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68 Cards in this Set
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Reactive Leukocytosis |
WBC count of 50K+
Increased Alakaline Phosphatase |
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Chronic Myeloginous Leukemia (CML) |
High number of Eosinophils and Basophils
High white count, but lower than Leukocytosis
9-22 translocation forms the BCR-ABL gene
May develop into a Blast Crisis which leads to leukemia |
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Acute Lymphocytic Leukemia (ALL) |
90% of leukemia cases for children, often appears at age 4. Many are cured. Down syndrome patients have a 15x risk of ALL
TdT: Terminal deoxynucleotidal transferase test; tells if the cells are lymphoblasts, but it dose no distinguishe between B or T cells. Look for CD10
L1 = Normal ALL L2 = Indented Nucleus L3 = Burkit's Lymphoma |
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Chronic Lymphocytic Leukemia (CLL) |
The clonal proliferation of B-cells
Leads to anemia, thrombocytopenia, and a marrow made up of malignant lymphocytes.
Only treated after anemia or thrombocytopenia manifest. Early aggresive treatment shortens life expectancy.
Blood smear with lymphocyhtosis (to many lymphs) and many small, mature lymphocytes in a patient over 50. Look for lymphs with nucleolis. Also will have many smudge cells
Check marrow for involvement
Low ZAP-70 = good prognosis
Look for elevated CD19, 20, and 5
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Prolymphocytic Leukemia |
Similar to CLL, but with more immature (pro)lymphocytes.
Dose not respond to therapy
Look for markers CD19, 20, and 24
Look for cookie-cutter nucleus. |
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Hairy Cell Leukemia |
Besides their "hairy" appearance, these cells acid phosphatase which is not inhibited by tartarate and are stained with TRAP.
Look for an enlarged spleen and a dry tap on a marrow assperation |
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Burkit's Lymphoma |
A rare sub-type of ALL.
An 8-14 trasnlocation leads to chronic activation of the myc gene which promotes growth
Look for distinct starry sky cytoplasm; darkly colored with big vacules
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Hodgkin's Lymphoma |
Causes the painless enlargement of the neck's lymph nodes.
Typically emerges in teens and young adults
Look for the reed sternberg "owl cell"
High cure chance if treated, but lethal otherwise |
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Esterases |
Specific = Granulocytes
Non-Specific = Monocytes (CD11b) |
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P. vera |
The most common myeloproliferative disorder
Increased platelets and leukocytes, with a massivley increased hematocrit
Diagnose: Increased RBC mass and JAK-2 Kinase |
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Multiple Myeloma |
Look for Roulex, RBC clumping and stacking due to excessive Paraprotein.
Increased Platelets, and Increased B-lymphocytes |
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Myelodisplasia |
Causes macrocytosis, hypersegmentation, pseudo Pelger Heuet, hyper/hypogranulation of white cells.
RARS has the best diagnosis, look for ringed sidroblasts. They have a ring of iron inclusions 3/4 around the nucleous
Can become AML |
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Acute Myelocytic Leukemia (AML) |
Has a high mortality rate, but dose not appear in those over 40.
Associated with Down's syndrome, benzene exposure, radiation exposure, and chemo therapy.
Causes myelodisplasia, myelorfirbosis, P. vera, ET, CML and aplastic anemia
Auer rods!!! |
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Acute Promyelocytic Leukemia (APL)(M3) |
15-17 translocation = Leukemia M3
Highly Lethal
Cytoplasmic granules contain thromboplastin which leads to DIC
RAR & PML genes fuse to create PML-RARA which stops white cell differentiation
Treated with All Trans Retinoic Acid (ATRA)
Stains with Sudom Black
Presents with auer rods which promote coagulation.
Treat with a massive dose of vitamin A |
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Acute Myelomonocytic Leukemia (M4) |
CD11b, CD13, CD33
Stains with Sudom black and non-specific esterase
Half way between a myelocyte and a monocyte, and has features of both |
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Chediak-Higashi |
Caused by a lack of dense (delta) platelet granules.
Autosomal recessive.
Symptoms: Partial oculocutanious albinism (albino), Pancytopenia, Frequen pyogenic bacteria infections, Giant lysosomal granules, Dense (delta) granule defficiency, Hemorrhage, Lymphocytic proliferation in the liver and spleen, Macrophage accumulation in tissues.
Has an accelerated phase with pancytopenia and sever hemorrhaging due to a loss of platelets. |
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May-Hagglin |
A rare autosomal dominant condition where thrombocytopenia occurs in half the sufferers along side giant platelets.
This is caused by a mutation of the MYH9 gene for the cytoskeleton.
Look for Dohel bodies |
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Wiskott-Aldrich |
Caused by a lack of dense platelet granules.
X-linked recessive.
Symptoms: Sever eczeam (itchy skin rash), reccurent infections particulary with pneumococcal (sepsis) due to not making antibodies to polycharides. Bloody Diarrhea
Thrombocytopenia due to poor multiplication, increased sequestering and destruction
Poor platelet aggregation with collagen and epithilial cells. |
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Bernard-Soulier |
Also called giant platelet syndrome.
Autosomal recessive mutation causes missing or deformed GP-Ib/IX/V
Heterozygotes are functionally well
Symptoms: First identified in childhood or infancy. Causes Mild to sever hemorrhage with Ecchymoses and bleeding gums. Enlarged platelets, thrombocytopenia, and lowered platelet survival. No response to ristocetin, and reduced response to thrombin. |
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Lymphoma |
B-cell from the terminal center of a lymph node become cancerous.
Reed-Sternberg cells
Low grade lymphomas are more likely to involve the marrow and have a lower survival chance
These B-cells do not undergo apoptosis
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Mycosis Fungoides |
Common form of cuntaneous T-cell Lymphoma
Progresses to Leukemia once it enters the blood, look for markers CD1, 2, 3, 4
Causded by a malfunction in CD4 helper cells found in the skin |
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Myelodysplastic |
??? |
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Hemoglobin by chain |
Embryonic: Gower 1 = e4 or z2e2, Gower 2 = a2e2, Portland = z2b2
Fetal = a2y2 (less than 2%)
Adult: A1 = a2b2 (95%), A2 = a2d2 (2%)
Glucose added to n-terminal of HbA on 6% of protein, making HbAc1 |
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Bad Hemoglobins |
Methemoglobin: Iron becomes F3+, it's oxidzided state. Dose not bind Oxygen well and is a brown blue color. Causes a left shif. Caused by exposure to oxidants, or by a genetic defect which causes the production of M-Hb
Sulfhemoglobin: irreversibly oxidizes hemoglobin. Results in a greenish color and cyanosis.
Carboxyhemoglobin: Carbon monoxide has a super high affinity for hemoglobin ans will out compete O2 for the binding site and never let go. |
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Iron deficiency anemia Vs Iron storage (inherited) anemia |
IDA: Total iron binding capacity (Norm): 300-360ug/dL
Serum Ferratin is a good way to check total iron stores. This drops quickly in early IDA
Causes microcytosis and hypochromicness along with target cells and elitptocytosis
MCV down, MCHC down, MCH down |
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CBC standard results! |
RBC# HCT = 47% HBG = 13-17 mg/dL MCH = 27-31 picograms MCHC = 32-36 g/dL MCV = 80-100Fl RDW = 11.5-14.5% TIBC = 300-360ug/dL Serum Ferratin = 100 +\- 60ug/L |
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Reticulocytes |
Normal = .5-1.5% or 25-75x10^9/L
RPI = (Retic% x Hct) /45 |
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Pernicious Anemia |
An autoimmune reaction to the peritle cells of the GI tract. These cells produce intrinsic factor which we need to absorb B12.
This condition presents very simmilarly to megaloblastic anemia, with Macocythic RBC and hypersegemented neutrophils. |
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Sideroblastic Anemia |
??? |
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Erythropoetin |
??? |
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Thalassemia |
Normal RDW |
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chronic inflamation anemia |
??? |
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Porphyria |
Delta-ALAS and ferrochelatase enzymes are disrupted and prevent the proper production of heme by the mitochondria |
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Hereditary Hemachromotosis |
To much iron is taken up by the body and stored in organ tissues to the point of toxicity.
This can be detected with a transferrin satruation test, or by looking for defects in the HFE gene. |
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Megaloblastic anemia |
Caused by a lack of DNA synthesis and cell division due to a deficiency in B12 and/or Folate
Hypersegemented neutrophils are a good indicator of this type of anemia. |
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RBC inclusions |
Holly-Jolly bodies contain RNA, look for a single big dot
Basophilic stippling is a good indicator of lead poisioning
Hynze Body are small inclusions made of denatured hemoglobin
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Extrinsic Vs Intrinsic hemolysis |
Intrinsic hemolysis occurs withing the blood vessels, and the released hemoglobin is taken up by haptoglobin, resulting in a lowered concentration
Extrinsic hemolysis is when RBCs are destroyed somewhere other than the blood vessels. A good example of this would be RBCs eaten be macrophages. Their hemoglobin is never released, so Haptoglobin levels are not chnaged. |
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Billyrubin Vs Uncongigated Billyrubin |
Bilirubin is responsible for giving urine it's yellow color.
Uncongigated bilirubin is produced by the macrophages of the spleen after the eat RBCs and breaks down their heme. Since this form is not water soluable, it has to be bound to albumin in order to be sent to the liver.
In the liver bilirubin is conjugated with glucoronic acid, making it water soluble"direct" bilirubin. Most of this is used in the bile. After being excreted as bile the bilirubin enters the lower intestines where bacteria de-conjugate it, creating urobilinogen, which is oxidized to urobilin. Some of this is excreted in the urine, making it yellow. |
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Beta-thallisymia |
Unpaired alpha chains precipitate in the RBC, causing it's destruction in the marrow or the peripheral blood. Babies are asymptomatic until they switch from fetal to adult hemoglobin after 4-6months |
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Alpha-Thallisymia |
Baby overproduces gama chains which precipitate as the cell ages. These cells are removed by the spleen. |
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G6PD deficiency |
Causes acute hemolytic anemia during infections, in response to some antibiotics or if favabeans are eaten
Associated with hynze bodies |
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Hemoglobin E |
Swaps a Glu for a Lys
Proper hemoglobin polymerization dose not occur.
Leads to mild anemia with microcytic RBC and target cells
HbEE (90% HbE) resembles thallsymia or IDA, smaller RBC
HbAE (35% HbE) |
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Hemoglobin H |
--/-a 3 bad gene. Unpaired B-chains for tetramers of HbH
Leads to mild,but chronic hemolytic anemia
RBCs are microcytic, hypochromic and show poikilocytosis (odd shapes)
Retic = 5-10%!!!
Replaces Hb Bart at birth
HbH = 5-40% of all Hb in adults |
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Hemoglobin Bart's |
--/-- no good alphas
Hb made up of only y chains, found in alpha thallysimia major.
Hb Bart's dose not give up bound O2, baby dies of anemia before or soon after birth. |
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Thrombotic Thrombocytic Purpura |
Casued by the spontanious aggregation of platelets and the activation of coagulation in the small blood vessels causing platelets to bind vWF and form dangerous microthrombi
These microthrombi can cause serious damage to the kidneys as they attempt to filter them.
Treat with plasampharesis, other wise is fatal |
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DIC test results |
Plt <200
PT >14s
PTT >35s
D-Dimer >500ng/mL
Fibrinogen <200mg/dL
PT and PTT prolonged, also a positive D-Dimer test |
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TTP Test results |
Neither PT or PTT prolonged!
No D-Dimer involved. |
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von Willebrand Factor |
vWF is stored in alpha granules and Weible-Palade bodies of vasculated endophilium.
vWF has 4 sites, the abundnat collagen binding site A3 Domain, the GPIb/IX/V platelet binding site, it's own antigen and the binding site for factor VIII
The antibiotic ristocetin can detect Bernard Soulier Syndrome and small vWF based on whether or not platelets clump to vWF, no clumping is negative. |
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vWF Disease |
Type 1: Quantity issue; 75% of cases, an autosomal dominant scrambling of the vWF gene. vWF and VIII reduced by a variable, but simmilar amount. Causes mild bleeding issues
Type 2: Qualitative; Abnormal functionality, such as being cleaved, Increased Plt affintiy, lowered Plt receptor binding, or no VIII binding site.
Type 3: Quantitative; no vWF produced
Use CBC to rule out thrombocytopenia, then narrow it down with PT, and PTT |
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Hemolytic Anemic Syndrome (HUS) |
Comes with hemolytic anemia and Kidney faliure after E. coli infection.
Bacterial toxins bind to RBCs and endothilial cells of the kidney, which triggers the cells destruction by T-cells or complement. |
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Hereditary Persistance of Fetal Hemoglobin |
Due to a possible mutation in the Beta Cluster, some people produce signifigant amounts of Fetal Hemoglobin past the normal shut off point.
This is a benign condition, but it dose benifit those with sickle cell, fetal hemoglobin won't sickle |
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Aspirin |
Permanently deactives a platelets cyclooxigenase by acytelating it.
This protein is still produced by epithelial cells, so aspirin only lessons platelet activation. |
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Prostacyclin |
This lipid molecule produced by endethelial cells inhibits the activity of platelets. |
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Coagulation inhibitors |
Protein C: binds activated factors V & VIII which are powerfuly pro-coagulant
Factor S: stabalizes protein C
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factor v ledin |
unlike normal factor v, this varient is not deactivated by factor c
PTT has a decreased clotting time |
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PT |
measures factors I (fibrinogin), II (Prothrombin), V, VII, X of the extrinsic (Tissue factor)path way of coagulation.
Monitores warfrin and vitamin K
Activate with Calcium and tissue factor |
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PTT |
Measures Factors I, II, V, VIII, IX, X, XI, XII of the Intrinsic (contact) pathway of coagulation.
Monitores heparin
A prolonged result is an indicator of Lupus anticoagulant.
activated with calcium, phospholipid, and kaloin
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Factor Deficiencies based on PT, and PTT |
V= PT: prolonged, PTT: prolonged
X = PT: prolonged, PTT: prolonged
XII = PT: Unchanged, PTT: Prolonged
Early Liver failure: PT: Prolonged, PTT: Normal
Late Liver Failure: PT: Prolonged, PTT: Prolonged |
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Lupus Anticoagulant |
Lupus Anticoagulant neutralizes the phospholipid used by PTT to activate, resulting in an extremley elongated clot time.
To test for Lupus, perform a mixing study with known normal Patient plasma. If lupus is the cause, then it still will not clot. |
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Grey Platelet syndrome |
Platelets have no alpha granules. The stuff normally inside of them is released into the marrow where it leads to myelofibrosis |
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GPIIb/IIIa |
Binding site on platelets for fibrinogen.
Absent in Glanzmann's Thrombasthenia, which noticably prevents clot retraction. |
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GPIb/IX/V |
Binds vWF
Absent in Bernard Soulier Syndrome |
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Vitamin K |
Adds the gla residues so that clotting factors can bind surfaces using calcium. this is done in the liver.
II, VII, XI, X and anti-coags S, C, Z |
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Factor 13 |
clot stabalizers
oozing wounds without it |
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Thrombin Time |
Measures the activity of Fibrinogen
Clots in about 22 s
Activated with bovine thrombin |
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Russel's viper venom |
Activates factor X in the presence of V and phospholipid
Sensetive to Lupus |
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Spherocytosis |
Genetic moleculer defects to the cytoskeletal proteins, such as Spectrin, Ankyrin, Band 3 and protein 4.2
RBCS are very fragile and lyse easily |
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Elliptocytosis |
A genetic disorder that most commonly causes defects in alpha and beta spectrin, which causes the RBC to loose it's structural integrety as it passes through narrow vessles. |
