Bone Marrow Failure Flashcards
True or false. The hypoproliferative anemias are normochromic, normocytic, or macrocytic, and are characterized by a low reticulocyte count.
True
True or false. Pancytopenia is frequent in bone marrow failure.
True
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.
Posthepatitis marrow failure
An acquired mutation in the ___ gene in a hematopoietic stem cell is required for the development of PNH.
PIG-A
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.
Fanconi anemia
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.
Dyskeratosis congenita
Its presentation is early in life with neutropenia with pancreatic insufficiency and malabsorption.
Schwachman-Diamond syndrome
In aplastic anemia, replacement of the bone marrow by ___ is apparent in the morphology of the biopsy specimen and MRI of the spine.
Fat
In aplastic anemia cells bearing the ___ antigen, a marker of early hematopoietic cells are greatly diminished.
CD34
It is the most common early symptom of aplastic anemia.
Bleeding
True or false. Lymphadenopathy and splenomegaly are highly atypicak of aplastic anemia.
True
The diagnosis of aplastic anemia is usually straightforward, based in the combination of ___.
Pancytopenia with a fatty bone marrow.
True or false. Aplastic anemia is a disease of the young and should be a leading diagnosis in the pancytopenic adolescent or young adult.
True
The major prognostic determinant of aplastic anemia is the___.
Blood count.
Severe aplastic anemia is characterized by 2 of these 3 parameters.
- absolute neutrophil count <500/ul
- platelet count <20000/ul
- corrected reticulocyte count <1% (or absolute reticulocyte count <60,000/ul)
True or false. Glucocorticoids are not of value as primaty therapy for aplastic anemia.
True.
This is the best therapy for the younger patient with aplastic anemia with a fully histocompatible sibling donor.
Hematopoietic stem cell transplantation
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.
Antithymocyte globulin in combination with cyclosporine
It is a flulike illness with a characteristuc cutaneous eruption and arthralgia, often develops 10 days after initiationg Horse ATG in aplastic anemia.
Serum sickness
These are the most important factors weighing decision between transplant and immunosuppression in aplastic anemia.
Increasing age and severity of neutropenia
In aplastic anemia, prophylactic platelet transfuion is made to attain the goal of playelet count of ___.
> 10,000/ul
This is characterized by anemia, reticulocytopenia, and absent or rare erythroid precursor cells in the bone marrow.
Pure red cell aplasia
This should be sought by radiographic procedures in patients suspected with pure red cell aplasia.
Thymoma.
Tumor excision is indicated, but anemia does not necessarily improve with surgery.
True or false. The diagnosis of parvovirus infection requires detection of viral DNA sequences in the blood (IgG and IgM antibodies are commonly absent).
True.
Myelodysplasia are a heterogenous group of hematologic disorders broadly characterized by both:
- Cytopenias due to bone marrow failure
2. A high risk of development of AML
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.
Cellular
Characteristic epidemiology of MDS
Elderly, mean ate onset is older than 70 years, male preponderance
This dominates the early course of MDS
Anemia
True or false. Fever and weight loss should point to a myeloproliferative rather than myelodysplastic process.
True
True or false. Children with Down syndrome are susceptible to MDS as well as leukemia.
True
