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Hematology Clinical Medicine - I

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1. Pathology for Thallessemia
Severe infections
Deletion or point mutation of 1 or more globin genes - caused by reduced synthesis of 1 or more alpha globin chains or beta which leads to microcytic hypochromic anemia - means that the globin chain synthesis is defective from mutated or missing 1 or
Encapsulated bacteria: Strep pneumonia and H. Influenza
Acyclovir - Phenytoin - Quinin - Amphotericin B - Levamisole - Digoxin - Ibuprofin - Amiodarone - Rifampin - ASA

2. PE for anemia
Arrest of bleeding - depends on vessel wall - platelets - and coagulation factors (fibrolytic factors)
Genetic and rare or acquired
Platelet problems - factor 10 and vwf deficiency - vitamin K deficiency - anti-thrombin III deficiency - protein C deficiency - acquired disorder or coagulation
Mild: tired - pale - often assymptomatic - pallor - jaudnice - tachycardia - palpitation - spleen enlargement - glossitis - cheilosis (sore in the mouth) - neurologic sxs

3. PE of bleeding patient
Skin: hematomas - petechiae - nose and mouth/ throat bleeding - LN: usually not involved - HLA: look for enlarged spleen - liver - MSS: hemathrosis (bleeding into the joints - swollen - tender)
4.5-10
Immature RBC right before it fully matures - Mature (spend 3 days in) red bone marrow and then circulate in the peripheral blood for 1 day and fully mature (extrusion of nucleaus) - 1% of RBC in the body
Variation in shape

4. Bone Marrow Cellularity
Temporary infections - trauma/ tissue injury - Acute Infections: WBC count increases - Other causes:Leukemia - Malignant Neoplasms - Drugs -
T and B lymph
Usually 40-45% - fat cells make up 50-55Q% - Rest are RBC precursor
Asymptomatic until 4 months of life

5. Erythrocytes
FE deficiency - have normal FE iron studies because cannot attach to Hb molecule
RBC - most munerous - Mature they are anucleated boconcave cells which allows more surface area for Hb - certain disease cause shape change: sickle cell anemia - ineffective erythropoiesis is a main feature in many blood diseases
98% - alpha and beta
What is stored in body M: 18-270 W: 18-160

6. Hemoglobin F
Less than 1 persent
Anti-thrombin III deficiency - Lupus anticoagulant - other conditions for hyper-coagulation
Lymphoid Leukemia - Mono - acute infections a/w viral diseases
HbA2: 2 alpha and 2 delta globin chains - in 2% of the population

7. Reticulocyte count
Programmed cell death - eliminates old - unnecessary - or unhealthy/ abnormal cells - defects in the pathway are important in causing CA
Platelets - In BM: very large - In blood: small irregular shaped cell fragments - involved in hemostasis
Immature red blood cells - normal: 0.5-1.5%: elevated in people with anemic anemia - low retic: anemia from decreased RBC production
Complete blood count with differential: tells what percentage of cells make up the blood

8. Granulocytes
Tetramer structure of proteins - proper arrangement of charged amino acids - interaction with low molecular weight substances
Have granules in the cytoplasm - neutrophil: 50-60%** most prominent - eosinophil: 1-4% - basophil: 0.5-1%
Lymphocytes: 20-40% - Monocytes: 2- 6% (circulate in the blood 1-3 days and mature in tissues)
Palpable - slightly raised areas of bleeding

9. Apoptosis
Protein encasement of heme: 2 chains per globin- 1 alpha and 1 non alpha - 4 globin chains per tetrameter - 2 alpha chains and a pair of non-alpha chains (beta or gamma)
Programmed cell death - eliminates old - unnecessary - or unhealthy/ abnormal cells - defects in the pathway are important in causing CA
Differentiate into whatever cell the body needs - self renewing - uncommitted cells - can differentiate into specialized cells such as progenitor cells
Normal adult Hg - 2 alpha and 2 beta globin chains - attached to each chain is heme - 95-98%

10. petechiae
Beta genes are on chromosome 11 - 1 gene on chromosome 11 for each globin chain - totatl 2 genes responsible for the 2 beta globin chains
90-91% water - 6-7% are proteins like Albumin - Remaining 2-3%: electrolytes - carbohydrates and fats - chemical messengers (growth factors) and gases like O2 and CO2
If platelets are below 40 -000 will spontaneously bleed and occurs where pressure it
T and B lymph

11. Blood is produced....
Deletion or point mutation of 1 or more globin genes - caused by reduced synthesis of 1 or more alpha globin chains or beta which leads to microcytic hypochromic anemia - means that the globin chain synthesis is defective from mutated or missing 1 or
Mild: tired - pale - often assymptomatic - pallor - jaudnice - tachycardia - palpitation - spleen enlargement - glossitis - cheilosis (sore in the mouth) - neurologic sxs
In the bone marrow
N - M - Meg - RBC

12. Neutropenia
Immature red blood cells - normal: 0.5-1.5%: elevated in people with anemic anemia - low retic: anemia from decreased RBC production
Low neutrophil count - seen in bone marrow/ blood diseases - drugs - chemicals (benzene) - radiation (affects bone marrow)
Lymphocytes: 20-40% - Monocytes: 2- 6% (circulate in the blood 1-3 days and mature in tissues)
Immature RBC right before it fully matures - Mature (spend 3 days in) red bone marrow and then circulate in the peripheral blood for 1 day and fully mature (extrusion of nucleaus) - 1% of RBC in the body

13. Proper O2 transport depends on...
Skin - tend to bleed also into the muscles and joints
Allergies - drug reactions (pCH) - parasitic diseases and tape worms
Tetramer structure of proteins - proper arrangement of charged amino acids - interaction with low molecular weight substances
Breaking open of the RBC's and release of HgB (spleen)

14. purpura
Palpable - slightly raised areas of bleeding
Genetic and rare or acquired
Baseline test - wellness screening - diagnosing certain condition - response to treatment and recovery - monitor certain health conditions
Hereditary spherocytosis - G6PD deficiency - sickle cell syndrome

15. What do growth factors do?
Skin - tend to bleed also into the muscles and joints
Bind to receptors on cells and initiate carrying of signals that generate transcription factors and the cell proliferates
Skin: hematomas - petechiae - nose and mouth/ throat bleeding - LN: usually not involved - HLA: look for enlarged spleen - liver - MSS: hemathrosis (bleeding into the joints - swollen - tender)
Hereditary spherocytosis - G6PD deficiency - sickle cell syndrome

16. HbF
Autosomal co-dominant traits who inherit a different abnormal allele from each parent - Alpha chain is present in HbA - HbA2 - HbF: alpha chain mutations cause abnormalities in all three - beta chains are present in HbA and HbA2
Sickle cell - G6PD - thalessemia
Standard for reporting the PT
Fetal Hg - 2 alpha and 2 gamma chains - 1%

17. Variations with HbA
High neutrophil count - due to acute - localized/ general bacterial infections - inflammation - myeloproliferative diseases
HbF: 2 alpha and 2 gamma
HbA2: 2 alpha and 2 delta globin chains - in 2% of the population
Deletion or point mutation of 1 or more globin genes - caused by reduced synthesis of 1 or more alpha globin chains or beta which leads to microcytic hypochromic anemia - means that the globin chain synthesis is defective from mutated or missing 1 or

18. Transferrin
Sickle cell - G6PD - thalessemia
low platelet count - skin bleeding - mucosa: especially bleeding of gums
Bind to receptors on cells and initiate carrying of signals that generate transcription factors and the cell proliferates
Plasma protein that binds to Fe and helps absorb it - 200-400

19. Serum Ferritin
What is stored in body M: 18-270 W: 18-160
RBC (erythrocytes) - WBC (leukocytes) - Platelet (thrombocytes) - blood cells are surrounded in Plasma
Severe infections
Have granules in the cytoplasm - neutrophil: 50-60%** most prominent - eosinophil: 1-4% - basophil: 0.5-1%

20. Decreased Platelets
ITP/TTP: platelets destroyed - bone marrow suppression - drugs - prosthetic heart valve: chew up platelets
7th month
Hgb in the blood is too low to fulfill the O2 demands of the body - can be a specific disease of a sign of disease progress (GI CA)
Erythrocytes - respond to tissue hypoxia and carry O2 to the body

21. Reticulocyte
Type of anemia characterized by smaller paler than usual RBC
Erythrocytes: 120 days - Platelets: 8-10 days - WBC: hours to few weeks - increase during infection
4 globin chaines: globin (each bines to 1 O2 molecule Heme: binds to iron
Immature RBC right before it fully matures - Mature (spend 3 days in) red bone marrow and then circulate in the peripheral blood for 1 day and fully mature (extrusion of nucleaus) - 1% of RBC in the body

22. von Willebrand Factor
Sickle cell - G6PD - thalessemia
Type of anemia characterized by smaller paler than usual RBC
large gylcoprotein - major adhesion molecule which allows platelets to stick - binding factor in factor VIII and prolongs its half life
Female: 12-16 gm/dl ***menopausal women have more Male: 14-17 gm/dl

23. Hgb anatomy
Female: 12-16 gm/dl ***menopausal women have more Male: 14-17 gm/dl
Protein encasement of heme: 2 chains per globin- 1 alpha and 1 non alpha - 4 globin chains per tetrameter - 2 alpha chains and a pair of non-alpha chains (beta or gamma)
Hemoglobulinopathy - inherited or acquired - Problem: RBC membrane defects so will get episodic or continuous RBC destruction - varies from mild to severe
low platelet count - skin bleeding - mucosa: especially bleeding of gums

24. HbA
N - M - Eo - Meg - B - RBC - stem
Bind to receptors on cells and initiate carrying of signals that generate transcription factors and the cell proliferates
Lymphoid Leukemia - Mono - acute infections a/w viral diseases
Normal adult Hg - 2 alpha and 2 beta globin chains - attached to each chain is heme - 95-98%

25. Bacteria that patients with sickle cell anemia are at risk for
HbA-2alpha and 2 beta chains
Autosomal co-dominant traits who inherit a different abnormal allele from each parent - Alpha chain is present in HbA - HbA2 - HbF: alpha chain mutations cause abnormalities in all three - beta chains are present in HbA and HbA2
Encapsulated bacteria: Strep pneumonia and H. Influenza
Confluent areas of bleeding right below the skin

26. Basophils are present in....
group of disorders affecting the structure - function - or production of Hgb - Hgb is important for normal O2 delivery to the tissues. If there is an abnormality then can cause hypoxia of the tissue.
Thalessemia - and sickle cell
Parasite infections
Immature RBC right before it fully matures - Mature (spend 3 days in) red bone marrow and then circulate in the peripheral blood for 1 day and fully mature (extrusion of nucleaus) - 1% of RBC in the body

27. Hemolysis

28. Hematopoietic Stem cells
Differentiate into whatever cell the body needs - self renewing - uncommitted cells - can differentiate into specialized cells such as progenitor cells
HbF: 2 alpha and 2 gamma
High neutrophil count - due to acute - localized/ general bacterial infections - inflammation - myeloproliferative diseases
What is stored in body M: 18-270 W: 18-160

29. Drug Inducted Thrombocytopenia
Acyclovir - Phenytoin - Quinin - Amphotericin B - Levamisole - Digoxin - Ibuprofin - Amiodarone - Rifampin - ASA
Bind to receptors on cells and initiate carrying of signals that generate transcription factors and the cell proliferates
Severe infections
Starts with vasoconstriction and this causes platelets to come to the site of damage and adhere to damaged collagen

30. Gestation HgB
Sickle cell - G6PD - thalessemia
Autoimmnr hypersplensm
HbF: 2 alpha and 2 gamma
Pathophysiologic basis: diminished product of increase destruction - Morphologic basis: measure of average RBC size

31. Hemolytic Anemia
Production of RBC - stimulated by decrease in O2 -kidneys produce hormone erythropoietin
4 globin chaines: globin (each bines to 1 O2 molecule Heme: binds to iron
Complete blood count with differential: tells what percentage of cells make up the blood
Cause by increase destruction of RBC in the peripheral blood faster than the bone marrow can produce them - decstruction is intrinsic: defect in production - extrinsic: produced healthy

32. Anemia
Hgb in the blood is too low to fulfill the O2 demands of the body - can be a specific disease of a sign of disease progress (GI CA)
group of disorders affecting the structure - function - or production of Hgb - Hgb is important for normal O2 delivery to the tissues. If there is an abnormality then can cause hypoxia of the tissue.
Megakaryocytes (growth stimulated by thrombopoieten) - help prevent hemorrhages
Have granules in the cytoplasm - neutrophil: 50-60%** most prominent - eosinophil: 1-4% - basophil: 0.5-1%

33. Clotting disorders
Anti-thrombin III deficiency - Lupus anticoagulant - other conditions for hyper-coagulation
N - M - Meg - RBC
Type of anemia characterized by smaller paler than usual RBC
Fetal Hg - 2 alpha and 2 gamma chains - 1%

34. Coombs Antiglobulin Test
Detects presence of Ag-AB complex: direct or indirect - diagnostic for: hemolytic dz of a newborn - acquired hemolytic anemia - transfusion reaction - blood that sensitized patient. RBC
Polycythemia vera - CHF/COPD - Drugs - Living at high altitudes
GF produced by liver and stimulates production of megakariocytes
Cellular Immunity: helper - killer cells: affected with HIV

35. Macrocytic cells
Less than 80 MCV - iron deficiency - thalessemia - siderblastic
7th month
lineage committed - create different blood cell lineages - can be very large - mostly all nucleaus. As they mature and differentiate - they become smaller in size and the nucleaus decreases in size - Ex: Lymphiod - meloid - and platelet lineage cells
Greater than 100 MCV - B12 deficiency - folic acid deficiency - pernicious anemia (form of B12)

36. Acquired Disorders
Complete blood count with differential: tells what percentage of cells make up the blood
volume % of RBC in blood - Female: 36-48 - Males: 42-52
55% of blood plasma - contains all the blood cells
Begins later in life - may be a medication related - may be related to underlying medical condition - may be idiopathic - may involve more than 1 system

37. Heme- Heme interction
Hb analysis and electrophoresis with functional assays for sickling - solubility - and O2 affinity are used for routine clinical evaluations
Autosomal co-dominant traits who inherit a different abnormal allele from each parent - Alpha chain is present in HbA - HbA2 - HbF: alpha chain mutations cause abnormalities in all three - beta chains are present in HbA and HbA2
GF produced by the kidney and is a glycoprotein - stimulates the production of RBC in response to tissue hypoxia - binds to specific receptors on the surface of RBC precursors
The property inherent in the arrangement of the heme and globin units that allows for oxygenation acquisition and delivery over a narrow range of tension

38. WBC
HbA2: 2 alpha and 2 delta globin chains - in 2% of the population
Standard for reporting the PT
Leukocytes - neutraphils - monocytes - basophils - lymphocytes - eosinophils--> 5 different cells divided into 3 classes - immune system and fights viral - bacterial - fungal - parasites - and allergens - classified by presence or absence of granules
Baseline test - wellness screening - diagnosing certain condition - response to treatment and recovery - monitor certain health conditions

39. Poikilocytosis
Autoimmnr hypersplensm
Severe infections
Abnormally shaped cells - schiztocyte - tear drop - oval
Hereditary disorder than can vary from mild to severe life threatening - common in areas with malaria and causes a problem with the function and structure of Hb - seen in populations from: SE asia - China - Mediterranean - lesser extent: Africa

40. Intrinsic hemolytic anemia
Immature red blood cells - normal: 0.5-1.5%: elevated in people with anemic anemia - low retic: anemia from decreased RBC production
RBC - most munerous - Mature they are anucleated boconcave cells which allows more surface area for Hb - certain disease cause shape change: sickle cell anemia - ineffective erythropoiesis is a main feature in many blood diseases
Skin - tend to bleed also into the muscles and joints
Thalessemia - and sickle cell

41. Platelet problem
90-91% water - 6-7% are proteins like Albumin - Remaining 2-3%: electrolytes - carbohydrates and fats - chemical messengers (growth factors) and gases like O2 and CO2
The flat bones: sternum - pelvis - proximal ends of the long bones
Increased retic with falling/ stable HgB - increase serum LDH - increase total bilirubin - transient hemoglobinuria
low platelet count - skin bleeding - mucosa: especially bleeding of gums

42. poikilocytosis
FE deficiency - have normal FE iron studies because cannot attach to Hb molecule
Variation in shape
GF produced by the kidney and is a glycoprotein - stimulates the production of RBC in response to tissue hypoxia - binds to specific receptors on the surface of RBC precursors
group of disorders affecting the structure - function - or production of Hgb - Hgb is important for normal O2 delivery to the tissues. If there is an abnormality then can cause hypoxia of the tissue.

43. Mild forms of thalessemia need to be differentiated from...
Sickle cell - G6PD - thalessemia
Anemia - hemodilution (over hydration) - leukemia - hemolytic reactions - drugs
Genetic and rare or acquired
FE deficiency - have normal FE iron studies because cannot attach to Hb molecule

44. Hemobglobin A
Polycythemia vera - CHF/COPD - Drugs - Living at high altitudes
Production of RBC - stimulated by decrease in O2 -kidneys produce hormone erythropoietin
Confluent areas of bleeding right below the skin
98% - alpha and beta

45. Bleeding disoders can be....
Holds platelets together - factor VIII activated it and has to tighten - trapping more platelets
High neutrophil count - due to acute - localized/ general bacterial infections - inflammation - myeloproliferative diseases
Fetal Hg - 2 alpha and 2 gamma chains - 1%
Acquired or inhereited

46. HgB/ HCT is increased with....
Deletion or point mutation of 1 or more globin genes - caused by reduced synthesis of 1 or more alpha globin chains or beta which leads to microcytic hypochromic anemia - means that the globin chain synthesis is defective from mutated or missing 1 or
Polycythemia vera - CHF/COPD - Drugs - Living at high altitudes
Mild: tired - pale - often assymptomatic - pallor - jaudnice - tachycardia - palpitation - spleen enlargement - glossitis - cheilosis (sore in the mouth) - neurologic sxs
Normal adult Hg - 2 alpha and 2 beta globin chains - attached to each chain is heme - 95-98%

47. IL-2
Plasma protein that binds to Fe and helps absorb it - 200-400
T and B lymph
Normal adult Hg - 2 alpha and 2 beta globin chains - attached to each chain is heme - 95-98%
Allergies - drug reactions (pCH) - parasitic diseases and tape worms

48. Blood consists of what cellular elements
RBC (erythrocytes) - WBC (leukocytes) - Platelet (thrombocytes) - blood cells are surrounded in Plasma
Platelets - In BM: very large - In blood: small irregular shaped cell fragments - involved in hemostasis
Less than 80 MCV - iron deficiency - thalessemia - siderblastic
Polycythemia vera - CHF/COPD - Drugs - Living at high altitudes

49. G-CSF
M- 75-175 F- 65-165
Programmed cell death (cyto C. and caspases)
Differentiate into whatever cell the body needs - self renewing - uncommitted cells - can differentiate into specialized cells such as progenitor cells
Growth factor that causes neutraphils to grow

50. IL-1
Erythrocytes: 120 days - Platelets: 8-10 days - WBC: hours to few weeks - increase during infection
Normal adult Hg - 2 alpha and 2 beta globin chains - attached to each chain is heme - 95-98%
T lymph - stem cell
Deletion or point mutation of 1 or more globin genes - caused by reduced synthesis of 1 or more alpha globin chains or beta which leads to microcytic hypochromic anemia - means that the globin chain synthesis is defective from mutated or missing 1 or