sideropenia (low serum iron) despite abundant iron stores
27
Acute phase reactants that contribute to Anemia of chronic infection
Hepcidin Ferritin Lactoferrin
28
Master regulatory hormone for systemic iron metabolism - inactivates ferroportin
Hepcidin
29
Storage form of iron
Ferritin
30
Transports iron from tissue to blood
Ferroportin
31
Type of anemia that Develops when the production of protoporphyrin or the incorporation of iron into protoporphyrin is prevented
Sideroblastic anemia
32
Nucleated RBC precursor with cytoplasmic iron granules
SIDEROBLAST
33
Anucleate RBC with iron granules
Siderocyte
34
Hallmark of Sideroblastic anemia
Ring Sideroblast
35
The rings in sideroblast is ?
Mitochondria loaded with iron
36
Most common type of anemia
Iron deficiency anemia
37
Pica-cravings for non food items (such as laundry starch or ICE (most common) - pagophagia Brittle hair Pillow Glossitis - these are clinical feature of what type of anemia
Iron deficiency anemia
38
Indirectly measures the concentration of transferrin by measuring its ability to bind iron
Total iron binding capacity
39
Reveals the body’s tissue IRON STORES Good indicator of IRON STORAGE status
Serum ferritin test
40
Useful in diagnosing iron deficiency (generally the first lab test to become abnormal when iron stores begin to decline)
Serum ferritin test
41
Serum ferritin test is measured using?
Radioimmunoassay
42
Iron deficiency stage Hgb: Normal Serum Iron: Normal TIBC: Normal Serum Ferritin Test: Low
Stage 1 (Storage Iron Depletion)
43
Iron deficiency stage Hgb: Normal Serum Iron: low TIBC: high Serum Ferritin Test: low
Stage 2 (transport iron depletion)
44
Iron deficiency stage Hgb: low Serum Iron: low TIBC: high Serum Ferritin Test: low
Stage 3 (functional iron depletion) Characterized by frank iron deficiency anemia
45
MICROCYTIC HYPOCHROMIC ANEMIA Serum Iron: HIGH TIBC: NORMAL Serum Ferritin Test: HIGH FEP: NORMAL
THALASSEMIA
46
MICROCYTIC HYPOCHROMIC ANEMIA Serum Iron: LOW TIBC: LOW Serum Ferritin Test: HIGH FEP: HIGH
ANEMIA OF CHRONIC INFECTION
47
MICROCYTIC HYPOCHROMIC ANEMIA Serum Iron: LOW TIBC: HIGH Serum Ferritin Test: LOW FEP: HIGH
IDA
48
MICROCYTIC HYPOCHROMIC ANEMIA Serum Iron: NORMAL TO HIGH OR LOW TIBC: NORMAL Serum Ferritin Test: NORMAL FEP: HIGH
LEAD POISONING
49
MICROCYTIC HYPOCHROMIC ANEMIA Serum Iron: HIGH TIBC: NORMAL Serum Ferritin Test: HIGH FEP: MIXED (HIGH AND LOW)
SIDEROBLASTIC ANEMIA
50
TYPE OF MACROCYTIC NORMOCHROMIC ANEMIA: Vit b12 deficiency Folate dificiency Acute erythroleukemia
Megaloblastic anemia
51
TYPE OF MACROCYTIC NORMOCHROMIC ANEMIA: Liver disease Alcoholism Bone marrow failure
Nonmegaloblastic anemia
52
TYPE OF MACROCYTIC NORMOCHROMIC ANEMIA: Hypersegmented neutrophils (>6 lobes) are present
Megaloblastic anemia
53
TYPE OF MACROCYTIC NORMOCHROMIC ANEMIA: Oval Macrocytes
Megaloblastic anemia
54
TYPE OF MACROCYTIC NORMOCHROMIC ANEMIA: Round macrocytes
Nonmegaloblastic anemia
55
Considered as root cause of megaloblastic anemia
Impaired DNA synthesis
56
DNA synthesis is dependent on ____ ; this structure cannot be made unless it receives a methyl group from methyl tetrahydrofolate or folic acid
Thymidine triphospahte (TTP)
57
Is the cofactor responsible for transferring the methyl group to methyl tetrahydrofolate
Vitamin b12
58
When thymidine triphosphate cannot be made, it is replaced by ; the synthesis of this structure results in nuclear fragmentation and destruction of cells and impaired cell division
Deoxyuridine triphosphate
59
Synthetic form of cobalamin
Cyanocobalamin
60
Naturally occurring cobalamin
Methyl cobalamin
61
In either folate or vit b12 deficiency, patients may experience :
Glossitis Gastritis, nausea, constipation
62
These two compounds are essential in the formation or thymidine triphosphate
Vitamin B12 and folic acid
63
Metabolically active form of Vit B12
HOLOTRANSCOBALAMIN
64
Due to differences in the arrangement of amino acids in the polypeptide chain ; qualitative globin synthesis defect
Hemoglobinopathies
65
Most commonly encountered hemoglobinopathies
Beta-hemoglobinopathies
66
HEMOGLOBINOPATHIES: BOTH B-genes are mutated ; HbA is absent ; abnormal hemoglobin becomes the predominant Hb type
Homozygous B-hemoglobinopathies
67
HEMOGLOBINOPATHIES: One of the B-genes is NORMAL ; other B-gene is mutated ; HbA is predominant than abnormal Hb
Heterozygous B-hemoglobinopathies
68
Examples of homozygous B-hemoglobinopathies
Sickle cell disease Hb C disease
69
Examples of heterozygous B-hemoglobinopathies
Sickle cell trait Hb C trait
70
Examples of abnormal hemoglobins present in certain B-hemoglobinopathies
Hb S Hb C Hb E
71
Most common and most severe abnormal hemoglobins present in B-Hemoglobinopathies
Hb S
72
ABNORMAL HB: Glutamic acid on the 6th position of the beta chain is replaced by valine
Hb S
73
2ND Most common abnormal hemoglobins present in B-Hemoglobinopathies
Hb C
74
3RD Most common abnormal hemoglobins present in B-Hemoglobinopathies
Hb E
75
SCREENING TESTS FOR Hb S
Sodium metabisulfite method Sodium dithionate tube test Hemoglobin Electrophoresis (Cellulose acetate)
76
Considered as the primary screening procedure to detect abnormal hemoglobins
Hemoglobin Electrophoresis (Cellulose acetate)
77
Confirmatory test for Hb S
Hemoglobin Electrophoresis (Citrate Agar)
78
ABNORMAL HEMOGLOBIN: Glutamic acid on the 6th position of the beta chain is replaced by lysine
Hb C
79
An Hb C crystal with WASHINGTON MONUMENT appearance ; found protruding the RBC membrane
Hb SC crystal
80
An Hb C crystal with BAR OF GOLD appearance ; found within RBC membrane
Hb CC crystals
81
Also known as hereditary leptocytosis and Mediterranean defect
Thalassemia
82
Mutations affecting the a- or b-globin gene - most clinically significant
Thalassemia
83
Screening tests for thalassemia
CBC PBS IRON STUDIES
84
CONFIRMATORY TEST FOR THALASSEMIA
MOLECULAR GENETIC TEST
85
Typically caused by deletion of one, two, three, or all four of the a-globin genes
Alpha thalassemia
86
ALPHA THALASSEMIA: One of the four genes is deleted
Silent carrier state
87
ALPHA THALASSEMIA: two of four genes are deleted
A-thalassemia trait (thalassemia minor)
88
ALPHA THALASSEMIA: three of four genes are deleted
Hb H disease (4 beta globin chain)
89
ALPHA THALASSEMIA: four of four genes are deleted
Hb Bart hydrops fetalis syndrome (Hb Bart Syndrome ; Alpha thalassemia major)
90
Most severe form of alpha thalassemia
Hb Bart syndrome
91
A type of thalassemia caused by mutations that affect the B-globin gene complex
Beta thalassemia
92
Most severe form of beta thalassemia ; transfusion depended
B-thalassemia major (Cooley’s anemia)
93
Transfusion independent type of beta thalassemia
B-thalassemia intermedia

HEMATOLOGY 1 (8 decks)

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