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Lecture 16
Bone Marrow Failure
| Question | Answer |
|---|---|
| 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. |
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UVAPATH4
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