Haematology Exam Questions - Coursework Example

Haematology Exam Questions Describe briefly how Full Blood Count analyser enumerates the following: i. Total white cell count The normal count of the total white cell count in adults is 4.0-10.0×109/L. A low count on the white cell may indicate that a patient is suffering from a spleen or liver disorder and presence of toxic substance whereas a high total white cell count may indicate tissue damage, inflammatory diseases or an infection. ii. Differential white cell count The ranges of the normal differential white cell count in adults in the different types of white cells are: 0.02-0.5×109/L in eosinophils, 1.0-3.0×109/L in lymphocytes, 0.05-0.1×109/L in basophils, 2.0-7.0×109/L in neutrophils and 0.2-1.0×109/L in monocytes. A low neutrophil count may be because of an infection and the condition reffered to as neutropenia and a low count also indicates a disorder of the bone marrow. High differential white cell count may reslt in leukemia or lymphoma. iii. Total red cell count Anormal total red cell count is 4.5-5.5×1012/L in adult males while in adult females it is 3.8-4.8×1012/L. A low count in these cells may be because of failure in bone marrow, loss of blood or over-dehydration. This indicates the presence of anaemia. Whereas a high number of the total red cell count may indicate presence of a disease such as a lung disease, congenital disease even dehydration. iv. Platelet count Normal platelet count is 150-400×109/L. A low platelet count may be because of a medical condition known asthrombocytopenia caused by usage of some drugs. High platelet counts on the other hand my result out of a medical condition also called thrombocytosis. v. Haemoglobin A normal haemoglobin count for adults is 130-170 g/L. A loe haemoglobin content may indicate that a person has anaemia and this conmditipn can be present with iron deficiency whereas a high haemoglobin content may lead to polycythaemia as this is normally associated with low concentration of haemoglobin. vi. MCV In adults, the normal MVC is 83-101fL. A high MVC may result from abnormalities of the bone marrow, chronic lung disease and nutritional deficiencies and all this may indicate that the patient is anemic. A low count on MVC on the other hand may be a s a result of bone marrow disorder, pregnancy or destruction of the blood cell and this causes anaemia and thelassaemia. (b) Define the term anaemia. Anaemia is a medical condition in which the number of the red blood cells is low. This low number of the red blood cells is as a result on the reduction in the production of the red blood cells and an increase in loss of the red blood cells. Production of red blood cells takes place in the bone marrow and a body needs vitamin B12, iron and folic acid in order to produce more of the red cells. Deficiency on these three components causes anaemia develops. (c) Explain the laboratory results you would expect in a case of iron deficiency anaemia for the following parameters and state reference ranges: i. Haemoglobin The concentration of haemoglobin will be low meaning that there is a poor circulation of oxygen into the body since the iron containing compound (haemoglobin) in the red blood cells have reduced. The normal levels of haemoglobin in adults males is 130-170 g/L and in adult females is 120-150 g/L. ii. Mean cell volume (MVC) MVC is an estimate in the volume of the red blood cells. In case of iron deficiency, the Mean Cell Volume will be a low. Normal mean cell volume in adults is 83-101fL. iii. Red Cell Count The red blood cell count is the estimation on the number of the red blood cell per litre of blood. In case of a deficiency in iron, there will be abnormally a low number of the red blood cell. The normal range in adult males is 4.5-5.5×1012/L and in females is 3.8-4.8×109/L. iv. Mean Cell Haemoglobin (MCH) In case of iron deficiency, the Mean Cell Haemoglobin is either low or high. The normal range of MCH is 27-32 pg in adults. v. Reticulocyte Count Reticulocyte count measures the circulation of the immature erythrocytes. In case if iron deficiency, there is a reduced reticulocyte count. The normal l count of reticulocyte is 0.5-1.5 % of the red blood count population. (d) Explain what features you would expect to see on a blood film from a patient with iron deficiency anaemia. Microcytosis, this is the appearance of the red blood cells such that they appear small. The shape or poikilocytosis of the red blood cells may have 10-30 projections, which are spiny, known as echinocytes. The blood cells are also pale in color (hypochromasia) because of insufficient haemoglobin. Sideroblasts which is an Immature siderocytes forming a ring pattern 2 (a) Describe what a normal neutrophil would look like if stained by the May Grunewald Giemsa method and give 5 causes of neutrophenia. When a normal neutrophil is smeared with a combination of May Grunewald and Giemsa stain, the neutrophil appears in a round or oval shaped cells when observed and the cells have granulated cytoplasm and eccentric nuclei that appears in a two to three lobed structure. In some cases, the cytoplasm is not visible when being observing through a microscope and when visible a dark blue ring observed while the stained nucleus has a dark red appearance Neutropenia is a medical condition characterized by a reduction in the number of neitrophils in the blood. The five causes that lead to neutropenia are as follows: Viral infections, such infections are HIV/AIDS, tuberculosis, and malaria; Vitamin deficiencies; Disease of the bone marrow resulting from myelodysplatic syndrome, myelofibrosis and leukemia; An increase in the destruction of the blood cells by spleen; medication through chemotherapy. (b) Briefly discuss the identifying features of a normal eosinophil and its function. Eosinophil have a large bright orange refractile granules , it’s hard for them to have more than two nuclear lobes like neutrophil which has three to four, the range of the absolute eosinophil is between 0-450 / µL. Eosinophil have several functions such as, they are capable of phagocytosis and are able to undergo an amoeboid motion. They are the site of an invading allergy and parasitic infestation response in that with a person or patient experiencing parasitic infections, they normally have a high eosinophil counts. They mitigate the allergic responses since they can neutralize histamine, stop reacting substances of anaphylaxis and it also inhibits degranulation of mast cell. 3 (a) Define the role of factor VIIIc and von willebrand factor in normal haemostasis and describe the classification of deficiency in each In normal haemostatis, coagulation is a very important key aspect when a patient is experiencing loss of bleed through bleeding because of tissue cavity. Factor VIIIc are the coagulation factors in haemostasis that facilitates the rapid clotting of blood inorder to stop the continuous bleeding. The deficiency of factor VIIIc is because of the development of some antibodies that act against the coagulation process and results in a fatal bleeding. One may inherit this deficiency or acquire it through a past accident or incident that had happened before such as the removal of tooth. Von willebrand factor is a multimeric glycoprotein, which has two important roles in normal hemostasis. The first role is that it is a bridging molecule for the normal platelet aggregation and adhesion at the points experiencing vascular injury. The other role is that is carries the factor VIII in the circulation thereby increasing the half-life of this coagulation factor by a great percentage. When a person has a qualitatively abnormal or deficient in von willebrand factor, he acquires a bleeding disorder known as von willebrand disease (VWD). One may also get this disease through inheritance. (b) Compare the main features of the coagulation disorders Haemophilia A and von willebrand disease. Include pathophysiology; mode of inheritance; and clinical presentation. Haemophilia A is a bleeding disorder that develops out of deficiency in factor VIII. A patient having a deficiency in factor VIII that contributes to the clotting processes experiences bleeding episodes. Reduction on these factors arises out of the development of antibodies primarily IgG that work against the clotting process of a bleeding s site. Haemophilia A occurs exclusively on males who have defective gene on his X chromosome. The male will transmit an abnormal X chromosome to his daughters and making them carriers while the Y-chromosomes transmitted to his sons and the sons are not affected. Clinically a patient with less than 1 percent is referred to be having severe disease, 1-5 percent as factor activity and greater than 5 percent to be having a mild and moderate disease. Generally, all males in a family are at the same level in deficiency since they have the same genes. When there is qualitatively abnormal or a deficient in von willebrand factor (VWF), a person is then likely to undergo a bleeding disorder known as von willebrand disease (VWD). Von willebrand factor plays the role of binding endothelial and platelets components thereby forming a bridge between the platelets and the vascular structure and between the platelets that are adjacent at the site of an endothelial injury moreover it helps in fibrin clot formation while acting as a protein carrier for factor VIII. The von willebrand disease is an inherited bleeding disorder and it affects quite a number of the population. Clinically, a few people only go for medical attention since the disease is of a mild nature and hard for victims to realize even when experiencing minor bleeding that is in excess such as experiencing a heavy menstrual. (c) Discuss the laboratory diagnosis of the above Diagnosis of haemophilia A is through blood tests in order to determine whether the clotting factors are at low levels or missing. In most cases when a patient is having this condition, there are three steps to consider, checking and reviewing the family tree and seeing whether there are others with the same conditions and if the result is positive then the patient is a carrier. Secondly, the clotting factor level in the blood of the patient is measured and if it is below 50 percent of the normal then probably the patient is a carrier having, a mild condition whereas factor levels above 50 percent also means that the patient is still a carrier only that there are elevated levels on the factor. The third test is also necessary and this is DNA testing, where the mutation that caused the disease are looked for in the patient. Von willebrand diseaseis fo three major types, the first type there is low von willebrand factor giving mild signs, the second type the von willebrand factor does no function normally giving more significant symptoms and the third type both the von willebrand factor are low and do not function normally giving severe signs. In diagnosis, there is first examination on the patient on his past medical history together with a physical examination such as checking on briises. Blood tests, specifically on von willebrand factor antigen, ristocetin cofactor activity, and factor VIII clotting activity, von willebrand factor multimers and platelet function test. This is done more than once since the results fluctuate over time. 1(a) Write an account of the Full Blood Count (FBC), including details on the type of the sample required, a description of the principles of analysis of the main parameters and the reference ranges. Explain the significance of abnormal values for the key parameters. Full Blood Count is a commonly preferred test performed on blood cells that can tell the health status of a person by showing high or low abnormality on the number of blood cells or showing the abnormal cells. It is important in diagnosis of some conditions and it gives a number of different features of the blood such as: Level of haemoglobin in the blood, measuring on the level of haemoglobin concentration in the blood helps in diagnosis of anaemia that caused by a deficiency in haemoglobin. Normal haemoglobin level in adults is between 130-170 g/L and in children is 120-150 g/L. Abnormally high concentration of haemoglobin is as a result of chronic lung disease because of an increase in production of red cells by the bone marrow Red cell count, this is the estimation per litre of blood on the red blood cells. The normal red cell count should be between 4.5-5.5×1012/L in adult males and 3.8-4.8×1012/L in adult females. Abnormally high number on the red cells in blood may be as a result of lung disease,kidney disease or dehydration and abnormally low number in the red cells may be as a result of anaemia, malnutrition or failure of the bone marrow. White cell count, this estimates the number of the white blood cells per a litre if blood. Normal white cells is 4.0-10.0×109/L. Abnormally low number of the white cells indicate spleen or liver disorder or viral infections whereas abnormally high levels of white cells count indicates a tissue damage , inflammatory disease or leukemia. Platelet count, it is a measure that estimates per litre of blood the number of platelets. It is for monitoring the medication that may be toxic to the bone marrow. Normal platelet count is between 150-400×109 / L in adults. When there is abnormally high, level of platelet count the condition referred to as thrombocytosis whereas abnormally low numbers gives a condition known as thrombocytopenia 2 (a) Describe the normal breakdown of red blood cells in the body The normal breakdown of the red blood cells in the body is a result of two mechanisms that are different. In the first mechanism, there is an increasing aging of the red blood cell also known as senescence. The other mechanism is because of the random process in destruction of the adhesion of the red blood cells. In the first mechanism, the senescence, the entire red blood cell die through wear and tear as there is a result of prolonged circulation of the red cells within intravascular space. Haemolysis results into the beak down of the cell as it shorten the circulation life span in the blood stream. (c) Using sickle cell anaemia as an example describe: i. The mechanism of anaemia This is a disorder in the blood cells caused by inherited abnormal haemoglobin. This abnormal haemoglobin causes the formation of sickle-shaped red blood cells that are fragile and capable of rapture and they only last in the body for 10-20 days other than the normal 120 days. When the red blood cells reduce in the blood stream because of rapture or haemolysis, anaemia develops. ii. The clinical features of anaemia Jaundice, this is the change in the color to yellowish on the white part of the eye and the skin. Swollen hands and feet, this comes because of blockage in the blood vessels. Shrinkage of the spleen, it occurs after the clogging of sickle cells in the spleen that filters blood cells that are abnormal in the blood. Delay in growth in childhood and adolescent also caused by sickle cells since the working red blood cells are few. Visual problems arise because of the clogging of the blood vessel that transports red blood cells into the retina of the eye by sickle cells. iii. Which laboratory tests would be carried out in the investigation and characterization, stating the expected results and reference ranges for the tests. Haemoglobin Electrophoresis is a test method undertaken by separating and measuring the abnormal and normal haemoglobin. In this test, haemoglobin is separated and and forms pigmented bands that are then put into comparison with the normal sample. The normal Hb S is 50%. Low levels will mean presense of anaemia. Erythrocyte sedimentation rate (ESR), this test is used to measure the time taken for erythrocyte to settle at the bottom of a tube. The sample is to be examined within a two-hour duration and clotting should not take place. A decreased in ESR will indicate sickle cell anaemia lor low plasma protein. The normal range is between 0-20 mm/hr. Serum ferritin test, ferritin is a protein that stores iron found in the reticuloendothelial cells. Normal range of ferritin in women is 20-120 NG/ml and in men is 20-300 NG/ml. A decrease in the level of serum ferritin indicates a chronic iron deficiency. Sickle cell test, this is used to detect the presence of sickle cells in the blood that are deformed and may cause a slow in the blood flow. Normal ranges of haemoglobin S should be 50% and high levels of Hb S about 70-98% the implicatiuon will be sickle cell anaemia. 6 Describe the impact of the 2005 Blood Safety and Quality Regulations on hospital blood banks. The impact of the 2005 Blood Safety and Quality Regulations on hospital blood banks was that the personnel in charge of the management of hospital blood banks are to follow some requirements and it included: To ensure that the personnel in charge of testing, storage and distribution of the blood must be qualified and is to receive regular training and be updated always on the emerging issues. And also that they are directly involved on the storage and distribution. To ensure that the testing and the procedures that the blood banks are to take are all validated. To maintain a system in the blood banks based on better principles of good practice. To make sure that all blood banks document all the procedures, training and the reference materials used for the purpose of inspection. The data of the recipient donor of blood is to be kept for not less than thirty years in order to ensure that there is a full traceability of the blood and its components. To ensure that accurate procedures are undertaken from the point of blood withdrawal to storage for the safety of human. An annual report submitted at the end of a reporting to the secretary of state detailing the measures that the hospital blood bank has in place that comply with the regulation and to provide the information on the system in place. It gave power to the secretary of state to inspect the hospital blood banks to ensure that the hospital blood banks management and the persons responsible comply with the regulation foe the safety of the public. Only the regulated hospitals were to deal with blood withdrawal and distribution to the public for the purpose of safety on donors and recipients. Before any withdrawal of blood and transfusion of blood to the recipient, tests undertaken first and a detailed report of the two documented. Q1 a) Give a brief description of the disease process in leukemia Leukemia is a cancer of the bone marrow. The bone marrow produces the blood cells and a patient suffering from leukemia means that there is production of abnormal blood cells. These abnormal blood cells are the white blood cells. The immature blood cells get damaged and this causes the blood cells to divide and grow rapidly, normally the functioning blood cells die after a while and are then replaced by new ones, the produced abnormal cells do not die and they accumulate occupying more and more space. As the abnormal cells occupy, it leaves no space for the normal cells to occupy and this results into either chronic or acute leukemia or lymphocytic and myelogenous leukemia. b) Describe the clinical presentation and the laboratory findings in a new patient diagnosed with chronic lymphocytic leukemia (CLL). This disease is most common in adults over 55 years of age but at times younger adults also get this disease. The most affected are the males and it is an incurable disease. In chronic lymphocytic leukemia, there is continuous accumulation of incompetent monoclonal lymphocytes. Patients with this disease develop complications that are associated with intrinsic immune dysfunction, which may results in immunodeficiency and autoimmune disorders. Patient has a swollen lymph node, fever, frequent infections, night sweats, and weight loss and become asymptomatic. The blood test done contained many white blood cells, more that 10,000 lymphocytes in cubic millimeter. Under microscopic view, the blood samples taken from the lymph nodes, bone marrow and blood had many immature cells that were replacing the normal cells making it hard to fight infections. c) Which are the main monoclonal antibodies that are included in a diagnostic immunophenotyping panel for chronic lymphocytic leukemia (CLL)? Explain your choices. Aletmtuzumab antibody, this targets the CD52 which is a protein at the surface of a matute lymphocytes. It attacks the B-cells of a chronic lymphocytic leukima. Ibritumomab tiuxetan antibody, this antibody binds itself into the CD20 antigen, found on the surface of normal B-cells. This kills the cells and allows production of a new population of the B-cells. d) Describe the term ‘inherited haemolytic anaemia’and using an example of your choice describe Inherited haemolytic anaemia is a type of anaemia that results from a faulty of one or more of the genes that controls the production of the red blood cell i. mechanism of anaemia Haemolytic anaemia is a blood disorder in which there is destruction of the red blood cells prematurely. The cells are broken down at a faster rate than their production in the bone marrow. The normal life span of a red blood cell is about 120 days and within this time the old red cells get destroyed and replace by new ones. With this anaemia the red blood cells do not love for long as the normal ones. ii. the clinical features of anaemia Abnormal paleness of the skin, yellowing of the skin, mouth and eyes, weakness in a patient, fever, dizziness confusion, increased heart rate and also enlargement of the liver and spleen. iii. What laboratory tests would be carried out stating the expected results and reference (normal) ranges for these test Complete Blood Count to measure haemoglobin count, this should be at the required rate. The normal range for haemoglobin is 130-170 g/L in adult males and 120-150 g/L in Red blood cell count, this is in order to measure the number of the red blood cells per litre of blood. Abnormal low number of the red cells will indicate anaemia due to blood loss. Normal count of the red cell is 4.5-5.5×1012/L in males and 3.8-4.8×1012/L in adult females. Mean Cell Haemoglobin (MCH), this measures the average red cells in the blood. With anaemia the MCH are low. The normal MCH count for adults is 27-32 pg. Q3 a) Define the role of factor VIIIc and von willebrand factor in normal haemostasis and describe the classification of deficiency in each In normal haemostatis, coagulation is a very important key aspect when a patient is experiencing loss of bleed through bleeding because of tissue cavity. Factor VIIIc are the coagulation factors in haemostasis that facilitates the rapid clotting of blood inorder to stop the continuous bleeding. The deficiency of factor VIIIc is because of the development of some antibodies that act against the coagulation process and results in a fatal bleeding. One may inherit this deficiency or acquire it through a past accident or incident that had happened before such as the removal of tooth. Von willebrand factor is a multimeric glycoprotein, which has two important roles in normal hemostasis. The first role is that it is a bridging molecule for the normal platelet aggregation and adhesion at the points experiencing vascular injury. The other role is that is carries the factor VIII in the circulation thereby increasing the half-life of this coagulation factor by a great percentage. When a person has a qualitatively abnormal or deficient in von willebrand factor, he acquires a bleeding disorder known as von willebrand disease (VWD). One may also get this disease through inheritance. (b) Compare the main features of the coagulation disorders Haemophilia A and von willebrand disease. Include pathophysiology; mode of inheritance; and clinical presentation. Haemophilia A is a bleeding disorder that develops out of deficiency in factor VIII. A patient having a deficiency in factor VIII that contributes to the clotting processes experiences bleeding episodes. Reduction on these factors arises out of the development of antibodies primarily IgG that work against the clotting process of a bleeding s site. Haemophilia A occurs exclusively on males who have defective gene on his X chromosome. The male will transmit an abnormal X chromosome to his daughters and making them carriers while the Y-chromosomes transmitted to his sons and the sons are not affected. Clinically a patient with less than 1 percent is referred to be having severe disease, 1-5 percent as factor activity and greater than 5 percent to be having a mild and moderate disease. Generally, all males in a family are at the same level in deficiency since they have the same genes. When there is qualitatively abnormal or a deficient in von willebrand factor (VWF), a person is then likely to undergo a bleeding disorder known as von willebrand disease (VWD). Von willebrand factor plays the role of binding endothelial and platelets components thereby forming a bridge between the platelets and the vascular structure and between the platelets that are adjacent at the site of an endothelial injury moreover it helps in fibrin clot formation while acting as a protein carrier for factor VIII. The von willebrand disease is an inherited bleeding disorder and it affects quite a number of the population. Clinically, a few people only go for medical attention since the disease is of a mild nature and hard for victims to realize even when experiencing minor bleeding that is in excess such as experiencing a heavy menstrual. (c) Discuss the laboratory diagnosis of the above Diagnosis of haemophilia A is through blood tests in order to determine whether the clotting factors are at low levels or missing. In most cases when a patient is having this condition, there are three steps to consider, checking and reviewing the family tree and seeing whether there are others with the same conditions and if the result is positive then the patient is a carrier. Secondly, the clotting factor level in the blood of the patient is measured and if it is below 50 percent of the normal then probably the patient is a carrier having, a mild condition whereas factor levels above 50 percent also means that the patient is still a carrier only that there are elevated levels on the factor. The third test is also necessary and this is DNA testing, where the mutation that caused the disease are looked for in the patient. Von willebrand diseaseis fo three major types, the first type there is low von willebrand factor giving mild signs, the second type the von willebrand factor does no function normally giving more significant symptoms and the third type both the von willebrand factor are low and do not function normally giving severe signs. In diagnosis, there is first examination on the patient on his past medical history together with a physical examination such as checking on briises. Blood tests, specifically on von willebrand factor antigen, ristocetin cofactor activity, and factor VIII clotting activity, von willebrand factor multimers and platelet function test. This is done more than once since the results fluctuate over time. Bibliography LINCH, D. C., & YATES, A. P. (1986). Haematology. Edinburgh, Churchill Livingstone. Read More