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Pathophysiology
Hematology Q
| Question | Answer |
|---|---|
| 1. What is Hematopoiesis? | The process of the formed elements that are produced of blood. |
| 2. How does the process of Hematopoiesis occur? | The process occurs through a series of intricate steps that initiate with stem cell. |
| 3. What does the stem cell produce? | The stem may produced red cells, all classes of granulocytes, monocytes, platelets and cells of the immune system |
| 4. Where does hematopoiesis occur? | In the yolk sack, then liver, and eventually the bone marrow. In normal adults it occurs in marrow and lymphatic tissues. |
| 5. What are the characteristics of RBC? | In humans, RBC takes the form of flexible biconcave disks, lack a cell nucleus, subcellular organelles and the ability to synthesize protein. |
| 6. Where is RBC developed? | Bone Marrow |
| 7. How does a RBC live? | About 120 days |
| 8. Does RBC have a red cell precursor? | Yes |
| 9. What is the initial recognizable red cell precursor for RBC? | pronormoblast |
| 10. What will the pronormoblast cell undergo? | This cell will undergo 4/5 division before, in result of production of 16-32 RBC |
| 11. Along with the division, what else occurs with the pronormoblast? | Concomitant with the stimulation of erythropoietin the early progenitor cell production can be amplified. |
| 12. What is Erythropoietin? | A dalton glycoprotein hormone that is produced and released by the peritubular capillary linining of the kidney |
| 13. Where is EPO produced? | liver and kidney |
| 14. What does EPO regulate? | It governs the day to day production of RBC |
| 15. What does EPO measure? | The ambient level of the hormone |
| 16. How is EPO measured? | By very sensitive immunoassays |
| 17. How is EPO production controlled? | By the tissue availability of oxygen |
| 18. What is the function of EPO? | To bind to receptors on the marrow erythroid precursors |
| 19. What can occur under the stimulus of EOP? | Red cell production can increase 4-5 fold in one week time |
| 20. What happens when hemoglobin falls below 10g per dl? | a stimulus to enhance EPO production is initiated |
| 21. What is a erythron? | A dynamic organ made of a pool of marrow erythroid precursor cells and a large mass of mature circulating red blood cells |
| 22. What is the function of erythron? | It depends on EPO and functional erythroid marrow, and adequate supply of nutrients for red cell production |
| 23. What are the disorders of hematological system? | Nutrient Disorders, Hemoglobinopathies, Metabolic Defects, Autoimmune Related Disorders, Disorders of Cell Production, Mylelothistic Disorder ( Malignancies), Hemolytic Anemias |
| 24. What are the 3 major nutrients of RBC production? | Iron, B12 and folate |
| 25. What does Iron binds to? | Iron binds to hemoglobin and facilitates the biological activity of EPO |
| 26. What does Iron Deficiency cause? | hypo-proliferative anemia |
| 27. What causes megaloblastic anemias? | Both folic and coblamin B12 |
| 28. What is the major role of Iron? | To carry oxygen as part of the heme protein that turns into hemoglobin |
| 29. What is Iron a critical element of? | Iron is a critical element in the cytochrome system in mitochrondria |
| 30. What happens to sans iron cells in Iron Deficiency Anemia? | Sans iron cells lose their capacity for electron transport and hemoglobin synthesis is impaired |
| 31. Where does the Iron absorbed from the diet circulate? | In the plasma bound to transferin |
| 32. What is a transferrin? | It is a bi-lobed glycoprotein with two binding sites |
| 33. Where is majority of the iron transported by transferring delivered? | To the erythroid marrow |
| 34. What is the clearance of tranferrin directly related to? | marrow activity and plasma Fe |
| 35. Where does the Iron transferrin complex circulate? | In the plasma until they interact with transferrin receptors on the surface of the erythroid marrow cells |
| 36. What has the greatest number of receptors? | The developing erythroblast |
| 37. Why is Iron made available upon interacting with the receptor? | For heme synthesis |
| 38. Where does excess of amounts of hemoglobin synthesis of Iron bind to? | storage protein |
| 39. How is ferritin formed? | By a storage protein apoferritin |
| 40. What occurs when apoferritin forms ferritin? | It is binds with the excess Fe |
| 41. Where else does Iron storage occur? | In the liver |
| 42. How does Iron incorporated into hemoglobin enter the circulation? | As a new red blood cell that are released in the circulation |
| 43. What does the iron incorporated into hemoglobin become a part of? | Becomes a part of red cell mass and cannot be re-utilized until red cell dies |
| 44. What is the life span of RBC? | 120 days |
| 45. What causes megaloblastic anemias? | Impaired DNA synthesis |
| 46. What kinds of cells are affected in megaloblastic anemia? | Cells with rapid turnover – hemopoietic precusors and gastrointestinal epithelial cells |
| 47. How are cells affected in megaloblastic anemia? | Cells are larger with greater RNA to DNA ratio |
| 48. What is the end result of megaloblastic anemias? | The end result is ineffective erythropoesis |
| 49. What does Hemoglobin Oxygen Dissociation Curve do? | It plots the proportion of hemoglobin in saturated form on its vertical axis against the prevailing oxygen tension on the horizontal axis |
| 50. What will changes in physiological condition do to the curve? | It will shift the curve to the left or right indication decreased or increase oxygen affinity |
| 51. What will happen to the curve is there is an increase temperature, 2,3-BPG, or pC02, or a decrease in pH? | It will shift the curve to the right, which demonstrated decrease oxygen affinity |
| 52. What does the right shift do? | It decreases the oxygen affinity of hemoglobin thus increasing the requirement oxygen to obtain the same saturation. |
| 53. What does the right shift enhance? | The ability of hemoglobin to release oxygen into the tissues |
| 54. Who has a higher oxygen affinity, adult hemoglobin or fetal hemoglobin? | Fetal hemoglobin, which shifts the curve to the left |
| 55. What does the fetal hemaglobin physiological ability to shift the curve to the left mean? | It compensates for the lower oxygen tension that are in the human placenta |
| 56. What is apparent at 6 weeks of fetal life? | 3 hemoglobin variants appear that are subsequently transformed into fetal hemoglobin |
| 57. When does fetal hemoglobin appear? | At 10 weeks and persistent until 38 weeks after which adult hemoglobin is predominately synthesized |
| 58. What causes Spleenic Sequestration Syndrome? | Caused by engorgement of RBCs in un-infarcted spleen. |
| 59. In who does SSS present in? | children under 5 years of age. |
| 60. If a child has a grossly enlarged spleen and profound anemia, what is possible? | shock is possible |
| 61. What is the first line of therapy? | Immediate transfusion with packed RBC |
| 62. What are the Classification of Hemoglobinopathies? | 1-Structural hemaglobinopathies, 2-Thalassemias, 3-Combination Thalassemia variants, 4-Hereditary Persistent of HgbF, and 5-Aquired Hemaglobinopathies |
| 63. What is structural hemoglobinopathies? | A specific single amino acid defects in the Beta chain |
| 64. What is Thalassemias? | A defective biosynthesis of the globin chains |
| 65. What hemaglobinopathies does no clinically present before six months of age? | B chain hemaglobinopathies |
| 66. What has a much higher oxygen affinity, Fetal Hemoglobin (gamma chain) or hemoglobin A? | Fetal hemoglobin (gamma chain) |
| 67. When does the gamma chain switch to B chain? | 6 months of age |
| 68. What can happen after 6 months of age? | A child can begin to express one of the B chain hemoglobinopathies |
| 69. What is B chain Hemoglobinopathies? | The B chain hemaglobinopathies are inherited as autosomal recessive due to a single amino acid substitution. |
| 70. What is the basic mechanism for sickle cell anemia? | The B chain changes from hemaglobin A to hemaglobin S from single amino acid substitution of valine for glutamic acid at position 6 of B chain. |
| 71. Does Sickle cell hemoglobin have a significantly decrease oxygen carrying capacity? | Yes |
| 72. In what conditions does sickle sell hemoglobin tend to lose their capability of deformability that predisposes them to a sickle? | Under adverse physiological conditions such as infection, surgery and stress. |
| 73. What occurs in the first 5 years of life to a sickle cell anemia patient? | Infarction of the spleen, which results in auto spleenectomy and is associated with immunodeficiency |
| 74. What does chronic hemolytic anemia result in a sickle cell anemia patient? | excessive number of RBC that must be catabolized which subsequently overloads the RE system and potentiates the spleenic dysfunction |
| 75. What are sickle RBC capable of? | Obstructing capillaries resulting in a pathology of increased adhesion and local microinfarction |
| 76. What does the process of microinfarction in sickle cell anemia patients involve? | Progressive and preferentially involves the spleen, the long bones, CNS and chest |
| 77. Is the process of microinfarction in sickle cell anemia patients progressive? | Yes |
| 78. What are the clinical syndromes of sickle cell anemia? | Hand Foot Syndrome, Spleenic Sequestration Syndrome, Pain Crises( tubular bones), Hypoplastic Crises, Chest Syndrome, CNS infarctions, Osteomyelitis, Sepsis |
| 79. When does the Hand Foot Syndrome occur? | It occurs in the 1st two years of life |
| 80. What does the Hand Foot Syndrome initiate? | It initiates pain and swelling of the small tubular bones of the hand and feet. |
| 81. What does a child with Hand Foot Syndrome present with? | Child presents with swollen hands or the inability to walk |
| 82. What is the treatment for Hand Foot Syndrome? | Treatment with analagesics and fluid therapy is usually effective |
| 83. What does pain crisis principally involve? | Pain crises principally involve the long bones |
| 84. Why does pain crisis occur? | These crises are result of capillary obstruction by increased number sickled red blood cells |
| 85. How are the pain crisis initiated? | These crises are initiated by infection, dehydration and stress. |
| 86. How are Pain Crisis treated? | Pain crises are treated with hydration and pain management. |
| 87. What causes Hypoplastic Crisis? | These crises are caused by intercurrent viral infection in particular Parvo B19. |
| 88. What does Parvo B19 initiate? | red cell production at the level of the bone marrow |
| 89. What does a patient with Hypoplastic Crisis present with? | Patient present with fever, lethargy, occasionally gastroenteritis and pallor |
| 90. What will a patient with Hypoplastic Crisis’ laboratory evaluation demonstrate? | A fall in Hgb, Hct and reticulocyte count. |
| 91. What is the cause of Chest Syndrome? | Chest syndrome is caused by multiple micro-infarctions in the lung resulting in perfusions defects that may result in respiratory failure |
| 92. What does a child with Chest Syndrome present with? | Children with chest syndrome present with acute chest pain, dyspnea, tachypnea, pallor and occasionally cyanosis |
| 93. How is Chest Syndrome best diagnosed? | Chest syndrome is best diagnosed by chest Xray( infiltrates) or more specifically with MRI |
| 94. Are children with sickle cell disease more susceptible to strokes? | Yes |
| 95. Are patients with more malignant genotypes( Central African) more prone to the devastating complication of stroke and major infections? | Yes |
| 96. Are all children with sickle cell disease have an increase prevalence to micro brain infarcts that may lead to both brain functional impairments or physiologic impairment? | Yes |
| 97. Should every child with sickle cell disease be monitored with transdermal Doppler yearly? | Yes |
| 98. Why are sickle cell patients susceptible to infection? | because of splenetic dysfunction, impairment of the RE system secondary to failure of clearing immune complexes from the RE system and possible Factor B deficiency |
| 99. What do children with sickle cell disease present with? | pneumococcal sepsis, H. influenza disease and osteomyelitis due to salmonella |
| 100. In hemoglobinopathies, what is critical for oxygen delivery? | Hemoglobin |
| 101. In hemoglobinopathies, what can happen to hemoglobin? | Hemoglobin can alter the shape, deformability and viscosity of RBC |
| 102. What are hemoglobinopathies? | Disorders that affect the structure, function or production of hemoglobin |
| 103. Where does neutrophils evolve from? | Neutrophils evolve in the marrow from the myeloblast into a mature neutrophil |
| 104. What does promyelocyte have? | The promyelocyte has a large nucleus with the development of primary granules |
| 105. What does a band form consist of? | The Band form has condensed band shaped nucleus and is the analog to the reticulocyte |
| 106. Is mature neutrophil is segmented? | Yes |
| 107. Where and how is neutrophils released? | The neutrophil is released from the bone marrow by G-CSF, steroid, infection( endotoxin) |
| 108. In what sort of pool does neutrophil circulate? | Circulating pool |
| 109. How are neutrophils induced to leave the circulating pool? | By inflammatory mediators |
| 110. How are neutrophils egressed from the circulation to the tissue? | Adhesion molecules |
| 111. The bone marrow represents how many pools? | 3 dynamic pools |
| 112. What are the three dynamic Marrow pools? | Circulating Pool, Tissue Pool, and Marginal Pool |
| 113. What is the marrow pool? | The Marrow pool is the development and storage facility for neutrophil and its’ precursors |
| 114. Where are the marrow pools? | Normally 90% of the marrow pool is in the bone marrow |
| 115. The circulating pool consists of two dynamic pools? | Yes |
| 116. What percent is the free flowing pool? | Approximately 50% |
| 117. What is marginal pool? | The marginal pool are neutropils that are in close contact with the endothelium |
| 118. How does Infection affect the marrow pools? | Infection expands the circulating and marginal pools as well as tissue pool. |
| 119. Does Infection expands the tissue pool? | Yes |
| 120. How does Epinephrine affect the marrow pools? | It expands the circulating pool and Contracts the marginal pool |
| 121. How does Steroid affect the marrow pools? | Expands the marginated pool and the circulating pool |
| 122. How does Leucocyte Adhesion Deficiency affect the marrow pools? | It expands the Circulating Pool and contracts the Tissue Pool and Marginated Pool |
| 123. What are the Plasma Cell disorders? | Basic Mechanism of Plasma Cell Dycrasias, Immunological Mechanisms Involved, Monoclonal gammapathies of Undetermined Significance(MGSU), Multiple Myeloma, Waldenstrom’s Macrogloblinemia, Heavy Chain Disease, Cryoglobulinemia |
| 124. How are each M protein characterized? | Each M protein is characterized by two Heavy Chains and two Light Chains of the identical class. |
| 125. How are the heavy chains designated? | use Greek letter |
| 126. How are the Light Chains designated? | Either Kappa or Lambda. |
| 127. Are Plasma Cell Disorder neoplastic? | Plasma Cell Disorders are either neoplastic or potentially neoplastic |
| 128. What are plasma cell disorders associated with? | These disorders are associated with the proliferation of a single clone of immunoglobulin secreting plasma cells derived from B cell immunocytes. |
| 129. How are plasma cell disorders characterized? | These disorder are characterized by immunologically homogenous proteins(M). |
| 130. What is the previous terminology for MGUS? | Benign Monoclonal Gammapathy |
| 131. How is MGUS defined? | A disease process that produces less than 3 g/dl of M protein and fewer than 10% plasma cells in the BM. |
| 132. What is there a absence of in MGUS? | lytic lesions, hypercalcemia, renal insufficiency and stablization of the M protein concentration. |
| 133. How is Multiple Myeloma characterized? | By the neoplastic proliferation of single clone of plasma cell engaged in the production a monoclonal immunoglobulin |
| 134. In Multiple Myeloma, what is involved in the process? | Involves bone marrow proliferation, with invasion of the adjacent bone. |
| 135. What are the features of Multiple Myeloma? | Anemia, hypercalcemia and renal insufficiency |
| 136. What does the Multiple Myeloma represent? 1 | % of all cancers and 10% of all hematologic cancers. |
| 137. What is the incidence of Multiple Myeloma? | The incidence is 4 per 100,000. |
| 138. What is the average age of onset of Multiple Myeloma? | 65 years. |
| 139. Is Multiple Myeloma more common in men or female? | It is more common( slightly in men) |
| 140. In Multiple Myeloma, is the incidence 2X greater in African Americans? | Yes |
| 141. What is the clinical manifestation of Multiple Myeloma? | bone pain that spares the patient during sleep, anemia, weakness and fatigue. |
| 142. What is the most common physical finding? | most common is pallor. |
| 143. In Multiple Myeloma, what is present from an allergic stand point? | generalized pruritis. |
| 144. How is Multiple Myeloma caused? | environmentally and hereditary |
| 145. What is Multiple Myeloma linked to? | To hydrocarbons and aliphatic chemical. Ex: Herbicides. |
| 146. How is Multiple Myeloma linked to family clusters? | Reported in first degree relative and in identical twins |
| 147. What are the basic Immunology plasma cells? | The plasma cell are cytoplasmic Ig+, CD38+, CD 138+, and PCA 1+ |
| 148. What does the smaller % of cells express? | CD 10, HLADR |
| 149. What does the 20% of cells express? | CD 20. |
| 150. Where does the premyeloma cell circulate? | In the microenvironment of the marrow |
| 151. What cells are reduced? | CD4 + Tcell |
| 152. What are the over produced myeloma? | Il6, TNFa, Il-1B |
| 153. What are the results of increased osteoclast activity? | Lytic lesions, osteopenia, and hypercalcemia and fractures |
| 154. How are the Osteoclast activated? | By the transmembrane receptor RANK( receptor activator of nfkb) |
| 155. What controls the osteoclast production? | Receptor RANK |
| 156. What is the newly discovered member of the TNf-R family? | Oteoprotegerin OPG or receptor of activator of RANK. |
| 157. How is Oteoprotegerin expressed? | osteoclasts, macrophages and dendritic cell |
| 158. What increases the receptor activator? | Cytokines Il-6, TNfa and Il-1b |
| 159. What increases osteoclast proliferation? | Cytokines Il-6, TNfa and Il-1b |
| 160. How is Oseosclerotic Myeloma (POEMS) characterized? | by polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes. |
| 161. What is the clinical features of Oseosclerotic Myeloma (POEMS)? | Chronic inflammatory demyelinating polyneuropathy, hepatomegaly, optic neuritis, gyneocomastia, testicular atrophy, and hyperpigmentation and hypertrichosis. |
| 162. In Oseosclerotic Myeloma (POEMS), do the patients have elevated mu protein? | Yes |
| 163. In Oseosclerotic Myeloma (POEMS), the patient does not have bone marrow involvement, anemia, hypecalcemia? | True |
| 164. What is Waldenstrom’s Macroglobulinemia the result of? | from an uncontrolled proliferation of lymphocytes and plasma cells resulting in a large lgM M protein. |
| 165. What are the clinical manifestations of Waldenstrom’s Macroglobulinemia? | weakness, fatigue, bleeding( oronasal), blurred vision , heart failure and recurrent infections. |
| 166. In Waldenstrom’s Macroglobulinemia, there is no lytic lesions and renal involvement. | True |
| 167. What are the physical examination of Waldenstrom’s Macroglobulinemia? | pallor, hepatospleenomegaly, retinal hemorrhages, exudates and vascular segementation.Sensimotor periheral neuropathy and extensive pulmonary involvment |
| 168. What is the most common physical examination? | Extensive pulmonary involvement |
| 169. What is evident in the laboratory for Waldenstrom’s Macroglobulinemia? | severe normocytic normochromic anemia. |
| 170. What is evident in electrophoresis for Waldenstrom’s Macroglobulinemia? | It reveals a tall narrow peak(g)mobility. |
| 171. T/F. In Waldenstrom’s Macroglobulinemia 75% of Ig M has k light chains? | True |
| 172. In Waldenstrom’s Macroglobulinemia, Is the bone marrow is hypocellular(apirate)? | Yes |
| 173. In Waldenstrom’s Macroglobulinemia , the biopsy is hypercellular with lymphoid, plasma cells and mast cells? | Yes |
| 174. Is the formation of Rouleaux present on Waldenstrom’s Macroglobulinemia? | Yes |
| 175. In Waldenstrom’s Macroglobulinemia patients, how many % produce cryoglobulins? | 10% |
| 176. In Waldenstrom’s Macroglobulinemia, what does the lymphoplasmacytic cells express? | CD 19, CD 20, and CD 22 |
| 177. How does Myeloma and Heavy Chain Disease differ? | These disease differ from myeloma due the production of M protein characterized only by portions of the heavy chain in the serum and urine. |
| 178. In Heavy Chain Disease, how are the Gamma HCD characterized? | By an M protein that contains on amino acid deletions and the CH1 domain |
| 179. What are the clinical manifestation of Heavy Chain Disease? | Severe anemia, coombs + anemia, hepatospleenomegaly and bone marrow(hypercellular) |
| 180. T/F. Alpha Heavy Chain Disease is the most common and occurs in Mediterranean region? | True |
| 181. What is Alpha Heavy Chain Disease most commonly present with? | GI disease mal-absorption, diarrhea, steatorrehea and weight loss. |
| 182. Is there a Pathology infiltration of the jejunum with plasma cells in Alpha Heavy Chain Disease? | True |
| 183. What is the diagnosis depend on in a patient of Alpha Heavy Chain Disease? | The identification alpha heavy chain |
| 184. Is Alpha Heavy Chain Disease a frequent, progressive and fatal disorder that responds to treatment with early diagnosis? | True |
| 185. What does the diagnosis of Mu Heavy Chain Disease depend on? | The detection of a mu heavy chain in the serum. |
| 186. What do the patients of Mu Heavy Chain Disease present with? | Chronic lymphocytic leukemia or lymphoma. |
| 187. Does a patients with Mu Heavy Chain Disease frequently present with? | Significant hypogammaglobulinemia |
| 188. In Cryglobinemia, what proteins are present? | Cryglobulins |
| 189. What are Cryglobulins? | Proteins that precipitate when cooled and dissolve with heat. |
| 190. How many types of patients are there? | Type 1 and Type 2 |
| 191. How is Type 1 disease characterized? | By IgM and Ig G. |
| 192. Are 80% of the patients with Type 1 Cryoglobulin asymptomatic? | Yes |
| 193. What is the range in Type 1 patients with monoclonal cryoglobulins? | 1-2 dL |
| 194. What does a Type 1 patient Cryoglobulins present with? | pain, purpura, and ulceration of skin on exposure to cold |
| 195. What is a Type 1 patient Cryoglobulin associated with? | WMG,MM, and MGUS |
| 196. How are the characteristized of a Type 2 Cryoglobulin? | IgM and polyclonal IgG/A. |
| 197. What is Type 2 Cryoglobulins associated with? | Severe renal disease glomerular damage, vasculitis, and lymphoproliferative disease |
| 198. What are the Causes of Neutropenia? | Decreased production, peripheral destruction, peripheral pooling and hereditary |
| 199. How is the production decreased? | drug induced Hematologic disorders( aplastic anemia) |
| 200. What is peripheral destruction? | Autoimmune disorders |
| 201. What are the Hereditary disorders? | Kostmann’s Syndrome, Shachman’s Syndrome, cyclic neutropenia |
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