Define the Following Terms of HEMATOLOGY-PATHOLOGY Flashcards

*Sickle cell screen (Induce sickling)
The patient most likely has sickle cell trait(Aa), which causes recurrent microscopic hematuria.In sickle cell trait, the percentage of sickle hemoglobin is 40% to 45%, and the remainder of hemoglobin is hemoglobin A.*There are no sickle cells in the peripheral smear in sickle cell trait,therefore, a sickle cell screen is required to induce sickling of RBCs containing sickle hemoglobin. However, the O2 tension in the renal medulla is low enough to induce sickling of RBCs in the peritubular capillaries. This causes microinfarctions in the renal medulla leading to hematuria.*There is also the potential for renal papillary necrosis and subsequent loss of both concentration and dilution.

*Chronic alcohol>Cirrhosis>Portal hypertension>Congestive splenomegaly with too much blood getting stuck in spleen>Secondary Hypersplenism(Portal hypretension is MCC of hypersplenism)>Spleen sequesters RBCs,WBCs,Platelets>Too much of it>Pancytopenia.<Contrast this with Hyposplenia(For example after autosplenectomy in Sickle cell patient or After surgical removal of spleen) where you would see Basophillic Nuclear remnants(DNA remnant) in RBCs known as Howell-Jolly bodies.BOTH of these have been tested so know them

*She likely has Von willebrand disease-Most common inherited bleeding diathesis, it is autosomal dominant condition that involves lack of Factor 8, so PTT can be prolonged(In this patient it is not, remember that oral contraceptives increase release of vWF and factor 8, so it can be partially responsible), however the ONLY sign of this disease can be Prolonged bleeding time.

*He is likely hetezygote that underproduces B-globin, but usually they still produce enough to usually to be asymptomatic.But most important thing here was HbA2 (> 3.5%) on electrophoresis(He had 5%), which is a complete giveaway for Thalassemia MINOR.

*ONLY PTT Will be increased, because patient likely has Hemophilia A-X linked condition where Factor VIII is deficient. lack of thrombin from the VIII:C deficiency prevented conversion of the fibrinogen to fibrin, producing a stable platelet thrombus held together by fibrin. When the patient rinsed his mouth out with lukewarm water, the temporary platelet plugs were dislodged causing serious bleeding..For mild hemophilia like this give Desmopression>Increases release of Factor VIII from ENDOTHELIAL cells.

*ANSWER was translocation between 2-8(Involves KAPPA LIGHT chain gene locus)

*platelet count normal, bleeding time normal, PT increased, PTT increased,both PT and PTT are prolonged because factors 2 and 10 are affected by warfarin(those are vitamin K dependent coagulation factors), yet more factors 2,7,9,10 are all affected by warfarin as they all are vitamin K dependent, factor 7 is only involved in Extrinsic pathway and generally we use PT to monitor warfarin(very testable fact), however remember that even though PT changes more, PTT is affected by warfarin too.

*Patient has primary fibrinolysis, which may occur in radical prostate surgery.In this setting, urokinase is released from the tissue causing activation of plasminogen and the release of plasmin.Plasmin degrades multiple coagulation factors (e.g., V, VIII, fibrinogen)>>This increases the PT and PTT, because factors V and VIII are in the final common pathway.The degradation products of fibrinogen interfere with platelet aggregation causing an increase in the bleeding time.The platelet count is not affected.*Test for fibrinogen degradation products is positive, however, d-dimer test is negative because fibrin clots are not present in primary fibrinolysis.all in all :platelet count normal, bleeding time increased, PT increased, PTT increased.

*platelet count normal, bleeding time increased, PT normal, PTT increased.Remember vWF helps platelets to adhere to damaged endothelial cells.(So lack of vWF>Increased bleeding time)*It also normally prevents degradation of factor8, so deficiency of vWF>deficiency of factor 8>Prolongation of PT.*Contraceptives increase factor 8 release and thus removes most of her symptoms.

*DECREASED HEMOGLOBIN CONCENTRATION.

It will give similar manifestations as WARFARIN overdose, basically you'll get BLEEDING DIATHESIS once old vitamin K dependent coagulation factors(2,7,9,10) are depleted(around 48 hours).
EMERGENCY treatment is FRESH FROZEN PLASMA(DIRECTLY give clotting factors/proteins) vitamin K will need DAYS to exert its effects.

Because it contains ANTIthrombin 3.

PROmyelocytes vs Myelocytes

Retention of RNA not dna.*this retention of RNA is due to LEAD mediated INHIBITION Of 5" NucleotidASE activity.

*DEATH of RBC PRECURSORS in Bone marrow>Ineffective hematopoiesis, this along with EXTRAvascular hemolysis is due to PRECIPITATION of UNpaired Alpha chains within RBC.(Unpaired cause no B-chain synthesis).vs
Most DIRECT effect of decreased B-chain synthesis is mIcrocytic/hypOchromic anemia.