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Clinical Medicine 2 > Platelet Disorders - Quantitative > Flashcards

Platelet Disorders - Quantitative Flashcards

1
Q
  • Platelets are fragments of larger (…)
  • They contrain several chemicals involved in the clotting process such as what?
  • What is their function?
A
  • megakaryocytes
  • serotonin, calcium, enzymes, ADP, platelet-derived growth factor
  • form tempory platelet plugs that help seal breaks in blood vessels
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2
Q

Circulating platelets are kept inactive and mobile by (…) and (…) from endothelial cells lining blood vessels (prevent from sticking)

A
  • nitric oxide (NO) and
  • prostacyclin
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3
Q
  • What is platelet formation regulated by?
  • They are formed in the myeloid line from (…)
  • This is known as what stage of the cell?
  • Mitosis occurs by no (…), resulting in large stage IV cells with (…) nucleus
  • The (…) sends cytoplasmic projections into the lumen of the capillary
  • projections break off into (…)
A
  • thrombopoietin
  • megakaryoblast
  • stage I megakaryocyte
  • cytokineses; multilobed
  • stage IV megakaryocyte
  • platelet fragments
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4
Q
  • How long does it take platelets to age and degenerate (lifespan)?
  • What percentage of platelets are sequestered in the spleen?
A
  • about 10 days
  • 20-30%
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5
Q
  • What do platelets stick to when a vessel is damaged?
  • Why don’t they stick to intact vessel walls?
  • What is secreted by endothelial cells that act to prevent platelet sticking?
  • What helps stabilize platelet-collagen adhesion?
A
  • collagen fibers that are exposed
  • collagen is not exposed
  • prostacyclins and nitric oxide
  • von willebrand factor
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6
Q
  • What happens to platelets when they become activated?
  • What messengers are released?
  • Platelet activation and aggregation is a positive feedback cycle, what does this mean?
  • Platelet plugs are fine for (…), but (…) will need an additional step
A
  • they swell, become spiked and sticky, and release chemical messengers
  • ADP, serotinin, thromboxane A2
  • as more platelets stick, they will release more chemicals, causing more platelets to stick and release more chemicals
  • small vessel tears, but larger breaks in vessels need additional step
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7
Q
  • What chemical messenger(s) released by platelets enhance vascular spasms and platelet aggregation?
  • What chemical messenger(s) released by platelets cause more platelets to stick and release their contents?
A
  • serotonin and thromboxane A2
  • ADP
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8
Q

What is the normal platelet count?

A

150,000-450,000

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9
Q
  • What is the platelet count for a patient to be considered to have thrombocytopenia?
  • What is the platelet count in individuals that can hemorrhage from minor traumas?
  • What is the platelet count in individuals that may have spontaneous bleeding?
  • What is the platelet count in individuals that may have severe bleeding that can be fatal?
A
  • < 100,000
  • < 50,000
  • < 15,000
  • < 10,000
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10
Q
  • What is another name for thrombocythemia?
  • What is the platelet count for patients to be considered to have thrombocythemia?
  • What is the cause of thrombocythemia?
  • What are the types?
  • What does thrombocythemia cause/do?
A
  • thrombocytosis
  • > 450,000
  • accelerated platelet production in bone marrow
  • primary or secondary (reactive)
  • intravascular clot formationg (thrombosis), hemorrhage, or other abnormalities
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11
Q

What may thrombocytopenia be caused by?

A
  • decreased platelet production
  • increased platelet destruction
  • hypersplenic sequestration
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12
Q

What are some general reasons for why there may be a decrease in platelet production?

A
  • congenital syndromes
  • acquried defects
  • ineffective thrombopoiesis
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13
Q

What is an example of a congenital syndrome that can lead to decreased platelet production?

A

Fanconi’s anemia

(congenital syndromes are rare for this)

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14
Q

Whatare the most common acquired defects that can lead to decreased platelet production?

A
  • aplastic anemia (viruses)
  • myelodysplastic syndromes o rmalignant bone marrow infiltration (myelofibrosis, multiple myelome, lymphoma, tumor metastases)

(may have an increase in WBC)

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15
Q

What are general things that can lead to ineffective thrombopoiesis?

A
  • toxins
  • drugs
  • acute viral infections
  • nutritional deficiencies
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16
Q
  • What toxins can lead to ineffective thrombopoiesis?
  • What drugs?
  • What acute viral infections?
  • What nutritional deficiencies?
A
  • alcohol, cocaine, cytotoxins, XRT (radiation)
  • thiazides, estrogens, sulfas, immune therapy
  • acute hepatitis, HIV, CMV, EBV, rubella
  • B12, folate or iron deficiencies
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17
Q

What is the most common cause of thrombocytopenia?

A

increased platelet destruction

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18
Q

What are some general reasons for increased platelet destruction?

A
  • immune destruction
  • nonimmune destruction
  • hypersplenic sequestration
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19
Q

What can cause immune destruction of platelets?

A
  • autoimmune diseases
  • drug induced antibodies
  • infections (HIV, viral hepatitis)
  • alloantibodies (post-transfusion)
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20
Q

What can lead to nonimmune destruction of platelets?

A
  • DIC: increased thrombin/microvascular thromboses, coagulation cascade
  • thrombotic microangiopathies: TTP, HUS
  • massive transfusions, prosthetic intravascular devices, extracorporeal circulation (CP bypass, hemodialysis)
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21
Q

What thrombotic microangiopathies can lead to nonimmune destruction of platelets?

A
  • TTP
  • HUS (hemolytic uremic syndrome)

(can lead to schistocytes)

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22
Q
  • What is hypersplenic splenic sequestration of platelets most commonly from?
A

alcoholic cirrhotic portal hypertension

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23
Q

What are some signs/symptoms of thrombocytopenia?

A
  • bleeding into superficial sites
  • easy bruising
  • “wet bleeding:” epistaxis, mucosa, gingiva, GI, GU, IV sites
  • “dry bleeding:” petechiae (esp. in areas of increased capillary pressure/trauma; face after coughing, buccal mucosa w/ chewing)
  • ecchymoses
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24
Q

What is a characteristic of severe thrombocytopenia?

A

petechiae

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25
Q

What platelet counts are associated with these symptoms/signs:
- asymptomatic
- easy brusing/post trauma bleeding
- risk for serious, excessive post-trauma bleeding
- risk for spontaneous bleeding, including intracranial (major cause of mortality)
- platelet transfusion; earlier if high risk for bleeding

A
  • 100,000-150,000
  • 50,000-100,000
  • 20,000-50,000
  • < 20,000
  • < 10,000-15,000
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26
Q

What are the sizes of petechiae, purpura, and ecchymoses?

A
  • petechiae: 1-2 mm
  • purpura: < 1 cm
  • ecchymoses: > 1 cm
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27
Q
  • What are small usually rounded red or purple spots that are approximately 1-2 mm in size, caused by bleeding under the skin?
  • What are these normally caused by?
  • What else can cause these?
A
  • petechiae
  • trauma (injuries, accidents, excessive coughing or extreme bouts of vomiting)
  • certain medications, some types of allergic rxns
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28
Q
  • Both (…) and (…) may be irregular in shape depending upon the location and amount of pooled blood
  • Both of these lesions are visible in (…) and the (…) who have fragile microvasculature
A
  • purpura and ecchymoses
  • children and the elderly
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29
Q

What may large purpura be caused by?

A

vascular problems, platelet disorders, meningitis, or vitamin C deficiency

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30
Q
  • When doing a physical exam on a patient with suspected thrombocytopenia, you should look at their (…) and (…) membranes
  • There should be a special focus for (…) and (…)
  • You should also look for signs of what 4 things?
A
  • skin and mucous membranes
  • LAD and hepatosplenomegaly
  • lymphome, hepatic disease, connective tissue disease, and infectious disease
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31
Q

What laboratory tests can be conducted for thrombocytopenia diagnosis?

A
  • CBC
  • peripheral blood smear
  • PT (INR), PTT
  • bone marrow
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32
Q
  • When getting a peripheral blood smear, what should you rule out?
  • Abnormalities of other cell lineages suggest what?
  • What does isolated thrombocytopenia suggest?
  • What does an elevated MPC indicate?
A
  • collection tube EDTA (can cause clumping)
  • marrow/production etiology
  • peripheral destruction
  • increased thrombopoiesis
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33
Q

What is indicated in ill, hospitalized, or postsurgical patients?

A

PT (INR), PTT

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34
Q
  • What is the normal PT, INR, and PTT
  • What conditions will PT, INR, and PTT be abnormal in?
  • What conditions with PT, INR, and PTT be normal in?
A
  • PT: 11-15 secs, PTT: 20-35 secs, INR: 1
  • DIC
  • TTP and HUS
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35
Q
  • What will a bone marrow examination show in thrombocytopenia?
  • When is a bone marrow definitely done?
A
  • nothing
  • WBC and RBC issues
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36
Q

What should you look for if thrombocytopenia is d/t sequestration of platelets?

A

splenomegaly
(make sure theres no malignancy)

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37
Q

If a patient has a platelet count less than 100,000:
- What does an abnormal blood smear or WBC/RBC abnormality suggest?
- What does a large spleen suggest?
- What else could be going on?

A
  • bone marrow disorders
  • hypersplenism
  • immune mediated, primary or secondary ITP, drug induced thrombocytopenia
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38
Q
  • In true thrombocytopenia, what do schistocytes on a peripheral smear suggest?
  • What if the smear is normal, what does it suggest?
A
  • TTP, HUS, DIC
  • isolated thrombocytopenia
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39
Q
  • What is the general treatment for thrombocytopenia for serious bleeding or anticipated major surgery?
  • One unit/bag of platelets should increase platelet count by (…)
  • Ideally, you want to increase platelets by (…)
A
  • platelet transfusion
  • 10,000-12,000
  • 20,000-50,000
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40
Q

What are complications of platelet transfusion?

A
  • pts become alloimmunized against platelet antigens
  • bacterial contamination of stored platelets, sensitive to cold storage
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41
Q

Platelet transfusion is contraindicated in what?

A
  • post transfusion purpura
  • TTP/HUS, HIT
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42
Q

When should you do platelet transfusions (below what number)?

A

below 20,000

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43
Q

What is the general treatment for thrombocytopenia d/t decreased platelet production?

A
  • address underlying cause
  • transfusions PRN
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44
Q

What is the treatment for thrombocytopenia d/t increased platelet destruction from ITP and secondary autoimmune thrombocytopenia?

A

treatment:
- address possible primary disorder
- prednisone for platelet counts < 50,000 w/ significant bleeding/risks or < 20,000 in general
- LED to maintain 50,000
- splenectomy (if this is the problem)
- if fails: danazol and immunosuppressive agents

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45
Q

What should be given if thrombocytopenia is caused due to increased platelet destruction(ITP/secondary autoimmune thrombocytopenia) and a splenectomy fails?

A
  • danazol
  • immunosuppressive agents
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46
Q

What is the treatment for drug-induced thrombocytopenia (increased destruction of platelets)?

A
  • discontinue drug
  • prednisone short course
  • platelet transfusion
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47
Q

What do you treat TTP with (non-immune thrombocytopenia)?

A

emergency plasmapheresis with FFP, orther recs

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48
Q
  • What is last line therapy for non-immune thrombocytopenia?
  • What is contraindicated for non-immune thrombocytopenia?
A
  • steroid use
  • transfusions
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49
Q

If patients have hypersplenism, why is treatment usually unnecessary?

A

decreased degree of thrombocytopenia

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50
Q
  • What is heparin induced thrombocytopenia (HIT) associated with?
  • Who is HIT more common in?
  • (…)% of patients with HIT are on unfractionated heparin and are at a (…) increased risk
  • (…)% of patients with HIT are on LMW heparin
A
  • associated with arterial and venous thromboses, not bleeding
  • more common in adults, slight increased incidence in females
  • 2-5%; 5-10X
  • 0.7%
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51
Q

What are the 2 forms of heparin induced thrombocytopenia (HIT)?

A
  • type 1: mild, transient decrease, benign
  • type 2: antibody mediated, high risk thrombosis
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52
Q

Describing the process of heparin-induced thrombocytopenia:
- platelets secrete a protein, (…), which binds to (…)
- (…) antibodies bind to this complex
- What does this process activate?
- Platelets aggregate and undergo (…) from circulation
- This process can lead to (…)

A
  • PF4, binds to heparin
  • IgG
  • more platelets, and then more antibodies (cyclic)
  • premature removal circulation
  • thrombosis
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53
Q

What are the risk factors that can increase risk of heparin-induced thrombocytopenia?

A
  • longer duration of exposure to heparin
  • the type of heparin
  • type of patient (surgical, esp. cardiac and orthopedic > risk than medical)
54
Q

Heparin induced thrombocytopenia should be suspected in patients with exposure to heparin for how long?

A
  • 4-14 days OR
  • prior to 3 months
55
Q

Heparin induced thrombocytopenia should be suspected in patients with an unexplained platelet count decrease to (…) below pre-treatment baseline

A

50%

56
Q

HIT should be suspected in patients with an onset of thrombocytopenia (…) days after heparin initiation

A

5-10 days

57
Q
  • HIT should be suspected in patients with evidence of (…) or (…)
  • HIT should be suspected in patients with (…) at heparin injection sites
  • HIT should be suspected in patients that have an (…) reaction during administration of heparin bolus
A
  • acute venous or arterial thrombosis
  • skin lesions/necrosis
  • acute anaphylactoid reaction
58
Q
  • What are the 4 T’s that aid in the diagnosis of HIT?
  • Probability scoring (…) involving judgement
  • High probability should be (…)
A
  • thrombocytopenia, timing of onset, thrombosis, other cause
  • 0-8
  • treated
59
Q

What are some heparin antibody tests that can be used to aid in diagnosis?

A
  • ELISA
  • Functional SRE (serotonin release assay)
60
Q

What HIT test is an enzyme immunoassay test with high sensitivity but low specificy so it doesn’t tell you its HIT?

A

ELISA

61
Q

What HIT test has high specificity and sensitivity so it tells you its HIT?

(this is the gold standard test for HIT)

A

functional SRA (serotonin release assay)

62
Q

What imaging modalities can be used for HIT?

A
  • doppler US of BLEs
  • UEs PRN
63
Q

What is the treatment for HIT?

A
  • D/C all heparin exposure
  • begin alternate anticoagulant
  • emerging data for anti-thrombin binder: Fondaparinux
64
Q

After discontinuing heparin exposure in HIT patients, there is still a (…)% risk of developing thrombosis within 30 days

A

50%

65
Q

What alternate anticoagulant therapies can be used with HIT patients after discontinuing heparin?

A
  • direct thrombin inhibitors; use single agent until platelet count returns to baseline (150)
  • initiate warfarin when count normalizes, overlap therapy until platelet count stable and INR at target
66
Q

There is now emerging data for anti-thrombin binder Fondaparinux to be used for HIT treatment, what is good about this drug?

A

increased efficacy and lower risk of bleeding

67
Q
  • Remember, HIT causes (…) and (…), not (…)
  • Consider use of (…) as DVT prophylaxis to prevent HIT
A
  • thrombocytopenia and clotting; not bleeding
  • LMW heparin (lower risk)
68
Q
  • What is an autimmune disorder caused by circulating antiplatelet autoantibodies (IgG)?
  • This occurs in either (…), at any (…)
  • The acute form of this is more common in (…) often after (…)
  • The chronic form of this is more common in (…)
A
  • immune thrombocytopenic purpura (ITP)
  • gender, any age
  • children, after viral URI
  • adults
69
Q

ITP coexists with other autoimmune diseases such as what?

(you will need to rule these out)

A
  • SLE
  • CLL
  • HIV
70
Q

What are the clinical manifestations associated with acute ITP?

A

abrupt appearance of petechiae and purpura of skin/mucous membranes

71
Q

What are the clinical manifestations of chronic ITP?

A

often asymptomatic, but may develop petechiae and purpura; generally excessive mucoidal bleeding

72
Q

Pertaining to chronic ITP:
- You should examine/ask about what?
- You should examine for what?
- Usually there is no (…) unless characteristic of underlying disorder such as lymphoma
- make sure to check in patients (…) or (…)

A
  • epistaxis, gingival bleeding, easy bruising, hematuria, melena, hematochezia, menorrhagia
  • hemorrhage in conjuctiva, retina and CNS
  • splenomegaly
  • eyes or mouth
73
Q

When working up acute ITP, what may the findings consist of?

A
  • decreased platelets (10,000-20,000)
  • eosinophilia
  • mild lymphocytosis
74
Q

When working up chronic ITP, what may the findings consist of?

A
  • platelet count 25,000-75,000
  • mild anemia
  • peripheral smear/bone marrow biopsy may show megakaryocytes
75
Q

What does treatment of ITP consist of?

A

treat symptoms and platelet count
- corticosteroids
- immunosuppressive/immunomodulators
- splenectomy
- thrombopoietin (TPO)

76
Q

What is the first line therapy in ITP as 80% of patients respond to this?

A
  • corticosteroids
  • high dose prednisone or dexamethasone for 4-6 cycles
77
Q

What immunosuppressives can be used for ITP treatment?

A
  • retuximab (monoclonal antibody)
  • IVIG, anti-D IG
78
Q

What ITP treatment is used for refractory patients and have good success with complete response rates (up to 70%)?

A

splenectomy

79
Q

What TPO medications can be used for ITP treatment? What do these do?

A
  • eltrombopag, romiplostasm
  • stimulates megakaryocytes (platelet precursors)
80
Q

In acute ITP, it may resolve spontaneously and (…) or (…) may help

A
  • steroids
  • splenectomy
81
Q

What else may you try with chronic ITP for treatment?

A

stem cell transplant

82
Q
  • What is a rare, often fatal, platelet consumption disorder associated with hemolytic anemia?
  • This disorder primarily affects who?
  • What is the incidence of this disorder in adults?
  • Which patients will have an increased incidence of this?
A
  • thrombotic thrombocytopenic purpura (TTP)
  • females b/w 18-50 yo
  • 2.9 cases/million per year
  • pts who are pregnant or who have HIV/AIDS
83
Q

How can people end up with thrombotic thrombocytopenic purpura (TTP)?

A
  • acquired: autoimmune disorder caused by autoantibody inhibition of ADAMTS13
  • hereditary: homozygous or compound heterozygous mutations of ADAMTS13
84
Q

How can people get TTP by acquiring it?

A
  • drug induced
  • infections
  • pregnancy
  • malignancies, ABMT
  • neurologic disorders
85
Q

What drugs can cause TTP?

A
  • PCN
  • antineoplastic agents
  • OCPs
  • quinine
86
Q

What should you consider in a pregnant patients especially if there are vague neuro, GI, or renal symptoms (obstetric triage)?

A

TTP

87
Q

What is the differential diagnosis if a patient has TTP (look alikes)?

A
  • DIC
  • sepsis
  • malignant HTN
  • vasculitis
  • eclampsia, pre-eclampsia
  • HUS, esp. in children after viral infection
  • gastroenteritis from serotoxin result of E. coli
88
Q
  • TTP often beings as (…) followed by (…) and (…) abnormalities
  • What do patients typically complain of?
A
  • flu-like; clinical and lab abnormalities
  • non-specific constitutional symptoms: weakness, N/V, abdominal pain
89
Q

What are some signs/symptoms of TTP?

A
  • purpura secondary to thrombocytopenia
  • jaundice, pallor from hemolysis
  • mucosal bleeding
  • fever
90
Q

What may be seen in about 2/3rds of patients with TTP?

A

fluctuating levels of consciousness (thrombotic occlusion of cerebral vessels - CVA)

91
Q

What are some end stage features of TTP?

A

renal failure and neurological events

92
Q

What is the classic pentad of clinical manifestations in 50% of patients with TTP?

A
  • fever
  • thrombocytopenia
  • hemolytic anemia
  • renal dysfunction (elevated creatine)
  • neurologic dysfunction (delirium)
93
Q

When working up a patient with suspected TTP, you should get a conprehensive (…) and (…)

A

history and physical

94
Q
  • What will a CBC show in a workup of a pt with TTP?
  • What will a peripheral blood smear show in a pt with TTP?
  • What will the levels of BUN/creatinine be in a patient with TTP?
A
  • severe anemia and thrombocytopenia (platelet count < 50 or 50% decrease from baseline)
  • RBC fragments (schistocytes)
  • elevated BUN/creatinine
95
Q
  • In TTP there will be evidence of (…)
  • In this case, what will be the levels of the retic count, indirect bilirubin, LDH, and haptoglobin?
  • What will a urinalysis show with a patient who has TTP?
  • There will be no evidence of (…)
A
  • hemolysis
  • elevated retic count, indirect bilirubin, LDH; decreased haptoglobin
  • hematuria and proteinuria
  • DIC (fibrin breakdown)
96
Q
  • What does hereditary TTP require documentation of?
  • Acquired TTP criteria requires presence of (…) and (…) w/o other cause
A
  • positive ADAMTS13 test results
  • hemolytic anemia and thrombocytopenia
97
Q
  • What is the standard therapy for acute TTP?
  • What does treatment of acute TTP consist of?
A
  • emergency large volume plasmapheresis
  • plasmapheresis, plasma infusion, high dose; corticosteroids
98
Q
  • Patients with acute TTP will have a daily plasma exchange (PLEX) for at least (…) and until platelet count returns to normal
  • This will consist of (…) in which the patient receives (…) units per session daily for (…) weeks
  • You will have to monitor patients closely for complications, especially with use of CVC in which cases?
A
  • 2 days
  • FFP (fresh frozen plasma); 20-30 units; 2-3 weeks
  • hypotensions, sepsis, hemorrhage, and thrombosis
99
Q
  • In treatment of acute TTP, plasma infusion is useful only if unable to quickly begin (…)
  • However, this can cause volume overload in (…)
A
  • plasma exchange
  • renal insufficiency
100
Q
  • If acute TTP is mild, (…) can be used alone
  • However, for moderate-severe cases, you can use this with (…)
A
  • prednisone
  • PLEX (plasma exchange)
101
Q

What are other acute treatment methods for TTP?

A
  • retuximab
  • platelet transfusions
  • splenectomy (in refractory cases)
  • if hereditary, plasma infusion to correct ADAMTS13
  • discontinue offending agents
102
Q

What is contraindicated in treatment of TTP except in severe cases with documented bleeding or facing surgery?

A

platelet transfusions

103
Q

What does chronic treatment of TTP consist of?

A
  • PLEX (plasmapheresis) if relapsing TTP
  • rituximab, cyclophosphamide (remission of TTP unresponsive to conventional tx reported)
  • splenectomy
104
Q

What is done while a patient is in TTP remission to decrease frequency of relapse?

A

splenectomy

105
Q
  • People with TTP who get PLEX have (…)% survival
  • Relapse occurs in (…)% of patients who have TTP in remission
A
  • > 80%
  • 20-40%
106
Q

What describes an elevated platelet count ( > 450,000) in peripheral blood?

A

thrombocytosis

107
Q
  • Extreme thrombocytosis is defined as having a platelet count greater than what?
  • What is a synonym for thrombocytosis?
A
  • 1 million
  • thrombocythemia
108
Q
  • How many cases out of 100,000 people per year are there with thrombocytosis?
  • What is the median age of diagnosis for thrombocytosis?
  • What is the female to male ratio for thrombocytosis?
A
  • 2.5 cases/100,000 per year
  • 60 yo
  • 2:1
109
Q

What is thrombocytosis caused by?

A
  • overproduction of platelets
  • clonal expansion of megakaryocytes
  • RBCs and WBCs
110
Q
  • What is reactive thrombocytosis?
  • What is clonal thrombocytosis?
A
  • overproduction of platelets
  • clonal expansion of megakaryocytes
111
Q
  • Reactive thrombocytosis is driven by excessive (…) induced by various stimuli, such as (…) or (…)
  • Which is more common, reactive or clonal thrombocytosis?
  • The etiology is unclear in essential/clonal thrombocythemia; DNA changes from chemical signals into the cell membrane may play a rold in (…)
A
  • cytokines; trauma or inflammation
  • reactive thrombocytosis (70% - 22%)
  • CMPD (JAK2 pathway)
112
Q
  • A high platelet count may be associated with (…) symptoms
  • What does this include?
A

vasomotor symptoms:
- HA, dizziness
- visual disturbances
- atypical CP
- limb dysesthesia
- thrombotic and bleeding complications
- erythromelalgia (rare condition affecting feet > hands)

113
Q
  • The degree of thrombocytosis doesn’t predict or generally correlate to the risk of (…)
  • (…) is common with myeloproliferative disorders
  • Coexistent leukocytosis and erythrocytosis are common with (…) and (…)
A
  • thrombosis
  • splenomegaly
  • PV and CML
114
Q

What is this describing:
- characterized by the presence of nonplatelet structures in the peripheral blood which are counted as platelets by the automated counters used in modern complete blood counts
- cytoplasmic fragments in pts with leukemia or lymphoma, or severe hemolysis, or burns can be counted falsely as platelets

A

spurious thrombocytosis

115
Q

What type of thrombocytosis are these disorders associated with:
- other anemias
- benign hematologic disorders
- chronic infections
- acute/chronic inflammatory disorders
- rheum disorders
- IBD
- pancreatitis
- trauma and tissue damage
- recent surgery
- MI
- renal disease
- excercise
- medications

A

reactive thrombocytosis

116
Q

What type of thrombocytosis are these associated with:
- CML
- PV
- AML
- ET
- PMF (primary myelofibrosis)
- other myelodysplastic syndromes

A

clonal thrombocytosis

117
Q

What is the differential diagnosis of thrombocytosis?

A
  • spurious
  • reactive
  • clonal
118
Q

What does the diagnosis of thrombocytosis require?

A
  • platelet counts > 600,000 on 2 separate occasions > 4 weeks apart
  • absence of Philadelphia chromosome (CML); ruling this out
  • exclusion of secondary causes
  • need to repeat CBC w/ peripheral smear and bone marrow biopsy to exclude spurious thrombocytosis
119
Q

What needs to be done to exclude spurious thrombocytosis?

A

repeat CBC w/ peripheral smear and bone marrow biopsy

120
Q
  • The work-up of thrombocytosis includes a comprehensive (…) and (…)
  • This is done to exclude many of common causes of (…) etiology
  • You need to examine specifically for what to make sure these things aren’t going on?
A
  • history and physical
  • reactive
  • acute blood loss, iron deficiency, acute/chronic infection or inflammation, medication use, asplenia, malignancy, trauma
121
Q
  • What will a CBC with peripheral smear show in pts with asplenia?
  • What will be seen in pts with PMF?
A
  • howell-jolly bodies and target cells
  • nucleated RBC, teardrop RBC and WBC precursors
122
Q
  • Why would you do a serum ferritin test in the work-up of thrombocytosis?
  • What tests can be done in this work-up as they are nonspecific markers of infection or inflammation?
  • What test can be done in the work-up of thrombocytosis that will be positive in CML?
A
  • rule out iron deficiency
  • serum C-reactive protein (CRP), ESR, and plasma fibrinogen
  • philadelphia chromosome or BCR-ABL rearrangment
123
Q
  • What will the levels of serum EPO assay be in PV and ET, so if they are these values, it won’t be thrombocytosis?
  • What disorders who a JAK2 mutation analysis be done?
A
  • low to normal
  • PV and ET
124
Q
  • What will a bone marrow chromosome analysis show in ET?
  • What will a bone marrow chromosome analysis rule out in the workup of thrombocytosis?
A
  • clusters of abnormal megakaryocytes and increased reticulin fibrosis
  • myelodysplastic syndrome, CML
125
Q

What is usually the first line treatment for thrombocytosis?

A

aspirin (low dose)

126
Q

What should patients with thrombocytosis who have vasomotor symptoms be treated with?

A

aspirin for < 100 mg/day

127
Q

What should the treatment of patients with thrombocytosis who are bleeding consist of?

A
  • discontinue platelet anti-aggregrating agent (ASA, NSAIDs)
  • evaluation for DIC and coagulation factor deficiency (acquired factor V deficiency, FFP infusion)
  • if extreme disease, von willebrands may occur -> immediate platelet apheresis with a platelet lowering agent
128
Q
  • What occurs in 20-30% of patients with thrombocytosis?
  • If platelet count is > 800,000, what should treatment consist of?
  • Initial work-up should include additional thrombophilic disease, which includes what?
  • Thrombosis d/t thrombocytosis should be treated with anticoagulant therapy for (…) months based on other thrombophilic defects
A
  • thrombosis
  • platelet apheresis + platelet lowering agent w/ goal < 400,000
  • protein C/S, antithrombin, anticardiolipin antibodies, mutations for factor V and II, and plasma homocysteine
  • 3-9 months
129
Q

What is the general treatment of chronic thrombocytosis?

A
  • smoking cessation and obesity management discussion
  • low dose ASA (81 mg/day)
  • cytoreductive therapy
130
Q

What is this describing:
- may be safe and effective for preventing vascular events
- also prevents recurrent vascular evens in high-risk pts and treatment of vascular evens

A

low dose ASA (81 mg/day) for treatment of chronic thrombocytosis

131
Q
  • What does cytoreductive therapy consist of for chronic thrombocytosis?
  • What may be safer and more effective that ^?
  • What should you monitor while providing this treatment?
  • (…) conversion is possible, < 1%
  • (…) may help in those who fail this treatment
A
  • hydroxyurea vs anagrelide
  • HU + ASA
  • LFTs and degree of neutropenia or anemia (w/ HU tx)
  • leukemic conversion
  • interferon alpha
132
Q

A patient is admitted with confusion and abdominal pain. She has a body temperature of 101.1 degrees and her laboratory studies demonstrate the following: leukocyte count 4,400/uL, hemoglobin 10.5 g/dL, platelet count 11,000/uL, D-dimer 0.2 ug/mL, and creatinine 1.1 mg/dL. The peripheral blood smear shows nucleated RBCs and fragmented RBCs (schistocytes). Which of the following should you recommend:
- IV immunoglobulin
- platelet transfusion
- platelet transfusion + RBC transfusion
- plasmapheresis
- antibiotics

A
  • plasmapheresis
    pt has thrombocytopenia and anemia associated w/ schistocytes on blood smear. Indicates she has microangiopathic hemolytic anemia. presence of fever and neuro symptoms suggests TTP.

Clinical Medicine 2 (9 decks)

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