Case 1

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In the case of acute blood loss, the loss of blood volume is compensated by movement of fluid from ISF to vascular volume. This reduction in oxygenation leads to increased secretion of erythropoietin from the kidneys. CFU-E is then produced in the marrow. What do these mature into after 5 days? A. Reticulocytes B. Erythroid progenitors C. Normal red blood cells D. Platelets E. Hemoglobin

A - After maturation is completed in 5 days, they will be released as new red blood cells in peripheral blood. This is to compensate for the significant loss of red blood cells due to the acute bleeding.

An elderly woman presents to the ER because she has been experiencing overall weakness, fatigue, has pale skin, and has been experiencing shortness of breath. She has not been in any accidents and seems completely normal otherwise. The physician orders a G6PD test, which came back with lower levels than normal. What does decreased G6PD levels most likely indicate that this patient has? A. Hemolytic anemia B. Asthma C. Jaundice D. COPD E. Hypoglycemia

A - G6PD activity levels correlate with the lifetime of a RBC. As G6PD activity decreases, oxidative damage accumulates, causing the RBC to lyse. When there are more lysed RBC's than the normal rate of production, the RBC count drops in the blood below normal values, leading to hemolytic anemia.

A patient arrives at the emergency room pale, fatigued, and with dyspnea on exertion. A blood smear is taken, and it reveals elliptical-shaped red blood cells. Further genetic testing reveals a mutation in a protein that is the root cause of the malformed shape. What protein mutation is primarily responsible for the change in shape? A. Spectrin B. Hemoglobin C. Band 3 protein D. Ankyrin E. Glycophorin C

A - Hereditary elliptocytosis is an autosomal dominant mutation affecting spectrin molecules. In this mutation, spectrin-to-spectrin lateral bonds are defective, causing the plasma membrane to be unable to rebound from deformation and progressively elongates, resulting in an elliptical erythrocyte.

A 1-year-old female is brought into the clinic after her parents have noticed that the child is easily fatigued and appears to be pale. It was also noted that the child was experiencing shortness of breath as well as excreting dark colored urine despite having adequate consumption of liquids. The parents noted that they are carriers of Thalassemia and are concerned that their child may have the disease. Lab tests indicate that the hemoglobin levels fall below the normal values, low MCHC values and MCV value is elevated. It is suspected that the child has Thalassemia major (Cooley's anemia). Which of the following genetic abnormalities does the child have? A. Homozygous β0- thalassemia mutation B. Heterozygous β0- thalassemia mutation C. α-thalassemia (αα/--) deletion D. Heterozygous β+- thalassemia mutation E. α-thalassemia (αα/α-) deletion F. Compound heterozygous β0- thalassemia and β+- thalassemia mutation

A - Homozygous β0- Thalassemia mutations result in Thalassemia major or Cooley's anemia. This occurs when there are two defective β0- hemoglobin genes resulting in the absent synthesis of β-globin chains from the affected allele. Patients with Thalassemia major inherit two mutated genes, one from each parent carrier making it a homozygous genotype. Babies born with Thalassemia major are usually healthy at birth but develop signs and symptoms within the first two years of life.

An 85-year-old female presents to the ER complaining of cough, chest pain, and weakness. She mentioned familial history of hemolytic anemia, being of autosomal dominant inheritance. Lab evaluation including CBC and reticulocyte count indicated moderate anemia with macrocytosis. Chest X-ray and EKG were within normal limits. Peripheral blood smear indicates increased stomatocytes and no spherocytes or schistocytes. Which of the following treatment/therapy options should be avoided in comparison to the treatments of other congenital red blood cell membrane abnormalities? A. Splenectomy B. Rituximab C. Transfusion D. Folate supplementations E. Cholecystectomy

A - Splenectomy should be avoided due to increased risk of thrombosis. The treatment option for a patient who has hereditary stomatocytosis should only be supportive care. All the other congenital red blood cell membrane abnormalities (HS, HE, HPP) are treated with splenectomy.

A patient presents to her physician and is diagnosed with 𝛃-thalassemia. Her lab results also show elevated levels of fetal hemoglobin (HbF). The physician explains that the elevated levels of HbF indicate she has hereditary persistence of fetal hemoglobin (HPFH), specifically, the nondeletion form of HPFH. Her father who also has 𝛃-thalassemia experiences a much more severe illness compared to his daughter and his lab results have never shown HPFH. Why does the patient with nondeletion HPFH experience a less severe form of 𝛃-thalassemia compared to her father? A. Due to increased production of 𝛾 chains B. Due to decreased production of 𝛾 chains C. Due to increased production of 𝛃 chains D. Due to decreased production of 𝛃 chains E. Due to increased production of 𝜶 chains

A - The patient has nondeletion HPFH that results in increased production of 𝛾 chains which compensates for the defective 𝛃-globin synthesis that occurs with 𝛃-thalassemia. Decreased production of 𝛾 chains would not result in a less severe form of 𝛃-thalassemia.

A 25 year old male arrives at his primary care physician's office for a routine checkup. Over the past few weeks, he has had increasing difficulty breathing exacerbated by exercise. A CBC was taken prior to arrival revealing a hematocrit level of 68%. He also appears to have a blood pressure of 148/78. What is the most likely cause of high blood pressure? A. Polycythemia vera B. Physiological polycythemia C. Macrocytic anemia D. Alpha-1 agonist E. Secondary polycythemia

A - This is primarily caused by Polycythemia Vera (Erythremia) which is a condition causing the increased synthesis of erythrocytes due to a genetic condition in which hemocytoblastic cells continue to generate plasma circulating erythrocyte. This causes increased blood viscosity which decreases venous return, but with an offsetting increase in cardiac output which increases venous return. However, beyond a certain limit, blood pressure regulatory mechanisms fail resulting in hypertension.

A patient presents to their physician complaining of minor fatigue and dizziness. Throughout the patients physical, the doctor finds out the patient is vegetarian. The physician decided to run a serum iron test and noticed decreased iron levels compared to the norm. The physician suspects an issue in the absorption of iron within the patient. What is the most likely supplement the physician would suggest to help increase the absorption of iron? A. Vitamin C B. Vitamin D C. Creatine D. Insulin E. Vitamin A

A - Vitamin C (ascorbic acid) increases the uptake of nonheme iron from the digestive tract. This patient is vegetarian so they are not getting as much readily absorbable heme from meat. This is all due to the fact plants tend to form insoluble precipitates with heme making it more difficult to absorb.

A 31-year-old female presented to the clinic with complaints of fatigue. A blood smear was ordered and showed that her RBCs look smaller and paler than normal RBCs. She was diagnosed with anemia due to iron deficiency. What could explain the altered appearance of these RBCs? A. Decreased heme synthesis B. Decreased DNA synthesis C. Insufficient oxygen circulating the blood D. Increased heme synthesis E. Patient has had a splenectomy

A - lack of iron results in decreased heme synthesis, which also decreases globin synthesis. Normal maturing red blood cells divide until their hemoglobin has reached their appropriate concentration. Iron-deficient red cells continue dividing past their normal stopping point, resulting in microcytic cells. The lack of hemoglobin also explains why RBCs look paler.

A 99-year-old woman presents to the Emergency Department after noting that she appears paler and her skin is being bruised more easily, with associated shortness of breath while spending time with family at home all weekend. The physician ordered a CBC and found the patient has macrocytic anemia due to an elevated MCV of 100 fL and elevated MCH of 45 pg. What is the major protein of RBC membranes that allows it to be flexible? A. Spectrin B. Glycophorin C C. Band 4.1 D. Ankyrin E. Actin

A - spectrin is viewed as the primary protein necessary to form a heterodimer with its subunits which is linked to the membrane lipid bilayer by interacting with either Ankyrin and Band 3, or Band 4.1, actin and glycophorin C. The spectrin cytoskeleton forms a network which reacts to mechanical stress placed on the RBC and causes the spectrin to rearrange.

A 28-year-old pregnant female presents to her OB-GYN clinic for a follow-up visit. She is currently in her first trimester, about 8 weeks along. At this point in gestation, what organ is the major organ involved in hemopoiesis? A. Yolk-sac B. Liver C. Bone marrow D. Spleen E. Red bone marrow

A - the first phase of hemopoiesis begins in yolk-sac in the third week of gestation. It is characterized by formation of "blood islands" within the wall of the yolk sac of the embryo. Since the patient is currently in her first trimester, the yolk sac would be the major organ involved in hemopoiesis.

A complete blood cell count (CBC) with differential is ordered for a patient. Upon receiving the results the ER physician looks at the results for the white blood cell (WBC) count. What types of cells are assessed when looking at the differential for this laboratory test? (One or more answers may be correct). A. Neutrophils B. Thrombocytes C. Eosinophils D. Basophils E. Erythrocytes F. Lymphocytes G. Monocytes H. Plasma cells

A, C, D, F, G - Neutrophils, eosinophils, basophils, lymphocytes and monocytes are all major types of leukocytes, or WBCs, that are reported in a WBC differential.

Which is true regarding the release and production of erythropoietin (EPO)? A. Erythropoietin is released from the kidney to stimulate red blood cell production. B. Erythropoietin is released from the spleen in response to low oxygen. C. The carotid sinus acts as an O2 sensor to trigger release of erythropoietin. D. Erythropoietin is released from the bone marrow where red blood cells develop. E.Erythropoietin is released from the kidney to decrease the production of RBC.

A- Erythropoietin is released by the kidneys in response to low oxygen being sensed in the tissues. Erythropoietin is a hormone that stimulates the maturation and multiplication of erythrocyte progenitor cells.

A 5yr old girl was recently adopted from Congo by an American couple. Unfortunately, the medical history of the child's parents was unknown. While in Congo, both adoptive parents caught malaria while the child did not. When they returned to the states, they found out that their daughter had microcytic anemia and was hypochromic. It has been found that there is a large excess of one subunit over the other. What class of disease does this fall under? A. Thalassemia B. Porphyria C. Pyruvate Kinase Deficiency D. G6P dehydrogenase deficiency

A- The daughter has been diagnosed with thalassemia which is a genetic disease which comes from the imbalance of hemoglobin subunits. This class of disease also provides resistance to malaria in the heterozygous state. This deletion of hemoglobin subunits can lead to a decreased size of RBC and decreased concentration of hemoglobin.

A 7-month-old African American female presents to the ER with swollen hands and feet, as well as extreme irritability and fussiness for 2 days. Child has decreased appetite and has had 2 wet diapers in the last 24 hours. The child was adopted by her parents and there is no known medical history of the child's birth or newborn screenings. Peripheral blood smear shows sickle cell shaped red blood cells. Why is the infant only now presenting with symptoms of this disease? A. Fetal hemoglobin inhibits the polymerization of sickle cell hemoglobin, therefore when levels begin to decline at 6 months of age, symptoms begin to manifest. B. The parents fed the child a fava bean and peas baby food 2 days prior which lead to the onset of symptoms C. Due to the decreased levels of fetal hemoglobin around 6 months of age, IgG antibodies will bind to red blood cells to destroy the membrane creating the sickle cell shape D. There has been a steady decline in vitamin B12 uptake in the patient due to decreased intrinsic factor production causing the symptoms the patient is presenting with E. The child has developed an inability to reabsorb iron which has caused the symptoms the child is presenting with.

A: Fetal hemoglobin is a suppressing agent against the sickle cell hemoglobin. When the levels of fetal hemoglobin begin to drop around 6 months old, there is the inability to prevent red blood cells from forming the characteristic sickle cell shape seen in sickle cell anemia. The symptoms of fussiness and irritability seen in the child are due to the pain crises, and swollen hands and feet are due to hand-foot syndrome, both of which are characteristic of sickle cell anemia along with the sickled red blood cells.

A 22 year old woman presents to her primary care physician with symptoms of weakness, fatigue, and pale skin. She has been feeling these symptoms for the past year, but has been too busy with her college courses to make an appointment. Her physician decides to order a blood test that measures the volume of packed erythrocytes in a sample of her blood. What is the specific name of the test the physician is ordering and how will they perform this test? A. Platelet Count; measured by analyzing a blood sample using automated technology. B. Hematocrit; measured by centrifuging a blood sample to which anticoagulants have been added, and then calculating the percentage of the centrifuge tube volume occupied by the erythrocytes compared to that of the whole blood. C. Creatinine; measured from a 24-hour urine sample and from a serum sample taken during the same time period. D. WBC Differential; diluting blood with a Unopette system that contains a diluent that lyses the red cells to remove them from view. A hemocytometer is charged with the diluted blood, and nuclei are counted in the appropriate areas of the grid using a light microscope. E. Hemoglobin test; measures the amount of protein in the blood that carries oxygen to the body's organs and tissues.

B - A hematocrit blood test measures the percentage of red blood cells within a total blood volume. A hematocrit blood test directly tests for anemia, which the patient likely has due to her weakness, fatigue, and pale skin. The hematocrit test is performed using a centrifuge, which is a machine that separates the blood into red blood cells (on the bottom), white blood cells (in the middle, also called the buffy coat), and plasma (on top). After the test is performed, the dark color in the test tube indicates the red blood cells (~45% of whole blood).

A patient presents to the ER with fatigue, pale skin, slight shortness of breath, and states he is feeling quite dizzy. His CBC shows low erythrocyte counts and a peripheral blood smear displays abnormal cell morphology. The ER physician orders a bone marrow aspiration to examine the patient's bone marrow under higher magnification. Which structure produces reticular fibers and sends sheet-like extensions into the substance of the hemopoietic cords to provide support for developing blood cells within bone marrow? A. Megakaryocytes B. Adventitial cell C. Transcellular diapedesis pore D. Yellow bone marrow E. Sinusoid

B - Adventitial cells, or reticular cells, send sheet-like extensions into the substance of the hemopoietic cords, which provide support for developing blood cells.

A 31-year-old pregnant female presents to the emergency department with complaints of fatigue, weakness, frequent mild diarrhea, and dyspnea. Her medical history reveals prior hospitalizations due to seizures and was prescribed phenytoin, an anticonvulsant. Her diet primarily consisted of carbohydrates-rich foods and fried vegetables for the past year, but is now concerned about her rapid weight loss. The physician orders a CBC and Diff, revealing elevated MCV levels along with low hemoglobin levels. The physician decides to do a fetal ultrasound, and diagnoses the fetus with spina bifida, a neural tube defect. Which biochemical abnormality is this patient most likely suffering from? A. Vitamin B12 deficiency B. Folate deficiency C. Decreased expression of transferrin receptors D. Increased Hepcidin levels E. Decreasing Methotrexate levels

B - Folate deficiency would be the most appropriate answer choice because megaloblastic anemia is present (elevated MCV), and neural tube defects are evident in the fetus. Pregnancy increases the risk of folate-deficiency-related anemia. Also, anticonvulsant medications can inhibit appropriate folate reabsorption.

A 27 year old female presents to her primary care physician for her yearly physical. Upon meeting with the patient, the physician notes that she appears to look healthy. During the exam, the patient notes that she is routinely physically active, and has no complaints to share with her physician. After lab workup, her CBC shows slightly low hematocrit, and low MCV. Following further testing, her physician suspects anemia caused by α - Thalassemia. Which of the following scenarios would exist in the patient? A. Formation of free gamma chain tetramers B. Deletion of 2 α - chains present on the same chromosome C. Presence of 4 α - chains D. Deletion of a singular α - chain E. Hemoglobin H disease

B - For patients with α - Thalassemia, there is a spectrum of how the disease may present. In the question, it was established that the patient has both slightly low hematocrit and low MCV. These points indicate that she is mildly anemic and is microcytic. If a patient is both anemic and microcytic, and is suspected of having α - Thalassemia, they present with a deletion of 2 α - chains, either on the same chromosome or on a different chromosome. This deletion is typically referred to as α - Thalassemia trait, and while patients often present with mild anemia and microcytosis, they do not appear with any abnormal physical signs.

A 53-year-old male presents to the clinic due to feeling fatigue, having shortness of breath, and chest tightness. The patient also has a history of smoking. A CBC report reveals high counts for RBC and hemoglobin. Which of the following blood cell types are most likely targets for the growth inducers and differentiation inducers? A. Multipotent Hematopoietic Stem Cells B. Colony-Forming Unit- Erythrocytes C. Reticulocytes D. Proerythroblasts E. Orthochromatic Erythroblasts

B - Growth factors and differentiation factors target committed stem cells that generate specific blood cell types, Colony-Forming Unit-erythrocytes are committed to generating erythrocytes, and are targets when there is a need for increased RBCs

A 24-year-old male comes into the emergency room after being rear-ended while driving to work. The impact of the collision caused him to hit his head on the steering wheel leaving him with a laceration on his forehead. He said while he was on his way to the hospital the bleeding gradually stopped on its own. The formed element responsible for reducing the blood flow at the site of injury is derived from which cells in the bone marrow? A. Erythrocytes B. Megakaryocytes C. Monocytes D. Basophils E. Eosinophils

B - Megakaryocytes in the bone marrow are responsible for the formation of thrombocytes (platelets). When blood vessels become broken or injured the platelet adhesion is promoted at the site of damage. This adhesion causes platelets to release serotonin, adenosine diphosphate (ADP), and thromboxane A₂. Serotonin works by causing the constriction of smooth muscle around blood vessels leading to the reduction of blood flow. ADP and thromboxane A₂ help promote platelet aggregation. As well, platelets mediate the conversion of fibrinogen into fibrin to create a mesh over the site of injury.

A 48 year old female presents to the clinic with concerns worsening respiratory illness despite recent oral antibiotics. Extensive lab work was obtained including WBC differential which was notable for neutrophilia. Neutrophils are differentiated from common myeloid progenitor stem cells. What maturation stage is unique to Neutrophils in comparison to the other granulocytes during granulopoiesis? A. Promyelocyte B. Band Cell C. Monocyte D. Megakaryocyte E. Pre-NK cell

B - Neutrophil, eosinophil, and basophil progenitors undergo similar steps of maturation during granulopoiesis, however the neutrophil line distinctly has the Band Cell stage. This stage is considered the immature stage before it develops into the mature neutrophil. In this immature phase the nucleus is elongated and has a horseshoe like appearance, whereas the mature neutrophil will have distinct nuclei with 3-5 lobes.

Which of the following platelet cell components includes microtubules, actin filaments, myosin, and actin-binding proteins? A. Peripheral zone B. Structural zone C. Organelle zone D. Membrane zone E. Gamma granules

B - Structural zone- consist of microtubules, actin filaments, and myosin

A 24 year old female presents with fatigue, shortness of breath, and pale skin. A CBC confirms that the patient is anemic. Her physician ordered a bone marrow smear to help identify the cause of her unexplained anemia. The sample shown below is obtained. Which of the following accurately describes the cell identified by the yellow star? A. The indicated cell functions as antigen presenting cells in the immune system. B. The cell functions as a platelet producing cell. C. The cell indicated is packed with hemoglobin. D. The cell contains proteins that are cytotoxic to protozoans and helminthic parasites. E. This cell is involved in synthesizing hormones, inflammatory mediators, and growth factors.

B - The cell indicated by the yellow star is a megakaryocyte. Megakaryocytes are large polyploid and hematopoietic cells that exhibit a large and irregular nuclear profile. Megakaryocytes are responsible for the production of blood platelets.

A 36 year old male presents to his primary care physician with a chief complaint of general malaise and fatigue that began 3 days ago. He also describes feeling short of breath and that he had 2 episodes of diarrhea yesterday. The patient has a wife and two kids and lives in Toronto, Canada. He works as a line cook at Chili's and in his free time he enjoys distance running and playing the bongo drums in his Latin-Jazz band. Physical exam and vital signs are overall unremarkable. Notable lab results include an elevated unconjugated bilirubin and an elevated reticulocyte level. The physician determines that the patient is anemic, what is the most likely mechanism for his anemia? A. Acute blood loss B. Repetitive physical trauma C. Severe dehydration D. Hereditary spherocytosis E. Malaria infection

B - The correct answer is B because the patient is likely suffering from an intravascular hemolysis due to his hobbies of distance running and playing the bongo drums.

A 22-year-old female presents to her physician with dizziness, fatigue, and lethargy. Upon examination, the physician notices the patient to have brittle nails, hair loss and shortness of breath. The patient reveals that she has been eating a plant-based diet for the last 5 years. After ordering a CBC and Diff the physician notices the patient's hemoglobin levels to be 10.1 g/dL. The physician tells the patient to incorporate which supplement into her diet? A. Biotin B. Ascorbic Acid C. Niacin D. Thiamin E. Vitamin E

B - The iron found in animal products is in the form of heme, which is readily absorbed. However, the iron in plants is found in the nonheme form and is not as readily absorbed. This is due to plants often containing oxalates, phytates, tannins, and other phenolic compounds that form insoluble precipitates with iron, thus preventing the absorption of iron. Not enough heme in the diet can result in an iron deficiency which can cause iron deficiency anemia. Vitamin C (ascorbic acid) can aid in increasing the uptake of nonheme iron from the digestive tract. Because the patient's hemoglobin levels are 10.1 g/dL we know that the patient is anemic due to her plant-based diet, and vitamin C (ascorbic acid) can aid in absorbing the nonheme iron she is getting from plants in her diet.

A 37-year-old male with pancytopenia undergoes a bone marrow biopsy. Which of the following biopsy characteristics would most likely lead to a diagnosis of aplastic anemia? A. Absence of RBC progenitors B. Hypocellular bone marrow C. Presence of neoplastic progenitors D. Hypercellular bone marrow E. Presence of few adipocytes

B - The presence of hypocellular bone marrow with pancytopenia is the key distinguishing factor between aplastic anemia and myeloid neoplasms.

A 25-year-old female has been taking rifampin daily to treat her latent tuberculosis infection for the past 2 months. She sees her family physician and complains about experiencing fatigue for the past 2 weeks and appears pale. She also presents with scleral icterus. She has previously been healthy before she was infected with tuberculosis. She does not have any genetic blood disorders. Which of the following would be seen in this patient? A. High IgM B. High IgG C. Low IgG D. High IgD E. Bite cells

B - This patient is experiencing classic autoimmune hemolytic anemia, which is mediated by IgG and the use of certain drugs such as rifampin. Therefore, high levels of IgG would be seen in this patient.

A scientist is experimenting with a new drug to treat individuals with beta-thalassemia. This drug is found to enhance and activate expression of fetal globin genes. What protein is this drug most likely interfering with? A. NADH B. KLF1 C. Spectrin D. NURD repressor complex

B - this regulates the expression of BCL11A. BCL11A blocks the interaction between LCR and y-globin gene promoter on the B-globin gene. So, by blocking KLF1's action you can reduce the expression of BCL11A and allow the expression of y-globin gene.

A 28 year-old pregnant Asian female arrives at the OBGYN clinic to undergo a regular routine checkup. An ultrasound was performed and abnormal findings were indicative of hydrops fetalis. The physician shortly after, requests for a neonatal screening for diseases. The results were suggestive for a type of thalassemia that is usually fatal in utero. The loss of the child in her pregnancy is most likely due to the absence of how many α-globin genes? A. One copy of the gene is deleted B. Loss of four α-globin genes C. No α-globin subunits deleted D. Loss of three α-globin genes E. Two copies are deleted

B - α-Thalassemia usually arises from complete gene deletions. The amount of deletions correlate with different clinical presentations. Specifically, the absence of four α-globin genes is associated with severe anemia and results in hydrops fetalis during pregnancy. α-Thalassemia is more present in Asian and African descent.

Identify the disease associated with the following blood smear slide and what is it caused by it? A. Sickle Cell Disease; single point mutation in the gene of Hemoglobin HbA2 B. Sickle Cell Disease; single point mutation in the gene of Hemoglobin HbA C. Sickle Cell Disease; single point mutation in the gene of Hemoglobin HbF D. Beta Thalassemia; single point mutation in the gene of Hemoglobin HbA2 E. Beta Thalassemia; single point mutation in the gene of Hemoglobin HbA

B- The following blood smear is from a sickle cell anemia patient that shows a "sickle" or "boat" shape cells determining it is Sickle Cell Disease and that is caused by a single point mutation in the gene that encodes for B-globin chain of hemoglobin A (HbA), which accounts for 96% of total hemoglobin.

Exposure of G6PD deficient red cells to high levels of oxidants causes the cross-linking of reactive sulfhydryl groups on globin chains, which become denatured, and form membrane-bound precipitates known as: A. Howell jolly bodies B. Heinz bodies C. β0 mutations D. Hb H bodies E. PIGA mutations

B- This question is testing the result of oxidative damage to erythrocyte hemoglobin. Heinz bodies are structures that are formed from the breakdown of hemoglobin. They present as dark inclusions within red cells and occur due to exposure to oxidative damage. Patients who have abnormalities in glutathione metabolism resulting from deficient or impaired enzyme function reduce the ability of red cells to protect themselves against oxidative injuries and lead to hemolysis and the formation of Heinz bodies

A 25-year-old female patient was involved in a serious car crash and presents to the ER with significant blood loss. Which of the following would the patient be expected to have as a compensatory mechanism for the blood loss? A. Decreased Erythropoietin B. Increased Reticulocyte Count C. Low Platelet Count D. Increased Hematocrit E. Decreased Iron

B: reticulocytes are immature RBCs and are increasingly pushed out of the bone marrow as compensation for the loss of mature RBCs

An experimenter is observing the stages of erythropoiesis for a patient who has defective common myeloid progenitor cells. These cells usually differentiate into erythrocytes under the influence of erythropoietin, IL-3, and IL-4. In normal development, what is the first microscopically recognizable precursor cell in erythropoiesis? A. common lymphoid progenitor cells B. Basophilic erythroblasts C. Proerythroblasts D. Polychromatophilic erythroblasts E. Orthochromatophilic erythroblast

C - A proerythroblast cell is a relatively large cell and it contains a large spherical nucleus. It is the first cell that is formed from Megakaryocyte/erythrocyte progenitor (MEP) cells. This stage lasts about 24 hours and is usually recognizable.

A 15 year male patient presents to a clinic with complaints of numbness and fatigue. It was determined that a group of cells within the stomach were not secreting intrinsic factors. What cells are responsible for the secretion of intrinsic factors? A. Chief cells B. Mucus cells C. Parietal cells D. S cells E. I cells

C - Absorption of vitamin B12 requires intrinsic factor, which is secreted by the parietal cells of the fundic mucosa

A 26 year old patient presents to a primary care physician with complaints of headache, fatigue and nausea. During the visit, the patient learns they are in the early stages of pregnancy. During what stage of fetal hemopoiesis do hemopoietic centers begin to appear on the liver of the developing embryo? A. Thrombopoiesis B. Bone marrow phase C. Hepatic phase D. Leukopoiesis E. Yolk sac phase

C - During the hepatic phase of fetal hemopoiesis, hemopoietic centers appear on the liver.

A 14-year-old male patient presents to the ED with symptoms of mild epigastric discomfort, nausea, flatulence, and diarrhea. Patients' vital signs are T: 37.2°C, P: 72 bpm, R: 18 breaths/minute, BP: 122/79 mm Hg. A stool sample confirms the presence of a parasite, Taenia solium in the patient's intestinal tract. After running a WBC differential, which white blood cell would most likely be elevated in this patient? A. Neutrophils B. Platelets C. Eosinophils D. Basophils E. Megakaryocytes

C - Eosinophilic-specific granules contain proteins that are cytotoxic to protozoans and helminthic parasites. These substances are associated with allergic reactions, parasitic infections, and chronic inflammation

A 56-year-old female patient with progressive kidney failure visits her nephrologist for a follow-up, while her peripheral edema and hyperkalemia has been properly addressed, she still complains of being easily fatigued and out of breath upon mild exertions, along with constant lower back pain, and she also appears pale; which of the following is most likely the direct mechanism behind her persistent signs and symptoms? A. Increased fluid retention due to a lack of plasma proteins B. Adrenal cortical atrophy due to a lack of stimulation from the pituitary C. Decreased secretion of erythropoietin from kidneys D. Decreased fluid reabsorption from renal tubules E. Decreased aldosterone sensitivity of renal tubules

C - Erythropoietin is a glycoprotein produced by kidneys to stimulate the production and maturation of red blood cells, especially in response to increased tissue demand or hypoxia, the patient is displaying signs and symptoms of anemia while suffering from kidney disease.

A 35-year-old male presents to the clinic due to feeling fatigued, dizzy and is exhibiting signs of dyspnea on mild exertion. After the physician asks about the patient's diet in the past week, the patient tells the physician he ate a dish made from fava beans when he visited his middle eastern family a week ago. After conducting a blood smear and running blood tests, what features of RBCs are not going to be present? A. Bite cells B. Membrane-bound inclusions composed of denatured hemoglobin C. Howell-Jolly bodies D. Heinz bodies E. Spherocytes

C - Howell-Jolly bodies are basophilic, small, nuclear remnants within RBCs that are typically seen in sickled cells and is not a feature of RBCs seen in cases of G6PD deficiency.

α-Thalassemia syndromes stem from combinations of deletions that remove one to four α-globin genes. Which of the following syndrome is caused by deletion of all four α-globin genes? A. Silent Carrier State B. Hemoglobin H (HbH) Disease C. Hydrops Fetalis D. α- Thalassemia trait E. -Thalassemia Minor

C - Hydrops fetalis, the most severe form of α-Thalassemia, is caused by deletion of all four α-globin genes.

A 29-year-old patient presents to the emergency room with generalized weakness, fatigue, fever, bleeding, and bruising. Unable to determine the cause of malaise immediately, the emergency room physician orders a Complete Blood Count (CBC). Within the thrombocyte, which of the following zones contain contents that play an important role in the initial phase of vessel repair, blood coagulation, and platelet aggregation? A. Structural Zone B. Membrane Zone C. Organelle Zone D. Peripheral Zone E. Coagulation Zone

C - Organelle Zone. The Organelle Zone consists of mitochondria, peroxisomes, glycogen particles, and at least three types of granules dispersed within the cytoplasm. The content of the granules, specifically the α granule, plays an important role in the initial phase of vessel repair, blood coagulation, and platelet aggregation.

A 24 year old male presents to the clinic with anemia, an enlarged spleen and a history of gall stones. Blood tests revealed that the patient has sphere shaped red blood cells due to a genetic defect which results in a spectrin deficiency of the blood cell membrane. Based on these findings, the patient likely has: A. Congenital methemoglobinemia B. Pyruvate Kinase deficiency C. Heredity Spherocytosis D. B+ Thalassemia E. Iron deficiency

C - Spherocytosis can result from mutations in any number of red blood cell membrane proteins (like spectrin). This leads to instability of the red blood cells (sphere shape) which would cause more of them to be destroyed in the spleen (resulting in the enlarged spleen). The gallstones were a result of the large amounts of bilirubin that were produced and stored in the gall bladder as a result of the hemolysis.

A 79 year old, postmenopausal women presents to primary care with anemia due to inhibition of the heme synthetic pathway enzymes. Assuming it is not congenital, which of the following is commonly associated with her sideroblastic anemia? A. Lead poisoning B. H. pylori C. Alcohol use D. Severe hypothyroidism E. Chronic transfusions

C - The anemia described and stated in the question stem is acquired sideroblastic. The most common cause of this is alcohol use, in which ethanol inhibits most of the enzymes in the heme synthetic pathway.

A 42 year old female presents to the Emergency department at her local hospital with a laceration on her left leg. Despite taking appropriate measures, the resident on call is unable to stop the bleeding. The patient tells the resident she has been diagnosed with leukemia and is currently undergoing chemotherapy as well. What underlying disease or condition is the patient suffering from that's likely preventing the resident from being able to stop the bleeding? A. Defect in the bone marrow reserve pool B. Increase in number of reticulocytes C. Thrombocytopenia D. Tissue plasminogen activator (TPA) was administered E. Bone marrow failure

C - Thrombocytopenia is when a patient suffers from low platelet count. The low platelet count hinders the ability to slow and limit blood loss as there is a lack of coagulation of the blood. This is especially a problem experienced by patients that suffer from immune system disorders or cancers, such as leukemia. Due to this added risk of bleeding, the maximum dosage of chemotherapeutic agents are limited for such patients.

A 32 year old female presents with fatigue and exercise intolerance. She has a history of menorrhagia (abnormally heavy bleeding at menstruation). A CBC reveals a mild microcytic anemia, a low normal WBC count, and a normal platelet count. A corrected reticulocyte count is less than 2%. Which of the following sets of laboratory data best represents the hematologic findings in this patient? Serum Iron TIBC % Saturation RBC Count Ferritin A. Normal Normal Normal High Normal B. Low Low Low Low High C. Low High Low Low Low D. High Low High Normal High E. Normal Normal Normal Low Normal

C - menorrhagia gives away the answer as a cause of iron deficiency in women.

A Northern European patient is diagnosed with an inherited disorder caused by defects in the red cell membrane that render red cells spheroid and vulnerable to splenic sequestration and destruction. Which of the answer choices is most likely to be mutated in this disorder? A. CD31 B. p53 C. Ankyrin D. MYO15A E. Tropomyosin

C: Ankyrin is one of the proteins involved in the structure of the red blood cell membrane and one of the most commonly mutated proteins in Hereditary Spherocytosis.

A young female patient is rushed to the emergency room with complaints of fatigue, severe abdominal pain and sensations of nausea as well as shortness of breath. The patient was successfully treated for megaloblastic anemia using two different types of therapies. One being a folate therapy and the other being a cobalamin therapy. No autoimmune responses were observed in the patient. Which of the listed cells, when damaged, would result in symptoms leading to megaloblastic anemia and the subsequent treatment described? A. Chief cells B. Reticulocytes C. IF antibodies D. Parietal Cells E. ECL cell

D - Cobalamin is another name used to describe vitamin B12. Vitamin B12 can only be absorbed by the gastrointestinal system when utilizing the secretions produced by Parietal cells. One of those secretions is intrinsic factor protein (IF) that helps with binding and absorbing cobalamin. If no autoimmune response is found within the patient, then that would not be indicative of pernicious anemia where treatment is lifelong. In this case, the patient was treated quickly with cobalamin and folate therapy favoring simply a megaloblastic anemia state. Since there is treatment using cobalamin, this indicates damage in the GI system resulting in a cobalamin deficiency which further results in megaloblastic anemia.

A 20-year-old female presents to her physician with dyspnea and fatigue for several months. Her past medical history is insignificant. She denies smoking, alcohol, and drug use and does not take any medications. She says her uncle had a 'blood disorder' but was killed before age 20 in a motor vehicle accident. Physical examination is notable for palpable spleen. Laboratory tests are as follows: Hematocrit: 22% WBC: 8,500 micro-L Plateletcount:180,000micro-L TotalBilirubin:7mg/dL SerumLDH:450U/L SerumHaptoglobin:15mg/dL reticulocyteCount:10% What is the most likely cause of her illness? A. Vitamin B6 deficiency B. Low level of indirect bilirubin C. Point mutation in her DNA that changes the sixth amino acid in the β-globin chain of hemoglobin from glutamate to valine. D. Defects in erythrocyte cytoskeletal proteins E. Iron deficiency

D - Defects in erythrocyte cytoskeletal proteins lead to hemolytic anemia. Shear stresses in the circulation result in the loss of pieces of the red cell membrane. As the membrane is lost, the red blood cell becomes more spherical and loses its deformability. As these cells become more spherical, they are more likely to lyse in response to mechanical stresses in the circulation, or to be trapped and destroyed in the spleen.

An 18-year-old female presents to the emergency room drowsy, short of breath and pale with her mom. Her mom tells the emergency physician that she collapsed a couple of hours after tooth extraction. For the tooth extraction, Benzocaine was used as the main topical anesthetic (Benzocaine is an oxidizing agent and affects cytochrome b5 reductase). Prior to procedure, there were no underlying conditions. Upon initial physical examination, the physician states that the patient has bilateral lung sounds; but seems to be getting even paler and losing consciousness. Her SpO2 is now 70% (cyanotic). As a response to her low oxygen saturation and with no gag reflex, she gets intubated and ventilated. However, the patient is not responding well and continues to desaturate in her oxygen levels. Family history was taken and there were no detected genetic disorders. Blood smears and lab tests were also done on this patient. What is the patient suspected to have based on case scenario? A. G6PD deficiency B. Pernicious anemia C. Porphyria Disorders D. Methemoglobinemia E. Pyridoxine deficiency

D - In the case description, it mentions that the topical agent is an oxidizing agent that specifically affects cytochrome b5 reductase which is an enzyme that is responsible in reducing ferric iron (Fe3+) to ferrous iron (Fe2+). However, this process is blocked; therefore, there is an accumulation of ferric iron which leads to the production of methemoglobin. Methemoglobin does not have the capabilities in carrying oxygen, leading to the patient being cyanotic.

A 24 year old patient returns from traveling outside of the country after spending time in Eastern Asia. After returning home she goes to her primary care physician and finds out she has contracted a parasitic infection presenting with an increased amount of eosinophils. What specific granule is most likely present in the blood? A. Lysosomal acid hydrolase B. Histamine C. Myeloperoxidase D. Major Basic protein E. Metalloproteinase

D - Major Basic protein. Specific granules of eosinophils contain the crystal body which include 4 types of proteins. These include major basic protein, eosinophil cationic protein, eosinophil peroxidase, and eosinophil-derived neurotoxin. Therefore, major basic protein is correct.

A 35-year-old male presents to the ED with unexplained abdominal pain, fatigue, and shortness of breath. He reports a two-week history of dark, red-colored urine, especially upon voiding in the morning. Flow cytometry is performed, and the patient is diagnosed with a rare, intravascular hemolytic anemia called paroxysmal nocturnal hemoglobinuria (PNH). A mutation causing dysfunction of which of the following proteins is most likely associated with the pathophysiology of PNH? A. Glucose-6-phosphate dehydrogenase (G6PD) B. Spectrin C. Hemosiderin D. CD59 E. β-globin

D - Many proteins are tethered to the cell membrane through a phospholipid called GPI. There are three GPI-associated proteins that are deficient in PNH, all of which are involved in the complement pathway regulation. CD59 is an enzyme inhibitor that regulates complement pathway activity in PNH. The other two GPI-associated proteins are CD55 and C8-binding protein, neither of which are listed.

A 19-year-old female with SS sickle cell disease arrives at the emergency room with a pain crisis concern. The patient claims that this incident is a perfect representation of her regular pain episodes, during which she feels dehydrated, and she feels discomfort in her chest, her abdomen and back, as well as each of her upper lateral thighs. She's been using oral pain medicine since the pain started yesterday, but it hasn't helped. Which of the following acute manifestations does this resemble? A. Chronic renal failure B. Aplastic crisis C. Avascular necrosis D. Vaso-occlusive crisis E. Hemoglobinopathies

D - Most of the acute complications of sickle cell disease are related to the vaso-occlusive crisis. Painful crisis is secondary to vaso-occlusive as it can occur anywhere, with the pain most commonly experienced in the extremities, chest, abdomen, and back. Also, painful crises are frequently precipitated by infections, dehydration, and rapid changes in temperature.

A 24-year-old medical student working in a lab researching sickle cell anemia is interested in how the structure of hemoglobin A is affected by the disease. Which statement is the most likely true regarding the formation of hemoglobin A of a normal individual? A. 1 succinyl-CoA + 2 glycine form a pyrrole molecule B. 2 succinyl-CoA + 1 glycine from a pyrrole molecule C. Protoporphyrin IX binds to heme to form the α or β hemoglobin chain. D. 4 pyrrole are needed to form protoporphyrin IX. E. Fe++ binds to heme to form the hemoglobin chain.

D - Pyrroles are the subunits of protoporphyrin IX, and 4 pyrroles bind together to form protoporphyrin IX.

A 30-year-old, otherwise healthy female goes to her physician's office after finding out she is pregnant with her second child. After birth, it was discovered that the first child was Rh(D+). Knowing that she is Rh(D-), what should be done if this fetus is Rh(D+)? A. No complications; continue with regular prenatal care. B. The woman should increase intake of folic acid to reduce the chances of erythroblastosis fetalis. C. Refusal of RhoGAM because there will be no harmful interactions between mother and fetus' blood. D. Administration of anti-D immunoglobulin (RhoGAM) to destroy any circulating Rh(D+) fetal erythrocytes that persist in the mother's blood. E. A blood transfusion should be given to the mother to increase circulation and blood volume.

D - Rh incompatibility may happen if an Rh(D-) mother and Rh(D+) baby's blood interact, potentially causing miscarriage and/or erythroblastosis fetalis. Mother will produce anti-D antibodies in response to the D antigen expressed on the fetal erythrocytes (Rh(D+) sensitization).

A 6-month-old is screened for sickle cell. The family is from Sub-Saharan Africa. Why might the child be positive for the sickle cell trait? A. The child is infected with malaria B. To prevent the missense point mutation from Val to Glu C. To prevent vaso-occlusive crises from occurring D. To be protected against plasmodium falciparum malaria E. To allow the oxygen dissociation curve to shift to the left for more oxygen delivery

D - Sickle cell trait is very common in certain African American populations to protect them from plasmodium falciparum malaria.

A 29 year old female presents to the emergency room with significant blood loss following a motor vehicle accident. She requires a blood transfusion, but her blood type is unknown. Which correctly describes the surface antigens found on the type of blood she should receive? A. Erythrocyte surface contains A antigen, B antigen, and Rh factor. B. Erythrocyte surface antigen consists of an additional sugar molecule N-acetylgalactosamine. No Rh factor present on the cell surface. C. Erythrocyte surface antigen consists of an additional galactose. No Rh factor present on the cell surface. D. Erythrocyte surface antigen consists of only the immunodominant core structure: N-acetylglucosamine, galactose, and glucose. No Rh factor present on the cell surface. E. Erythrocyte surface antigen consists of only the immunodominant core structure: N-acetylglucosamine, galactose, and glucose. There is also an Rh factor present on the cell surface.

D - This describes O- blood, which is known as the "universal donor." O- erythrocytes have no surface antigens (no A and B antigens and no Rh factor). It just contains the immunodominant core structure of O antigen. It is the only blood type that should be used for a patient with unknown blood type as it will not cause a transfusion reaction.

A 35-year-old male comes to see the physician with complaints of fatigue and decreased appetite. When taking a medical history, the physician sees that the patient has intestinal spurs. The physician concludes that this patient is suffering from megaloblastic anemia. What vitamin is the patient most likely deficient in that causes this type of anemia? A. Vitamin K B. Vitamin C C. Vitamin E D. Vitamin B E. Vitamin A

D - Vitamin B is the correct answer. The patient has intestinal sprue, which means that the patient cannot absorb vitamin B compounds efficiently. This can lead to a slower production of RBCs from the bone marrow.

Which of the following choices makes up the majority of blood plasma? A. Albumin B. Fibrinogen C. Free O2 D. Water E. Glucose

D - Water makes up 91-92% of blood plasma.

A 70-year-old presents with megaloblastic anemia, increased serum homocysteine and methylmalonic acid, and antibodies to intrinsic factor. What is this person most likely deficient in? A. Folate B. Vitamin D C. Vitamin C D. Vitamin B12 E. Iron

D - in megaloblastic anemia, elevated serum methylmalonic acid and the presence of antibodies to intrinsic factor are specific for B12 deficiencies.

A 68 year old female was sent to the emergency department after labs done at her primary care physician's office indicated a low thrombocyte count. A problem with the development of which of the following progenitor cells are likely to have contributed to her lab results? A. Granulocyte/monocyte progenitor B. Erythrocyte progenitor C. Common lymphoid progenitor D. Megakaryocyte progenitor E.Monocyte progenitor

D- Megakaryocyte progenitor. Thrombocytes are derived from megakaryocytes, which are derived from MEP. The other options derive into different cells

A 47-year old female with acute myeloid leukemia presents to her oncologist for a bone marrow biopsy one week after starting chemotherapy. What would be the characteristics of the bone marrow examination? A. The bone cellularity would be between 60% and 80%. B. The bone cellularity should be normocellular. C. The bone cellularity will be indicative of the ratio between megakaryocyte and sinusoid. D. The bone cellularity yields a hypocellular bone marrow. E. The bone cellularity is undetermined pending a smear preparation.

D- The bone cellularity yields a hypocellular bone marrow. Hypocellular bone marrow occurs after chemotherapy.

A newborn is admitted to the NICU for recurring infections. The doctor discovers the newborn was born without a thymus. Which of the following immune cells is most likely affected by this? A. pre-T lymphocytes B. B lymphocytes C. Natural Killer cells D. Mature T lymphocytes E. Neutrophils

D- The correct answer is mature T lymphocytes as they are the only immune cell that must travel to the thymus to become a mature lymphocyte.

A 60-year-old Caucasian male from Scandinavia is being seen by his primary care physician for a routine appointment. He often experiences lightheadedness when standing up or with exertion. He is also complaining of some loss of appetite, pale skin, and dyspnea (shortness of breath) upon exertion. He is diagnosed with having autoimmune gastritis that impairs the production of intrinsic factor, which is required for vitamin B12 uptake from the gut. What specific form of anemia is this condition associated with? A. Hemolytic Anemia B. Aplastic Anemia C. Sickle cell Anemia D. Pernicious Anemia E. Thalassemia

D. Although somewhat more prevalent in Scandinavia and other Caucasian populations, pernicious anemia occurs in all racial groups. It is a disease of older adults; the median age at diagnosis is 60 years, and it is rare in people younger than 30 years of age. Pernicious anemia is believed to result from an autoimmune attack on the gastric mucosa. Histologically, there is chronic atrophic gastritis marked by loss of parietal cells. Destruction of parietal cells means impaired production of intrinsic factors. Since Vitamin B12 is an essential coenzyme for RBC and DNA synthesis, failure of absorption of Vitamin B12 in the gut due to lack of intrinsic factor results in Pernicious Anemia.

A patient has been diagnosed with a clinical syndrome that is caused by a deletion of 3 alpha globin genes, leaving one normal alpha-globin gene left. What is the clinical syndrome the patient is diagnosed with and what does this syndrome lead to? A. Alpha-thalassemia trait. Microcytosis, minimal or no anemia and no abnormal physical signs. B. Silent carrier state. Asymptomatic but can have slight microcytosis C. Beta-thalassemia. Hemolytic anemia D. HbH disease. Tissue hypoxia and severe anemia E. Hydrops fetalis. Severe tissue anoxia that leads to death in utero or shortly after birth.

D. HbH disease. Tissue hypoxia and severe anemia. HbH disease is a hemoglobin H disease caused by the deletion of 3 alpha globin genes. In HBH, you only have one normal alpha gene so the synthesis of the alpha chain is reduced and 3 beta globin tetramers also known as HbH form. HbH has a very high affinity for oxygen but it can't deliver oxygen so HbH causes tissue hypoxia. HbH oxidizes which causes precipitation leading to the formation of intracellular inclusions. The inclusions promote phagocytosis in the spleen.

A 31 year old male patient presents to the emergency department with complaints of fatigue, shortness of breath, and pallor that began three days ago. The patient notes that he recently was diagnosed with a Parvovirus B19 infection shortly before symptom onset. Test results from a complete blood count indicate a low hematocrit level and a slightly elevated WBC count which is attributed to the recent Parvovirus B19 infection. Which of the following disorders is most likely associated with the patient's symptoms? A. Megaloblastic anemia B. Aplastic anemia C. Iron deficiency anemia D. Pure red cell aplasia E. Anemia of chronic inflammation

D. Pure red cell aplasia is associated with different mechanisms of onset depending on onset of the anemia. In acute cases, such as our patient case, pure red cell aplasia is associated with Parvovirus B19 infections. In chronic cases, not relevant to our patient, it is a result of many factors such as thymomas, large granular lymphocytic leukemia, etc...

A 65 year old male goes to see their physician for a checkup, and upon examination the physician realizes that the patient appears very pale. The physician sends the patient for a blood test. The results revealed that the patient has low serum vitamin B12 levels and increased homocysteine and methylmalonic acid serum levels. From this information, the physician diagnoses the patient with megaloblastic anemia. Which of the following is most likely the reason for his condition. A. Increased secretion of pepsin in the stomach B. Decreased intrinsic factor release from intestinal chief cells C. Increased release of vitamin B12 from haptocorrin-vitamin B12 complexes in the duodenum D. Decreased intrinsic factor release from gastric parietal cells E. Increased vitamin B12 increase due to parasitic infections

D: A decrease in intrinsic factor will prevent vitamin B12 from being absorbed in the ileum. A decrease in vitamin B12 absorption reduces the conversion of homocysteine to methionine because vitamin B12 is a cofactor for the reaction. This leads to the increased serum levels of homocysteine and methylmalonic acid, which in turn cause megaloblastic anemia.

A 28 year old female in her second trimester presents to the emergency department complaining of shortness of breath and palpitation. An ultrasound is carried out showing the presence of multiple abnormal fluid collections in the fetus. The physician decides to do a fetal blood sampling which reveals a deletion of all four α-globin genes. Which of the following genetic conditions is most likely to have resulted from this finding? A. Hemoglobin H Disease B. Silent Carrier State C. α-Thalassemia Trait D. Hydrops Fetalis E. Paroxysmal Nocturnal Hemoglobinuria

D: Hydrops fetalis is caused by the deletion of all four α-globin genes and is the most severe form of α-thalassemia.

A 12-year-old patient comes to the ER with her parents complaining of fatigue and increased abdominal pain. Upon doing some bloodwork, her reticulocyte count is elevated as well as high iron levels, decreased hepcidin, and low hemoglobin. The parents then reveal that they adopted her from Cyprus and as a baby she was diagnosed with Beta-Thalassemia. What is the most likely cause of her increased abdominal pain? A. The patient's kidneys have a deposition of insoluble alpha-globulin chains causing blockages and increased pain B. Due to her decreased hemoglobin, her kidneys have begun to atrophy and have shunted blood to more vital areas like her heart and brain C. The increased reticulocyte count means her white cells have not fully matured yet and their morphology is not suitable for movement in the vasculature D. The increased iron deposition has caused blockages in sinusoid organs like her liver causing abdominal pain E. Her abdominal organs are being hypoperfused as evidenced by her low hemoglobin levels necessitating a blood transfusion

D: This answer is correct because the accumulation of iron will deposit into organs like the liver heart and pancreas causing a more insoluble mass

A 57-year-old female presents to her primary care physician with chief complaints of fatigue and shortness of breath. The physician noted she appears very pale during the physical exam. Lab tests show she has significantly low RBCs, hematocrit, and hemoglobin levels. The physician diagnoses her with anemia. Which of the following bone marrow locations does a majority of the RBC production occur in this patient? A. Tibia B. Femur C. Liver D. Humerus E. Sternum

E - After the age of 20-25, RBCs are formed in the membranous bones, such as the vertebrae, ribs, ilia, and sternum. This is because the bone marrow in these bones are red bone marrow, which produce RBCs. The production of RBCs from these locations will decrease as age increases.

A patient goes to see their primary care physician with complaints of weakness, fatigue, and weight loss. A blood smear is performed, and the red blood cells are found to be larger than normal with irregular and oval shapes. Pernicious anemia is suspected. What deficiency would the patient have that would be the most direct cause of this type of anemia? A. Interleukin-3 B. Folic Acid C. Vitamin B6 D. HIF-1 E. Vitamin B12

E - Blood smear shows maturation failure of red blood cells, which could indicate B12 or folic acid deficiency, but pernicious anemia is mentioned, making the most correct choice vitamin B12.

James, age 7, is brought in to see the doctor by his parents after experiencing severe sunburn after visiting the beach for the first time. James has lived a very sheltered life following a previous diagnosis of porphyria, which has caused neurological symptoms. In an attempt to have a fun family outing, his parents took him to the beach without realizing his condition also renders him extremely photosensitive. Through what mechanism does porphyria cause photosensitivity? A. Decreased heme synthesis leads to toxic levels of free iron, which damages the skin when exposed to light. B. Decreased heme synthesis causes decreased hemoglobin, leading to pernicious anemia. Without adequate red blood cells, the skin becomes more sensitive to sunlight. C. Accumulation of heme synthesis intermediates interferes with the synthesis of melanin, which pigments the skin to provide protection from sunlight. D. Decreased heme synthesis decreases levels of glutathione reductase, inhibiting the body's ability to remove reactive oxygen species (ROSs) that are produced when exposed to sunlight. E. Accumulated heme synthesis intermediates react with light to form porphyrins, which react with molecular oxygen to form oxygen radicals that damage the skin.

E - Porphyrinogens makeup the intermediates of the heme synthesis pathway and convert to porphyrins when exposed to light. Porphyrins are highly reactive, and after reacting with molecular oxygen produce oxygen radicals, a reactive oxygen species that can oxidize many important cellular compounds leading to tissue damage.

A 24 year old female visits her doctor's office with initial symptoms of malaise and fatigue. Given the pale appearance of the patient, the physician suspects anemia to be the primary cause of the patient's symptoms. Which single lab value would be most beneficial in determining if the anemia is due to failure to produce blood cells or blood loss (hemolysis or hemorrhage)? A. Hematocrit B. Hemoglobin C. Hemoglobin A1C D. Reticulocyte Count E. RPI

E - RPI or reticulocyte production index is the most correct answer because this lab value multiplies the patients reticulocyte count by the ratio of the patient's hematocrit to a normal hematocrit value. This essentially normalizes the patients reticulocyte count to their degree of anemia. A high RPI would indicate an appropriate response to RBC loss where a low RPI would indicate that there is a problem in the production of new red blood cells.

A patient is suffering from cancer and has received several sessions of radiation therapy. He now presents with anemia and has been suffering from chronic recurrent infections. Upon physical examination, he has no notable splenomegaly. Bone marrow biopsy was obtained which showed hypocellular marrow containing mainly fat cells. What is the most likely diagnosis? A. Polycythemia B. Folate deficiency C. Pernicious anemia D. Sickle cell anemia E. Aplastic anemia

E - This question is asking about the clinical features of aplastic anemia. People who receive radiation are at risk for marrow aplasia which can result in aplastic anemia. Aplastic anemia is characterized by anemia, thrombocytopenia, and neutropenia. Neutropenia can cause those suffering from aplastic anemia to deal with chronic recurrent infections. Hypocellular bone marrow containing mainly fat cells is characteristic of aplastic anemia.

A patient presents to their physician stating worms are viewable in their stool, it is determined the patient has a parasitic helminth infection. Bloodwork was drawn, indicating a high level of eosinophils in the CBC. What is the function of an eosinophil? A. Red cell loss B. Degrade microbial structures & remodel damaged tissues C. Impaired DNA synthesis D. Phagocytose cells that enter inflammatory site & consume necrotic host cell debris E. Release granules with hydrolytic enzymes & cationic proteins

E - function of an eosinophil is to release granules with hydrolytic enzymes & cationic proteins which are toxic to parasitic worms. A high level would be seen, as the body is trying to fight off the parasite.

In the ICU, a new born baby is showing worsening symptoms of jaundice. Through testing, the patient's blood smear shows red blood cells as small, dark-staining red cells lacking the central zone of pallor. What form of anemia is the patient likely diagnosed with? A. Iron deficiency anemia B. Glucose-6-Phosphate Dehydrogenase Deficiency Anemia C. Blood loss anemia D. Sickle Cell Disease E. Hereditary Spherocytosis

E- Hereditary Spherocytosis can be seen as red blood cells as small, dark-staining red cells lacking the central zone of pallor due to the lipid bilayer shedding membrane fragments as red cells age in the circulation.

A blood smear is taken from a patient with suspected severe anemia. It is revealed that the cells within the smear are hypochromic and contain several alpha-globin aggregates. The physician orders a blood transfusion for the patient. What complication might arise within the patient when given the blood transfusion? A. Increased absorption of dietary iron in the liver B. Decreased absorption of dietary iron in the liver C. Decreased absorption of dietary iron in the stomach D. Primary hemochromatosis E. Secondary hemochromatosis

E- This question is testing on the pathogenesis of B-thalassemia. In severe cases of B-thalassemia, anemia occurs from both the lysis of red blood cells that contain insoluble alpha-globin aggregates (in a normal cell, two alpha and two beta-globin proteins are configured together) and ineffective erythropoiesis (maturation of red blood cells) due to erythroblasts dying within the bone marrow. The body will try and self correct its anemia by producing more erythrocyte precursors. However, these erythrocyte precursors produce hormones that favor iron absorption from the gut. In cases of severe anemia, a blood transfusion may be a favorable treatment to consider. Repeated blood transfusions will also contribute to iron accumulation within the tissues. Both of these forces lead to severe iron accumulation in the tissues, a term we call secondary hemochromatosis.

A 23yo female who is 19 weeks pregnant, presents to the emergency room with complaints of fatigue and early contractions. Patient states that she's been having contractions spaced out every six hours since yesterday. However, the frequency has shortened since early this morning, and she is now having contractions every 45 minutes. Patient has a pale appearance and has a bilateral conjunctival icterus. Patient reports working at an auto body shop. The attending physician has ordered a CBC exam which reveals that patient is anemic. She ended up miscarrying that night. Based on the information in the passage, which form of anemia is the patient most likely suffering from? A. Macrocytic, caused by folate deficiency B. Normochromic due to a mutation leading to thalassemia C. Macrocytic due to lead poisoning D. Microcytic due to red cell metabolic defects E. Microcytic due to lead poisoning

E-Patient stated that she works at an auto body shop where she most likely has been exposed to lead. Of the answer choices, only E represents the form of morphologic anemia caused by lead poisoning.

A 25-year-old female presents to the ED with complaints of shortness of breath, fatigue, dizziness, and hot flashes. The patient is 33 weeks pregnant; her blood pressure is elevated at 136/91, and her temperature is 97.8. The patient states she hasn't been able to eat properly the past week and says she has been "snacking" on pieces of chalk and bits of clay that her 2-year-old daughter brought back from the daycare. The attending physician suspects a deficiency and orders a peripheral blood smear among other tests. She also suspects the patient may undergo preterm labor and wishes to keep her under observation for the next 24 hours. According to the patient's symptoms, match the correct deficiency with the results from the peripheral blood smear. A. Aplastic anemia, hyper segmented granulocytes. B. Fanconi anemia, sickled red blood cells. C. Glucose-6-Phosphate Dehydrogenase Deficiency, spherocytes. D. Thalassemia, macro-ovalocytes. E. Pernicious anemia; bite cells. F. Iron deficiency anemia; Hypochromic microcytic red cells.

Iron deficiency anemia; characterized by hypochromic microcytic red cells containing a narrow rim of peripheral hemoglobin. Iron deficiency anemia is most prevalent in young females of child-bearing ages, amongst others. The 25-year-old female patient who is 33 weeks pregnant with her second child falls aptly under this category. Moreover, two major symptoms that signify an iron deficiency are severe fatigue and pica, a condition in which affected individuals have a craving for non-foodstuffs such as clay, or food ingredients such as flour. This is resultant of depletion of iron from the central nervous system - seen especially in individuals with an increased physiological demand for iron intake, such as pregnancy.

A 34 year old male patient comes to the emergency room with abdominal pain and diarrhea. A stool sample is taken and he is diagnosed with a helminth parasitic infection. If a complete blood cell count (CBC) with differential is ordered for the patient, what count would most likely be high? A. basophils B. eosinophils C. neutrophils D. erythrocytes E. lymphocytes

b - Eosinophils participate in immunologic responses and phagocytose antigen-antibody complexes. The eosinophil count in blood samples is usually high in individuals with allergies and parasitic infections. They specifically play a major role in host defense against helminthic parasites.

go to Histology, Group 9 question 5, link on back of card

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