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
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
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
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%
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
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
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
8
Q
What is the normal platelet count?
A
150,000-450,000
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
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
11
Q
What may thrombocytopenia be caused by?
A
- decreased platelet production
- increased platelet destruction
- hypersplenic sequestration
12
Q
What are some general reasons for why there may be a decrease in platelet production?
A
- congenital syndromes
- acquried defects
- ineffective thrombopoiesis
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)
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)
15
Q
What are general things that can lead to ineffective thrombopoiesis?
A
- toxins
- drugs
- acute viral infections
- nutritional deficiencies
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
17
Q
What is the most common cause of thrombocytopenia?
A
increased platelet destruction
18
Q
What are some general reasons for increased platelet destruction?
A
- immune destruction
- nonimmune destruction
- hypersplenic sequestration
19
Q
What can cause immune destruction of platelets?
A
- autoimmune diseases
- drug induced antibodies
- infections (HIV, viral hepatitis)
- alloantibodies (post-transfusion)
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)
21
Q
What thrombotic microangiopathies can lead to nonimmune destruction of platelets?
A
- TTP
- HUS (hemolytic uremic syndrome)
(can lead to schistocytes)
22
Q
- What is hypersplenic splenic sequestration of platelets most commonly from?
A
alcoholic cirrhotic portal hypertension
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
24
Q
What is a characteristic of severe thrombocytopenia?
A
petechiae
25
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
- 100,000-150,000
- 50,000-100,000
- 20,000-50,000
- < 20,000
- < 10,000-15,000
26
What are the sizes of petechiae, purpura, and ecchymoses?
- petechiae: 1-2 mm
- purpura: < 1 cm
- ecchymoses: > 1 cm
27
- 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?
- petechiae
- trauma (injuries, accidents, excessive coughing or extreme bouts of vomiting)
- certain medications, some types of allergic rxns
28
- 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
- purpura and ecchymoses
- children and the elderly
29
What may large purpura be caused by?
vascular problems, platelet disorders, meningitis, or vitamin C deficiency
30
- 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?
- skin and mucous membranes
- LAD and hepatosplenomegaly
- lymphome, hepatic disease, connective tissue disease, and infectious disease
31
What laboratory tests can be conducted for thrombocytopenia diagnosis?
- CBC
- peripheral blood smear
- PT (INR), PTT
- bone marrow
32
- 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?
- collection tube EDTA (can cause clumping)
- marrow/production etiology
- peripheral destruction
- increased thrombopoiesis
33
What is indicated in ill, hospitalized, or postsurgical patients?
PT (INR), PTT
34
- 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?
- PT: 11-15 secs, PTT: 20-35 secs, INR: 1
- DIC
- TTP and HUS
35
- What will a bone marrow examination show in thrombocytopenia?
- When is a bone marrow definitely done?
- nothing
- WBC and RBC issues
36
What should you look for if thrombocytopenia is d/t sequestration of platelets?
splenomegaly
(make sure theres no malignancy)
37
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?
- bone marrow disorders
- hypersplenism
- immune mediated, primary or secondary ITP, drug induced thrombocytopenia
38
- In true thrombocytopenia, what do schistocytes on a peripheral smear suggest?
- What if the smear is normal, what does it suggest?
- TTP, HUS, DIC
- isolated thrombocytopenia
39
- 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 (...)
- platelet transfusion
- 10,000-12,000
- 20,000-50,000
40
What are complications of platelet transfusion?
- pts become alloimmunized against platelet antigens
- bacterial contamination of stored platelets, sensitive to cold storage
41
Platelet transfusion is contraindicated in what?
- post transfusion purpura
- TTP/HUS, HIT
42
When should you do platelet transfusions (below what number)?
below 20,000
43
What is the general treatment for thrombocytopenia d/t decreased platelet production?
- address underlying cause
- transfusions PRN
44
What is the treatment for thrombocytopenia d/t increased platelet destruction from ITP and secondary autoimmune thrombocytopenia?
**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
45
What should be given if thrombocytopenia is caused due to increased platelet destruction(ITP/secondary autoimmune thrombocytopenia) and a splenectomy fails?
- danazol
- immunosuppressive agents
46
What is the treatment for drug-induced thrombocytopenia (increased destruction of platelets)?
- discontinue drug
- prednisone short course
- platelet transfusion
47
What do you treat TTP with (non-immune thrombocytopenia)?
emergency plasmapheresis with FFP, orther recs
48
- What is last line therapy for non-immune thrombocytopenia?
- What is contraindicated for non-immune thrombocytopenia?
- steroid use
- transfusions
49
If patients have hypersplenism, why is treatment usually unnecessary?
decreased degree of thrombocytopenia
50
- 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
- associated with arterial and venous thromboses, not bleeding
- more common in adults, slight increased incidence in females
- 2-5%; 5-10X
- 0.7%
51
What are the 2 forms of heparin induced thrombocytopenia (HIT)?
- type 1: mild, transient decrease, benign
- type 2: antibody mediated, high risk thrombosis
52
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 (...)
- PF4, binds to heparin
- IgG
- more platelets, and then more antibodies (cyclic)
- premature removal circulation
- thrombosis
53
What are the risk factors that can increase risk of heparin-induced thrombocytopenia?
- longer duration of exposure to heparin
- the type of heparin
- type of patient (surgical, esp. cardiac and orthopedic > risk than medical)
54
Heparin induced thrombocytopenia should be suspected in patients with exposure to heparin for how long?
- 4-14 days OR
- prior to 3 months
55
Heparin induced thrombocytopenia should be suspected in patients with an unexplained platelet count decrease to (...) below pre-treatment baseline
50%
56
HIT should be suspected in patients with an onset of thrombocytopenia (...) days after heparin initiation
5-10 days
57
- 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
- acute venous or arterial thrombosis
- skin lesions/necrosis
- acute anaphylactoid reaction
58
- What are the 4 T's that aid in the diagnosis of HIT?
- Probability scoring (...) involving judgement
- High probability should be (...)
- thrombocytopenia, timing of onset, thrombosis, other cause
- 0-8
- treated
59
What are some heparin antibody tests that can be used to aid in diagnosis?
- ELISA
- Functional SRE (serotonin release assay)
60
What HIT test is an enzyme immunoassay test with high sensitivity but low specificy so it doesn't tell you its HIT?
ELISA
61
What HIT test has high specificity and sensitivity so it tells you its HIT?
| (this is the **gold standard test** for HIT)
functional SRA (serotonin release assay)
62
What imaging modalities can be used for HIT?
- doppler US of BLEs
- UEs PRN
63
What is the treatment for HIT?
- D/C all heparin exposure
- begin alternate anticoagulant
- *emerging data for anti-thrombin binder: Fondaparinux*
64
After discontinuing heparin exposure in HIT patients, there is still a (...)% risk of developing thrombosis within 30 days
50%
65
What alternate anticoagulant therapies can be used with HIT patients after discontinuing heparin?
- 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
There is now emerging data for anti-thrombin binder Fondaparinux to be used for HIT treatment, what is good about this drug?
increased efficacy and lower risk of bleeding
67
- Remember, HIT causes (...) and (...), not (...)
- Consider use of (...) as DVT prophylaxis to prevent HIT
- thrombocytopenia and clotting; not bleeding
- LMW heparin (lower risk)
68
- 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 (...)
- immune thrombocytopenic purpura (ITP)
- gender, any age
- children, after viral URI
- adults
69
ITP coexists with other autoimmune diseases such as what?
| (you will need to rule these out)
- SLE
- CLL
- HIV
70
What are the clinical manifestations associated with acute ITP?
abrupt appearance of petechiae and purpura of skin/mucous membranes
71
What are the clinical manifestations of chronic ITP?
often asymptomatic, but may develop petechiae and purpura; generally excessive mucoidal bleeding
72
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 (...)**
- epistaxis, gingival bleeding, easy bruising, hematuria, melena, hematochezia, menorrhagia
- hemorrhage in conjuctiva, retina and CNS
- splenomegaly
- eyes or mouth
73
When working up acute ITP, what may the findings consist of?
- decreased platelets (10,000-20,000)
- eosinophilia
- mild lymphocytosis
74
When working up chronic ITP, what may the findings consist of?
- platelet count 25,000-75,000
- mild anemia
- peripheral smear/bone marrow biopsy may show megakaryocytes
75
What does treatment of ITP consist of?
**treat symptoms and platelet count**
- corticosteroids
- immunosuppressive/immunomodulators
- splenectomy
- thrombopoietin (TPO)
76
What is the first line therapy in ITP as 80% of patients respond to this?
- corticosteroids
- high dose prednisone or dexamethasone for 4-6 cycles
77
What immunosuppressives can be used for ITP treatment?
- retuximab (monoclonal antibody)
- IVIG, anti-D IG
78
What ITP treatment is used for refractory patients and have good success with complete response rates (up to 70%)?
splenectomy
79
What TPO medications can be used for ITP treatment? What do these do?
- eltrombopag, romiplostasm
- stimulates megakaryocytes (platelet precursors)
80
In acute ITP, it may resolve spontaneously and (...) or (...) may help
- steroids
- splenectomy
81
What else may you try with chronic ITP for treatment?
stem cell transplant
82
- 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?
- 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
How can people end up with thrombotic thrombocytopenic purpura (TTP)?
- acquired: autoimmune disorder caused by autoantibody inhibition of ADAMTS13
- hereditary: homozygous or compound heterozygous mutations of ADAMTS13
84
How can people get TTP by acquiring it?
- drug induced
- infections
- pregnancy
- malignancies, ABMT
- neurologic disorders
85
What drugs can cause TTP?
- PCN
- antineoplastic agents
- OCPs
- quinine
86
What should you consider in a pregnant patients especially if there are vague neuro, GI, or renal symptoms (obstetric triage)?
TTP
87
What is the differential diagnosis if a patient has TTP (look alikes)?
- DIC
- sepsis
- malignant HTN
- vasculitis
- eclampsia, pre-eclampsia
- HUS, esp. in children after viral infection
- gastroenteritis from serotoxin result of E. coli
88
- TTP often beings as (...) followed by (...) and (...) abnormalities
- What do patients typically complain of?
- flu-like; clinical and lab abnormalities
- non-specific constitutional symptoms: weakness, N/V, abdominal pain
89
What are some signs/symptoms of TTP?
- purpura secondary to thrombocytopenia
- jaundice, pallor from hemolysis
- mucosal bleeding
- fever
90
What may be seen in about 2/3rds of patients with TTP?
fluctuating levels of consciousness (thrombotic occlusion of cerebral vessels - CVA)
91
What are some end stage features of TTP?
renal failure and neurological events
92
What is the classic pentad of clinical manifestations in 50% of patients with TTP?
- fever
- thrombocytopenia
- hemolytic anemia
- renal dysfunction (elevated creatine)
- neurologic dysfunction (delirium)
93
When working up a patient with suspected TTP, you should get a conprehensive (...) and (...)
history and physical
94
- 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?
- severe anemia and thrombocytopenia (platelet count < 50 or 50% decrease from baseline)
- RBC fragments (schistocytes)
- elevated BUN/creatinine
95
- 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 (...)
- hemolysis
- elevated retic count, indirect bilirubin, LDH; decreased haptoglobin
- hematuria and proteinuria
- DIC (fibrin breakdown)
96
- What does hereditary TTP require documentation of?
- Acquired TTP criteria requires presence of (...) and (...) w/o other cause
- positive ADAMTS13 test results
- hemolytic anemia and thrombocytopenia
97
- What is the standard therapy for acute TTP?
- What does treatment of acute TTP consist of?
- emergency large volume plasmapheresis
- plasmapheresis, plasma infusion, high dose; corticosteroids
98
- 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?
- 2 days
- FFP (fresh frozen plasma); 20-30 units; 2-3 weeks
- hypotensions, sepsis, hemorrhage, and thrombosis
99
- In treatment of acute TTP, plasma infusion is useful only if unable to quickly begin (...)
- However, this can cause volume overload in (...)
- plasma exchange
- renal insufficiency
100
- If acute TTP is mild, (...) can be used alone
- However, for moderate-severe cases, you can use this with (...)
- prednisone
- PLEX (plasma exchange)
101
What are other acute treatment methods for TTP?
- retuximab
- platelet transfusions
- splenectomy (in refractory cases)
- if hereditary, plasma infusion to correct ADAMTS13
- discontinue offending agents
102
What is contraindicated in treatment of TTP except in severe cases with documented bleeding or facing surgery?
platelet transfusions
103
What does chronic treatment of TTP consist of?
- PLEX (plasmapheresis) if relapsing TTP
- rituximab, cyclophosphamide (remission of TTP unresponsive to conventional tx reported)
- splenectomy
104
What is done while a patient is in TTP remission to decrease frequency of relapse?
splenectomy
105
- People with TTP who get PLEX have (...)% survival
- Relapse occurs in (...)% of patients who have TTP in remission
- > 80%
- 20-40%
106
What describes an elevated platelet count ( > 450,000) in peripheral blood?
thrombocytosis
107
- Extreme thrombocytosis is defined as having a platelet count greater than what?
- What is a synonym for thrombocytosis?
- 1 million
- thrombocythemia
108
- 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?
- 2.5 cases/100,000 per year
- 60 yo
- 2:1
109
What is thrombocytosis caused by?
- overproduction of platelets
- clonal expansion of megakaryocytes
- RBCs and WBCs
110
- What is reactive thrombocytosis?
- What is clonal thrombocytosis?
- overproduction of platelets
- clonal expansion of megakaryocytes
111
- 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 (...)
- cytokines; trauma or inflammation
- reactive thrombocytosis (70% - 22%)
- CMPD (JAK2 pathway)
112
- A high platelet count may be associated with (...) symptoms
- What does this include?
vasomotor symptoms:
- HA, dizziness
- visual disturbances
- atypical CP
- limb dysesthesia
- thrombotic and bleeding complications
- erythromelalgia (rare condition affecting feet > hands)
113
- 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 (...)
- thrombosis
- splenomegaly
- PV and CML
114
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
spurious thrombocytosis
115
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
reactive thrombocytosis
116
What type of thrombocytosis are these associated with:
- CML
- PV
- AML
- ET
- PMF (primary myelofibrosis)
- other myelodysplastic syndromes
clonal thrombocytosis
117
What is the differential diagnosis of thrombocytosis?
- spurious
- reactive
- clonal
118
What does the diagnosis of thrombocytosis require?
- 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
What needs to be done to exclude spurious thrombocytosis?
repeat CBC w/ peripheral smear and bone marrow biopsy
120
- 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?
- history and physical
- reactive
- acute blood loss, iron deficiency, acute/chronic infection or inflammation, medication use, asplenia, malignancy, trauma
121
- What will a CBC with peripheral smear show in pts with asplenia?
- What will be seen in pts with PMF?
- howell-jolly bodies and target cells
- nucleated RBC, teardrop RBC and WBC precursors
122
- 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?
- rule out iron deficiency
- serum C-reactive protein (CRP), ESR, and plasma fibrinogen
- philadelphia chromosome or BCR-ABL rearrangment
123
- 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?
- low to normal
- PV and ET
124
- What will a bone marrow chromosome analysis show in ET?
- What will a bone marrow chromosome analysis rule out in the workup of thrombocytosis?
- clusters of abnormal megakaryocytes and increased reticulin fibrosis
- myelodysplastic syndrome, CML
125
What is usually the first line treatment for thrombocytosis?
aspirin (low dose)
126
What should patients with thrombocytosis who have vasomotor symptoms be treated with?
aspirin for < 100 mg/day
127
What should the treatment of patients with thrombocytosis who are bleeding consist of?
- 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
- 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
- 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
What is the general treatment of chronic thrombocytosis?
- smoking cessation and obesity management discussion
- low dose ASA (81 mg/day)
- cytoreductive therapy
130
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
low dose ASA (81 mg/day) for treatment of chronic thrombocytosis
131
- 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
- hydroxyurea vs anagrelide
- HU + ASA
- LFTs and degree of neutropenia or anemia (w/ HU tx)
- leukemic conversion
- interferon alpha
132
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
- 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.*
