Hematology 1. Hematopoiesis (abrevated) a. ERP i. Controlled 1. Decreased O2 a. Increase ERP 2. Hormone a. Erythropoietin i. Glycoprotein produced in the kidney ii.

Acts on ECFC iii. 1-4 units in urine a day iv. none in plasma is normal v. men have higher ERP cause of testosterone than women vi. ERP in urine inversely proportional to hemoglobin vii. Can produce 10 normal 3. Kidney disease creates a severe anemic ii. In utero 1. Intrauterine life a. Mesoblastic period (2-4 weeks) i. Primitive blood cells that are nucleated and they make embryonic hemoglobin ii. Comes from meschymal tissue iii. These cells form blood island that get connected by endothelial cells b. Hepatic period (2-3 months) i. Liver dominant site c. Myeloin period (5-9 months) i. Bone marrow starts to predominate iii. extra uterine hematopoiesis 1. red marrow is active marrow 2. yellow is fatty inactive marrow 3. age 20 a. sternum b. pelvis c. ribs d. vertebrare e. skull f. proximal ends of the long bones iv. extra medullary hematopoiesis 1. BM damage 2. Liver and spleen pick up slack and produce blood cells a. Splenomegaly b. Hepatomegaly

v. Bone Marrow 1. large reticular cells a. macrophages, support and compartmentalize i. hold in BM till mature ii. get rid of abnormals 2. space where cells develop a. cords 3. myloid erythroid ratio a. normal i. 2:1 to 4:1 b. abnormal i. increase 1. bacterial infection 2. WBC leukemia ii. Decrease 1. Sever infection 2. Anemic recovery 3. RBC leukemia b. Erythron i. Pituitary problems/ adrenal cotex/corticosteroids 1. Hyer/hypo active ii. Testosterone 1. increases iii. Estrogen 1. Decreases 2. Red cell maturation 1. Stem cell CD34 2. ECFU a. Un recognizable 3. Pronormoblast a. Big b. Can undergo mitosis c. 5-10% of NRBS in BM 4. Basophilic normoblast a. Smaller b. No nucleoli c. Can undergo mitosis d. 15-20% of NRBC in BM 5. Ploycromataphilic normoblast a. Smaller still b. Start to see Hb productions c. Can undergo mitosis d. 20-30% of NRBC in BM 6. Orthochromic normoblast a. Normal color

3. Hemoglobin a. Carries O2 and CO2 b. Heme i. Active part ii. Protophyrin ring iii. Contains central iron atom 1. Must me in 2+ state iv. Made in the mitochondria of NRBC c. Globin i. Group of protiens synthesis by ribosomes ii. Six normal chains are possible 1. Alpha 2. Beta 3. Gamma 4. Delta 5. Epsilon 6. Zeta d. Hemoglobin complex i. 4 heme and one globin ii. carries 4 oxygen molecules iii. oxyhemoglobin 1. carrying oxygen iv. reduced hemoglobin 1. carrying CO2 e. embryonic Hb i. grower 1 (first 3 months) 1. zeta/epsilon ii. Grower 2 1. Alpha/epsilon iii. Portland 1

i. 65-80% Hb b. pyknotic nucleus c. no mitosis d. 50% of NRBC in BM 7. Reticulocyte a. Slight blue left b. No nucleus c. Supravital stain see the RNA i. New methyl blue d. Can be seen in PB i. 0.5-1.5% e. anemia can be 5-20% f. 2-4 day span i. 3 in BM and 1 PB 8. Red cells a. 120 life span normal

1. Zeta/gamma iv. Fetal Hb (6-9 months) 1. Alpha/gamma f. Birth to adult i. Hb A1 1. Alpha/beta ii. Hb A2 1. Alpha/gamma iii. Hb F 1. Alpha/delta iv. Hb A1c 1. 3-6% of A1 2. glucose on terminal valine g. Oxygen dissociation i. Bohr affect 1. Saturation is equivalent to pH 2. At tissues a. Shift down and right 3. At lungs a. Shift up and to the left h. Electrophorisis i. 4. Spleen a. Sluggish blood flow b. Largest amount of MPS cells and Lymphs c. Blood flow through spleen i. Splenic artery ii. Trabecular 1. Lots of lyphs iii. Follicular artery of white pulp iv. Sinuses v. Red pulp granulocytes vi. Penicullar artiolies 1. MPS cells vii. Venous sinus viii. Splenic vain ix. Out d. Fuction i. Produce lyphs ii. Store iron and normal metabolites iii. Store abnormal metabolites 1. Melanin 2. Carbon iv. Phagocytize foreign particles and microorganisms v. RBC membrane maturation vi. RBC destruction

1. Culling 2. Pitting vii. Pooling and sequestering platelets and leukocytes e. Splenomegaly i. Consumption of glucose ii. Increased acidity iii. Increased exposure to MPS 1. Increased culling and pitting iv. Leukopenia v. Thrombocytopenia vi. Treatment 1. splenectomy 2. irradiate 5. Red cell morphology a. Anisocytosis i. Macrocyte ii. Microcyte b. Poiklocytosis i. Spherocytosis 1. Hereditary spherocytosis a. Auto dom gene b. Sodium in with water and potassium out c. Fine if not in spleen d. 14-19 day life span e. mostly white ppl f. symptoms i. and all hemolytic anemia symptoms ii. chronic anemia 2. other places found i. acquired hemolytic anemia ii. chemical poising iii. severe burns iv. hyperactive spleen ii. Sbistocytes 1. Fragments 2. Mechanical destruction 3. DIC 4. March hemaglobinuria iii. Leptocytes 1. Targets a. Floppy not normally mature membrane b. Decreased osmotic fragility c. Liver diseae d. Post splenectomy iv. Ovalocytes 1. auto dom

a. Membrane defect b. Ppl are fine in most cases c. 15% have hemolytic anemia v. Burr cells 1. Sharp points in PB 2. Found in a. Stomach cancer b. Peptic ulcers c. HA d. Cirrhosis e. Uremia vi. Crenated 1. artifact vii. Acanthocytes 1. Irregular projections thorny apple 2. Recessive gene a. Severe defect in lipid metabolism b. Decreased beta lipoproteins c. Visual and neurological disturbances d. Decreased osmotic fragility e. anemia viii. Stomatocytes 1. Mouths 2. Auto dom gene 3. Leads to HA 4. Membrane defects and swollen on one side with sodium and loss of potassium 5. See in a. Liver disease b. Rh null patients ix. Dacryocytes 1. Pear shaped 2. Anemia 3. cell comes from spleen and liver x. Sickle cell 1. Inherited 6. Red cell inclusions a. RNA i. Retics can have normally ii. Supra vital stain iii. Chronic HA b. Basophilic stippling i. RNA/Ribosmes c. Howell jolly body i. 1-2 distinct dots ii. nuclear material remints

iii. abnormal mitosis iv. feulgen stain d. Cabot ring i. Single circles or figure 8 ii. Cant see with phase microscopy iii. Wright stain + neg with feulgen iv. Spindle fiber left over v. Sever anemia e. Heinz Ehrlichs bodies i. Precipitated oxidized hemoglobin ii. No with wrights stain f. Siderotic granuals i. Small dots ii. Iron contain spots iii. In wrights stain there called pappenhimer bodies iv. Perls Prussian blue and you can call them this g. Malaria i. Ring stage usually 7. Anemia a. Blood loss i. Acute 1. Greater than 1000ml 2. At 2 hours your Hb and Hct drop cause fluid returns 3. 2-3 days a. Retic shower/ even orthochromic b. Increase WBC, platelets 4. Decreased serum iron 5. 10-12 days a. normalize ii. Chronic 1. Develop iron deficiency anemia b. Decreased ERP i. Deficiency 1. Iron a. Comes from i. Food in Fe 3+ 1. Reduce to 2+ with HCL or vit C b. Store i. Bone marrow 1. 20-25% Sideroblasts normal ii. Liver iii. Spleen iv. Lasts 3-4 years c. Causes i. Diet 1. Total iron is 4-5mg

2. Absorb 10-20mg per day ii. Colon inflammation iii. Small intestine inflammation iv. Diarrhea v. Gastrectomy d. Sequence i. In the body 1. Storage iron depleted 2. Plasma serum decreases 3. Total iron binding capacity increases 4. Normal red blood cell production decreases 5. Microcytic hypochromic anemia ii. In the lab 1. Serum iron decreased 2. TIBC increased 3. BM iron decreased 4. Positive response to Iron 5. Microcytic hypochromic cells iii. Normals 1. Serum iron a. 75-100ug/dl 2. TIBC a. 250-400ug/dl 2. Vitamin B12 a. Causes i. Pernicious anemia 1. Decreased intrinsic factor 2. Cause a. Antibody to intrinsic factor or parietal cells 3. Population a. 40-70 year old some kids b. NE,GB, scandanavia c. More in females 4. Symptoms a. Weight loss b. Smooth sore tongue c. Neurological problems d. Nerve damage e. Loss of appetite 5. Lab findings a. Megloblastic Bone marrow b. Macrocytic normocromic c. Ovalocytes

ii.

iii. iv. v. vi.

3. Folic acid a. Same as pernicious anemia only with neurological problems b. Stored in liver c. 3 month supply d. absorbed in small intestine with needed liver enzymes e. pregnancy increased need i. causes neural tube defects f. certain drugs are antagonists g. norm i. 3-16ug/L 4. Vitamin B6 a. Raw material for heme b. Microcytic hypochromic c. Refractory to iron d. Some ppl have high requirement e. Caused by TB drugs ii. Abnormal Bone marrow function 1. Aplastic a. Pancytopemia b. Aquired i. Drug induced/BM damage c. Congentital form (fanconis anemia)

d. 12hrs after B12 injection i. normoblastic 6. Shilling test (Historical test) a. Saturate receptors with B12 injection b. Oral dose tagged with isotope c. Measure in 24hour urine i. Less than 7.5% in urine and impaired d. Repeat with intrinsic factor Diphyullbothrium latum 1. Fish tape work and competes for B12 2. Stool exam Malabsorbtion 1. Gluten intolerance 2. steaterrhea gastrectomy chronic liver disease 1. metabolize stores diet

i. Recessive ii. Rare iii. Decreased DNA repair iv. AML v. 30-40 year life span 2. Myelophthisic a. Invasion of bone marrow by tumor, TB, Leukemia 3. Siderablastic a. Refractory (defect in utilization) i. Iron in tissues b. Common in 50-75 year olds c. Normochromic hypochromic d. Terminate in leukemia e. Associated with toxins like lead poising iii. Secondary to chronic disorders 1. Long term inflammation disorders a. Impaired iron metabolism b. Does not stimulate ERP c. Macrophage and lyph activation i. Cytokines 1. Decreased ERP with increased destruction 2. Renal insufficiency a. Less released from kidneys 3. Chronic liver disease a. Iron/B12/folic acid stored b. Lipid production for membranes c. Sequester cells d. Enzymes needed to metabloze B12/Folic acid c. Intracorpuscular defects i. Abnormal membrane function (SEE MEMBRANE DEFECTS) 1. Hereditary spherocytosis 2. Ovalocytes 3. Stomatocytes 4. acanthocytes ii. Enzyme deficiency 1. G-6PD a. 11% of black males b. susceptible to oxidizing drugs c. Hb precipitates as Heinz body i. Supra vital stain d. Sex linked deficiency with X chromosome 2. Pyruvate kinase a. Generates 2 ATP b. Recessive

c. Increased destruction by lack of energy for sodium pumps in spleen d. Varied severity iii. Hemaglobinopthay 1. Qualitative a. Abnormal Hemaglobin complex i. Carboxyhemoglobin 1. CO2 binds more rapidly and firmly than oxygen 2. Cherry red blood 3. Treat with hyper baric chamber 4. Normal 1-3% 5. Syptoms at 20-30% 6. Death at 40% ii. Methhemoglobin 1. Oxidized Fe3+ 2. Brown color blood 3. Treatment with reducing substances a. New methy b. Ascorbic acid 4. Congenital and acquired iii. Sulfhemaglobin 1. Sulfur attached which is permanent 2. Lavender blood 3. Wont be measured 4. Need transfusion b. Abnormal hemoglobin change in amino acids i. Hemoglobin S 1. General a. Sickle cell anemia b. Decreased oxygen makes long rigid rods c. 2 alpha 2 beta with valine at position 7 d. infections the leading cause of death e. Trait (8-10%) i. Protection against malaria ii. Make alpha 1, alpha 2, F and S iii. 20-40% is S rest is A1

iv. no symptoms or anemia v. only extreme conditions can cause syptoms f. Disease(0.3-1.3%) i. Have alpha 1, F, and S ii. 15-20 red cell life iii. increased HbF iv. targets,ovalocytes, Drepanocytes v. diagnosis with electrophoresis 2. testing a. sodium metabisulfate with blood on slide sealed check 2 times look for sickling i. doesnt separate SS or SA b. solubility i. read with color after lysis and reducing ii. not qualitative c. electrophoresis 3. treatment a. can cause syptoms so only treat with crisis b. painkillers and oxygen c. vasodilators d. blood transfusion e. hydroxyurea i. increases Hb F f. BM transplant g. Gene therapy ii. Hemoglobin C 1. Homo is rare a. Similer to sickle cell b. Moderate anemia c. Predom in blacks d. Intraerythrocytic crystals i. Not oxygen dependent e. 100%target cells 2. hetero

iii. iv.

v.

vi.

2. Quantitative a. Hereditary persistence of Hemoglobin F i. By 3 years should be at adult level of Hb F ii. Homo 1. Almost all Hb F iii. Hetero 1. 15-35% Hb F iv. Kleihauer betke test 1. Citrate phosphate buffer 2. Counter stain with eosin 3. Solid color is HbF 4. Controls a. Normal PB i. All ghost cells b. Cord blood i. Some ghost some solid ii. Baby and mom blood c. HPHbF i. Equal F in all cells

a. asymptomatic but increased target cells Hemoglobin D 1. Increased targets asyptomatic Hemoglobin E 1. Homo a. Mild anemia b. Microcytes c. Targets 2. Hetero a. asyptomatic Hemoglobin SC 1. No beta chains 2. Less serious than SS 3. Washington monument projections 4. Positive sickle prep 5. Electrophoresis a. S,C,F A2 Hemoglobin SD 1. No Alpha 1 2. Moderate anemia 3. Sickle cell prep positive 4. Travel together on electrophoresis have to change buffer and gel

d. Thal i. Unequal F in cells 5. Alkali denaturation test of singer a. Read absorb of Hb solution b. Add KOH c. Read again d. HbF more readily oxidized b. Thalassemia i. General 1. Heme is same globin chain decressed 2. Microcytic hypochromic ii. Alpha 1. Phillipines/SE asia (25%) 2. 4 genes 3. Major a. --/-b. incompatible with life c. predominance of Hb Barts i. no oxygen to tissue 4. intermediate a. --/-A b. hemoglobin H disease i. 4 Beta chains ii. high affinity for oxygen iii. denatures easy c. testing i. supra vital stain ii. retic count at 10,20,1hr,24hr iii. denature of hb 5. minor a. A/-A b. relatively mild c. decreased A1 d. normal A2,F e. 5-15% barts f. some Hb H 6. silent carrier a. AA/Ai. 25% African Americans iii. Beta 1. Italian/greek 2. 5-10%

8. Polycythemia a. Absolute i. Primary 1. Greater than 5.7 million female 2. Greater than 6.4 million male 3. Cause a. JAK 2 mutation i. Codes for mutated protein kinase

3. 2 genes 4. Major (codeys anemia) a. 2 possible genes b. B+/B+ i. 30% A1 c. B+/B0 i. Mod to sever ii. 10% A1 iii. fatal in childhood d. B0/B0 i. No normal A1 ii. Fatal in childhood e. Symptoms i. Microcytic/hypocro mic ii. Increased A2,F iii. Decreased A1 iv. Excess alpha chains make Heinz bodies 5. Minor a. B+/B i. Mild b. B0/B i. Mild to moderate ii. Sometimes called intermediate c. Refractory to iron d. Double RBCC e. Increased A2 f. Normal F c. Hemoglobin S thalassemia i. More S than A1 ii. B0/S no A1 iii. Syptoms of both d. Hemoglobin C thalassemia i. Symptoms of both ii. Mostly C, increased F decreased A1, normal A2

ii. Activates ERP receptor iii. Increased Bcl-XL 1. Anti aprotic protien 2. Cells dont die 4. Stem cell is proliferative 5. 8-10 million with mutation 6. diagnosed 50-60 7. men more common 8. extra medullary hematopoiesis 9. heart overworked 10. cant predict if they will clot or bleed 11. decreased size and content in red cells 12. terminate in leukemia 13. therapy a. hydroxuria b. bisulfan c. phosphate 32 d. phlebotomy e. can live 20+ years ii. Secondary 1. Increased red cells 2. Causes a. Hypoxia b. Pulmonary disease c. Heart dieses d. Smoking e. Tumor near ERP tissues f. Congential adrenal hyperplasia iii. Familial 1. Only red cells 2. Treat with phlebotomy 3. Associtated with abnormal Hb 4. 23 DPG inheratied b. Relative i. Decreased plasma ii. Shock,dehydration 9. Luekocyte a. General i. Normal 1. 5-10000/cumm 2. percentages a. 40-75 segs b. 20-45 lyphs c. 2-10 mono d. 0-10 stabs e. 1-6 eos

ii. live in 1. BM,lungs,liver,spleen 2. Lyphs in throid,thymus,nodes,spleen b. Neutrophils i. Function 1. Motile 2. First line against bacterial infections a. Attracted by chemo taxis 3. Have lysosomes with protolytic enzymes 4. Pyrogens cause fever ii. maturation 1. CD34 2. GM CFU 3. Myeloblst 4. Promyelocyte 5. Myelocyte a. Specific granuals 6. Metamylocyte a. Kidney shaped 7. Band 8. Seg c. Basophils i. Fuction 1. Similer to tissue mast cell 2. Receptors for IgE a. Have cytokines for IgE synthesis by B lyphs b. Involved in IgE mediated immune reactions c. Seen is asthma 3. Granuals have histamine a. Increase gastric secretions 4. Chemotaxic factors bring eos, other WBC ii. maturation d. Eosinophils i. Function 1. Sluggishly motile 2. Histamases a. Anti histimines 3. Vasoconstrictor 4. Allergic reactions 5. Defend against parasites 6. Migrate to places with external exposure 7. Dependent on hormone control 8. Thorn test a. Give ACTH b. Normal should have decreased by 50%

f. 0-3 baso

ii. maturation e. Monocytes i. Function 1. Called a macrophage in a tissue 2. Phagocytic but slower than segs 3. Clean up crew 4. MPS 5. Attracted by chemotaxis a. T cells put migration inhabition factor i. Keeps monos there ii. Injest and kill microorganisums 1. Esp intracellular 6. Lipases in granuals break down lipids 7. Process antigens and present to lyphocytes ii. Maturation 1. CD34 2. GM CFU 3. Monoblast a. No granuals 4. Promonoblast a. Dust like granuals 5. monocyte f. Lymphocytes i. Types 1. Antigen receptors on cell surfaces 2. T cell (60%-80% of blood lymphs) a. Made in thymus b. Look same after activation c. T4 i. Helper cell d. T8 i. Suppressor cell ii. T Cytotoxic cells 1. Recognize and destroy virus infected cells 3. B cell (15-30% of blood lymphs) a. Made in bone marrow b. Look different after activation c. IgM or IgD suface antigens 4. Natural killer cells a. 10% b. virally infected and tumor cells c. dont need sensitization d. sheep red cells will surround these called rosette 5. Activated lymph a. Associated with viruses look diffrent

ii. Maturation 1. CD34 2. Lyphoid stem cell 3. Lymphoblast 4. Prolymphocyte 5. Lymphocyte 10. Leukocyte variation a. Increased number of cell/cells i. Leukoid reaction 1. Secondary to other cause with an increase in WBC 2. Transient 3. Difference between this and CGL/CML a. NAP test b. Normal i. 15-150 c. Leukoid reaction i. increased d. CML/CGL i. 0-15 4. Stress increases WBCC ii. Neutrophilia 1. Bacterial infection 2. Inflammation 3. Tissue damage 4. C-AML/C-AGL iii. Lymphocytosis 1. Viral infection 2. Whooping cough 3. Infectious lymphocytosis 4. Infectious mononucleosis 5. Secondary stage of syphilis 6. A-CLL iv. Monocytosis 1. Reactivation of TB 2. Recovery of acute infection 3. A-CMonL v. Eosinophilia 1. Acute allergic disorder 2. Parasitic infection 3. Infectious diseases with rashes 4. Copper poising 5. Black widow spider bite vi. Basophilia 1. Whooping cough 2. Allergic reactions 3. CGL

b. Decreased number of cell/cells i. Neutropenia 1. Long term infection 2. BM damage 3. Aleukemic leukemia a. BM full but stuck there ii. Lymphopenia 1. Radiation poisning 2. Excess corticosteroids from adrenal glands on kidneys a. Lysis lymphoid tissue 3. Cushing syndrome a. Overactive adrenal corticoid function iii. Eosinopenia 1. Stress, epinephrine from medulla and glucorticoids a. Cushings 2. Shock a. Major surgery b. Electric shock 3. Severe burns and blood loss 4. Thorn test c. Abnormal neutrophils i. Hyper segmented 1. Greater than 6 lobes 2. Pernious anemia 3. Undritz anomaly a. Giant neutrophilic leukocytosis ii. Hyposegmented 1. Less than 2 lobes 2. Pueglur huet anomaly a. Dom but all normal 3. Acquired a. Prequel to CGL or Mylofibrosis iii. Toxic granulation 1. From hurried maturation 2. Long standing infection 3. Drug reactions iv. Dohle bodies 1. Patches of blue 2. Seen with toxic granulation 3. Residual RNA d. Anomalies/syndroms/diseases i. May-hegglin 1. Dom 2. Giant platlets 3. Thrombocytopenia a. Normal function

4. 25% have tendency to bleed ii. Alder-rielly 1. Recessive 2. Giant granules 3. Missing enzyme to degrade a. Mucopolysaccharides 4. Related with Gargoylism 5. Death before age 10 iii. Chedike- hagiashi 1. Recessive 2. Gray green inclusion bodies in segs 3. Red purple in stabs, lyphs, monos 4. Abnormal lysosomes 5. Albinism 6. Susceptible to pyrogenic infection 7. Decreased platelet adhesion iv. Lipid enzyme deficiencys 1. Gauchers a. Resessive b. Massive splenomegaly c. Yellow brown skin d. Gauchers cells i. Contain glucocerebrsides e. Adult i. Most commen ii. Live 15-30 years after f. Infant i. Retared development ii. CNS problems g. Juvenile i. Neurological problems ii. Chronic h. Associated with hip replacement i. Treat with enzyme replacement 2. Neimen-pick a. Recessive b. Only infants c. Deficiency of sphingomyelinase d. Honecomb e. Cherry red spot on eye f. Death by 3 years old 3. Tay sachs a. Recessive b. Ashkenazi jews c. Affects CNS d. Missing enzyme hexosaminidase A

e. Increased Gangliosides f. Foamy cytoplasm g. Death by 3-4 years e. Infectious virus i. Infectious mononucleosis 1. General a. Acute b. Caused by epstien barr virus c. Children and young adults 2. Clinical a. 80% have fever b. swollen neck lyph nodes c. sore throut d. gradual syptoms e. splenomegaly f. hepatomegaly g. rash h. jaundice i. liver enzymes increased 3. Lab a. Paul bunnell test i. Doesnt show heterophile AB b. Davidson differential i. Guinea pig antigen 1. Absorbs Foresman ii. Beef antigen 1. Absorbs IM iii. Sheep red cells 1. Agglutination c. 2 spot test i. horse kidney 1. absorbs foresman ii. beef rbc stroma 1. absorbs IM iii. horse red cells 1. agglutination d. 1 spot test i. GP kidney and horse cells ii. Agglutination is positive for IM e. Can cause i. Viral hep ii. Burkilts lymphoma iii. CA perinasal sinuses 4. Cytological a. Lots of reactive lyphs b. Increased lyphs slight mono increase

ii. Infectious lymphocytosis 1. General a. Children less than 10 b. Flu like c. 12-20 day incubation d. 100,000 white count e. small mature with no smudge cells f. virus i. 2 adenovirus and enterovirus (coxsackie type A) g. absence of Blasts in Bone marrow h. homogeneity f. Immune Antibody related diseases i. Lupus Erthematasis 1. General a. Auto immune against nucleoproteins i. IgG b. Stable at room temp and have to heat to 65C to destroy c. LE cells i. Phagocyte engulfs cell whos nucleous is homogenious glob ii. Dependent on complement iii. Women of child bearing age d. Discoid i. Facial dermis ii. Aggravated by sun iii. Topical steroid treatment iv. Negative LE prep e. Systemic i. Whole body ii. Need to rule out Rheumatoid arthritis iii. Problems 1. Nephritis 2. Inflammation of the three linings of heart 3. Fluid in lungs a. Commen in men 4. Neurological problems 5. Inflamtion of intestines 6. Low grade fever iv. Lab 1. Pancytopenia 2. Decreased a. Albumin b. Alpha 2

3. 4. 5. 6. 7. 8.

c. Beta d. Alpha 1 Increased a. Gamma globulins b. Cryoglobulins Complement levels decreased Positive for LE prep in 80% Tart cells are normally found Rapid latex test ANA fluorescent antibody test a. Only one only for lupus

ii. Plasma cell disorders 1. General a. Maturation i. PlasmaBlast 1. No peri-nuclear zone ii. Proplasmacyte 1. Peri-nuclear zone 2. Corser chromatin iii. Plasma cell 1. Perinuclear zone 2. Finer chromatin b. Antibodies i. IgG 1. Smallest 2. Pass through placenta ii. IgM 1. Largest 2. Cold antibodies iii. IgD 1. B lyph surface receptors iv. IgE 1. Allergic responce v. IgA 1. secretions 2. Multiple myeloma a. Plasma cell leukemia b. Ab to nothing c. Invades BM cause fracturing i. Sheets of plasma cells in bone marrow ii. Normal is 0-2% d. Invades tissues as well e. Hyperprotienemia i. Susceptible to infection f. Proteinuria i. Bence-jones = light chains

g. Hypercalcemia i. From breaking down bone h. Prognosis i. 2-4 years i. 60 year old males i. do spot xrays and BM transplant ii. give calcium 3. Waldenstroms macroglobulinemia a. Same ad multiple myeloma except no bone degradation and it is IgM which clumps platelets b. Sia water test for screening c. See electrophoresis for IgM d. Treat with chemo 4. General ones a. Monoclonal gammopathy i. Replication of cloning plasma cells b. Polyclonol i. Many different kinds ii. Beta and gamma are increased g. Leukemia i. AGL/AML 1. Most severe 15-20 yrs 2. Greater than 30% blasts in BM which is diagnostic 3. Usually 30-60% 4. Monocytes and RBC and platelets are effected 5. Auer rods a. Abnormal fusion of lysosomes i. 100% chance NLL 6. cause sudden hemorage cause they get stuck in small blood vessels 7. 5-20% cured ii. CGL/CML 1. 20-45 2. splenomegaly 3. all kinds of granulocytes 4. NAP test a. Less than 15 5. Philidelphia chromosome 6. Blast crisis a. 2/3 myloblasts b. 1/3 lymphoblasts c. less than 2 months before death if not resolved i. interferon iii. Pro granulacytic 1. Non specific granuals

iv. v. vi.

vii.

viii.

ix. x. xi.

xii. xiii.

2. DIC, fragile cells than break up and start DIC Monocytic 1. Acute more popular Myelomonocytic 1. Progress to pure myelocytic 2. Skin and gum involment Acute Lymphocytic 1. 50-90% lypho blasts 2. sudden onst 3. 60% cured 4. greater than 90% in remission 5. can see in CSF and meninges Chronic lyphocytic 1. Middle to old age 2. Enlarged lyph nodes 3. Lots of smudge cells 4. Hyper mature lymphs 5. Life expectancy is almost normal 6. Some develop autoimmune HA Erythrocytic 1. Acute disorder that involves RBC and granulocytes 2. Myeloproliferative disorder 3. Three stages a. Erythroid b. Polyphasic c. Myloid AML 4. Diagnosis a. More than 50 of bone marrow is NRBC with 30% myeloblasts b. Multi nucleated red cells c. Less than a year of survival Megakaryocytic 1. Acute greater than 30% megakaryoblasts Stem cell leukemia 1. Non existant cause we have cell markers Hairy cell 1. Men over 40 2. Massive spleenomegally 3. B lyph has frayed edges 4. Positive trap test 5. Chronic with long rremissions Myeloproliferative disorder 1. Can effect any cell 2. Associated with extramedullary hematopoiesis Mylofibrosis with myloid metaplasia 1. Bone marrow replaced with fibrotic tissue

h. Platlets i. General 1. Less than 1% of bone marrow is megakaryocytes ii. Maturation 1. Megakaryoblast a. Scanty cytoplasm 2. Promegakarocyte a. Clumped cytoplasm some granular 3. Megakaryocyte a. Loblular nucleous b. Close to sinus iii. Contents 1. Thromasthenin a. Causes clot retraction 2. Ca++ a. Important in coagulation 3. Glycogen a. Storage form of glucose 4. ATP/ADP a. Energy and aggregation 5. Epinephrine/serotoinin a. Aggregation/vasoconstriction 6. PF3 for intrinsic coagulation 7. PF4 for platlete polymerization 8. Enzymes that meld platles together iv. Function 1. Stick to damged blood vessels a. Adhesion 2. Stick to eachother a. Aggregation 3. Maintain vascular integrity 4. Platelet plug a. Connective tissue acts as support i. Contains 1. Elastin fibers, smooth muscle, involuntary vasoconstictants b. Basement membrane i. Fine network of fibrils 1. Contain a. Collegen i. Activates adhesion and aggregation c. Endothielial cells i. Inert shield between layers

2. Massive spleenomegally 3. Abnormal platlets

v. Adhesion 1. Prefer to stick to collegen a. Factor 8 acts as a bridge between collegen b. Disease i. Willebrands 1. Cant stick to collegen 2. Shape change a. Disk to sphere to spiney i. Shrink and relese contents 3. Exogenous ADP causes primary aggregation 4. Release reaction 5. Secondary aggregation a. Releases Ca++ and enzymes 6. Viscus metamorphosis 7. Primary hemostatic plug a. PF3 starts intrinis coagulation 8. Intrinsic coagulation a. Fibrin to clot 9. Vasoconstriction from serotonin vi. Testing 1. Estimates 2. Platlet count 3. Bleeding time a. Depends on capillary function 4. Tourniquet test a. Depended on blood vessel function b. Number c. Or function 5. Clot retraction a. Normal number and thrombasthein 6. Platlet aggregation a. With spec 7. Platlet adhesiveness a. Mix glass beads after first count and count again should be less

d. Lining endothelial cells is prostacyclin i. Platlet inhibitor