Lecture 11 Chapter 19 Flashcards
What is the reticulocyte count when you have anemia?
Low/decreased
What is the ratio of myeloid cells to adipocytes (fat cells)
Younger: more myeloid than fat cells
Middle age: equal ratio
Older: more fat cells than myeloid
What type of bone marrow and label the arrows
Normal bone marrow
Top: adipocytes (fat cells)
Bottom: myeloid cells
What type of bone marrow and label the arrow
Hypocellular
Spongy tissue
What can you do with an aspiration sample
Can make a smear and can look at what cells are there
Can send for full cytometry
Can tag the antigens on the cells to exactly identify amount of cells
Quantitative numbers
What can you do with a biopsy sample?
Can make smear by placing one slide on top and one on bottom and sliding it between (imprinting)
Let dry and stain
Maintains the architectural organization of the bone marrow
Can see the location in respect to the trabeculae of the spongy bones
What is this type of bone marrow, when is it normal, and when is it a problem/what does it indicate?
Hypercellular
Normal in 5 year olds
Not normal in adults - sign of leukemia
What do you have when there is a decrease in all cells (RBCs, WBCs, hemoglobin, platelets, hematocrit)
Pancytopenia
What do you have when you have a decrease in only RBCs
Erythrocytopenia
What type of anemia comes from pancytopenia
Aplastic anemia
What conditions can occur when you experience erythrocytopenia and how do you determine it
Pure red cell anemia
Congenital dyserythropoietic anemia (CDA)
Anemia of chronic renal insufficiency
Must perform a bone marrow biopsy and aspiration. Cannot determine from blood smear
What is another way of saying bone marrow failure (what condition)
Aplastic anemia
Inherited aplastic anemia can cause what 4 conditions?
Fanconi anemia
Dyskeratosis congenital
Amegakaryocytic thrombocytopenia (CAMT)
Schwan-diamond syndrome
What is the most common inherited aplastic anemia?
Fanconi anemia
What makes schwan-diamond syndrome unique?
It involves the pancreas so the enzymes amylase and lipase will be elevated
Majority of aplastic anemias are due to
Acquired conditions
What % of aplastic anemia cases have unknown origin?
70%
What are the known causes of acquired aplastic anemia
Pregnancy
Radiation
Drugs
Virus: Epstein Barr, hepatitis, ParvoVirus, HIV
Cancer: myelophthistic anemia
Hemotherapy drugs
Heavy duty antibiotics (older people)
What is the common cause of aplastic anemia in young people
Viral infection
Epstein Barr virus (infectious mononucleosis)
Symptoms of aplastic anemia
Low platelets = prone to bleeding
Low RBCs = symptoms of anemia
Low WBCs = prone to infections
What causes pancytopenia?
Destruction of stem cells (I.e. drug targets stem cells)
Cells die prematurely
Ineffective hematopoiesis (formation of blood cellular components)
Decrease production of growth factors and hormones (I.e. erythropoietin, thrombopoietin)
Infiltration of marrow spaces by cancer cells (myelophthistic anemia) - metastasis of cancer cells to other organs, divide, and take over where normal cells live
What is ParvoVirus B-19 and what happens to the cells
Autoimmune disease
Body’s immure cells make WBCs that make antibodies against a foreign antigen but those antibodies attack both the foreign antigen and your stem cells
What is apoptosis
It kills cells
Ex: fetus’ hands are like paddles, apoptosis kills the cells between the fingers to create hands
What is the primary etiologic reason for aplastic anemia
Cases that direct target the stimuli on the stem cells in your bone marrow
What is the secondary etiologic reason for aplastic anemia
Origin of problem started outside the bone marrow
Primary reasons for secondary aplastic anemia
Radiation, drugs (antibiotic and chemotherapy), viruses (Epstein Barr, hepatitis, parvovirus, HIV)
Symptoms of pancytopenia/aplastic anemia
Petechia
Purpura
Blue spots on body
Susceptible to infection
Anemia/anemia symptoms
Rash
Bone marrow hypocellular
With aplastic anemia, what happens to the serum iron and percentage of transferrin saturation and why?
Serum iron will be normal or increase because iron will still be made, but won’t be used as much so it will get stored. Transferrin saturation will increase. The iron in the serum is going to be normal or the upper level of normal or increased
Main causes of aplastic anemia
Hepatitis
Liver disease
Viruses that affect the liver
Explain why Fanconi anemia cannot get repaired like other gene mutations
Proteins for Fanconi anemia are one of those groups of proteins that are responsible for the correction and repairing of the problems in the DNA
How does Fanconi anemia effect the body
Problems with division of cells/stem cells
Aplastic anemia
Short statute
Significantly delayed mental development
Inverted thumbs
Malformed toenails
What is the confirmation assay for Fanconi anemia
When 4 chromosomes come together and create a Maltese cross
What kills those with Fanconi anemia
Aplastic anemia - they become prone to infection, develop an infection, have low immure system, so they die
What is the inherited aplastic anemia that pertains to a mutation in the telomeres
Dyskeratosis congenita
What do telomeres do?
They maintain the integrity of the chromosome
What makes us more prone to mutations/diseases as we get older
As we get older, our telomeres get shorter
How many genes can have the Fanconi anemia mutation
21 genes
How many genes can have the dyskeratosis congenita mutation
13 genes
What are some other problems that occur with dyskeratosis congenita before aplastic anemia diagnoses
Cognitive problems, developmental defects, anatomical issues, leukoplakia (accumulation of white substance on tongue, malformed nails
About 50% of what protein is affected by dyskeratosis congenita
Dyskerin (DCK1)
What is refractive anemia
Refers to the classes of anemia that do not respond to any treatment
What is the only way to confirm refractive anemias (pure red cell aplasia)
Bone marrow studies
How does someone get pure Red cell aplasia?
It can be genetic or acquired
What is the name of the genetic pure red cell aplasia and how does it work
Diamond-Blackfan syndrome/anemia
Has a mutation in the genes that are responsible for the maintenance of the ribosomes, which are very critical for protein synthesis
All of the acquired pure red cell aplasia’s are?
Fall into the class of autoimmune diseases
What will you see on a blood smear for congenital dyserythropoietic anemia
+4 poikilocytosis
Macrocytic cells
Congenital dyserythropoietic anemia
CDA type I
Congenital dyserythropoietic anemia
CDA type II
Congenital dyserythropoietic anemia
CDA type III familial
Congenital dyserythropoietic anemia
CDA type III sporadic
Congenital dyserythropoietic anemia
CDA variants
What is associated with/is the secondary cause of myelophthistic anemia
Cancer
What is extramedullary hematopoiesis
Once someone has melanoma, it spreads everywhere (ie lungs, liver, bone marrow). If it starts dividing in the bone marrow, it will kick out the cells already residing there. Precursors and item cells go to spleen, liver, and lungs and will begin dividing in those environments now
What is the most important morphology to see in myelophthisic anemia and what is also an indicator
Dacryocytes (“tear drop” cells)
nRBCs
Hypocellular bone marrow
Brown stain in bone marrow showing melanoma
Other form of bone marrow failure with kidneys
Anemia of chronic renal insufficiency
Most likely have kidney disease and can not make erythropoietin
Signs and symptoms of anemia of chronic renal insufficiency
Increase in waste products in blood, such as urea and creatinine
Any time there is an increase in urea (and other bile salts) it causes
An abnormal morphology of RBCs = echinocytes
Echinocytes = old blood or diseases affecting the kidneys
