Hematology II: Exam 5
What are the therapy options for Chronic Acquired Pure Red Cell Aplasia?
- Transfusions - Thymectomy - Immunosuppression - Remove the drug
What are the symptoms/side effects of Fanconi anemia?
- abnormal chromosome fragility - dysplasia of the bones - renal abnormalities - dwarfism - mental retardation - microcephaly, hypogonadism, skin hyperpigmentation - risk of developing cancer
What are the lab findings of Anemia of Chronic Kidney Disease?
- decreased hemoglobin when BUN is increased - decreased: -- erythropoietin -- RBC survival -- folate levels - moderate anisocytosis - moderate to severe poikilocytosis - NRBC - Increased serum creatinine -- Schistocytes, burr cells
What are therapy options for the Diamond-Blackfan Syndrome?
- RBC transfusions - adrenal cortocoid steroids - chelation therapy to decrease serum iron
What therapy is considered for Anemia of Chronic Kidney Disease?
- Renal transplant - dialysis - iron and folate supplements - erythropoietin
What are the lab findings of aplastic anemia?
- Pancytopenia - decreased hemoglobin - NRBC and Teardrop red cells - increased Hemoglobin F - Increased levels of the "i" antigen - increased erythropoietin - increased serum iron
What are the lab findings for Diamon-Blackfan Syndrome?
- decreased reticulocyte count - increased erythropoietin - normal or slightly increased WBC - Normal or increased platelet count - Normal levels of Vitamin B12 or Folate - increased hemoglobin F (5-25%) - increased serum iron and ferritin levels
What is the purpose of ATP?
1. Used for cation transport across the cell membrane 2. maintain membrane deformability 3. maintain biconcave shape
What is Transient-Erythroblastopenia of Childhood (TEC)?
A slowly developing anemia that occurs in early childhood and is characterized by a gradual onset of pallor.
Which cancers are associated with Myelophthisic anemia? (more than one is correct) a. prostrate b. breast c. testicular d. stomach e. leukemia
A, B, D - prostrate - breast - stomach
In pyruvate kinase deficiency, loss of ATP causes what? a. Loss of K+ and gain of Na+ and Ca++ b. Unable to maintain cation jump c. cell membrane becomes damaged d. cell becomes dehydrated *multiple answers
All of the above
What is Myelophthisic anemia?
An anemia where the bone marrow is replaced or infiltrated by fibrotic or neoplastic cells
True or False: Heinz Bodies and spherocytes are seen in pyruvate kinase deficiency?
False
True or False: Pyruvate Kinase deficiency is life threatening to neonates and causes little to no jaundice.
False: it causes severe jaundice
What is favism?
Hemolytic anemia caused by fava beans
When is the Rapoport-Luebering Shunt stimulated?
Hypoxia
What does the Quantitation of G6PD measure?
Measures the rate of reduction of NADP to NADPH at 340 nm in a spectrophotometer
What therapy is used for pyruvate kinase deficiency?
No specific therapy, but RBC transfusions can be used along with a splenectomy in severe cases.
The hexose monophosphate shunt catabolizes what? a. 10% glucose b. 10% glycogen c. 50% glucose
a. 10% glucose
Aplastic anemia: what must the reticulocyte count be? a. <1% b. <2% c. <3% d. <5%
a. <1%
What are the stipulations regarding the absence of Hemoglobin F in Transient-Erythroblastopenia of Childhood (TEC)? a. >1 year of age b. >5 years of age c. <1 year of age d. <5 years of age
a. >1 year of age
Which of the following is *not* associated with Acquired Acute Red Cell Aplasia? a. ALL b. Rheumatoid arthritis c. CLL d. Hodgkins lymphoma
a. ALL
What does Hypoproliferative Anemia mean? a. Bone Marrow Failure b. Platelet failure c. A pre-leukemia anemia b. A post-leukemia anemia
a. Bone Marrow Failure
What can cause the reduction of NADP ---> NADPH? a. G6PD enzymes b. glucose c. ATP d. ADP
a. G6PD enzymes
Blister cells come from which red blood cell inclusion? a. Heinz bodies b. howell- jolly bodies c. pappenheimer bodies
a. Heinz bodies
Glutathione is maintained by the conversion of what? a. NADPH--> NADP b. NADP ---> NADPH c. Glucose---> ATP
a. NADPH---> NADP
Which Pure Red Cell Aplasia is *rare*? a. chronic acquired pure red cell aplasia b. acute acquired pure red cell aplasia d. inherited pure red cell aplasia
a. chronic acquired pure red cell aplasia
Which of the following describes Pure Red Cell Aplasia? a. decrease in erythroid precursors in the bone marrow b. temporary suppression of erythropoiesis after a viral infection in normal children c. reduction of cells formed in a normal bone marrow that results from a neoplastic disease process
a. decrease in erythroid precursors in the bone marrow
What are some clinical findings of Pyruvate kinase deficiency? a. Jaundice b. gallstones c. kidney stones d. hemoglobinuria e. anemia f. splenomegaly g. asthma *multiple answers
a. jaundice b. gallstones d. hemoglobinuria e. anemia f. splenomegaly
What indicates a G6PD deficiency in fluorescent spot tests? a. lack of fluorescence b. fluorescence
a. lack of fluorescence
What does the hexose monophosphate shunt do? a. maintains adequate levels of reduced glutathione b. provides roc with 2,3-BPD c. produces ATP
a. maintains adequate levels of reduced glutathione
How do mature blood cells appear in Aplastic Anemia? a. normal b. microcytic c. macrocytic d. polychromatic
a. normal
Myelophthisic anemia: what does the WBC count look like? a. normal or decreased b. normal or increased c. increased d. decreased
a. normal or decreased
What are the lab findings of pyruvate kinase deficiency? a. normochromic/normocytic anemia b. hypo chromic/normocytic anemia c. normochromic/microcytic anemia
a. normochromic/normocytic anemia
Which of the following describes schistocytes? a. physical or mechanical trauma to the red blood cell b. red cell membrane has been removed by phagocytes in the spleen or shed from the cell c. increased bone marrow activity d. replacement of the red blood cells at the same rate that they are being destroyed
a. physical or mechanical trauma to the red blood cell
What are some lab tests used to diagnose G6PD deficiency? a. Fluorescent spot test b. dye reduction test c. ascorbate cyanide test d. cytochemical staining e. quantitative of G6PD f. Molecular methods *Multiple answers
all of the above
What causes Aplastic anemia? a. failure of spleen to destroy bad RBCs b. bone marrow failure to send out enough RBCs c. failure of kidneys to filter RBCs d. failure of bone marrow to produce enough RBCs
d. failure of bone marrow to produce enough RBCs
Which of the following describes spherocytes? a. physical or mechanical trauma to the red blood cell b. red cell membrane has been removed by phagocytes in the spleen or shed from the cell c. increased bone marrow activity d. replacement of the red blood cells at the same rate that they are being destroyed
b. red cell membrane has been removed by phagocytes in the spleen or shed from the cell
Regarding intravascular hemolysis, hemoglobin that is released binds to ____________. a. sodium b. potassium c. ferritin d. haptoglobin
d. haptoglobin
What age group is affected by Transient-Erythroblastopenia of Childhood (TEC)? a. 1 month-12 months b. 1 month- 10 years c. 2 years-5 years d. 5 months- 3 years
b. 1 month-10 years
What does the Rapoport-Luebering Shunt provide the red blood cell with? a. NADPH b. 2,3-BPD c. ATP
b. 2,3-BPD
What percentage of the bone marrow is fat in Aplastic anemia? a. 50% b. 70% c. 85% d. 45%
b. 70%
What is pyruvate kinase necessary to generate? a. ADP b. ATP c. NAD
b. ATP
G6PD deficiency patients have what red blood cell inclusion? a. Howell-jolly bodies b. Heinz bodies c. pappenheimer bodies
b. Heinz bodies
When was G6PD recognized? a. world war II b. Korean war c. Vietnam war
b. Korean War from anti-malarial drugs
What is the most common disorder in the Glycolytic pathway? a. G6PD deficiency b. Pyruvate Kinase Deficiency c. IgA deficiency
b. Pyruvate Kinase Deficiency
Which lab test is not specific for G6PD? a. dye reduction b. ascorbate cyanide c. quantitation d. fluorescence spot
b. ascorbate cyanide
Which age group is inherited forms of aplastic anemia common in? a. adolescents b. children c. adults d. elderly
b. children
Which of the following can be found in Myelophthisic anemia? a. hypersegmented neutrophils b. giant platelets c. blasts d. neutropenia
b. giant platelets
What cells are replaced by fat in a stem cell disorder? a. hepatic cells b. hematopoetic cells c. mycocyte cells d. lymphatic cells
b. hematopoetic cells
What should G6PD deficient patients avoid? a. sunlight b. oxidizing agents c. fava beans d. lima beans *multiple answers
b. oxidizing agents c. fava beans
What acquired deficiency can be seen in some leukemias and myelodysplastic disorders? a. G6PD deficiency b. Pyruvate Kinase Deficiency c. IgA deficiency
b. pyruvate kinase deficiency
Which of the following describes compensated hemolytic anemia? a. physical or mechanical trauma to the red blood cell b. replacement of the red blood cells at the same rate that they are being destroyed c. destruction of the red blood cells exceeds the bone marrow ability to replace the red blood cells d. red blood cells are hemolyzed within the blood vessels
b. replacement of the red blood cells at the same rate that they are being destroyed
When does the test for G6PD deficiency need to be performed? a. 24 hours after episode b. 1 year after episode c. 2-3 months after episode d. 5-6 months after episode
c. 2-3 months after episode
How many cases of Fanconi Anemia have there ever been? a. 300 cases b. 3000 cases c. 2000 cases d. 500 cases
c. 2000 cases
Aplastic anemia: what must the granulocyte count be? a. <0.2 x10^9/L b. <1.0 x10^9/L c. <0.5 x10^9/L d. <0.8 x10^9/L
c. <0.5x10^9/L
What is the most common deficiency in the hexose monophosphate shunt? a. 2,3-BPD deficiency b. NADPH deficiency c. G6PD deficiency
c. G6PD deficiency
What is absent in Transient-Erythroblastopenia of Childhood (TEC)? a. serum creatinine b. schistocytes c. Hemoglobin F d. Hemoglobin A
c. Hemoglobin F
What is absent in Diamond-Blackfan Syndrome? a. spontaneous mutation b. Vitamin B12 and folate c. Leukopenia or thrombocytopenia d. Hemoglobin F
c. Leukopenia or thrombocytopenia
What is another term for Aplastic anemia? a. Addison's anemia b. Sickle cell anemia c. Pancytopenia d. Ferropenia
c. Pancytopenia
What is the gold standard test? a. Dye reduction b. Fluorescent spot c. Quantitation of G6PD d. Ascorbate Cyanide
c. Quantitation of G6PD
Which age group is acquired forms of aplastic anemia common in? a. adolescents b. children c. adults d. elderly
c. adults
Acquired aplastic anemia: what can be a symptom in severe cases? a. liver failure b. kidney disease c. cardiac problems d. breathing issues
c. cardiac problems
What is key clinical finding with a G6PD deficient patient? a. blood stool b. chest pain c. dark/black urine d. all of the above
c. dark/black urine
Which of the following describes uncompensated hemolytic anemia? a. physical or mechanical trauma to the red blood cell b. replacement of the red blood cells at the same rate that they are being destroyed c. destruction of the red blood cells exceeds the bone marrow ability to replace the red blood cells d. red blood cells are hemolyzed within the blood vessels
c. destruction of the red blood cells exceeds the bone marrow ability to replace the red blood cells
In the glycolytic pathway 90% of _____ is utilized for the energy of the cell? a. NADPH b. ADP c. Glucose
c. glucose
Glycose is catabolized to lactic acid and produces ATP in what pathway? a. Hexose Monophosphate b. Kreb's Cycle c. Glycolytic pathway
c. glycolytic pathway
Which of the following describes increased reticulocytes? a. physical or mechanical trauma to the red blood cell b. red cell membrane has been removed by phagocytes in the spleen or shed from the cell c. increased bone marrow activity d. replacement of the red blood cells at the same rate that they are being destroyed
c. increased bone marrow activity
Which of the following describes intravascular hemolysis? a. premature RBC destruction are removed from circulation by phagocytes in the tissues b. destruction of RBCs in the tissue or bone marrow c. red blood cells are hemolyzed within the blood vessels d. none of the above
c. red blood cells are hemolyzed within the blood vessels
Which of the following describes Myelophthisic anemia? a. decrease in erythroid precursors in the bone marrow b. temporary suppression of erythropoiesis after a viral infection in normal children c. reduction of cells formed in a normal bone marrow that results from a neoplastic disease process
c. reduction of cells formed in normal bone marrow that results from a neoplastic disease process
What is Fanconi anemia?
chronic failure of bone marrow due to rare genetic anemia from defects in DNA repair
Aplastic anemia: what must the platelet count be? a. <5 x10^9/L b. <30 x10^9/L c. <10 x10^9/L d. <20 x10^9/L
d. <20 x10^9/L
How much radiation causes irreversible damage? a. >1000 rads b. >2000 rads c. >500 rads d. >4000 rads
d. >4000 rads
What do red blood cell enzymes do? a. protect from oxidative damage b. provide cell with energy c. destroy antigens d. A and B only
d. A and B only
What is the most sensitive screening test? a. Dye reduction b. Fluorescent spot c. Quantitation of G6PD d. Ascorbate Cyanide
d. Ascorbate Cyanide
What could develop from Aplastic Anemia? a. PNH b. leukemia c. myelodisplasia d. all of the above
d. all of the above
What must be decreased in order to have Aplastic anemia? a. reticulocytes b. granulocytes c. platelets d. all of the above e. none of the above
d. all of the above
Which of the following can be an underlying disease of Myelophthisic anemia? a. Carcinoma b. lymphoma c. tuberculosis d. all of the above
d. all of the above
Which of the following is associated with Acquired Acute Red Cell Aplasia? a. Viral b. Bacterial c. Chemical and drugs d. all of the above
d. all of the above
Which of the following laboratory findings is associated with hemolytic anemia? a. decreased M:E ratio b. increased reticulocytes (RPI>2) c. decreased haptoglobin d. all of the above
d. all of the above
Which of the following is *not* considered a treatment for Aplastic Anemia? a. remove causative agent b. bone marrow transplant c. immunosuppression d. exchange transfusion
d. exchange transfusion
Who has the most severe deficiencies with Pyruvate Kinase Deficiency? a. adult b. elders c. teens d. infants
d. infants
Which of the following describes osmotic fragility test? a. red cell membrane exhibits abnormalities in cation permeability b. combined deficiency of spectrin and ankyrin c. complement regulatory proteins are missing d. measures the RBC resistance to hemolysis by osmotic stress
d. measures the RBC resistance to hemolysis by osmotic stress
When do symptoms of Scwachman-Bodian-Diamond-Blackfan Syndrome show up? a. after 2 years of life b. within 2 weeks of life c. within 1 month of life d. within 1 year of life
d. within 1 year of life
What is associated with Myelophthisic anemia? a. bone marrow tumors b. splenomegaly c. metastatic disease from solid tumors d. A and B e. A and C
e. A and C
Which of the following is not affected by Pure Red Cell Aplasia? a. WBC precursors b. Erythrocyte precursors c. Platelet precursors d. A and B e. A and C
e. A and C
What type of anemia is shown in hypoproliferative anemia? a. normocytic/normochromic b. microcytic/hypochromic c. macrocytic/normochromic d. microcytic/normochromic e. A and C only f. B and D only g. all of the above
e. A and C only
Which of the following is considered an autoimmune disorder for aplastic anemia? a. suppress stem cell growth and differentiation b. rheumatoid arthritis c. abnormal T-lymphocytes d. all of the above e. A and C only
e. A and C only
What could be characteristic of the bone marrow for other causes of Pancytopenia? a. normocellular b. hypercellular c. infiltrated with abnormal cellular elements d. A and B only e. all of the above
e. all of the above
Which of the following is associated with Leukoerythroblastic? a. NRBC b. abnormal RBC morphology c. immature leukocytes d. A and C only e. all of the above
e. all of the above
Which is more common: intravascular or extravascular hemolysis?
extravascular hemolysis
What is increased in pyruvate kinase deficiency? a. haptoglobin b. Heinz bodies c. unconjugated bilirubin d. LD e. reticulocytes f. C, D, E only g. B, C, D only h. all of the above
f. C, D, E only
What can cause destruction in stem cells? a. drugs. b. viruses c. chemicals d. autoimmune e. A, B, C only f. all of the above
f. all of the above
Which of the following are clinical findings for acquired forms of Aplastic anemia? a. asymptomatic to severe symptoms b. may present with a general sign of anemia c. altered hemostasis d. fever and infection that is prolonged e. A, C, D only f. all of the above
f. all of the above
Which of the following is not an acquired from of aplastic anemia? a. leukemia b. myelodysplastic syndromes c. transplants d. graft vs. host disease e. B and D f. All of the above are forms of aplastic anemia
f. all of the above are forms of aplastic anemia
What all can a defect in stem cells be due to? a. destruction b. genetic mutations c. vitamin B12 or folate deficiencies d. disruption of cells' environment e. decreased production f. infiltration g. all of the above
g. all of the above
What are the clinical findings of hemolytic anemia? a. jaundice b. gallstones c. dark or red urine d. pallor/fatigue e. skeletal abnormalities f. A, B and C only g. all of the above
g. all of the above
Which of the following is considered an acquired form of aplastic anemia? a. autoimmune disorder b. idiopathic c. environmental exposure d. metabolic e. paroxysmal nocturanl hemoglobinuria (PNH) f. cancer, transplants g. A, B, C only h. all of the above i. A, C, and F only
h. all of the above
What is thymoma?
malignant tumor of the thymus
What is Scwachman-Bodian-Diamond-Blackfan Syndrome?
rare congenital disorder characterized by exocrine pancreatic insufficiency, bone marrow dysfunction, skeletal abnormalities and short stature
