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3rd year > Anemia of bone marrow failure and systemic disorders > Flashcards

Anemia of bone marrow failure and systemic disorders Flashcards

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1
Q

is the reduction or cessation of blood cell production affecting one or more cell lines.

A

Bone marrow failure

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2
Q

decreased numbers of circulating RBC, WBC, and platelets.

A

Pancytopenia

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3
Q

Pathophysiology of Bone marrow failure

A
  • Destruction of hematopoietic stem cells as a result of injury.
  • premature senescence and apoptosis of hematopoietic stem cells as a result of genetic mutations.
  • Ineffective hematopoiesis caused by stem cell mutations or Vitamin b12 or folate deficiency.
  • Disruption of the bone marrow microenvironment that supports hematopoiesis.
  • loss of hematopoietic tissue as a result of infiltration of the marrow space with abnormal cells.
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4
Q

a bone marrow failure syndrome resulting from damaged or defective stem cells

A

aplastic anemia

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5
Q

Who provided the first case report of aplastic anemia?

A

Ehrlich in 1888

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6
Q

Who gave the name aplastic anemia?

A

Vaquez and Aubertin in 1904

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7
Q

Characteristics of aplastic anemia

A
  1. Pancytopenia
  2. Reticulocytopenia
  3. Bone marrow hypocellularity
  4. Depletion of hematopoietic stem cells.
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8
Q

underdevelopment or incomplete development of a tissue or organ.

A

Hypoplasia

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9
Q

failure of a tissue to develop

A

aplasia

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10
Q

anemia with no known cause

A

idiopathic acquired aplastic anemia

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11
Q

anemia associated with an identified cause

A

secondary acquired aplastic anemia

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12
Q

Bone Marrrow if

A

less than 25% bone marrow cell or less than 50% normal cellularity with less than 30% hematopoetic cells

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13
Q

there are no immature myeloid cells in the peripheral blood.

A

aplastic anemia

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14
Q

is the classic drug associated with
marrow aplasia

A

Chloramphenicol

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15
Q

PRIMARY:

A
  1. Fanconi Anemia (Congenital)
    - Inborn due to inheritance
  2. Idiopathic aplastic anemia (Acquired)
    - Unknown cause
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16
Q

Secondary

A
  • Drugs
  • Chemicals
  • Radiation
  • Immune mechanism
  • Infections (Hepatitis C, viral diseases, millary tuberculosis, Brucellosis, Parasites
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17
Q

Caused either directly by lymphocytes or by some humoral factors

A

Immune Mechanisms

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18
Q

Rare, inherited form of aplastic anemia

A

Fanconi Anemia

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19
Q

A rare condition that involves either an idiopathic or an immune mechanism

A

ACQUIRED PURE RED CELL APLASIA

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20
Q

Some patients may have an Ig inhibitor of erythroid precursors or erythropoietin inhibitor

A

ACQUIRED PURE RED CELL APLASIA

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21
Q

A rare congenital disorder defined as a
normochromic- normocytic anemia with normal leukocyte and platelet count and a marked decrease in marrow normoblasts

A

DIAMOND BLACKFAN ANEMIA

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22
Q

A common finding in 94% of patients with carcinoma

A

MYELOPHTHISIC ANEMIA

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23
Q

is the presence of circulating nucleated rbc (NRBC) and immature leukocytes in the peripheral blood

A

LEUKOERYTHROBLASTIC REACTION

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24
Q

Aplastic anemias which have a number of identified causative factors and agents.

A

SECONDARY APLASTIC ANEMIAS

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25
Seen in long term, low dose irradiation where radiation may damage the stem cells
Radiation
26
Caused either directly by lymphocytes or by some humoral factors
Immune Mechanisms
27
Laboratory Findings in Peripheral Blood
- Pancytopenia - Normocytic-Normochromic type of anemia with slight macrocytosis - Normal red cell morphology - Reticulocyte Count is decreased - Reticulocyte Production Index is severely decreased - NO IMMATURE CELLS in the peripheral blood
28
Laboratory Findings in Bone marrow
- Hypoplastic or aplastic - Increased fat replacement - Decreased hematopoietic cells - Principal cells present are lymphocytes and plasma cells - No increased number of immature cells - Patchy areas of cellularity
29
ANEMIA OF ABNORMAL IRON METABOLISM
- Microcytic - Hypochromic
30
is necessary for the synthesis of normal oxygen carrying hemoglobin.
Iron
31
Used to evaluate patient with suspected PNH or suspected congenital dyserythpoietic anemia
Ham's test
32
TREATMENT for APLASTIC ANEMIA
- Bone marrow transplantation - Patient should receive prior treatment with cyclophosphamide to reduce the risk of rejection - Blood transfusion - Immunosuppressive Therapy (antithymocyte Globulin ATG (antilymphocyte globulin ALG) - [ ] Adrogens (stimulate erythropoiesis and to lesser degree, granulopoiesis and platelets)
33
are a diverse group of genetic disorders
Thalassemia
34
QUANTITATIVE reduction in globin chain synthesis for hemoglobin.
Thalassemia
35
the most common single gene disorders in the world population
Thalassemia
36
Thalassemias are very common in the shares of the
Mediterranean and Africa through the Middle East, the Indian subcontinent, Myanmar (Burma), and Southeast Asla (including the Philippines).
37
The geographic distribution follows that of the malaria parasite
Plasmodium falciparum
38
The types of thalassemia are
- a-thalassemia - B-thalassemia - Y-thalassemia - S-thalassemia
39
are the main components of a globin
Alpha and Beta
40
The genetics of thalassemia is centralized to the structures of CHROMOSOME
11& 16
41
is caused by a GENE DELETION
A thalassemia
42
is caused by POINT MUTATIONS in the DNA code
ẞ thalassemia
43
SPECIAL HEMATOLOGY TESTS in Thalassemia
- Bone marrow Hyperplastic red cell precursors - Hemoglobin electrophoresis on cellulose acetate at alkaline pH- varied results - Citrate agar electrophoresis (acid pH)
44
THALASSEMIA
Microcytic, Hypochromic WBC & platelet not involved
45
Most important test to differentiate Iron deficiency from thalassemia
Ferritin
46
Most often related to iron defiency or folate deficiency
anemia of pregnancy
47
is characterized by retarded growth and secondary sexual defiency
Hypogonadism
48
Transferrin saturation
>60%
49
Serum Iron
>160ug/dL
50
is known to lower concentration of Hb A, and Hb H as well as serum iron and ferritin which may mask the diagnosis of heterozygous b thalassemia and Hb H disease
Concurrent iron deficiency anemia
51
Caused by a deletion of all 4 a globin chains (-- /--)
BART'S HYDROPS FETALIS
52
In BART'S HYDROPS FETALIS, Both parents of affected individuals have ______ a thalassemia
heterozygous
53
Infants who are liveborn are underweight, edematous, demonstrate ascites, and have distended abdomens with marked hepatosplenomegaly.
BART'S HYDROPS FETALIS
54
Deletion of 3 or 4 a globin genes (- -/-α)
HbH DISEASE
55
The disorder results from the decreased synthesis of a chains with resultant formation of unstable Hb H
HbH DISEASE
56
2 gene deletion
a THALASSEMIA MINOR
57
a THALASSEMIA MINOR genotypic forms
- Heterozygous a (- -/αα) - Homozygous a' (-α/-α)
58
Homozygous a thalassemia is common in
American blacks and in the Mediterranean
59
is rare in blacks
Heterozygous aº
60
Associated with one a gene deletion (-α/αα)
THE SILENT CARRIER
61
Also known as Heterozygous at thalassemia
THE SILENT CARRIER
62
An unbalanced globin chain synthesis due to the LACK OF, OR THE REDUCED PRODUCTION OF b CHAINS that causes an excess of a chain which is very unstable
ẞ THALASSEMIA
63
is composed of normal a chains and two abnormal a chains formed by fusion of N-terminal end of the d chain and the C-terminal of the b chain
Hb Lepore
64
Hb Lepore consists of 3 types:
- Hb Lepore-Baltimore - Hb Lepore-Boston (most common) - Hb Lepore-Hollandia
65
related to body’s low oxygenation
Hypothyroidism
66
anemia occurs due to deranged cortisol secretion that have multiple effects on bloods cells
Adrenal abnormalities
67
Manifests with severe anemia within the 1st year of life with a difficult clinical course
ẞ THALASSEMIA MAJOR
68
Describes certain ẞ thalassemias which are clinically milder than thalassemia major but more severe than thalassemia minor
ẞ THALASSEMIA INTERMEDIA
69
Types of ẞ THALASSEMIA INTERMEDIA
Homozygous B+ thalassemia (B+/B+) Homozygous Sß thalassemia (Sß°/SB°) Doubly heterozygous off thalassemia varieties
70
Caused by deletion on the S and B structural genes on chromosome 11
Homozygous Sß thalassemia (Sß°/SB°)
71
Only Hb F and Hb Lepore are seen in Hgb electrophoresis
Doubly heterozygous off thalassemia varieties
72
An asymptomatic ẞ thalassemia disorder in which there is little or no associated anemia, although peripheral blood erythrocyte morphology is significantly abnormal
ẞ THALASSEMIA MINOR
73
Describes a form of ẞ thalassemia in which no clinical or laboratory abnormality is usually detected
ẞ THALASSEMIA MINIMA (SILENT ẞ THALASSEMIA TRAIT)
74
a heterogeneous group of inherited disorders characterized by increased levels of Hb F in adults in the absence of usual clinical and hematologic features of thalassemia
HEREDITARY PERSISTENCE OF FETAL HEMOGLOBIN
75
Sometimes classified as a Thalassemia Minor
HEREDITARY PERSISTENCE OF FETAL HEMOGLOBIN
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by deletion or inactivation of the b and d structural gene complexes
HEREDITARY PERSISTENCE OF FETAL HEMOGLOBIN
77
all cells contain increased levels of HbF as determined by the acid elution test
Pancellular HPFH
78
only a subpopulation of cells contain HbF
Heterocellular HPFH

3rd year (34 decks)

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