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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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