platelet disorders part 1

week 8

what are the two categories of platelet disorders?

quantitative and qualitative

what are the quantitative platelet disorders? (8)

May-Hegglin, Bernard sulier, HIT, ITP, TTP, HUS, DIC, ET

which quantitative platelet disorders are congenital?

May-Hegglin, Bernard Soulier

anything less than _____ platelets is a thrombocytopenia

100X10³

how long do platelets live?

7-10 days

the spleen stores _____ of all platelets

1/3

what are the symptoms of mucocutaneous hemorrhage?

petechiae, purpura, ecchymoses, gum bleeding, epistaxis, hematemesis

what three symptoms are associated with primary hemostasis disorders?

petechiae, purpura, ecchymoses

what are the six most common causes of mucocutaneous bleeding?

thrombocytopenia due to cancer treatment, qualitative platelet disorders, vWD, scurvy, telangiectasia, Kasabach-merrit syndrome

what is Kasabach merrit syndrome?

growth of a vascular tumor that eats platelets and clotting factors resulting in thrombocytopenia in newborns

what are the two types of quantitative platelet disorders?

thrombocytopenia and thrombocytosis

in thrombocytopenia _____ is impaired due to ___ platelet count

primary hemostasis, low

what are the three types of thrombocytopenias?

impaired/decreases function production, Destruction, abnormal distribution

what types of things can cause impaired/decreased platelet production?

alcohol use, chemo, B12 deficiency, cancer

what disorders/conditions are due to thrombocytopenia from destruction of platelets?

DIC, HUS, HIT

what causes abnormal platelet distribution?

splenic sequestration

May Hegglin syndrome is a _____ _____ _____ platelet disorder. It causes the formation of ____ platelets and ____ in PMN’s and lymphs. It is the result of a mutated _____ gene.

congenital, thrombocytopenic, quantitative, abnormally large, Dohle bodies, MYH9

HIT or Heparin induced thrombocytopenia is an _____ ______ _______ platelet disorder. IgG antibodies bind to _____ + ______ and cause clots to form. This disorder occurs ____ days after Heparin treatment and platelets will drop _____%

acquired, thrombocytopenic, quantitative, platelets, factor 4, 5-10, >50

ITP in children is an _____ _______ platelet disorder that develops _____ weeks after viral infection. The immune system _____ platelets in the ____ causing _____. symptoms include _____, _____, _____, and a _____ in an otherwise healthy child

acute, thrombocytopenic, quantitative, 1-3, attacks, spleen, thrombocytopenia, bruising, petechiae, nose bleeds, low platelet count

ITP in adults is a _____ ______ platelet disorder. It lasts longer than ____ months and is most common in women over _____ yrs old. Symptoms include ______ and _____. Its caused due to the _____ attacking _____.

chronic, thrombocytopenic, 12, 60, persistent thrombocytopenia, bleeding, immune system, platelets

TTP is a life threatening ______ platelet disorder where abnormal ___ form in small blood vessels due to a severe deficiency of the ______ enzyme which normally breaks ____ down into smaller pieces. Lack of this enzyme causes excessive _____ and _____. Platelet count will be ___ PT and PTT will be ____ and RBVC’s will become ____.

thrombocytopenic, clots, ADAMSTS 13, vWF, Platelet adhesion, aggregation, low, normal, schistocytes

HUS is a rare and life threatening _____ platelet disorder most often seen in _______ with gastrointestinal bacteria that create the _______. The toxins damage the small blood vessels in the _____ causing the creation of _____ and _____. Platelet count will be ____ and the _____ ratio will be high

thrombocytopenic, children, shiga toxin, kidney, small blood clots, schistocytes, low, BUN: creatinine

DIC is a _____ platelet disorder that can form as a secondary condition due to complications like _____. Its where a lot of little ____ form and spread throughout the body using up all of the _______.

thrombocytopenic, sepsis, clots, clotting factors

reactive thrombocytosis is when there are between _________ and _______ platelets. It’s a secondary condition following _______, _______, _______, or ______.

450X10³, 800X10³, inflammation, surgery, trauma, blood loss.

ET or essential thrombocythemia is a ______ platelet disorder caused by ___________ like _____, ______, _______, or _____. The platelet count will be above ______ due to the persistent ________ of ________ due to a ________ issue. Platelets will vary in size and appearance. ______ over ______ yrs old are most likely to have this condition. In order to have ET the mutation ______ must be present.

quantitative, myeloproliferative disorders, ET, PCV, PM, CML, 450X10³, overproduction, platelets, clonal, women, 60, JAK2 CALR MPL

why is thrombocytosis an interference for platelet testing?

difficult to obtain PPP, membrane phospholipids in platelets neutralize LA, secretion of F5 F7 and vWF desensitize PT and aPTT, secretion of P4 from alpha granules neutralizes heparin and shortens aPTT

Glanzmann Thrombasthemia is a _____ platelet disorder that affects platelet ______ and _______. It is caused by a _______ _______ mutation in the genes for _____ and ______ platelet receptors. symptoms include ______, ______, ______, _____, and abnormal _______ tests. platelet count and morphology will be _____.

qualitative, aggregation, clot retraction, autosomal recessive, GP2b, GP3a, easy bleeding, gum bleeding, bruising, nose bleeds, aggregation, normal

Bernard Soulier syndrome is a ______ and a _____ disorder. It is an _____ ______ disorder that causes a deficiency in the _______ platelet receptor. It causes a disorder of platelet ______ as well as _____ platelets and a _______ life span. The disorder has a platelet count between _____ and _____ and is most often diagnosed in _____. symptoms include _______, ________, ________, and a normal ____ and _____.

quantitative, qualitative, autosomal recessive, GP1b-9-5, adhesion, huge, decreased, 30X10³, 200X10³, gum and nose bleeds, can’t bind vWF properly, Ristocetin doesn’t fix it, PT, aPTT

Chediak-Higashi syndrome is a ______ platelet disorder. It causes the abnormal _______ of leukocytes which leads to impaired ______ which causes impaired _______ and an impaired _______ system. This condition causes partial ______, Huge ______ and _____, blue/green _____ in WBC’s and life threatening complications

qualitative, fusion, primary hemostasis, granule secretion, immune system, albinism, platelets, granules, crystals, WBC’s

Asprin’s chemical name is _____ or ____. It is used to treat _____, ____, ____ and to reduce the insidance of another _____, ____, ____ after a first. The therapy is measured via the _____ tests. Asprin inhibits ______ and _____ leading to less platelet _____. It also stops ____. Asprin only effects platelets _____ not platelets _____.

acetylsalycilic acid, ASA, pain, inflammation, fever, MI, stroke, thrombosis, PFA 100, cyclooxygenase, Thromboxane A2, vasoconstriction, to be made, circulating.

what Lab tests are ran to assess platelets for disorders?

PLT count, PLT function, Bleeding time, CT, PFA-100, PLT aggregometry light transmitance, PLT whole blood aggregometry, PLT lumiaggregometry

how is light transmittance PLT aggregometry measured?

light transmittance increases as platelets aggregate

how is whole blood aggregometry measured?

electrical impedance increases as platelets aggregate

how does PLT Lumiaggregometry get measured?

ATP and PLT granule release