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Hematology Clinical Medicine - I

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1. Alpha globin chain genetics
Lymphoid Leukemia - Mono - acute infections a/w viral diseases
Alpha genes are on chromosome 16 - 2 genes on chromosome 16 for every alpha globin chain - total of 4 genes responsible for the 2 alpha globin chains
Acyclovir - Phenytoin - Quinin - Amphotericin B - Levamisole - Digoxin - Ibuprofin - Amiodarone - Rifampin - ASA
M- 75-175 F- 65-165

2. Clotting disorders
Anti-thrombin III deficiency - Lupus anticoagulant - other conditions for hyper-coagulation
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)
XRT (radiation and Chemo - inherited immune disorders
Male: 14-17 Female: 12-15

3. Anemia developes because
Excessive loss of RBC - inadequate production of RBC (problem with bone marrow) - increased destruction of RBC - secondary to chronic disease
In the bone marrow
Differentiate into whatever cell the body needs - self renewing - uncommitted cells - can differentiate into specialized cells such as progenitor cells
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

4. Lab findings for Hemolytic anemia
Increased retic with falling/ stable HgB - increase serum LDH - increase total bilirubin - transient hemoglobinuria
FE deficiency - Hemoglobinopathies - sideroblastic anemia - lead poisoning
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
Low WBC count - Bone marrow depression: drug related (H2 blockers) - primary bone marrow disorder - immune associated neutropenia (chemo) - fungal infection of bone marrow (HIV)

5. IL-2
Hemoglobulinopathy - inherited or acquired - Problem: RBC membrane defects so will get episodic or continuous RBC destruction - varies from mild to severe
N - M - Meg - RBC
T and B lymph
T lymph - stem cell

6. Thallassemia Syndormes
Plasma protein that binds to Fe and helps absorb it - 200-400
Breaking open of the RBC's and release of HgB (spleen)
Mild: tired - pale - often assymptomatic - pallor - jaudnice - tachycardia - palpitation - spleen enlargement - glossitis - cheilosis (sore in the mouth) - neurologic sxs
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

7. ecchymosis
Abnormal size of cells and vary in abnormality
4.5-10
8% of body weight- 5L in women and 5.5L in males
Confluent areas of bleeding right below the skin

8. Bone Marrow Cellularity
Usually 40-45% - fat cells make up 50-55Q% - Rest are RBC precursor
volume % of RBC in blood - Female: 36-48 - Males: 42-52
Bind to receptors on cells and initiate carrying of signals that generate transcription factors and the cell proliferates
FE deficiency - have normal FE iron studies because cannot attach to Hb molecule

9. Intrinsic hemolytic anemia
Thalessemia - and sickle cell
T and B lymph
Erythrocytes: 120 days - Platelets: 8-10 days - WBC: hours to few weeks - increase during infection
volume % of RBC in blood - Female: 36-48 - Males: 42-52

10. Hematopoietic Stem cells
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
Differentiate into whatever cell the body needs - self renewing - uncommitted cells - can differentiate into specialized cells such as progenitor cells
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
Platelet problems - factor 10 and vwf deficiency - vitamin K deficiency - anti-thrombin III deficiency - protein C deficiency - acquired disorder or coagulation

11. IL-1
Plasma protein that binds to Fe and helps absorb it - 200-400
Temporary infections - trauma/ tissue injury - Acute Infections: WBC count increases - Other causes:Leukemia - Malignant Neoplasms - Drugs -
T lymph - stem cell
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

12. Decreased Platelets
Primary function is to support O2 transport - hard to bind the first O2 to Hgb but once it does it becomes easier to bind - It must bind at the partial pressure of O2 of alveolus and then it releases it to the tissue at the partial pressure of O2 at
Programmed cell death - eliminates old - unnecessary - or unhealthy/ abnormal cells - defects in the pathway are important in causing CA
ITP/TTP: platelets destroyed - bone marrow suppression - drugs - prosthetic heart valve: chew up platelets
Sickle cell - G6PD - thalessemia

13. PE for anemia
Abnormal size of cells and vary in abnormality
Mild: tired - pale - often assymptomatic - pallor - jaudnice - tachycardia - palpitation - spleen enlargement - glossitis - cheilosis (sore in the mouth) - neurologic sxs
CBC with Diff - PT: prothrombin time ***important it analyzes clotting ability of 5 plasma coagulation factors - INR: internatoinal normalized ratio (1-2) - PTT: partial thromboplastin time (30-45sec): analyzes the first stage of clotting - bleeding
Parasite infections

14. PE of bleeding patient
If platelets are below 40 -000 will spontaneously bleed and occurs where pressure it
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
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)
98% - alpha and beta

15. Extrinsic hemolytic anemia
Pathophysiologic basis: diminished product of increase destruction - Morphologic basis: measure of average RBC size
RBC (erythrocytes) - WBC (leukocytes) - Platelet (thrombocytes) - blood cells are surrounded in Plasma
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
Autoimmnr hypersplensm

16. Hemolytic Anemia
Starts with vasoconstriction and this causes platelets to come to the site of damage and adhere to damaged collagen
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)
Tiredness - easily fatigued - DOE - palpitation - dizziness - bleeding - sores on mouth and tongue
Hemoglobulinopathy - inherited or acquired - Problem: RBC membrane defects so will get episodic or continuous RBC destruction - varies from mild to severe

17. anisocytosis
Cellular Immunity: helper - killer cells: affected with HIV
7th month
Variation in size
HbA-2alpha and 2 beta chains

18. Bleeding disoders can be....
55% of blood plasma - contains all the blood cells
Acquired or inhereited
4.5-10
Platelet problems - factor 10 and vwf deficiency - vitamin K deficiency - anti-thrombin III deficiency - protein C deficiency - acquired disorder or coagulation

19. Neutropenia
Platelets - In BM: very large - In blood: small irregular shaped cell fragments - involved in hemostasis
Low neutrophil count - seen in bone marrow/ blood diseases - drugs - chemicals (benzene) - radiation (affects bone marrow)
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

20. Transferrin
Temporary infections - trauma/ tissue injury - Acute Infections: WBC count increases - Other causes:Leukemia - Malignant Neoplasms - Drugs -
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
Plasma protein that binds to Fe and helps absorb it - 200-400
Bind to receptors on cells and initiate carrying of signals that generate transcription factors and the cell proliferates

21. Major Adult Hgb
Programmed cell death - eliminates old - unnecessary - or unhealthy/ abnormal cells - defects in the pathway are important in causing CA
HbA-2alpha and 2 beta chains
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.
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

22. Detection of hemoglobinopathy
The flat bones: sternum - pelvis - proximal ends of the long bones
Hb analysis and electrophoresis with functional assays for sickling - solubility - and O2 affinity are used for routine clinical evaluations
Fetal Hg - 2 alpha and 2 gamma chains - 1%
liver produces most coagulation factors excepts for VIII - vWF - - liver damage: impaired synthesis of most clotting factors - fibrinogen - plasminogen - malabsoprtion of vitamen K - portal HTN - Labs: prolonged PT - advanced increase PT - PTT - Tx:

23. Platelets
Palpable - slightly raised areas of bleeding
Erythrocytes - respond to tissue hypoxia and carry O2 to the body
Megakaryocytes (growth stimulated by thrombopoieten) - help prevent hemorrhages
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)

24. Progenitor cells
Anti-thrombin III deficiency - Lupus anticoagulant - other conditions for hyper-coagulation
HbA2: 2 alpha and 2 delta globin chains - in 2% of the population
MCV: 80-100 - sickle cell anemia - G6PD
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

25. Bacteria that patients with sickle cell anemia are at risk for
N - M - Eo - Meg - B - RBC - stem
Hemoglobulinopathy - inherited or acquired - Problem: RBC membrane defects so will get episodic or continuous RBC destruction - varies from mild to severe
Parasite infections
Encapsulated bacteria: Strep pneumonia and H. Influenza

26. WBC
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
HbA-2alpha and 2 beta chains
Holds platelets together - factor VIII activated it and has to tighten - trapping more platelets
FE deficiency - have normal FE iron studies because cannot attach to Hb molecule

27. Iron Serum test
M- 75-175 F- 65-165
volume % of RBC in blood - Female: 36-48 - Males: 42-52
HbA-2alpha and 2 beta chains
Thrombocytopenia - Henoch- Scholein Purpura - Scurvy - ITP - TTP - DIC - Hemophilia A and B - Von Willebrands disease - Vit. K deficiency - Liver disease

28. CBC with differential
98% - alpha and beta
Complete blood count with differential: tells what percentage of cells make up the blood
Erythrocytes - respond to tissue hypoxia and carry O2 to the body
Low WBC count - Bone marrow depression: drug related (H2 blockers) - primary bone marrow disorder - immune associated neutropenia (chemo) - fungal infection of bone marrow (HIV)

29. What do growth factors do?
Less than 1 persent
N - M - Meg - RBC
Bind to receptors on cells and initiate carrying of signals that generate transcription factors and the cell proliferates
Low neutrophil count - seen in bone marrow/ blood diseases - drugs - chemicals (benzene) - radiation (affects bone marrow)

30. Hematopoiesis
Confluent areas of bleeding right below the skin
Baseline test - wellness screening - diagnosing certain condition - response to treatment and recovery - monitor certain health conditions
Allergies - drug reactions (pCH) - parasitic diseases and tape worms
Process by which all blood cells form - develop - and are replaced in the body - blood production

31. Hemolytic Anemia
Bind to receptors on cells and initiate carrying of signals that generate transcription factors and the cell proliferates
Platelet problems - factor 10 and vwf deficiency - vitamin K deficiency - anti-thrombin III deficiency - protein C deficiency - acquired disorder or coagulation
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)
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. RBC
Humeral immunity: Ab production
Thrombocytopenia - Henoch- Scholein Purpura - Scurvy - ITP - TTP - DIC - Hemophilia A and B - Von Willebrands disease - Vit. K deficiency - Liver disease
Erythrocytes - respond to tissue hypoxia and carry O2 to the body
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

33. Variations with HbA
Megakaryocytes (growth stimulated by thrombopoieten) - help prevent hemorrhages
HbA2: 2 alpha and 2 delta globin chains - in 2% of the population
55% of blood plasma - contains all the blood cells
Female: 12-16 gm/dl ***menopausal women have more Male: 14-17 gm/dl

34. Plasma
T and B lymph
55% of blood plasma - contains all the blood cells
150 -000-450 -000
Have granules in the cytoplasm - neutrophil: 50-60%** most prominent - eosinophil: 1-4% - basophil: 0.5-1%

35. G-CSF
Growth factor that causes neutraphils to grow
large gylcoprotein - major adhesion molecule which allows platelets to stick - binding factor in factor VIII and prolongs its half life
1-2% - alpha and gamma
Platelets - In BM: very large - In blood: small irregular shaped cell fragments - involved in hemostasis

36. Mean Corpuscular Volume
Temporary infections - trauma/ tissue injury - Acute Infections: WBC count increases - Other causes:Leukemia - Malignant Neoplasms - Drugs -
Primary function is to support O2 transport - hard to bind the first O2 to Hgb but once it does it becomes easier to bind - It must bind at the partial pressure of O2 of alveolus and then it releases it to the tissue at the partial pressure of O2 at
MCV - index for classifying anemia - Norma: 80-100
55% of blood plasma - contains all the blood cells

37. Fibrin
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
Breaking open of the RBC's and release of HgB (spleen)
Holds platelets together - factor VIII activated it and has to tighten - trapping more platelets
What is stored in body M: 18-270 W: 18-160

38. Pathology for Thallessemia
Asymptomatic until 4 months of life
Programmed cell death (cyto C. and caspases)
Anti-thrombin III deficiency - Lupus anticoagulant - other conditions for hyper-coagulation
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

39. bleeding disorders
Thrombocytopenia - Henoch- Scholein Purpura - Scurvy - ITP - TTP - DIC - Hemophilia A and B - Von Willebrands disease - Vit. K deficiency - Liver disease
MCV: 80-100 - sickle cell anemia - G6PD
Cellular Immunity: helper - killer cells: affected with HIV
8% of body weight- 5L in women and 5.5L in males

40. Anemia
Female: 12-16 gm/dl ***menopausal women have more Male: 14-17 gm/dl
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
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)
Normal adult Hg - 2 alpha and 2 beta globin chains - attached to each chain is heme - 95-98%

41. HbF
Immature red blood cells - normal: 0.5-1.5%: elevated in people with anemic anemia - low retic: anemia from decreased RBC production
CBC with Diff - PT: prothrombin time ***important it analyzes clotting ability of 5 plasma coagulation factors - INR: internatoinal normalized ratio (1-2) - PTT: partial thromboplastin time (30-45sec): analyzes the first stage of clotting - bleeding
Fetal Hg - 2 alpha and 2 gamma chains - 1%
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

42. Reticulocyte count
FE deficiency - have normal FE iron studies because cannot attach to Hb molecule
N - M - Eo - Meg - B - RBC - stem
1-2% - alpha and gamma
Immature red blood cells - normal: 0.5-1.5%: elevated in people with anemic anemia - low retic: anemia from decreased RBC production

43. Blood Facts
7th month
8% of body weight- 5L in women and 5.5L in males
Lymphocytes: 20-40% - Monocytes: 2- 6% (circulate in the blood 1-3 days and mature in tissues)
1-2% - alpha and gamma

44. Bleeding D/O laboratory testing
150 -000-450 -000
7th month
CBC with Diff - PT: prothrombin time ***important it analyzes clotting ability of 5 plasma coagulation factors - INR: internatoinal normalized ratio (1-2) - PTT: partial thromboplastin time (30-45sec): analyzes the first stage of clotting - bleeding
Variation in size

45. B12
It is bound to intrinsic factor: protein produced and secreted by gastric parietal cells - B12 is absorbed in the terminal ilium and stored in the liver
98% - alpha and beta
Plasma protein that binds to Fe and helps absorb it - 200-400
Female: 12-16 gm/dl ***menopausal women have more Male: 14-17 gm/dl

46. Microcytic cells
Immature red blood cells - normal: 0.5-1.5%: elevated in people with anemic anemia - low retic: anemia from decreased RBC production
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%
Less than 80 MCV - iron deficiency - thalessemia - siderblastic

47. INR
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
Standard for reporting the PT
Platelets - In BM: very large - In blood: small irregular shaped cell fragments - involved in hemostasis
Allergies - drug reactions (pCH) - parasitic diseases and tape worms

48. Proper O2 transport depends on...
Increased retic with falling/ stable HgB - increase serum LDH - increase total bilirubin - transient hemoglobinuria
Have granules in the cytoplasm - neutrophil: 50-60%** most prominent - eosinophil: 1-4% - basophil: 0.5-1%
Tetramer structure of proteins - proper arrangement of charged amino acids - interaction with low molecular weight substances
Genetic and rare or acquired

49. Hgb anatomy
Complete blood count with differential: tells what percentage of cells make up the blood
XRT (radiation and Chemo - inherited immune disorders
Anemia - hemodilution (over hydration) - leukemia - hemolytic reactions - drugs
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)

50. Specific disease of anemia
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
M- 75-175 F- 65-165
Alpha genes are on chromosome 16 - 2 genes on chromosome 16 for every alpha globin chain - total of 4 genes responsible for the 2 alpha globin chains
Sickle cell - G6PD - thalessemia