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Hematology > BONE MARROW FAILURE > Flashcards

BONE MARROW FAILURE 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

destruction of hematopoietic stem cells due to injury by drugs, chemicals, radiation, viruses, or autoimmune mechanisms

A

BONE MARROW FAILURE

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3
Q

premature senescence and apoptosis

A

BONE MARROW FAILURE

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4
Q

ineffective hematopoiesis due to stem cell mutations or vitamin B12 or folate deficiency

A

BONE MARROW FAILURE

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5
Q

disruption of the bone marrow microenvironment that supports hematopoiesis

A

BONE MARROW FAILURE

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6
Q

decreased production of hematopoietic growth factors or related hormones

A

BONE MARROW FAILURE

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7
Q

loss of normal hematopoietic tissue

A

BONE MARROW FAILURE

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8
Q

include pancytopenia, reticulocytopenia, bone marrow hypercellularity, and depletion of hematopoietic stem cells

A

APLASTIC ANEMIA

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9
Q

Acquired Aplastic Anemia
Two major categories:

A
  1. Idiopathic acquired aplastic anemia - 70%
  2. Secondary acquired aplastic anemia - 10%-15%
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10
Q

CD34+ cells have increased expression of Fas receptors that mediate apoptosis and increased expression of apoptosis- related genes

A

Acquired Aplastic Anemia

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11
Q

present at an earlier age and may have characteristic physical stigmata

A

Inherited Aplastic Anemia

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12
Q

Inherited Aplastic Anemia

A
  1. Fanconi Anemia
  2. Dyskeratosis Congenita
  3. Shwachman-Bodian-Diamond Syndrome
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13
Q

is a chromosome instability disorder characterized by aplastic anemia, physical abnormalities, and cancer susceptibility

A

Fanconi Anemia

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14
Q

skeletal abnormalities (thumb malformations, radila hypoplasia, microcephaly, hip dislocation, scoliosis)

A

Fanconi Anemia

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15
Q

skin pigmentation (hyper-/hypopigmentation, cafe-au-lait lesions)

A

Fan coni Anemia

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16
Q

short stature, abnormalities of the eyes, kidneys, and genitals

A

Falcon anemia

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17
Q

low birth weight and developmental delay

A

Fanconi Anemia

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18
Q

characterized by mucocutaneous abnormalities, bone marrow failure, and pancytopenia

A

Dyskeratosis Congenita

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19
Q

triad of abnormal skin pigmentation, dystrophic nails, and oral leukoplakia

A

Dyskeratosis Congenita

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20
Q

multisystem abnormalities: pulmonary fibrosis, liver disease, developmental delay, short stature, microcephaly, prematurely gray hair or hair loss, immunodeficiency dental carries, periodontal disease.

A

Dyskeratosis Congenita

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21
Q

is an inherited multisystem disorder characterized by pancreatic insufficiency, cytopenia, skeletal abnormalities, and a predisposition for hematologic malignancies

A

Shwachman-Bodian-Diamond Syndrome

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22
Q

a rare disorder of erythropoiesis characterized by a selective decrease in erythrocyte precursors in an otherwise normal bone marrow

A

PURE RED CELL APLASIA

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23
Q

Acquired Pure Red Cell Aplasia

A

Transient Erythroblastopenia of Childhood

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24
Q

the acquired form of PRCA in young children

A

Transient Erythroblastopenia of Childhood

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25
Q

Congenital Pure Red Cell Aplasia:

A

Diamond-Blackfan Anemia

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26
Q

a congenital erythroid hypoplastic disorder of early infancy

A

Diamond-Blackfan Anemia

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27
Q

approximately half of patients have: physical anomalies (craniofacial dysmorphisms, short stature, neck and thumb malformations)

A

Diamond-Blackfan Anemia

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28
Q

are a heterogeneous group of rare disorders characterized by refractory anemia, reticulocytopenia, hypercellular bone marrow with markedly ineffective erythropoiesis, distinctive dysplastic changes in bone marrow erythroblasts

A

CONGENITAL DYSERYTHROPOIETIC ANEMIA

29
Q

is due to the infiltration of abnormal cells into the bone marrow and subsequent destruction and replacement of normal hematopoietic cells

A

MYELOPHTHISIC ANEMIA

30
Q

common complication of CKD is anemia

A

ANEMIA OF CHRONIC KIDNEY DISEASE

31
Q

burr cells are common peripheral blood film findings

A

ANEMIA OF CHRONIC KIDNEY DISEASE

32
Q

Primary cause of anemia in CKD:

A
  1. Inadequate renal production of EPO
  2. Uremia
  3. Hemodialysis and frequent blood draws 4. Chronic inflammation and a restricted diet
33
Q

results when the rate of RBC destruction exceeds the increased rate of RBC production

A

HEMOLYTIC ANEMIA

34
Q

caused by the defects in proteins that disrupt the vertical interactions between transmembrane proteins and the underlying protein cytoskeleton

A

HEREDITARY SPHEROCYTOSIS

35
Q

results from gene mutation

A

HEREDITARY SPHEROCYTOSIS

36
Q

the defects in vertical membrane protein interactions cause RBCs to lose unsupported lipid membrane over time because of local disconnections of the lipid bilayer and underlying cytoskeleton

A

HEREDITARY SPHEROCYTOSIS

37
Q

Three key clinical manifestations of hereditary spherocytosis

A

anemia, jaundice, splenomegaly

38
Q

demonstrates increased RBC fragility in blood specimens in which the RBCs have decreased surface area-to-volume ratios

A

Osmotic fragility

39
Q

cells with decreased surface area:volume ratio have limited capacity to expand in hypotonic solutions, hence undergo lysis

A

Osmotic fragility

40
Q

EMA is a fluorescent dye that binds to transmembrane proteins band 3, Rh, RhAg, and CD47 in the RBC membrane

A

Eosin-5’-maleimide (EMA) binding test

41
Q

characteristic finding: elliptical or cigar-shaped RBCs on the peripheral blood film

A

HEREDITARY ELLIPTOCYTOSIS

42
Q

considered a severe form of HEREDITARY ELLIPTOCYTOSIS

A

Hereditary pyropoikilocytosis

43
Q
  • RBCs show thermal sensitivity.
  • RBCs fragment at 41°C to 45°C
A

Hereditary pyropoikilocytosis

44
Q
  • or Southeast Asian Ovalocytosis
A

HEREDITARY OVALOCYTOSIS

45
Q

a condition caused by mutation in the gene for band 3 that results in increased rigidity of the membrane

A

HEREDITARY OVALOCYTOSIS

46
Q

due to a defect in membrane cation permeability that causes RBCs to be overhydrated

A

OVERHYDRATED HEREDITARY STOMATOCYTOSIS

47
Q

the RBC membrane is excessively permeable to sodium and potassium at 37°C

A

OVERHYDRATED HEREDITARY STOMATOCYTOSIS

48
Q

or hereditary xerocytosis

A

DEHYDRATED HEREDITARY STOMATOCYTOSIS

49
Q

due to a defect in membrane cation permeability that causes RBCs to be dehydrated

A

DEHYDRATED HEREDITARY STOMATOCYTOSIS

50
Q

RBC membrane is excessively permeable to potassium

A

DEHYDRATED HEREDITARY STOMATOCYTOSIS

51
Q

potassium leaks out of the cell

A
52
Q

Other Hereditary Membrane Defects

A

I. Familial Pseudohyperkalemia
II. Cryohydrocytosis
III. Rh Deficiency Syndrome

53
Q

is a term used to describe a group of rare inherited disorders characterized by neurologic impairment and acanthocytes on the PBS

A

Neuroacanthocytosis

54
Q

Under Neuroacanthocytosis

A

I. Abetalipoproteinmeia
II. McLeod Syndrome
III. Chorea acanthocytosis

55
Q

characterized by fat malabsorption, progressive ataxia, neuropathy, retinitis pigmentosa, and acanthocytosis

A

Abetalipoproteinmeia

56
Q

an X-linked disorder caused by mutations in the KX gene

A

McLeod Syndrome

57
Q

characterized by chorea, hyperkinesia, cognitive impairments, and neuropsychiatric symptoms

A

Chorea acanthocytosis

58
Q

caused by an acquired clonal hematopoietic stem cell mutation that results in circulating blood cells that lack CD55 and CD59

A

PAROXYSMAL NOCTURNAL HEMOGLOBINURIA

59
Q

the absence of CD55 and CD59 on the surface of the RBCs renders them susceptible to spontaneous lysis by complement

A

PAROXYSMAL NOCTURNAL HEMOGLOBINURIA

60
Q

mutation in the PIGA gene

A

PAROXYSMAL NOCTURNAL HEMOGLOBINURIA

61
Q

Laboratory diagnosis for PAROXYSMAL NOCTURNAL HEMOGLOBINURIA

A

I. Sugar water test
II. Sucrose hemolysis test
III. Acidified serum test/ Ham test

62
Q

RBCs are particularly vulnerable to oxidative damage and subsequent hemolysis during oxidant stress

A

GLUCOSE-6-PHOSPHATE DEHYDROGENASE DEFICIENCY

63
Q

is the most common RBC enzyme defect

A

GLUCOSE-6-PHOSPHATE DEHYDROGENASE DEFICIENCY

64
Q

one of the important intracellular enzymes needed to protect hemoglobin and other cellular proteins and lipids from oxidative denaturation

A

G6PD

65
Q

catalyzes the first step in a series of reactions that detoxify hydrogen peroxide formed from oxygen radicals

A

G6PD

66
Q

confers protection against life-threatening P. falciparum and P. vivax

A

GLUCOSE-6-PHOSPHATE DEHYDROGENASE DEFICIENCY

67
Q

Laboratory diagnosis for GLUCOSE-6-PHOSPHATE DEHYDROGENASE DEFICIENCY

A

Ascorbate cyanide screening test

68
Q

detects deficiencies in the pentose phosphate pathway

A

Ascorbate cyanide screening test

Hematology (28 decks)

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