Pathophysiology Organ System Essay
HEMATOLOGY ORGAN SYSTEMS
SMALL GROUP ANSWER KEY
Case 1
1. Iron deficiency vs thalassemia vs anemia of chronic disease.
2. Serum iron, TIBC, ferritin.
3. Blood loss, likely GI. A GI evaluation is indicated.
4. Slow response (weeks) to oral iron.
5. Incorrect diagnosis, non-compliance, continued blood loss.
Case 2
1. Anemia of chronic disease vs iron deficiency.
2. Serum iron, TIBC, ferritin.
3. Consistent with ACD, but also iron deficiency with inflammation.
4. Bone marrow iron stain could resolve the possibilities.
5. Trial of oral iron could be considered.
Case 3 1. Macrocytic anemia.
2. B12, folate deficiency, liver disease, reticulocytosis, myelodysplasia.
3. Blood smear, serum B12, red cell folate levels, Schilling …show more content…
test.
4. Parenteral B12.
5. Not in the absence of symptoms.
Case 4 1. MCV = Hct/liter = 103 fl rbc x 1012/liter Hemolytic anemia Reticulocytosis, indirect hyperbilirubinemia 2. Compensated hemolytic anemia. Decreased reticulocyte count -~ worsened anemia 3. Family history of anemia or gallstones. 4. Autosomal dominant. 5. Blood smear, osmotic fragility test. 6. Splenectomy. Increased risk of infection. 7. Blood smear shows Howell-Jolly bodies.
Case 5
Fetal hemoglobin production ameliorates sickle cell disease.
Autosplenectomy due to recurrent splenic infarction.
Encapsulated organisms (salmonella, pneumococcus, etc.).
Vascular occlusion of viscera. Bone, kidney, brain, etc. Hypoxemia, dehydration, acidosis
Hydroxyurea.
6. All children will have Hb AS, which is not associated with significant clinical problems.
Case 6
Fetal hemoglobin production.
Splenectomy.
3. Iron overload due to rbc transfusions and non-compliance with iron chelation therapy.
Less severe disease.
Reduced hemolysis and decreased marrow expansion. 6. p-chains are more soluble, so less precipitation of 6-chains occurs with decreased hemolysis.
Case 7
1. Iron deficiency vs thalassemia.
Thalassemia
Exclude iron deficiency, β-thalassemia 3.
Asians with α-thalassemia may carry two deleted a genes on the same chromosome. Blacks with α-thalassemia may carry one deleted a gene on the same chromosome.
Case 8
1. Platelet count.
2. Bone marrow examination will distinguish abnormal platelet production from increased platelet destruction.
3. Platelet aggregation test to identify a qualitative platelet disorder.
Case 9
Intrinsic pathway deficiency vs inhibitor. 2. Without: factor XII, prekallikrein, or HMWK deficiency. With: factors VIII, IX, or XI deficiency.
VIII, IX, then XI. 5. 50% of girls will be carriers; 50% of boys will be hemophiliacs.
Case 10 1. Hemostatically normal vs abnormal. Likely hemostatic disorders with normal screening lab results include vWD and inherited platelet dysfunction. 2. vW studies, platelet aggregation test.
Case 11
DIC vs liver disease. 2. Yes; a positive test indicates DIC. Factor VIII should be low in DIC and increased in liver disease.
No; vitamin K would have minimal clinical impact in this case.
Case 12
IV heparin monitored with PTT tests.
Subtherapeutic heparin PE; supratherapeutic bleeding.
Coumadin monitored with PT tests.
Yes; factor V Leiden, proteins C, S, and antithrombin III
deficiencies.
Case 13 1. Macrocytic anemia disorders, especially myelodysplasia. 2. Bone marrow with cytogenetics; B12/folate levels. 3. Myelodysplasia (refractory anemia); survival 2-5 years. 4. Supportive care (rbc transfusions).
Case 14 1. Leukemoid reaction (infection) vs CML. 2. CML - very left-shifted with basophils. Cytogenetics (Philadelphia chromosome). 3. Low LAP = CML. 4. Imatinib is the drug of choice. Other options include Hydroxyurea, interferon, bone marrow transplantation. 5. Cytogenetics looking for the Philadelphia chromosome (9:22 translocation), and bcr-abl translocation. 6. Splenomegaly.
Case 15
Bone marrow, abdominal CT scan.
This intermediate-grade lymphoma requires chemotherapy.
This is a curable malignancy.
If localized → XRT; if not, alternative chemotherapy.
Case 16
Correction of coagulopathy and antibiotics. Bleeding, infection.
Promyelocyte granules contain tissue factor → DIC.
Differentiating agent - all-trans-retinoic acid, then chemotherapy.
Good. Better than other subtypes.
SMALL GROUP ANSWER KEY
Case 1
1. Iron deficiency vs thalassemia vs anemia of chronic disease.
2. Serum iron, TIBC, ferritin.
3. Blood loss, likely GI. A GI evaluation is indicated.
4. Slow response (weeks) to oral iron.
5. Incorrect diagnosis, non-compliance, continued blood loss.
Case 2
1. Anemia of chronic disease vs iron deficiency.
2. Serum iron, TIBC, ferritin.
3. Consistent with ACD, but also iron deficiency with inflammation.
4. Bone marrow iron stain could resolve the possibilities.
5. Trial of oral iron could be considered.
Case 3 1. Macrocytic anemia.
2. B12, folate deficiency, liver disease, reticulocytosis, myelodysplasia.
3. Blood smear, serum B12, red cell folate levels, Schilling …show more content…
test.
4. Parenteral B12.
5. Not in the absence of symptoms.
Case 4 1. MCV = Hct/liter = 103 fl rbc x 1012/liter Hemolytic anemia Reticulocytosis, indirect hyperbilirubinemia 2. Compensated hemolytic anemia. Decreased reticulocyte count -~ worsened anemia 3. Family history of anemia or gallstones. 4. Autosomal dominant. 5. Blood smear, osmotic fragility test. 6. Splenectomy. Increased risk of infection. 7. Blood smear shows Howell-Jolly bodies.
Case 5
Fetal hemoglobin production ameliorates sickle cell disease.
Autosplenectomy due to recurrent splenic infarction.
Encapsulated organisms (salmonella, pneumococcus, etc.).
Vascular occlusion of viscera. Bone, kidney, brain, etc. Hypoxemia, dehydration, acidosis
Hydroxyurea.
6. All children will have Hb AS, which is not associated with significant clinical problems.
Case 6
Fetal hemoglobin production.
Splenectomy.
3. Iron overload due to rbc transfusions and non-compliance with iron chelation therapy.
Less severe disease.
Reduced hemolysis and decreased marrow expansion. 6. p-chains are more soluble, so less precipitation of 6-chains occurs with decreased hemolysis.
Case 7
1. Iron deficiency vs thalassemia.
Thalassemia
Exclude iron deficiency, β-thalassemia 3.
Asians with α-thalassemia may carry two deleted a genes on the same chromosome. Blacks with α-thalassemia may carry one deleted a gene on the same chromosome.
Case 8
1. Platelet count.
2. Bone marrow examination will distinguish abnormal platelet production from increased platelet destruction.
3. Platelet aggregation test to identify a qualitative platelet disorder.
Case 9
Intrinsic pathway deficiency vs inhibitor. 2. Without: factor XII, prekallikrein, or HMWK deficiency. With: factors VIII, IX, or XI deficiency.
VIII, IX, then XI. 5. 50% of girls will be carriers; 50% of boys will be hemophiliacs.
Case 10 1. Hemostatically normal vs abnormal. Likely hemostatic disorders with normal screening lab results include vWD and inherited platelet dysfunction. 2. vW studies, platelet aggregation test.
Case 11
DIC vs liver disease. 2. Yes; a positive test indicates DIC. Factor VIII should be low in DIC and increased in liver disease.
No; vitamin K would have minimal clinical impact in this case.
Case 12
IV heparin monitored with PTT tests.
Subtherapeutic heparin PE; supratherapeutic bleeding.
Coumadin monitored with PT tests.
Yes; factor V Leiden, proteins C, S, and antithrombin III
deficiencies.
Case 13 1. Macrocytic anemia disorders, especially myelodysplasia. 2. Bone marrow with cytogenetics; B12/folate levels. 3. Myelodysplasia (refractory anemia); survival 2-5 years. 4. Supportive care (rbc transfusions).
Case 14 1. Leukemoid reaction (infection) vs CML. 2. CML - very left-shifted with basophils. Cytogenetics (Philadelphia chromosome). 3. Low LAP = CML. 4. Imatinib is the drug of choice. Other options include Hydroxyurea, interferon, bone marrow transplantation. 5. Cytogenetics looking for the Philadelphia chromosome (9:22 translocation), and bcr-abl translocation. 6. Splenomegaly.
Case 15
Bone marrow, abdominal CT scan.
This intermediate-grade lymphoma requires chemotherapy.
This is a curable malignancy.
If localized → XRT; if not, alternative chemotherapy.
Case 16
Correction of coagulopathy and antibiotics. Bleeding, infection.
Promyelocyte granules contain tissue factor → DIC.
Differentiating agent - all-trans-retinoic acid, then chemotherapy.
Good. Better than other subtypes.