Lecture 11 Chapter 19 Flashcards by Mikayla Cleary

What is the reticulocyte count when you have anemia?

Low/decreased

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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

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What type of bone marrow and label the arrows

Normal bone marrow
Top: adipocytes (fat cells)
Bottom: myeloid cells

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What type of bone marrow and label the arrow

Hypocellular
Spongy tissue

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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

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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

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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

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What do you have when there is a decrease in all cells (RBCs, WBCs, hemoglobin, platelets, hematocrit)

Pancytopenia

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What do you have when you have a decrease in only RBCs

Erythrocytopenia

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What type of anemia comes from pancytopenia

Aplastic anemia

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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

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What is another way of saying bone marrow failure (what condition)

Aplastic anemia

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Inherited aplastic anemia can cause what 4 conditions?

Fanconi anemia
Dyskeratosis congenital
Amegakaryocytic thrombocytopenia (CAMT)
Schwan-diamond syndrome

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What is the most common inherited aplastic anemia?

Fanconi anemia

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What makes schwan-diamond syndrome unique?

It involves the pancreas so the enzymes amylase and lipase will be elevated

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Majority of aplastic anemias are due to

Acquired conditions

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What % of aplastic anemia cases have unknown origin?

70%

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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)

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What is the common cause of aplastic anemia in young people

Viral infection
Epstein Barr virus (infectious mononucleosis)

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Symptoms of aplastic anemia

Low platelets = prone to bleeding
Low RBCs = symptoms of anemia
Low WBCs = prone to infections

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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

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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

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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