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Hematology Center
ITP

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ITP
Iron Chelation
IRON DEFICIENCY
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Treatment of ITP
When to treat?
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Steroids:
prednisone 1 mg/kg/day

--Glucocorticoid therapy is considered inappropriate initial treatment when the platelet count is >50,000 & the patient is either asymptomatic or has only minor purpura.
--Expect PLT count to increase within one week of treatment (in responding patients). Peak values will be achieved by 2-4 weeks. Patients who have not had response by then are unlikely to respond to prednisone & should be considered for splenectomy.
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Immune globulins
IVIG 1 g/kg X1

IVIg is appropriate initial treatment only for patients with platelet counts <50,000 who have severe, life-threatening bleeding. IVIg is inappropriate initial treatment for patients with platelet counts of 30,000 to 100,000 who are asymptomatic or who have only minor purpura.

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Anti-(Rh) D


The only clinically important adverse effect of anti-Rh(D) is alloimmune hemolysis. All Rh (D)+ patients develop a positive direct antiglobulin test after treatment, accompanied by a transient (1 to 2 weeks) decrease in Hgb concentration of about 0.5 to 2 g/dL. Although in two studies 4% to 24% of patients had a Hgb concentration of <10 g/dL after 7 to 14 days,48,74 RBC was not required.

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Splenectomy

Most studies suggest that approximately two thirds of patients achieve and sustain a normal platelet count after splenectomy and require no additional therapy. Most other patients experience a lesser increase or only transient normalization of platelet counts, with approximately half of the relapses occurring within 6 months of splenectomy.

splenectomy is appropriate in the following situations: (1) patients who have had the diagnosis for 6 weeks, have a platelet count <10,000, and have no bleeding symptoms, & (2) patients who have had the diagnosis for 3 months, have experienced a transient or incomplete response to primary treatment, have a platelet count of <30,000, and are either bleeding or not bleeding.

Splenectomy is inappropriate in nonbleeding patients who have had the diagnosis for 6 months and have a platelet count >50,000 and low hemostatic risk (eg, not engaged in potentially traumatic activities).

Prophylaxis for Splenctomy
If an elective splenectomy is planned, it is appropriate to provide preoperative prophylaxis with IVIg or oral glucocorticoid therapy in patients with platelet counts <20,000 to reduce the risk of intraoperative and postoperative bleeding.
Preoperative prophylaxis considered inappropriate when platelet counts >50,000, using IVIg , oral or parental glucocorticoid therapy, or anti-D. Platelet transfusions are considered inappropriate as preoperative prophylaxis for platelet counts >10,000.

ID prophylaxis for spelenectomy:
At least 2 weeks before elective splenectomy, patients should be immunized with polyvalent pneumococcal vaccine, Hemophilus influenzae b vaccine, & quadrivalent meningococcal polysaccharide vaccine.
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High-dose steroids
Dexamethasone, 40 mg/d for 4 days, repeated Q 4 weeks X 6 cycles

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Azathioprine

150 mg PO QD for a median duration of 6 months

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Danazol

400 mg PO QD X 5 months
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Pulse cytoxan
Cytoxan 1.0 to 1.5 g/m2 IV X 1 total dose: 1-4 (average 2 doses)

Ref:
[Blood 85(2): 351-358, 1995. --- 65%CR, 20%PR, 15%failure]

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Pulse decadron & vincristine:

Vincristine 2 mg IV X 1 Q week
Decadron mg IV X 4 Q month

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Antibody test to diagnosis ITP?
Studies have shown that anti-platelet antibody has a high sensitivity but a very low specificity in patients with ITP.

WHEN TO TREAT?
** Patients with counts >50,000 do not routinely require treatment; they should not be given glucocorticoids or IVIg as routine initial treatment.
** IVIg is also inappropriate as initial treatment in patients with counts >30,000 who are asymptomatic or have only minor purpura
** Treatment is indicated in patients with platelet counts <20,000 to 30,000, and those with counts <50,000 and significant mucous membrane bleeding (or risk factors for bleeding, such as hypertension, peptic ulcer disease, or a vigorous lifestyle).
** Hospitalization is appropriate for patients with platelet counts <20,000 who have significant mucous membrane bleeding.
** When ITP symptoms persist after primary treatment (glucocorticoid) and splenectomy, further therapy is recommended in patients with platelet counts <30,000 who have active bleeding.