Which of the following is not true of Hgb C disease?
B) hemoglobin C can be separated from other hemoglobins at an alkaline pH
(T/F) Thalassemia syndromes are the result of decreased production of globin chains of the hemoglobin molecule, resulting in a microcytic, hypochromic anemia.
True
Which of the following choices describes how beta thalassemia differs from sickle cell disease?
C) Underproduction of the beta chain of the hemoglobin molecule
Hereditary persistence of fetal hemoglobin (HPFH) is characterized by the persistence of fetal hemoglobin into adulthood. Select the best description from the clinical manifestations listed below.
B) No significant abnormalities for heterozygous inheritance; minor symptoms for homozygous
Which of the following electrophoretic results is consistent with a diagnosis of sickle cell trait?
A) Hgb A: 60% Hgb S: 38% Hgb A2: 2%
(T/F) Cold agglutinin syndrome is characterized by acrocyanosis, autoagglutination of blood at RT and may follow infection with Mycoplasma pneumoniae.
True
Which would best distinguish immune hemolytic anemia from hemolytic anemias due to other causes?
C) positive DAT
Which is NOT a characteristic of warm autoimmune hemolytic anemia?
A) Positive Donath Landsteiner test
Which of the following phrases regarding PCH does not apply?
D) involves extravascular hemolysis
Painful crises in patients with sickle cell disease occur as a result of:
D) vasoocclusion
(T/F) The following set of values follows the rule of three:
RBC: 2.82 x 10^6/ul
Hgb: 8.5 g/dl
Hct: 26.1%
MCV: 92.7 fl
MCH: 30.3 pg
MCHC: 32.7 g/dl
True
Which of the following results are most likely in the first phase of iron deficiency anemia?
A) Decreased ferritin, normal TIBC, normal serum iron
Which of these occurs in the second phase of iron deficiency anemia?
D) Transport iron depletion
Which anemia is caused by a defect in iron utilization, resulting in impaired insertion of iron into heme, with iron accumulating in mitochondria?
A) Sideroblastic anemia
Which rare condition produces photosensitivity, neuropathy and central nervous system disturbances?
A) Porphyria
Which of the following best describes pernicious anemia?
D) Autoantibodies to instrinsic factor
Which of the following conditions is an acquired stem cell disorder where RBCs lack GPI anchors on their surface, rendering them susceptible to complement fixation and lysis?
A) Paroxysmal nocturnal hemoglobinuria
A defective sodium and potassium pump where red cells in a peripheral smear appear empty across the center is responsible for which type of anemia?
B) Stomatocytosis
Which antibody is the biphasic antibody responsible for the phenomenon known as paroxysmal cold hemoglobinuria (PCH)?
A) Anti P IgG
Discuss the cause and major symptoms of sickle cell anemia. (2 pts) Include in your answer the typical red cell morphologies you would observe in a peripheral smear. (3 pts)
Sickle cell anemia is caused by receiving a mutated gene for the beta hemoglobin chain from both parents (I think it's a valine subs at pos 6?). This causes an improper folding of the protein, and thus a malformation of the heme molecule in its entirely. Major symptoms include major anemia as well as hepatosplenomegaly and accumulation of the sickled cells in various organs AND vasoocclusion. Transfusions will likely be required to maintain quality of life.
If the disease is present (again, both genes are affected) then many of your cells will become sickled due to this malformed hemoglobin. You would also tend to see several target cells in the smear, and likely hypochromic cells if they are "correctly" shaped.
Describe two patient populations who are most likely to suffer from iron deficiency anemia. (2 pts) What
would you typically see on their peripheral blood smear? Be sure to include RBC morphology in your answer.
(2 pts)
Patients with special dietary restrictions (eg. vegans) and anyone with a GI absorption disorder (including autoimmunity) are likely to suffer from iron deficiency anemia.
On a smear we would expect to see many microcytic cells with a heightened central pallor (hypochromic), but generally normal WBCs and platelets. As the severity of the disease increases (stores are depleted, etc) the RBC population will become significantly more dismorphic and hypochromic, as well as declining in number more substantially.
Briefly explain the mechanism behind lead poisoning, including how it leads to a secondary sideroblastic anemia.
Lead is substituted for iron in hemoglobin, rendering the hemoglobin unable to transport oxygen and an improper formation during erythropoesis. Furthermore, the displaced iron is now free (or in excess) and abnormally deposits itself in the cells and marrow, leading to sideroblasts (and therefor sideroblastic anemia).
Name the two deficiencies which cause megaloblastic anemia. (2 pts) Describe the effect of megaloblastic
anemia on erythrocytic and myelocytic cells. (2 pts)
B12 deficiency and folate deficiency both cause megaloblastic anemia.
Megaloblastic anemia results in a macrocytic anemia and a decrease in production of all cell types, because the deficiency results in a lack of specific nucleotides required for proper cell synthesis.
(T/F) Warm autoimmune hemolytic anemia is often associated with chronic lymphocytic leukemia.
True
(T/F) In drug induced autoimmune hemolytic anemia, the autoantibody produced reacts against red blood cell antigens, not the drug itself.
True
Identify the cells in the bone marrow specimen
Ringed sideroblasts
Given the following results, calculate the MCV, MCH and MCHC for this patient.
RBC: 5.0, Hgb: 15.0, Hct: 45.0
MCV = hct/RBC x10 = 90
MCH = hgb/RBC x10 = 30
MCHC = hgb/MCV x100 = ~33%
An inreased osmotic fragility test would be associated with which of the following conditions?
