Hubbard Flashcards

1
Q

Distinction between TTP and DIC

A

PT/PTT in TTP are normal early in the disease, but later tests suggest DIC

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2
Q

Treatment for TTP - 100% life saving

A

plasmapheresis

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3
Q

“pseudohemophilia” with mucocutaneous bleeding

A

Factor8-vWD

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4
Q

Platelet aggregation test (vWF)

A

ristocetin

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5
Q

Osler Weber Rendeu Syndrome is defined as __ and also called ___

A

Also called: Hereditary Hemorrhagic Telangiectasia

-Caused by thinning of the vessel walls causing telangiectatic formations and AV malformations

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6
Q

Fever + neuro + thromboCP + renal + microangiopathic hemolytic anemia = ?

A

Thrombotic Thrombocytopenia Purpura

Hemolytic Uremic Syndrome

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7
Q

Define microagiopathic anemia (MAHA) and example diseases (extravascular HA)

A

RBCs damaged when they pass through obstructed or narrowed vessels.

  • DIC, TTP/HUS, SLE, malignant HTN
  • PBS = **schistocytes
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8
Q

Define macroagiopathic anemia and example diseases (extravascular HA).

A

Prosthetic heart valves and Aortic Stenosis may cause HA secondary to MECHANICAL destruction of RBCs.
-PBS=schistocytes

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9
Q

Examples of infections that increase RBC destruction (extravascular HA).

A

Malaria, babesia

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10
Q

Pathogenesis of Thrombotic Thrombocytopenia Purpura

A
  • Hereditary – ADAMTS13 gene mutation
  • Acquired – autoantibodies at ADAMTS13
  • “Von Willebrand factor-cleaving protease” (degrades vWF) = ADAMTS13.
  • ADAMTS13 inhibition = decreased vWF degredation = increased platelet aggregation and thrombosis
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11
Q

Treat Hemophilia A with

Treat Hemophilia B with

A

Factor 8

Factor 9

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12
Q

CD#;ch# in hereditary hemorrhagic Telangiectasia.

Notable location of the hemorrhages.

A

CD105, ch9 –> codes for ENDOGLIN

-Hemorrhages on the vermilion border of lip and tongue

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13
Q

What is the most common cause of hypercoagulabilty?

A

Initiation of warfarin therapy because PRotein C and S are depleted prior to other factors, resulting in increased coagulablity.

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14
Q

Antiphospholipid syndrome is an anti/pro coagulant?
What do you use to diagnose.
***What condition is present in 50% of cases, but is NOT necessary to diagnose?
Tx with what?

A

PROcoagulant
-Use DRVVT (diluted Russell Viper Venom Test) or PTT test
**NOT NECESSARY to have connective tissue disease to have this.
Tx: hydroxychloroquine

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15
Q

Presence of what two factors increase risk of DVT or PT by 35-fold?

A

OCP + heterozygous Factor5 Leiden = synergistic relationship

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16
Q

Pts with vWD show up first with what symptom?

A

mucocutaneous bleeding

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17
Q

Treatment of vWD - does what? 1) cryoprecipitate 2) DDAVP

A
  1. Cryoprecipitate - replaces vWF

2. DDAVP - causes release of vWF from endothelium

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18
Q

Clinical features of ____?

  • Easy bleeding & bruisability
  • Hematomas from bleeding into soft tissues and muscles
  • Hemarthroses – frequent, many patients have 1 or 2 target joints which have recurrent bleeds
  • Patients are significantly inc risk for bleeding during and after surgery
A

Hemophilia A

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19
Q

Treatment of Hemophilia A

A
  • Factor replacement for bleeds, surgical prophylaxis
  • Recombinant factor replacements – hepatitis and AIDS less of a problem
  • Factor VIII inhibitors – antibodies to factor VIII that may complicate care
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20
Q

What deficiency can occur in patients taking warfarin, on chornic antibiotics, rat poison ingestion, or severe liver disease?

A

Vit K deficeincy

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21
Q
Characteristics of \_\_\_\_
o	Bleeding/hemorrhage
o	Prolonged PT
o	Def of factors II, VII, IX, X, Protein C & S
o	Anticoagulants = C & S
A

Vit K deficiency

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22
Q

Treatment for Vit K deficiency. Use which when?

A
  • Vit K replacement (intact liver)

- FFP (used when liver dz or acute hemorrhage)

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23
Q

What is the only endothelial syndrome associated with hemostatic complications?

A

Hereditary Hemorrhagic Telangectasia

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24
Q

Clinical presentation for…
o Minimal to early death from recurrent pulmonary emboli
o Chronic leg ulcers, deep vein thrombosis, recurrent LE thrombophlebitis
o DVT inc in pregnancy

A

Antithrombin 3 deficiency.

tendency to clot more/hypercoagulable

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25
Q

Antithrombin 3 deficiency treatment

A
  • Prophylactic treatment with anticoagulants
  • Patients with DVT should receive heparin but in higher doses
  • AT-III replacement therapy, if don’t respond to heparin
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26
Q

Action of protein C

A

inactivates Factors 5 and 8

27
Q

Use what in Protein C deficiency to decrease risk of thromboembolic disease?

A

warfarin

28
Q

What is antithrombin 3? What amplifies it?

A

serine protease that inhibits activation of thrombin.

  • Heparin amplifies activity
  • In absence of antithrombin3, hypercoagulablity
29
Q

Most common cause of hypercoagulable state from deficiency in Protein C or S

A

initiation of warfarin therapy

30
Q

Factor 5 Leiden is what classification of disease?

A

thrombotic

31
Q

Treatment of Factor 5 Leiden (Defect)

-No prior episodes v. prior episodes

A

o No prior episodes: monitor; DVT prophylaxis and risk reduction
o Prior episodes: add lifelong anticoagulation

32
Q

Three test used to diagnose antiphospholipid Syndrome

A
  1. Prolonged phospholipid dependent coagulation test (PTT)
  2. Lack of correction in mixing studies using normal plasma
  3. Neutralization of inhibitor with excess phospholipid
33
Q

What is the most common preventable cause of death?

A

DVT

34
Q

What is Virchow’s Triad?

A

vessel wall damage, blood coaguability, altered blood flow

35
Q

What is this?

  • Body makes antibodies to virus
  • Antigens on virus look like glycoproteins on platelets
  • Now platelets are circulation with antibodies on it platelets getting destroyed
  • Spleen should be big
A

Acute Immune Thrombocytopenic Purpura

36
Q

Management of Acute ITP

A

o PREDNISONE 1mg/kg/day
o Begin tapering does after a week
o If this doesn’t work immune globulin
o If person is a type one diabetic – sugar will be bouncing off the wall if on prednisone, so - 1)Put him on IV IG Or 2) give SPLENECTOMY (can be partial)

37
Q

What is this?

Dolle bodies, increased toxic granulations, no schistocytes, PT/PTT abnormal

A

DIC

38
Q

Management of DIC

A

o Treat underlying cause
o Until that’s fixed – fresh frozen plasma, cryoprecipitate
o Platelet transfusion
o Red cell count drops then transfuse red cells

39
Q

What is this?

*****White/painful fingers + extremely high platelets (1.2M) =

A

Erythromyalgia

also, possibly blurred vision

40
Q

Treatment of erythromyalgia

A

aspirin

41
Q

What is this?

  • causes hypercalcemia
  • suppresses levels of uninvolved immunoglboins
  • they have weird infection problems b/c no antibodies around, just ONE antibody immunoglobin that is involved in one major way
  • myeloma cells produce osteoclast activating factors that causes elevated calcemia
  • causes suppression of osteoblastic activity lytic bone lesions lots of free calcium
A

Multiple myeloma

42
Q

**What test MUST you do when you suspect Multiple myeloma?

A

Skeletal survey, NOT A BONE SCAN

Also, se M spike

43
Q

***treatment of multiple myeloma

A

thalidomide or linolidamide (angiogenesis inhibitor)
+
dexamethosone (steroid)

44
Q

Using bisphosmate in MM patients can cause what?

A

Myeloma kidney

45
Q

Ducher + congo red =

A

Amyloidosis (seen in pts with plasma cell dyscrasia)

Dutcher bodies

46
Q

heavy chain disease + truncated alpha chains = ?
(Presents with mesenteric adenopathy, diarrhea/malabsorption probelms, etc.)

Treat this with…?

A

alpha heavy chain disease

treated with ANTIMICROBIALS, NOT chemo

47
Q

Indicative of __?
• Chromosome 5,7
• Trisomy 8
• Inversion 1

A

MDS

48
Q

Treat 5q-Syndrome with what?

A

lenalidomide

49
Q

“Moth eaten appearance”

A

MM

50
Q

80yoM presents with sausage linking of the veins in eye. Electrophoresis shows gamma peak.

A

Waldenstrom (hyperviscosity), LPL

51
Q

Parvo and thymomas can cause what?

A

pure red cell aplasia

52
Q

***treatment for aplastic anemia

A

**cyclosporin and anti-thymocyte globulin

53
Q

Maltese cross = ? and can cause ___

A

babesiosis = HA

54
Q

A pt has high risk MDS. The only curative modality for this is?

Pt has severe aplastic anemia - curative modality?

A

allogenic stem cell transplantation

55
Q

MDS presents with?

MPD presents with?

A

MDS - cytopenia, infection

MPS - anemia, SM, B symptoms

56
Q

hydroxyuria does what?

A

Increases HbF - use in sickle cell

57
Q

When are increases in HbF beneficial in Sickle cell anemia?

A
  • painful episodes, Acute Chest Syndrome, Osteonecrosis, Leg ulcers
58
Q

Heavy chain disease - look for what in plasma cells?

A

Vacuolization

59
Q

Type of Heavy chain disease that can be aggressive or asymptomatic, almost always associated with anemia, HSM, LAD

A

Gamma

60
Q

Type of Heavy chain disease that can be MC type, MCly involving GI tract

A

Alpha

61
Q

Type of Heavy chain disease that can be Bence Jones proteinuria, hypogammalobulinemia

A

Mu

62
Q

Tx for sideroblastic anemia

A

paradoxine or alcohol cessation

63
Q

sickle cell tx - children need abx prolylaxis?

A

no. only for neonates