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Heme/Lymph Exam > RBC Disorders > Flashcards

RBC Disorders Flashcards

1
Q

blood - characteristics

A

*homogenous multifunctional specialized fluid connective tissue
*mature cellular elements ONLY: granulocytes, lymphocytes, platelets, RBCs
*specialized proteinaceous plasma fluid: immunoglobulins, coagulation factors, etc

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2
Q

bone marrow - characteristics

A

*heterogeneous site for hematopoiesis; reservoir for proliferation and differentiation
*% hematopoiesis - predictable cellularity (10% decrease in celullarity per decade of life)
*myeloid:erythroid ratio (3:1) - predictable cellular composition

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3
Q

lymphoreticular organs - characteristics

A

*immune surveillance of “interior” body surfaces and components of internal fluids
*lymph nodes, MALT, spleen, liver

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4
Q

anemia - defined

A

*red blood cell “deficiency” with inadequate O2-carrying capacity for proper metabolic function
*characterized by hemoglobin < 95% of the reference interval for age, sex, and geographic location

note - anemia is a SECONDARY MANIFESTATION of other disease process

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5
Q

3 mechanistic classifications of anemias

A
  1. blood loss (hemorrhage)
    -acute (e.g. trauma)
    -chronic (e.g. slow loss, GI/GU)
  2. RBC destruction (hemolytic anemias)
    -intrinsic abnormalities (membranopathies, enzymopathies, hemoglobinopathies)
    -extrinsic abnormalities (immune mediation, RBC fragmentation syndromes)
  3. impaired RBC production (non-hemolytic anemias)
    -erythroblast maturation defects (vitamin deficiencies, hemoglobin synthesis defects)
    -stem cell production failure
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6
Q

morphologic classification: MICROcytic anemias

A

*DECREASED HEMOGLOBIN SYNTHESIS
*normal cellular proliferation and DNA synthesis
*PALER, SMALLER cells
*can result from decreased synthesis of heme or improper synthesis of globins

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7
Q

top 4 causes of microcytic anemias

A
  1. IRON DEFICIENCY***
  2. anemia of chronic disease
  3. thalassemias
  4. sideroblastic anemia
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8
Q

morphologic classification: MACROcytic anemias

A

*DEFECTIVE DNA SYNTHESIS
*asynchrony b/w nuclear and cytoplasmic maturation
*gigantic cells with immature chromatin: MEGALOBLASTS
-larger, normochromic red cells
-hypersegmented nuclei of granulocytes

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9
Q

top 4 causes of macrocytic anemias

A
  1. vitamin B12 deficiency
  2. folate deficiency
  3. drug-induced (e.g. methotrexate, dilantin, sulfa, AZT)
  4. other (alcoholism, liver disease, MDS, reticulocytosis)
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10
Q

morphologic classification: NORMOcytic anemias

A

*NORMAL cell size
*various pathogenetic mechanisms
-acute hemorrhage
-RBC enzyme defects (e.g G6PD deficiency)
-RBC membrane defects
-bone marrow disorders
-hemoglobin variants
-autoimmune hemolytic anemia

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11
Q

calculation for MCV (mean corpuscular volume)

A

Hct / RBC count

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12
Q

normal MCV range

A

80-100 fL

note - < 80 is microcytic; > 100 is macrocytic

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13
Q

calculation for MCHC (mean corpuscular hemoglobin concentration)

A

Hb / Hct

note - MCHC is the mean concentration of hemoglobin in RBC

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14
Q

reticulocytosis

A

a normal increase in reticulocytes in the peripheral blood in response to anemia (indicates that the bone marrow is properly compensating for the anemia)

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15
Q

iron deficiency anemia (IDA) - epidemiology

A

*primary cause of defective heme synthesis
*MOST COMMON CAUSE OF ANEMIA WORLDWIDE
*about 20% of women, 50% of pregnant women; 3% of men

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16
Q

iron deficiency anemia (IDA) - pathophysiology in infants/children

A

dietary insufficiency

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17
Q

iron deficiency anemia (IDA) - pathophysiology in adults

A

*CHRONIC BLOOD LOSS
*malabsorption
*menstruation
*blood donation
*hemoglobinuria

18
Q

iron deficiency anemia (IDA) - clinical features

A

*insidious, slowly progressive
*fatigue, irritability, dizziness, headache, breathlessness
*PICA: craving/ingestion of unusual substance
*impaired neuromuscular activity
*brittle, pitted nails
*atrophy of lingual papillae, burning/sore mouth
*dysphagia, gastritis

19
Q

iron deficiency anemia (IDA) - hematologic findings

A

*Hb usually < 8 g/dL (severe microcytic)
*low MCV and MDHC
*high RDW
*reticulocytosis - mild
*thrombocytosis
*bone marrow: erythroid hyperplasia mild/moderate

20
Q

iron deficiency anemia (IDA) - lab findings (CBC & serum iron panel)

A

*CBC: low Hb, low Hct, low RBC count, low MCV, high RDW, decreased reticulocytes

*iron studies:
-low serum iron
-LOW SERUM FERRITIN
-high TIBC
-low transferrin saturation %

*bone marrow iron stores depleted (not enough iron in the body)

21
Q

iron deficiency anemia (IDA) - RBC size & appearance

A

*pale cells, large central pale zones
*low MCV (<80)

22
Q

megaloblastic anemias

A

*2 types: folate deficiency anemia and vitamin B12 deficiency anemia
*characterized by macrocytic RBCs & hypersegmented neutrophils in peripheral blood

23
Q

megaloblastic anemias - diagnostic approach

A

*moderate to severe macrocytic anemias
*MCV range from 100-150 fL (macrocytic)
*MCHC normal
*hypersegmentation of neutrophils

24
Q

megaloblastic anemias - image

A

*hypersegmented neutrophils (>5 lobes) are an early sign of megaloblastic anemia, indicating a folate deficiency or vitamin B12 deficiency

25
Q

megaloblastic anemias - bone marrow diagnosis

A

*hypercellular bone marrow
*large cells, immature nuclei with mature cytoplasm
WARNING - may be mis-diagnosed as leukemia

26
Q

spherocytosis - image

A

*relatively no central pale zone
*spheres instead of flat, biconcave discs

27
Q

hemolytic anemias - overview

A

*anemias caused by hemolysis of red blood cells
*reduction of normal RBC lifespan
*hemolytic state accompanied by compensatory hyperplastic bone marrow

28
Q

laboratory tests for hemolysis

A

*elevated serum bilirubin (demonstration of clinical jaundice)
*decreased serum haptoglobin
*increased plasma hemoglobin
*positive Coomb’s test indicates autoimmune hemolysis
*urine hemoglobin & hemosiderin (dark urine)
*increased LDH
*look at reticulocyte count to see if bone marrow is compensating

29
Q

hereditary spherocytosis - overview

A

*intrinsic RBC membrane defect
*renders RBCs spherical, less deformable, vulnerable to splenic destruction and hemolysis
*autosomal dominant/recessive inherited disorder

30
Q

hereditary spherocytosis - clinical features

A

*chronic hemolytic anemia with reticulocytosis
*episodes of mild jaundice
*splenomegaly
*gallstones
*acute hemolytic crisis: fever, abdominal pain

31
Q

hereditary spherocytosis - diagnosis

A

*normocytic anemia
*SPHEROCYTES
*very high MCHC
*reticulocytosis
*increased osmotic fragility of RBCs
*elevated LDH and total bilirubin

32
Q

pure red cell aplasia - overview

A

*red blood cell failure
*destruction of erythropoietic cell elements
*can be acquired (including parvovirus B19) or congenital
*presents with s/s of anemia (fatigue, pallor)

33
Q

pure red cell aplasia - diagnostic approach

A

*peripheral blood: severe normocytic, normochromic anemia with marked reticulocytopenia
*bone marrow: complete/near absence of erythroblasts; increased M:E ratio

34
Q

aplastic anemia - overview

A

*HEMATOPOIETIC STEM CELL FAILURE
*destruction of ALL hematopoietic bone marrow elements
*majority of cases are idiopathic

35
Q

aplastic anemia - clinical features

A

*progressive fatigue, dyspnea, palpitations
*bleeding (bruising, etc)
*infections

36
Q

aplastic anemia - diagnostic approach

A

*peripheral blood: marked pancytopenia (low counts of all blood cell types)
*bone marrow: marked panhypoplasia (empty marrow)

37
Q

aplastic anemia - bone marrow image

A

“empty marrow”

38
Q

thalassemias - overview

A

*QUANTITATIVE defective production of globin (protein is normal but there is not enough)
*absence/decrease of one chain
*relative excess of other chain manifests as:
1. peripheral blood -> hemolysis
2. bone marrow -> ineffective hematopoiesis/apoptosis

39
Q

hemoglobinopathies - overview

A

QUALITATIVE defective production of globin (makes enough but not made “correctly”)

40
Q

HbS/β0 (alpha2βSβ0) sickle/beta thalassemia - characteristics

A

*indistinguishable from sickle cell disease
*severe microcytic anemia
*Hb quantities:
-HbA: 0%
-HbA2: > 5%
-HbS: < 90%
-HbF: < 30%

41
Q

HbS/β+ (alpha2βSβ+) sickle/beta thalassemia - characteristics

A

*mild sickling disease
*mild to moderate microcytic anemia
*Hb quantities:
-HbA: 15-30%
-HbA2: > 3.5%
-HbS: ~70%
-HbF: < 20%

42
Q

what are “target cells” a clue for

A

possibly a thalassemia

Heme/Lymph Exam (38 decks)

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