RBC path Flashcards

1
Q
\_\_\_\_ system
Blood cell forming system
Lymph tissue 
Bone Marrow
Red bone marrow 
Yellow bone marrow 
Circulating blood
A

Hematopoietic System

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2
Q

Process in which red and white blood cells are
produced
Red bone marrow

A

Hematopoiesis

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3
Q
 Erythropoiesis
 Regulated by kidneys
Erythropoietin
 1% of RBCs replaced daily
 Life span
120 days (4 months)
A

Erythrocytes

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4
Q

 Immature red blood cells
 Reticular network of RNA in cytoplasm
 Normal range: 0.5% to 1.5%
 Indicator of bone marrow activity
 Reticulocytosis – elevated number of reticulocytes in blood
 Reticulocyte count should be appropriate to the clinical situation

A

Reticulocytes

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5
Q

Heme – non-protein portion
Iron porphyrin - 4 pyrrole rings + iron
Globin – protein portion
HbA (Adult Hb) – 2 alpha, 2 beta
HbF (Fetal Hb) – 2 alpha, 2 gamma
Normal adult red cells contain mainly HbA

A

Hemoglobin

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6
Q

 A reduction in the erythron – a reduction in the total red cell mass
below normal limits
 Reduction in the oxygen carrying capacity of the blood leading to
tissue hypoxia
 Usually diagnosed based on:
Inadequate numbers of erythrocytes (low hematocrit - the ratio
of packed red cells to total blood volume)
Inadequate level of hemoglobin – the hemoglobin concentration
of the blood

A

Anemia

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7
Q

The lifespan of a red blood cell is about ____ days

A

120 days

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8
Q

Increased RBC destruction
Decreased RBC production
Blood loss

A

Anemia

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9
Q
Clinical Features of \_\_\_\_\_:
Pallor – pale skin and mucosa
Lethargy – lack of energy, fatigue, weakness
Dyspnea – labored breathing, SOB
Tachycardia, arrhythmia, chest pain
Koilonychia - spoon-shaped nails 
Atrophic glossitis 
Cognitive problems, dizziness
Cold extremities
Headache
A

Anemia

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10
Q

 A hemoglobinopathy
 Inherited, mis-sense mutation of beta chain
 A single AA substitution of valine for glutamic acid
 Forms a new, abnormal hemoglobin, Hemoglobin S - HbS
 Sickle cell disease – homozygous HbS
 Sickle cell trait - heterozygous, a less serious condition

A

Sickle cell anemia

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11
Q

 Individuals with sickle cell trait (heterozygous for HbS) have a survival
advantage in malaria-endemic areas
 Homozygous normal – increased mortality due to malaria
 Heterozygous – survival advantage
 Homozygous – increased mortality due to sickle cell disease
 About 8% of African Americans are heterozygous (sickle cell trait)
 1 in 600 African Americans are homozygous (sickle cell disease)

A

HbS

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12
Q
Clinical Consequences of 
\_\_\_\_\_\_\_
Increased susceptibility to infection with 
encapsulated organisms
Pneumococcus pneumoniae
Hemophilus influenzae
A

Splenectomy

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13
Q
“Mediterranean” anemia
Group of inherited diseases
Quantitative problem - too few globins synthesized
Underproduction of normal globin proteins due to 
mutations in regulatory genes
 Ineffective production of 
globin chains 
Alpha globin chains (α
Thalassemia)
Beta globin chains (β
Thalassemia)
 Regular transfusions – iron 
overload – organ damage
 Bone deformities – expansion 
of marrow space
 Splenomegaly - splenectomy
 Impaired growth 
 Bone marrow transplantation
A

Thalassemia

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14
Q

Two genes involved in making beta chain (one from
each parent)
Severity depends on number of affected beta chain
genes
One gene – beta-thalassemia minor - beta-thalassemia
trait
Mild disease
Two genes – beta-thalassemia major (Cooley’s anemia)
Severe disease

A

Beta Thalassemia

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15
Q

 Four genes involved in making alpha chain (two from each parent)
 Severity depends on number of affected alpha chain genes
 One gene – asymptomatic carrier
 Two genes – alpha-thalassemia minor - alpha-thalassemia trait
 Mild disease
 Three genes – hemoglobin H disease
 Moderate to severe disease
 Four genes – alpha-thalassemia major – (lethal)

A

Alpha Thalassemia

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16
Q

ABO mismatch leads to _______

A

intravascular hemolysis

17
Q
 Rh- mom
 Rh+ fetus
 Fetal RBCs cross the placenta and 
enter the maternal circulation 
during birth trauma
 Prophylactic anti-Rh (D) immune 
globulin (Rhogam) within 72 hours 
of delivery
 Rhogam lyses fetal RBCs in the 
maternal circulation – effectively 
removing any available antigen, 
so the mom doesn’t develop anti-
Rh antibodies
A

Erythroblastosis Fetalis:

1st Pregnancy at Delivery

18
Q
2nd Pregnancy at Delivery  
 Rh- mom, with anti-Rh from prior 
pregnancy
 Rh+ fetus 
 Anamnestic response rapidly 
produces anti-Rh (IgG)
 Anti-Rh IgG crosses placenta 
and lyses fetal RBCs
 Rh-mediated hemolytic disease
A

Erythroblastosis Fetalis:

19
Q

Immunoglobulin
Administered to Rh-negative women after pregnancies
in which they carried Rh-positive fetuses
Anti-D antibodies

A

Rhogam:

Rhesus Immune Globulin - RhIg

20
Q

 Rh incompatibility
 Hemolytic anemia in utero
 Rh-negative mother develops antibodies against Rh-positive erythrocytes of
fetus
 Antibodies cross placenta and hemolyze fetal erythrocytes
 High levels of bilirubin and biliverdin
 Deposition in developing teeth
 Only primary teeth affected

A

Erythroblastosis Fetalis

21
Q

Clinical Features of __________

 Anemia caused by immune destruction of erythrocytes
 Erythroblasts in peripheral blood
 Hyperbilirubinemia
 Kernicterus (bilirubin encephalopathy) if bilirubin reaches a high
levels
 Developmental dental defects reported

A

Erythroblastosis Fetalis

22
Q

 X-linked disease; most common human enzyme defect (African-American male population)
 Most are asymptomatic; at risk for non-immune hemolytic anemia upon exposure to oxidative stress
 Oxidative stress: infections, drugs (aspirin)
 G6PD / NADPH / Glutathione pathway - maintains supply of reduced glutathione to scavenge free
radicals (anti-oxidant)
 Red cells sustain damage from oxidizing free radicals (phagocytosed in spleen)
 All individuals with Favism (hemolysis due to Broad Beans(fava)) are G6PD deficient
 Survival advantage in Malaria endemic environments

A

Glucose-6-Phosphate

Dehydrogenase Deficiency

23
Q

Protozoal disease – primarily Plasmodium falciparum
Female Anopheles mosquito vector, human reservoir
Reproduction in red cells – showers of organisms
produce shaking, chills and fever
Hemolytic anemia
High morbidity, mortality

A

Malaria

24
Q
 Most common anemia in US
 Lack of Fe most common nutritional 
deficiency in the world
 Microcytic, hypochromic
 Seen most often in females – more iron 
lost in menses than replaced by nutrition
 Treated with iron supplements
 When in males, suspect internal 
bleeding
A

Iron Deficiency Anemia

25
Q

Dietary lack
Impaired absorption
Increased requirement
Chronic blood loss

A

Iron deficiency

26
Q

Iron Deficiency is Usually Caused by

______ or ______

A

Dietary Lack or Blood Loss

27
Q
Scandinavian, Northern European women
Severe Fe-deficiency anemia
Mucosal atrophy - atrophic glossitis 
Esophageal webs - dysphagia
Increased risk for squamous cell carcinoma
Esophagus
Oropharynx
Posterior Oral Cavity
A

Plummer-Vinson Syndrome

28
Q
Absorption of \_\_\_\_\_\_\_ requires intrinsic 
factor, which is 
secreted by the 
parietal cells of the 
stomach
A

vitamin

B12

29
Q

Autoimmune disease
Not due to dietary deficiency of B12
A form of megaloblastic anemia caused by
autoimmune gastritis and failure of intrinsic factor
production leading to vitamin B12 deficiency
Loss of ability to absorb Vitamin B12
Vitamin B12 (cobalamin) required for normal folate
metabolism and DNA synthesis

A

Pernicious anemia

30
Q

Megaloblastic anemia
Dietary deficiency of folic acid
Folate required for DNA synthesis

A

Folic acid deficiency

31
Q

 Marrow aplasia secondary to supression of multipotent myeloid
stem cells (erythrocyte, leukocyte and thrombocyte series),
resulting in pancytopenia
 May be caused by known myelotoxic agents (eg. whole body
radiation)
 Antineoplastic drugs (alkylating agents, antimetabolites)
 Benzene
 Chloramphenicol
 Pathogenesis may involve T cell attack on myeloid stem cells
 Prognosis unpredictable
 Transfusion, bone marrow transplant

A

Aplastic Anemia

32
Q

 An increase in the RBC mass
 Relative polycythemia – dehydration – decreased plasma volume with normal red
cell mass
 Absolute polycythemia – a true increase in red cell mass
 Primary polycythemia (polycythemia vera)
 Erythropoietin-independent
 Acquired, clonal stem cell disorder (a chronic myeloproliferative disorder)
 Secondary polycythemia
 Erythropoietin-dependent
 Compensatory response to tissue hypoxia
 Chronic lung disease
 Cigarette smoking
 Residence at high altitude
 Paraneoplastic syndromes

A

Polycythemia (Erythrocytosis)