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Lecture 16

Bone Marrow Failure

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.
Created by: UVAPATH4