Bone Marrow Failure
Quiz yourself by thinking what should be in
each of the black spaces below before clicking
on it to display the answer.
Help!
|
|
||||
|---|---|---|---|---|---|
| A acquired for inherited condition defined as the inability to produce 2 or more of the normal blood elements. | Bone marrow failure
🗑
|
||||
| A condition defined as failure of pluripotential stem cells to produce red cells, white cells, and megakaryocytes. Both hematopoietic stem cells and progenitors are deficient in number. | Aplastic anemia
🗑
|
||||
| What is the inheritance pattern of Faconi Anemia? | Autosomal X-linked recessive (>12 FANC genes)
🗑
|
||||
| A bone marrow failure syndrome characterized by skin hyperpigmentation, cafe au lait spots, short stature, triangular face, abnormal thumbs/radii, microcephaly, abnormal kidneys, and decreased fertility. | Faconi Anemia
🗑
|
||||
| What test is diagnostic for Faconi Anemia? | Increased chromosome intrastrand cross-link breakage
🗑
|
||||
| What hematologic pathologies are associated wtih Fanconi Anemia? | Pancytopenia, macrocytosis, anemia, hypocellular marrow, MDS, leukemia, solid tumors
🗑
|
||||
| A bone marrow failure syndrome characterized by dyskeratotic nails, lacey reticular rash, and oral leukoplakia. | Dyskeratosis congenita
🗑
|
||||
| What is the inheritance pattern of Dyskeratosis Congenita? | X-linked inheritance or autosomal dominant inheritance
🗑
|
||||
| What hematologic pathologies are associated wtih Dyskeratosis Congeita? | Macrocytosis, anemia, thembocytopenia, neutropenia, hypocellular marrow, MDS< leukemia, solid tumors
🗑
|
||||
| A bone marrow failure syndrome characterized by short stature, abnormal thumbs, and pure red cell aplasia. | Diamond-Blackfan Anemia
🗑
|
||||
| What finding is diagnostic of Diamond-Blackfan Anemia? | Elevated red cell adenosine deaminase
🗑
|
||||
| What is the inheritance pattern of Diamond-Blackfan Anemia? | Autosomal dominant
🗑
|
||||
| A bone marrow failure syndrome characterized by short stature, malabsorption, neutropenia, pancreatic insufficiency, myeloid hypoplasia, MDS, and leukemia. | Schwachman-Diamond Syndrome
🗑
|
||||
| What finding is diagnostic of Schwachman-Diamond Syndrome? | Low serum trypsinogen and isoamylase
🗑
|
||||
| What is the inheritance pattern of Schwachman-Diamond Syndrome? | Autosomal recessive
🗑
|
||||
| What is the most common inherited bone marrow failure syndrome? | Fanconi anemia
🗑
|
||||
| What is the pathogenesis of Fanconi Anemia? | Defective DNA repair
🗑
|
||||
| What level of irradiation can cause complete hematopoietic failure? | 300-500 Grays
🗑
|
||||
| (T or F) Marrow suppression by benzene is dose related. | True.
🗑
|
||||
| (T or F) Aplastic anemia is primarily an immune-mediated disorder. | True.
🗑
|
||||
| Which hepatitis infection is most commonly associated with bone marrow failure? | Hepatitis B
🗑
|
||||
| What viral infection is a major cause of pure red cell aplasia? | Parvovirus B19
🗑
|
||||
| Clinical symptoms of aplastic anemia | (1) Weakness (2) Fatigue (3) Fever (4) Recurrent infections (5) Easy bruising (6) Bleeding (7) Purpura
🗑
|
||||
| Physical findings of aplastic anemia | (1) Pallor (2) Petechiae (3) Purpura (4) Ecchymoses
🗑
|
||||
| Laboratory findings of aplastic anemia | Pancytopenia with decreased reticulocytes
🗑
|
||||
| Findings of aplastic anemia on bone marrow biopsy | (1) Sparse cellularity (<25%) (2) Small islands of predominantly erythroid cells (3) other marrow elements are absent or sparsely distributed
🗑
|
||||
| How is pancytopenia secondary to hypersplenism from asplastic anemia. | Pancytopenia secondary to hypersplenism has a hyperplastic bone marrow rather than hypocellular bone marrow in aplasia.
🗑
|
||||
| What is the criteria on peripherial blood for severe pancytopenia? | Pancytopenia with at least two of the following: Neutrophils <500/uL, Platelets <20,000/uL, Reticulocytes <1% or 20,000/uL
🗑
|
||||
| What is the criteria for bone marrow for severe aplastic anemia? | (1) Severe hypocellularity (<25%) (2) No significant marrow fibrosis
🗑
|
||||
| What is defined as very severe aplastic anemia? | Neutrophil count < 200 uL
🗑
|
||||
| What is the first line of therapy for aplastic anemia? | Erytrhopoietin and G-CSF
🗑
|
||||
| Pathogenesis of Paroxysmal Nocturnal Hemglobinuria (PNH) | Deficiencies due to decreased production of glycosylphophatidylinsitol (GPI) anchor that links complement regulatory proteins to the cell membrane. This increases red cell sensitivity to complement mediated lyses, bleeding, thrombosis.
🗑
|
||||
| What gene is defective in Paroxysmal Nocturnal Hemoglobinuira (PNH)? | PIGA (phosphatidylinosotol glycan class A gene
🗑
|
||||
| What is the complement regulatory proteins deficient in Paroxysmal Nocturnal Hemoglobinuira (PNH)? | (1) Decay Accelerating Factor (CD 55) (2) Membrane Inhibitor of LYses (CD 59)
🗑
|
||||
| What hematologic disorder is characterized by episodes of hemolysis and hemoglobinuria that occurs more often at night but may be precipitated by infection, operations, and transfusions? | Paroxysmal Nocturnal Hemoglobinuira (PNH)
🗑
|
||||
| What are the flow cytometry analysis findings for Paroxysmal Nocturnal Hemoglobinuira (PNH)? | Flow cytometry analysis for GPI linked proteins show lack of at least 2 of these proteins: CD55, CD59, CD16.
🗑
|
||||
| What is the therapy for Paroxysmal Nocturnal Hemoglobinuira (PNH)? | (1) Blood transfusion (2) Folate supplemenation (3) Iron supplementation/chelation (4) Anticoagulation (5) Bone marrow transplation (6) Immunosuppressive therapy (7) Hematopoietic growth factors
🗑
|
||||
| What hematologic malignancy is associated with Paroxysmal Nocturnal Hemoglobinuira (PNH)? | Paroxysmal Nocturnal Hemoglobinuira (PNH) increases the risk of acute myeloid leukemia.
🗑
|
||||
| (T or F) Paroxysmal Nocturnal Hemoglobinuira (PNH) may progress to aplastic anemia. | True.
🗑
|
Review the information in the table. When you are ready to quiz yourself you can hide individual columns or the entire table. Then you can click on the empty cells to reveal the answer. Try to recall what will be displayed before clicking the empty cell.
To hide a column, click on the column name.
To hide the entire table, click on the "Hide All" button.
You may also shuffle the rows of the table by clicking on the "Shuffle" button.
Or sort by any of the columns using the down arrow next to any column heading.
If you know all the data on any row, you can temporarily remove it by tapping the trash can to the right of the row.
To hide a column, click on the column name.
To hide the entire table, click on the "Hide All" button.
You may also shuffle the rows of the table by clicking on the "Shuffle" button.
Or sort by any of the columns using the down arrow next to any column heading.
If you know all the data on any row, you can temporarily remove it by tapping the trash can to the right of the row.
Embed Code - If you would like this activity on your web page, copy the script below and paste it into your web page.
Normal Size Small Size show me how
Normal Size Small Size show me how
Created by:
UVAPATH4
Popular Medical sets