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Terms in this set (90)
Group of acquired or constitutional (hereditary) disorders
Can be ____________/______________ or _______________/_________________. (Cell size and color)
Whats is hematopoietic tissue replaced by?
What kind of bone marrow?
What other terms are used for Hypoproliferative anemia?
Defect is due to what?
depletion, damage, or inhibition of stem cells and/or progenitor cells
What Red blood cell line is effected?
Erythroid Progenitor cell
(Pure Red cell Aplasia)
Are WBCs and Platelets Normal?
Decrease in all 3 cells lines
Acquired is in _____.
Constitutional is in ___________.
Diagnostic criteria for Aplastic Anemia
Bone marrow cellularity <25% plus atleast 2 of the following
1) Granulocytes <0.5x10^9/L
3)Anemia with RPI<1
Normal cellularity should be what percent cells, and what percent fat?
Term that means No environmental link or cause can be found
Which is Idiopathic? Acquired or Constitutional
What percent of Acquired Aplastic Anemia cases are Idiopathic?
What are the causes of Aplastic Anemia
What % of Acquired Aplastic Anemia are associated with Drug exposure/therapy?
What develops before anemia or thrombocytopenia in Acquired Aplastic Anemia due to Drug exposure/therapy?
How do drugs cause Acquired Aplastic Anemia?
Accumulates in the bone marrow and injures stem cells
What kinds of chemical agents can cause Acquired Aplastic Anemia?
Toxic chemicals in industry and agriculture
How long may it take for Acquired Aplastic Anemia to develop after exposure to chemical agents?
Weeks, Months, or Years
How can an individual be exposed to Ionizing Radiation?
2)Military Nuclear Tests
3)Therapeutic Regimens for Malignancy
The effects of Ionizing Radiation depend on what?
What are Infectious Agents?
How do Viral Infections and Bacterial Infections cause Acquired Aplastic Anemia?
Infect stem cells
What is triggered when viral/bacterial infections infect stem cells?
How do Autoimmune disorders cause Acquired Aplastic Anemia?
Suppression of Stem cell growth and differentiation
How does Immunosuppressive Therapy cause Acquired Aplasic Anemia?
Damages all proliferating cells
What is a common type of Constitutional Aplastic Anemia?
Is Fanconi Anemia Acquired or Hereditary?
Molecular defect causing abnormal chromosomal fragility
Fanconi anemia involves what 5 complex Congenital anomalies
1)Progressive Bone Marrow Hypoplasia
2)Abnormal bone development resulting in dwarfism
3)Renal abnormalities and other organ malfunctions
Is Fanconi Anemia slowly or quickly progressive from birth?
What is the age range at which Fanconi Anemia appears?
What clinical signs of Pancytopenia does Fanconi Anemia exhibit?
What is a possible curative treatment for Fanconi Anemia?
Stem Cell Transplant
What % chance is there of developing cancer in an individual who has Fanconi Anemia?
What kind of cancer can develop in an individual who has Fanconi Anemia?
1)Acute non-lymphocytic leukemia
How is Fanconi Anemia definitively diagnosed?
What does chromosomal karyotyping look for?
1)Increase in spontaneous chromosomal breakage
Clinical Finding in Aplastic Anemia
2)Petechia and hemorrhages
What are symptoms of Aplastic Anemia related to?
The peripheral blood for Aplastic Anemia will show mild to moderate _______________ & ___________ as well as relative _______________
What is necessary to observe when diagnosing Aplastic Anemia?
What will the observation of Bone marrow show in an individual with aplastic anemia?
3)Mature Blood cells appear normal
Other abnormal Finding in Aplastic Anemia
1)EPO is ofter increased
2)Increased serum iron
3)>50% saturation of transferrin
What are 5 forms of therapy for aplastic anemia?
1)Remove Causative Agent/Supportive Car (1st aim in acquired aplastic anemia)
2)Hematopoietic growth factors
4)Stem Cell transplant
5)Bone Marrow transplant
Syndrome characterized by selective aplasia of erythroid lineage (Hypoproliferation of the erythroid progenitor)
Pure Red Cell Aplasia
Forms of Pure Red Cell Aplasia
1)Acquired (Acute or Chronic)
2)Inherited (Diamond-Blackfan syndrome)
Laboratory Findings in Pure Red Cell Aplasia
Temporary depression of erythropoiesis occurs in what type of Pure Red Cell Aplasia?
What are the causes of Acquired Acute Pure Red Cell Aplasia?
2)Drugs and Chemicals
Is the RBC lifespan increased or decreased during Aplastic Crisis of Acquired Acute Pure Red Cell Aplasia
What Anemias are present during the Aplastic Crisis of Acquired Acute Pure Red Cell Aplasia
What is Aplastic Crisis of Acquired Acute Pure Red Cell Aplasia preceded by?
2)Upper respiratory complaints
How is Acquired Acute Pure Red Cell Aplasia treated?
Transfusion of packed RBCs
Does Acquired Chronic Pure Red Cell Aplasia occur in children or adults?
Middle aged Adults
What does Acquired Chronic Pure Red Cell Aplasia occur in association with?
2)Autoimmune hemolytic anemia or diseases
3)Hematologic neoplasms (CLL & Hodgkin Disease)
What should you do in Acquired Chronic Pure Red Cell Aplasia is Drug induced?
Remove the drug
What are therapy options for Acquired Chronic Pure Red Cell Aplasia?
1)Transfusion of packed RBCs
2)Thymectomy if enlarged thymus
Rare congenital progressive RBC aplasia
Does Diamond-Blackfan Syndrome occur in children or adults?
Very young children
With Diamond-Blackfan Syndrome, symptoms of anemia begin to occur at what age?
Is there or is there not Leukopenia or Thrombocytopenia with Diamond-Blackfan Syndrome
No leukopenia or thrombocytopenia
Are congenital defects present in Diamond-Blackfan Syndrome?
Is there predisposition to cancer with Diamond-Blackfan Syndrome?
Are the EPO levels consistently increased or decreased with Diamond-Blackfan Syndrome?
What are the erythroid progenitor cells that have an intrinsic defect with Diamond-Blackfan Syndrome?
BFU-E & CFU-E
What does the bone marrow show in Diamond-Blackfan Syndrome?
The anemia with Diamond-Blackfan Syndrome is severe _________/________ (cell size and color)
Are there increased or decreased Reticulocytes with Diamond-Blackfan Syndrome
What 5 things are shown in the diagnosis of Diamond-Blackfan Syndrome?
1)No spontaneous breakage with chromosomal karyotyping
2)Vitamin B12 and Folate levels are normal
3)Increased Serum iron and Serum ferritin
4)Transferrin is 100% saturated
Therapy for Diamond-Blackfan Syndrome
Another Hypoproliferative disorder
Renal Disease Pathophysiology
2)EPO normal but bone marrow does not respond
Is there increased or decreased RBC survival in Renal Disease?
Where/When is there blood loss in renal disease?
What anemia is present with renal disease? (size and color )
The Hgb level in renal disease
Retic Production Index in Renal disease
~1 (very low)
Are there nRBCs in renal disease?
Yes (seen in peripheral smear)
There is moderate to severe poikilocytosis in renal disease showing what kinds of shapes...
Are WBCs and Platelets Increased, Decreased, or Normal in Renal Disease?
What does the Bone marrow show in renal disease?
The BUN in renal disease is...
Is serum creatinine increased or decreased in renal disease?
Is serum ferritin increase or decreased in renal disease?
Therapy for renal disease
1)Renal transplantation and/or hemodialysis
3)Iron and Folate supplements
4)Doses of EPO
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