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

Anemia of bone marrow failure and systemic disorders Flashcards

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
Q

Seen in long term, low dose irradiation where radiation may damage the stem cells

A

Radiation

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26
Q

Caused either directly by lymphocytes or by some humoral factors

A

Immune Mechanisms

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27
Q

Laboratory Findings in Peripheral Blood

A
  • 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
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28
Q

Laboratory Findings in Bone marrow

A
  • 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
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29
Q

ANEMIA OF ABNORMAL IRON METABOLISM

A
  • Microcytic
  • Hypochromic
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30
Q

is necessary for the synthesis of normal
oxygen carrying hemoglobin.

A

Iron

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31
Q

Used to evaluate patient with suspected PNH or suspected congenital dyserythpoietic anemia

A

Ham’s test

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32
Q

TREATMENT for APLASTIC ANEMIA

A
  • 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
Q

are a diverse group of genetic disorders

A

Thalassemia

34
Q

QUANTITATIVE reduction in globin chain synthesis for hemoglobin.

A

Thalassemia

35
Q

the most common single gene disorders in the world population

A

Thalassemia

36
Q

Thalassemias are very common in the shares of the

A

Mediterranean and Africa through the Middle East, the Indian subcontinent, Myanmar (Burma), and Southeast Asla (including the Philippines).

37
Q

The geographic distribution follows that of the malaria parasite

A

Plasmodium falciparum

38
Q

The types of thalassemia are

A
  • a-thalassemia
  • B-thalassemia
  • Y-thalassemia
  • S-thalassemia
39
Q

are the main components of a globin

A

Alpha and Beta

40
Q

The genetics of thalassemia is centralized to the structures of CHROMOSOME

A

11& 16

41
Q

is caused by a GENE DELETION

A

A thalassemia

42
Q

is caused by POINT MUTATIONS in the DNA code

A

ẞ thalassemia

43
Q

SPECIAL HEMATOLOGY TESTS in Thalassemia

A
  • Bone marrow Hyperplastic red cell precursors
  • Hemoglobin electrophoresis on cellulose acetate at alkaline pH- varied results
  • Citrate agar electrophoresis (acid pH)
44
Q

THALASSEMIA

A

Microcytic, Hypochromic
WBC & platelet not involved

45
Q

Most important test to differentiate Iron deficiency from thalassemia

A

Ferritin

46
Q

Most often related to iron defiency or folate deficiency

A

anemia of pregnancy

47
Q

is characterized by retarded growth and secondary sexual defiency

A

Hypogonadism

48
Q

Transferrin saturation

A

> 60%

49
Q

Serum Iron

A

> 160ug/dL

50
Q

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

A

Concurrent iron deficiency anemia

51
Q

Caused by a deletion of all 4 a globin chains (– /–)

A

BART’S HYDROPS FETALIS

52
Q

In BART’S HYDROPS FETALIS, Both parents of affected individuals have ______ a thalassemia

A

heterozygous

53
Q

Infants who are liveborn are underweight,
edematous, demonstrate ascites, and have distended abdomens with marked hepatosplenomegaly.

A

BART’S HYDROPS FETALIS

54
Q

Deletion of 3 or 4 a globin genes (- -/-α)

A

HbH DISEASE

55
Q

The disorder results from the decreased synthesis of a chains with resultant formation of unstable Hb H

A

HbH DISEASE

56
Q

2 gene deletion

A

a THALASSEMIA MINOR

57
Q

a THALASSEMIA MINOR genotypic forms

A
  • Heterozygous a (- -/αα)
  • Homozygous a’ (-α/-α)
58
Q

Homozygous a thalassemia is common in

A

American blacks and in the Mediterranean

59
Q

is rare in blacks

A

Heterozygous aº

60
Q

Associated with one a gene deletion (-α/αα)

A

THE SILENT CARRIER

61
Q

Also known as Heterozygous at thalassemia

A

THE SILENT CARRIER

62
Q

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

A

ẞ THALASSEMIA

63
Q

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

A

Hb Lepore

64
Q

Hb Lepore consists of 3 types:

A
  • Hb Lepore-Baltimore
  • Hb Lepore-Boston (most common)
  • Hb Lepore-Hollandia
65
Q

related to body’s low oxygenation

A

Hypothyroidism

66
Q

anemia occurs due to deranged cortisol secretion that have multiple effects on bloods cells

A

Adrenal abnormalities

67
Q

Manifests with severe anemia within the 1st year of life with a difficult clinical course

A

ẞ THALASSEMIA MAJOR

68
Q

Describes certain ẞ thalassemias which are clinically milder than thalassemia major but more severe than thalassemia minor

A

ẞ THALASSEMIA INTERMEDIA

69
Q

Types of ẞ THALASSEMIA INTERMEDIA

A

Homozygous B+ thalassemia (B+/B+)
Homozygous Sß thalassemia (Sß°/SB°)
Doubly heterozygous off thalassemia varieties

70
Q

Caused by deletion on the S and B structural genes on chromosome 11

A

Homozygous Sß thalassemia (Sß°/SB°)

71
Q

Only Hb F and Hb Lepore are seen in Hgb
electrophoresis

A

Doubly heterozygous off thalassemia varieties

72
Q

An asymptomatic ẞ thalassemia disorder in which there is little or no associated anemia, although peripheral blood erythrocyte morphology is significantly abnormal

A

ẞ THALASSEMIA MINOR

73
Q

Describes a form of ẞ thalassemia in which no clinical or laboratory abnormality is usually detected

A

ẞ THALASSEMIA MINIMA (SILENT ẞ
THALASSEMIA TRAIT)

74
Q

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

A

HEREDITARY PERSISTENCE OF FETAL
HEMOGLOBIN

75
Q

Sometimes classified as a Thalassemia Minor

A

HEREDITARY PERSISTENCE OF FETAL HEMOGLOBIN

76
Q

by deletion or inactivation of the b and d structural gene complexes

A

HEREDITARY PERSISTENCE OF FETAL HEMOGLOBIN

77
Q

all cells contain increased levels of HbF as determined by the acid elution test

A

Pancellular HPFH

78
Q

only a subpopulation of cells contain HbF

A

Heterocellular HPFH

3rd year (19 decks)

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