Bone Marrow failure

Question 1

This may result due to bone marrow failure

Answer 1

Thrombocytopenia - bleeding and increased bruising
Decreased RBC and Hgb - pallor, fatigue, cardiovascular complications
Neutropenia- increased life-threatening bacterial or fungal infections

Question 2

It a result of fatal bone marrow failure syndrome that leads to cessation of hematopoiesis

Answer 2

Aplastic anemia

Question 3

Tow major categories of acquired aplastic anemia

Answer 3

Idiopathic -no known cause (70%)

Secondary - identified cause (10-15%)

Question 4

Ages of this particular group is at higher risk of developing aplastic anemia

Answer 4

• Occur at any age but with peak incidince at 15-25 yes old

Question 5

Secondary Aplastic anemia is associated with

Answer 5

exposure to certain drugs, chemicals, radiations, or infections.

Note: related to degree of exposure and doses

Question 6

Acquired aplastic anemia occurs occasionally as complications of infection with what viruses?

Answer 6

HIV
Hepatitis viruses
Epstein-Barr virus
Human parvovirus

Question 7

Test Result for px with aplastic anemia:

Note: RBC is macrocytic or normocytic

Answer 7

↓

• Neutrophil count (toxic granulation is seen)
• Absolute lymphocyte count
• percent and absolute reticulocyte count

↑

• serum iron
• transferrin saturation q
• serum erythropoietin
• thrombopoietin
• Colony stimulating factor (G-CSF)
• GM-CSF
• MCV / normal

Question 8

Patients with alplastic anemia tends to have shorter ________ in their peripheral blood granulocytes compared with age-matched controls

Answer 8

Telomeres

Note: Telomeres protect ends of chromosomes from damage and erosion, and cells with abnormally short telomers undergo proliferation arrest and premature apoptosis.

Question 9

Symptoms for aplastic anemia in in conditions of

Reticulocytopenia
Thrombocytopenia
Neutropenia

Answer 9

Reticulocytopenia
- pallor, fatigue, tachycardia, hypotension, cardiac failure, or even death

Thrombocytopenia
- petechiae, bruising, mucosal bleeding, hemorrhages

Neutropenia
- bacterial or fungal infections

Question 10

Treatment for Aplastic anemia: True or False

Platelets should not be transfused at levels greater than 10,000 μl unless the px is bleeding

Answer 10

True

Question 11

What is the most accurate choice of treatment in px with severe aplastic anemia who’s younger than 40 yrs old and has a HLA identical sibling

Answer 11

Hematopoietic stem cell transplantation

given that he/she has HLA (human leukocyte antigen) identical sibling

Question 12

What is the most accurate choice of treatment in px with severe aplastic anemia who’s younger than 40 yrs old that doesn’t have HLA identical sibling?

Answer 12

Immunosuppressive therapy consisting of antithymocyte globulin and cyclosporine

Question 13

It is a rare hereditary disorder in the category of inherited bone marrow failure syndromes. It mainly affects the bone marrow and thus results in decreased production of all types of blood cells

Answer 13

Fanconi anemia (FA)

Question 14

Clinical findings for Fanconi anemia

Answer 14

• short stature
• skeletal abnormalities
• skin pigmentation (hyper/hypo pigmentation)
• Abnormalities of eyes, kidneys, and genitals

Question 15

Increased levels of these can be seen in px with Fanconi anemia

Answer 15

Fetal hemoglobin and alpha fetoprotein

Question 16

A type of inherited aplastic anemia characterized by:

chromosome instability disorder
pancytopenia
macrocytic ability
hypocellular bone marrow

Answer 16

Fanconi Anemia

Question 17

A type of inherited aplastic anemia characterized by:

mucocutaneous abnormalities
very short telomeres
pancytopenia
macrocytic rbc

Answer 17

Dyskeratotis congenita

Question 18

A type of inherited aplastic anemia characterized by:

pancreatic insufficiency / decreased pancreatic enzyme secretion
cytopenia (either of the three cells)
skeletal abnormalities
predisposition for hematologic malignancies

Answer 18

Swachman- Bodian-Diamond Syndrome

Question 19

A type of inherited aplastic anemia that causes gastrointestinal malabsorption due to decrease in pancreatic enzymes such as trypsinogen and isoamylase.

Answer 19

Shwachman-Bodien-Diamond Syndrome

Question 20

It is a rare disorder of blood production in which the bone marrow, the spongy tissue in the center of the bones, fails to function in an adequate manner resulting in anemia

Answer 20

Pure Red Cell aplasia

Question 21

The primary cause of anemia in Chronic kidney disease (CKD)

Answer 21

inadequate renal production of erythropoietin and uremia

Question 22

Anemia is a common implication of this disease

Answer 22

Chronic Kidney disease