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Heme and Lymph Final > DSA: Platelet Disorders > Flashcards

DSA: Platelet Disorders Flashcards

1
Q

thrombocytopenia happens in what two conditions?

A
  • decreased production of platelets

- increased destruction/sequestration of platelets

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2
Q

Thrombocytosis occurs in what two general conditions?

A
  • overproduction of platelets (ET)

- Increased platelet release form marrow/storage areas in response to an inflammatory stimulus (reactive thrombocytosis)

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3
Q

What are the causes that will give us thrombocytopenia due to decreased platelet production?

A
  • TCP due to bone marrow failure
  • bone marrow infiltration
  • chemotherapy and irradiation
  • nutritional deficiencies (folate or B12)
  • cyclic thrombocytopenia
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4
Q

EOD for thrombocytopenia due to bone marrow failure

A
  • may be congenital or acquired

- most congenital marrow failure disorders present in childhood

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5
Q

clinical findings for bone marrow failure

A
  • aplastic anemia

- in MDS, there is cytopenias but the marrow cellularity is not decreased

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6
Q

What is more suggestive of MDS?

A

-macrocytosis, ringed sideroblasts, dysplasia of hematopoietic elements, or cytogenetic abnormalities

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7
Q

What are the causes of thrombocytopenia because of increased platelet destruction?

A
  • Immune thombocytopenia
  • Thrombotic Microangiopathy
  • Heparin-induced thrombocytopenia
  • DIC
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8
Q

EOD for immune thrombocytopenia

A
  • isolated thrombocytopenia
  • assess for any new causative meds and HIV and hep C infections
  • ITP is a diagnosis of exclusion
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9
Q

Clinical findings for ITP

A
  • mucocutaneous bleeding
  • bruising
  • nosebleeds
  • isolated thrombocytopenia
  • infection is excluded
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10
Q

Tx for ITP

A

-if <20,000, treat them, if not, just observe
-short course of corticosteroids and maybe IVIG
-

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11
Q

EOD for thrombotic microangiopathy (TMA)

A
  • Microangiopathic hemolytic anemia and thrombocytopenia, in the absence of another plausible explanation
  • fever, neuro abnormalities, and kidney disease may happen too, but not required for diagnosis
  • kidney dysfunction is more common and more severe in HUS
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12
Q

What diseases do TMA’s include?

A

TTP and HUS fuh sho

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13
Q

What antibodies will TTP have?

A

ADAMTS13

-it normally cleaves vWF

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14
Q

What is HUS usually related to?

A

the shiga-toxin from E. Coli O157:H7

-they usually have diarrhea or something like that

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15
Q

clinical findings in TMA

A

the typical pentad for TTP

-microangiopathic hemolytic anemia, thrombocytopenia, fever, kidney disease, and neuro system abnormalities

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16
Q

Tx of TMA

A

immediate administration of plasma exchange!!!

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17
Q

EOD for Heparin-Induced Thrombocytopenia

A
  • thrombocytopenia within 5-14 days of exposure to heparin
  • decline in baseline platelet count of 50% or greater
  • thrombosis occurs in up to 50% of cases; bleeding is uncommon
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18
Q

What does HIT result from

A

IgG antibodies to heaprin-platelet factor 4 (PF4) and PF2 complexes
-leads to prothrombotic state…. takes up all the platelets

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19
Q

Tx for HIT

A
  • immediately discontinue all forms of heparin
  • do ultrasound to rule out DVT
  • DTI.. direct thrombin inhibitor
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20
Q

What is used for prophylaxis or tx of HIT?

A

Argatroban

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21
Q

What is used for percutaneous coronary intervention

A

Bivalirudin

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22
Q

EOD for Disseminated intravascular coagulation

A
  • frequent cause of thrombocytopenia in hospitalized patients
  • pronlonged activated partial thromboplastin time and prothrombin time
  • thrombocytopenia and decreased fibrinogen levels
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23
Q

Clinical findings of DIC?

A
  • bleeding at multiple sites
  • trousseau syndrome for malignancy related DIC
  • prolonged PT and PTT
  • elevation in D-dimer
  • schistocytes
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24
Q

Tx of DIC

A
  • treat the underlying disorder that’s causing it
  • evacuation of uterus for HELLP
  • trousseau: treat underlying malignancy
  • immediate intiation of chemo for APL (acute promyelocytic leukemia) associate DIC
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25
Q

What is HELLP syndrome

A

form of DIC that sucks a lot of ass

  • hemolysis, elevated liver enzymes, low platelets,
  • peri partum women
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26
Q

Drug induced thrombocytopenia

A

-7 to 14 days after exposure
-just stop…
chemotherapeutic agents do this a lot

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27
Q

Posttransfusion purpura

A
  • rare
  • sudden onset TCP in someone who recently had transfused Red cells
  • multiparous women
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28
Q

von Willebrand disease type 2B

A
  • leads to chronic, mild TCP via abnormal vWF molecule that binds platelets with increased affinity
  • aggregation and clearance
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29
Q

Platelet sequestration

A
  • 1/3 of platelets are sequestered in the spleen

- so, it is probably big in TCP

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30
Q

Pregnancy

A

-expansion of blood volume

31
Q

Infection or sepsis

A

-just treat with antimicrobials

32
Q

Pseudothrombocytopenia

A
  • results from EDTA anticoagulant-induced platelet clumping

- this phenomenon usually disappears when blood is collected in a tube containing citrate anticoagulant

33
Q

Bernard-Soulier syndrome (BSS)

A
  • auto recessive

- reduced or abnormal platelet membrane expression of glycoprotein Ib/IX

34
Q

Glanzmann thrombasthenia

A
  • IIb/IIIa receptors are messed up
  • they bind fibrinogen to vWF
  • auto recessive
35
Q

Storage pool disease

A

defects in release of alpha or dense (delta) platelet granules, or both

36
Q

lab values for BSS

A
  • large platelets
  • prolonged bleeding time
  • addition of normal platelets does the trick
37
Q

labs for Glanzmann

A

-marked impairment of aggregation in response to stimulation with typical agonists

38
Q

Albinism associated storage pool disease

A

defective dense granules in disorders of oculocutaneous albinism such as Hermansky-Pudlak and Chediak-Higashi syndromes

39
Q

non albinism associated storage pool disease

A

-defective dense granules in Ehlers-Danhlos and Wiskott-Aldrich syndromes

40
Q

Quebec platelet disorder

A
  • mild TCP
  • abnormal platelet factor V molecule
  • prolonged bleeding time
  • platelet transfusion doesn’t work here
41
Q

Tx of Qualitative platelet disorders

A
  • transfusion of normal platelets

- can use DDAVP, antifibrinolytic agents, and recombinant huan activated factor VII too

42
Q

EOD for congenital disorders of platelet function

A
  • usually diagnosed in childhood
  • family history is usually positive
  • may be diagnosed in adulthood when there is excessive bleeding
43
Q

Hemophilia A EOD

A

congenital deficiency of coagulation factor VIII

44
Q

Hemophilia B EOD

A

congenital deficiency of coagulation factor IX

45
Q

Hemophilia EOD

A
  • recurrent hemarthroses and arthropathy
  • risk of development of inhibitory antibodies to factor VIII or factor IX
  • in many older patients, infection with HIV or hepatitis C virus from receipt of contaminated blood products
46
Q

Is Hemophilia X-linked or autosomal

A

X-linked recessive!!!

-so, lots of male patients

47
Q

how do we separate severe, mild, and moderate hemophilia?

A
  • <1%
  • > 5%
  • between 1-5%
48
Q

tx of hemophilia

A

plasma-derived or recombinant factor VIII or IX products

-DDAVP for mild kinds

49
Q

What is DDAVP again?

A

desmopressin acetate

-can be used for mild hemophilia

50
Q

vWD EOD

A
  • the most common inherited bleeding disorder

- vWF binds platelets to subendothelial surfaces, aggregates platelets, and prolongs the half-life of factor VIII

51
Q

most common type of vWD?

A

type 1

-quantitative abnormality

52
Q

symptoms of vWD

A

mild to moderate platelet-type bleeding

  • type 3 is severe
  • type 2 is aight and arises in childhood
53
Q

tx of Vwd

A

type 1: DDAVP
type 2: DDAVP, vWF product
Type 3: vWF product!!!

54
Q

factor XI deficiency

A
  • hemophilia C
  • auto recessive
  • Jews
  • FFP is the treatment if XI isn’t available
  • adjunctive aminocaproic acid
55
Q

Which type of vWD is the only one that has RIPA (ristocetin-induced platelet aggregation)?

A

Type 2b

-large multimers dereased or absent

56
Q

What is DIC caused by?

A

excessive tissue factor exposure or release

  • results in decreases in the activity of clotting factors
  • Pt and PTT are pronlonged
57
Q

What is Fondaparinux and how does it work

A
  • it’s an anticoagulant
  • no effective neutralizing agent
  • works to indirectly inhibit factor Xa through binding to antithrombin
  • metabolized by the kidneys so watch out
58
Q

What does Heparin do?

A
  • parenteral anticoagulant
  • binds to antithrombin 3 and inhibits Xa
  • makes thrombin and antithrombin meet up
  • LMWH is cleared by renal and is more predictable
59
Q

What does warfarin do?

A
  • Vit K antagonist
  • inhibits the activity of the Vit K dependent carboxylase that is important for the posttranslational modification of coagulation factors II, VII, IX , and X
60
Q

What is Dabigatran?

A

-a direct acting oral anticoagulant (DOAC)

61
Q

What is Rivaroxaban?

A

-oral direct factor Xa inhibitor

62
Q

When is unfrationated heparin indicated?

A

if concomitant thrombolysis is being considered

63
Q

how long should DVT/PE patients be on anticoag therapy?

A

3 months minimum

64
Q

What guides the dosing of Warfarin?

A

the international normalized ratio

-INR

65
Q

What do we have to do to a PE/DVT patient first before we give them a DOAC (oral anticoag)?

A
  • they must first recieve 5-10 days of parenteral anticoagulation
  • then transitioned to the oral agent
66
Q

What do we add on to people who have a very high risk PE: PE with persistent hemodynamic instability?

A

add thrombolytic therapy along with the anticoagulant

-**should only be considered in patients who have a low risk of bleeding

67
Q

EOD for Essential thrombocytosis

A
  • elevated platelet count in absence of other causes
  • normal red blood cell mass
  • absence of bcr/abl gene (Ph chromsome)
68
Q

Symptoms of ET

A
  • 50 to 60 y/o
  • elevated platelet count
  • risk of thrombosis rises with age
  • eythromelalgia
  • mucosal bleeding is less common
  • paresthesias
69
Q

What is ET symptoms like erythromelalgia reliably relieved by?

A

aspirin

70
Q

what does the bone marrow look like on ET?

A

lots of megakaryocytes

71
Q

what do we need to differentiate ET from

A
  • =CML

- THat’s why we have to test for the philadelphia chromosome

72
Q

Tx of ET

A
  • control the platelet count

- treatment of choice is HYDROXYUREA

73
Q

ET prognosis

A

good

-major source of morbidity is thrombosis

Heme and Lymph Final (14 decks)

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