Bone Marrow Failure

Question 1

True or false. The hypoproliferative anemias are normochromic, normocytic, or macrocytic, and are characterized by a low reticulocyte count.

Answer 1

True

Question 2

True or false. Pancytopenia is frequent in bone marrow failure.

Answer 2

True

Question 3

This account for 5% of aplastic anemia. Patients are usually young men who have recovered from a bout of liver inflammation 1-2 months earlier; the subsequent pancytopenia is very severe.

Answer 3

Posthepatitis marrow failure

Question 4

An acquired mutation in the ___ gene in a hematopoietic stem cell is required for the development of PNH.

Answer 4

PIG-A

Question 5

It is an autosomal recessive disorder, manifests as congenital developmental anomalies, progressive pancytopenia, and an increased risk of malignancy. Patients have typically short stature, café au lait spots, and anomalies involving the thumb, radius and genitourinary tract.

Answer 5

Fanconi anemia

Question 6

It is characterized by the triad of mucous membrane leukoplasia, dystrophic nails, reticular hyperpigmentation,a dn with the development of aplastic anemia in childhood. This is due to the mutations in the genes of the telomere repair complex.

Answer 6

Dyskeratosis congenita

Question 7

Its presentation is early in life with neutropenia with pancreatic insufficiency and malabsorption.

Answer 7

Schwachman-Diamond syndrome

Question 8

In aplastic anemia, replacement of the bone marrow by ___ is apparent in the morphology of the biopsy specimen and MRI of the spine.

Answer 8

Fat

Question 9

In aplastic anemia cells bearing the ___ antigen, a marker of early hematopoietic cells are greatly diminished.

Answer 9

CD34

Question 10

It is the most common early symptom of aplastic anemia.

Answer 10

Bleeding

Question 11

True or false. Lymphadenopathy and splenomegaly are highly atypicak of aplastic anemia.

Answer 11

True

Question 12

The diagnosis of aplastic anemia is usually straightforward, based in the combination of ___.

Answer 12

Pancytopenia with a fatty bone marrow.

Question 13

True or false. Aplastic anemia is a disease of the young and should be a leading diagnosis in the pancytopenic adolescent or young adult.

Answer 13

True

Question 14

The major prognostic determinant of aplastic anemia is the___.

Answer 14

Blood count.

Question 15

Severe aplastic anemia is characterized by 2 of these 3 parameters.

Answer 15

• absolute neutrophil count <500/ul
• platelet count <20000/ul
• corrected reticulocyte count <1% (or absolute reticulocyte count <60,000/ul)

Question 16

True or false. Glucocorticoids are not of value as primaty therapy for aplastic anemia.

Answer 16

True.

Question 17

This is the best therapy for the younger patient with aplastic anemia with a fully histocompatible sibling donor.

Answer 17

Hematopoietic stem cell transplantation

Question 18

In aplastic anemia, the standard regimen of ____ induces hematologic recovery (independence from transfusion and a leukocyte count adequate to prevent infection) in 60-70% of patients.

Answer 18

Antithymocyte globulin in combination with cyclosporine

Question 19

It is a flulike illness with a characteristuc cutaneous eruption and arthralgia, often develops 10 days after initiationg Horse ATG in aplastic anemia.

Answer 19

Serum sickness

Question 20

These are the most important factors weighing decision between transplant and immunosuppression in aplastic anemia.

Answer 20

Increasing age and severity of neutropenia

Question 21

In aplastic anemia, prophylactic platelet transfuion is made to attain the goal of playelet count of ___.

Answer 21

> 10,000/ul

Question 22

This is characterized by anemia, reticulocytopenia, and absent or rare erythroid precursor cells in the bone marrow.

Answer 22

Pure red cell aplasia

Question 23

This should be sought by radiographic procedures in patients suspected with pure red cell aplasia.

Answer 23

Thymoma.

Tumor excision is indicated, but anemia does not necessarily improve with surgery.

Question 24

True or false. The diagnosis of parvovirus infection requires detection of viral DNA sequences in the blood (IgG and IgM antibodies are commonly absent).

Answer 24

True.

Question 25

Myelodysplasia are a heterogenous group of hematologic disorders broadly characterized by both:

Answer 25

1. Cytopenias due to bone marrow failure

2. A high risk of development of AML

Question 26

In MDS, anemia, often with thrombocytopenia and neutropenia, occurs with dysmorphic and usually (cellular or acellular?) bone marrow, which is evidence of ineffective blood cell production.

Answer 26

Cellular

Question 27

Characteristic epidemiology of MDS

Answer 27

Elderly, mean ate onset is older than 70 years, male preponderance

Question 28

This dominates the early course of MDS

Answer 28

Anemia

Question 29

True or false. Fever and weight loss should point to a myeloproliferative rather than myelodysplastic process.

Answer 29

True

Question 30

True or false. Children with Down syndrome are susceptible to MDS as well as leukemia.

Answer 30

True