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HEMATOLOGY 2πŸ™ > QUANTITATIVE RED CELL DISORDERS PART II > Flashcards

QUANTITATIVE RED CELL DISORDERS PART II Flashcards

1
Q
  • are macrocytic anemias in which DNA is unimpaired
  • lack hypersegmented neutrophils and oval macrocytes in the peripheral blood and megaloblasts in the bone marrow
A

Macrocytic nonmegaloblastic anemias

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2
Q

Normocytic normochromic anemia

A

Bone marrow failure

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3
Q

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

A

CD34+ aplastic anemia

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4
Q

-elevated blood and bone marrow cytotoxic T cells with an oligoclonal expansion of specific T-cell clones
- increased T cell production of such cytokines as interferon-Ξ³ and tumor necrosis factor-Ξ±
- upregulation of T-bet
- increased TNF-Ξ± receptors on CD34+ cells
- improvement in cytopenias after immunosuppressive therapy

A

Autoimmune pathophysiology: Acquired Aplastic Anemia

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5
Q

Symptoms:
- insidious-onset anemia with pallor, fatigue, and weakness
- tachycardia, hypotension, cardiac failure, death
- petechiae, bruising, epistaxis, mucosal bleeding, menorrhagia, retinal hemorrhages, intestinal bleeding, intracranial hemorrhage
- fever and bacterial/fungal infections

A

APLASTIC ANEMIA

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6
Q

decreased neutrophils, monocytes, and platelets

A

APLASTIC ANEMIA

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7
Q

RBCs are macrocytic or normocytic

A

APLASTIC ANEMIA

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8
Q
  • toxic granulation in neutrophils may be observed
  • serum iron level and percent transferrin saturation are increased
  • erythroid, granulocytic, and megakaryocytic cells are decreased/ab
A

APLASTIC ANEMIA

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9
Q

present at an earlier age and may have characteristic physical stigmata

A

Inherited Aplastic Anemia

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10
Q
  • is a chromosome instability disorder characterized by aplastic anemia, physical abnormalities, and cancer susceptibility
A

Fanconi Anemia

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11
Q

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

A

Fanconi Anemia

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12
Q
  • skin pigmentation (hyper-/hypopigmentation, cafe-au-lait lesions)
  • short stature, abnormalities of the eyes, kidneys, and genitals
  • low birth weight and developmental delay
A

Fanconi Anemia

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13
Q

-characterized by mucocutaneous abnormalities, bone marrow failure, and pancytopenia

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

Dyskeratosis Congenita

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14
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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15
Q

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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16
Q

3 types of Inherited Aplastic Anemia

A
  1. Fanconi Anemia
  2. Dyskeratosis Congenita
  3. Shwachman-Bodian-Diamond Syndrome
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17
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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18
Q

Acquired Pure Red Cell Aplasia:
Transient Erythroblastopenia of Childhood

A

Take note

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19
Q

the acquired form of PRCA in young children

A

Transient Erythroblastopenia of Childhood

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20
Q

Diamond-Blackfan Anemia

A

Congenital Pure Red Cell Aplasia:

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21
Q
  • a congenital erythroid hypoplastic disorder of early infancy
  • approximately half of patients have: physical anomalies (craniofacial dysmorphisms, short stature, neck and thumb malformations)
A

Diamond-Blackfan Anemia

22
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

23
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

24
Q
  • common complication of CKD is anemia
  • burr cells are common peripheral blood film findings
A

1ANEMIA OF CHRONIC KIDNEY DISEASE

25
Q
  1. Inadequate renal production of EPO
  2. Uremia
  3. Hemodialysis and frequent blood draws
  4. Chronic inflammation and a restricted diet
A

ANEMIA OF CHRONIC KIDNEY DISEASE

26
Q

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

A

HEMOLYTIC ANEMIA

27
Q

has a rapid onset and is isolated (sudden), episodic, or paroxysmal

A

Acute hemolysis

28
Q

may not be evident if the bone marrow is able to compensate, but it may be punctuated over
time with hemolytic crises that cause the anemia

A

Chronic hemolysis HEMOLYTIC ANEMIA

29
Q

are passed to offspring by mutant genes from the parents

A

Inherited HEMOLYTIC ANEMIA

30
Q

develop in individuals who were previously hematologically normal but acquire an agent of condition that lyses RBCs

A

Acquired HEMOLYTIC ANEMIA

31
Q

destruction of the RBCs due to a defect within the RBCs themselves

A

Intrinsic hemolytic conditions

32
Q
  • arise from outside the RBC, typically substances in the plasma or conditions affecting the anatomy of the circulatory system
A

Extrinsic hemolytic conditions

33
Q

takes place most often within the bloodstream; occurs by fragmentation

A

Intravascular hemolysis -

34
Q

can refer either to lysis within the macrophage and not in the bloodstream or to the fact
that most of the macrophages are in tissues

A

Extravascular hemolysis

35
Q

occurs when RBCs are engulfed by macrophages and lysed by their digestive enzymes

A

Macrophage-mediated

36
Q

Mechanisms to salvage hemoglobin iron and prevent oxidation reactions during fragmentation hemolysis:

A
  1. Haptoglobin
  2. Hemopexin
  3. Albumin
37
Q

Clinical features:
- fatigue, dyspnea, dizziness
- signs of pallor and tachycardia
- jaundice
- splenomegaly, gallstones
- bone deformities in children

A

HEMOLYTIC ANEMIA

38
Q

IN SERUM 🩸
Total bilirubin = INCREASED
Indirect bilirubin = INCREASED
Direct bilirubin = WRI
LDH = INCREASED
Haptoglobin = DECREASED
Free hemoglobin = INCREASED
Hemopexin = DECREASED

A

Fragmentation hemolysis and Macrophage- mediated hemolysis

39
Q

Plasma Color. Coffee-brown

A

Fragmentation hemolysis

40
Q

Urine

Urobilinogen = INCREASED
Free hemoglobin (+)
Methemoglobin (+)
Prussian blue staining of urine sediment (+)
Urine color = Root beer/beer-colored

A

Fragmentation hemolysis

41
Q

Urine

Urobilinogen = INCREASED
Free hemoglobin (-)
Methemoglobin (-)
Prussian blue staining of urine sediment (-)
Urine color

A

Macrophage- mediated hemolysis

42
Q

Anticoagulated whole blood

Hgb,Hct,RBC count DECREASED
Schistocytes POSITIVE
Spherocytes NEGATIVE
Glycated hemoglobin DECREASED

A

Fragmentation hemolysis

43
Q

Anticoagulated whole blood

Hgb,Hct,RBC count DECREASED
Schistocytes NEGATIVE
Spherocytes POSITIVE
Glycated hemoglobin DECREASED

A

Macrophage- mediated hemolysis

44
Q

SPECIAL TESTSπŸ§ͺ
Endogenous carbon monoxide INCREASED
Erythrocyte llife span DECREASED

A

Fragmentation hemolysis
Macrophage- mediated hemolysis

45
Q
  • caused by the defects in proteins that disrupt the vertical interactions between transmembrane proteins and the underlying protein cytoskeleton
  • results from gene mutation
A

HEREDITARY SPHEROCYTOSIS

46
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

47
Q

Symptoms:
Three key clinical manifestations:
anemia, jaundice, splenomegaly

A

HEREDITARY SPHEROCYTOSIS

48
Q

Laboratory findings:
Hallmark: spherocytes on the peripheral blood film
- increased MCHC hyperchromia
- decreased serum haptoglobin
- increased serum indirect bilirubin and lactate dehydrogenase

A

HEREDITARY SPHEROCYTOSIS

49
Q
  • demonstrates increased RBC fragility in blood specimens in which the RBCs have decreased surface area-to-volume ratios
  • cells with decreased surface area:volume ratio have limited capacity to expand in hypotonic solutions, hence undergo lysis
A

Osmotic fragility HEREDITARY SPHEROCYTOSIS

50
Q

Anticoagulant: Osmotic fragility

A

Heparin

51
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

HEMATOLOGY 2πŸ™ (12 decks)

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