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1954 Part XII Hemostasis and Thrombosis
induce platelet activation through release of platelet-derived RANTES 9a. Mithoowani S, Gregory-Miller K, Goy J, et al: High-dose dexa-
(regulated on activation, normal, T-cell expressed and secreted), a methasone compared with prednisone for previously untreated primary
proinflammatory and immunomodulatory chemokine involved in immune thrombocytopenia: a systematic review and meta-analysis.
multiple immunologic processes, including Ig synthesis and regula- Lancet Haematol 3(10):e489–e496, 2016.
tion of Th1/Th2 cytokine homeostasis. In some patients with PTP, 10. Beck CE, Nathan PC, Parkin PC, et al: Corticosteroids versus intravenous
the alloantibodies have been shown to interfere with cell-fibrinogen immune globulin for the treatment of acute immune thrombocytopenic
interaction, thereby increasing the risk of bleeding. purpura in children: a systematic review and meta-analysis of randomized
The stimulation of specific anti–HPA alloantibodies could in turn controlled trials. J Pediatr 147(4):521–527, 2005.
initiate the formation of platelet-reactive autoantibodies. Production 11. Kojouri K, Vesely SK, Terrell DR, et al: Splenectomy for adult patients
of pan-reactive antibodies has been shown to correspond with the with idiopathic thrombocytopenic purpura: a systematic review to assess
period of greatest thrombocytopenia and serologic analyses of PTP long-term platelet count responses, prediction of response, and surgical
cases demonstrated the presence of reactive IgG and IgM antibodies complications. Blood 104(9):2623–2634, 2004.
against GPIIb/IIIa, GPIX, and GPIa/IIa; however, only HPA-specific 12. Arnold DM, Dentali F, Crowther MA, et al: Systematic review: efficacy
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IgG antibodies persisted. The presence of autoantibodies can help and safety of rituximab for adults with idiopathic thrombocytopenic
to explain the destruction of both donor and recipient platelets. purpura. Ann Intern Med 146(1):25–33, 2007.
13. Chugh S, Lim W, Crowther MA, et al: Rituximab plus standard of care
for treatment of primary immune thrombocytopenia: a systematic review
Management and meta-analysis. Lancet Haematol 2:e75–e81, 2015.
14. Patel VL, Mahevas M, Lee SY, et al: Outcomes 5 years after response to
Treatment of PTP should be initiated even before serologic test results rituximab therapy in children and adults with immune thrombocytope-
for anti–HPA-antigens are available. The primary goal of treatment nia. Blood 119(25):5989–5995, 2012.
is to abbreviate the period of severe thrombocytopenia and minimize 15. Kuter DJ, Bussel JB, Lyons RM, et al: Efficacy of romiplostim in patients
the risk of bleeding. Multiple treatments are often administered with chronic immune thrombocytopenic purpura: a double-blind ran-
simultaneously or in rapid succession because of the desperate nature domised controlled trial. Lancet 371(9610):395–403, 2008.
of the disorder when it is severe. 16. Cheng G, Saleh MN, Marcher C, et al: Eltrombopag for management of
Patients with PTP require admission to hospital for close obser- chronic immune thrombocytopenia (RAISE): a 6-month, randomised,
vation, supportive treatments, and rapid management of bleeding phase 3 study. Lancet 377(9763):393–402, 2011.
symptoms should they occur. Nonessential transfusions should be 17. Arnold DM, Smith JW, Kelton JG: Diagnosis and management of neonatal
discontinued. The mainstay of therapy is high dose IVIg, which is alloimmune thrombocytopenia. Transfus Med Rev 22(4):255–267, 2008.
30
followed by a platelet count increase after 3–4 days. High-dose 18. Warner MN, Moore JC, Warkentin TE, et al: A prospective study of
corticosteroids have also been tried as a means of inhibiting RES protein-specific assays used to investigate idiopathic thrombocytopenic
phagocytosis and reducing IgG synthesis. Patients with bleeding purpura. Br J Haematol 104(3):442–447, 1999.
should be transfused with HPA-compatible platelets. While awaiting 19. Bakchoul T, Bertrand G, Krautwurst A, et al: The implementation of
the results of antigen typing, HPA-1a–negative platelet transfusions surface plasmon resonance technique in monitoring pregnancies with
can be administered to reduce further antibody production. Patients expected fetal and neonatal alloimmune thrombocytopenia. Transfusion
with PTP may be at increased risk of transfusion reactions such as 53(9):2078–2085, 2013.
fever, dyspnea, and allergic reactions. Plasma exchange should be con- 20. Ghevaert C, Rankin A, Huiskes E, et al: Alloantibodies against low-
sidered in patients who do not respond to IVIg and corticosteroids. frequency human platelet antigens do not account for a significant
proportion of cases of fetomaternal alloimmune thrombocytopenia:
evidence from 1054 cases. Transfusion 49(10):2084–2089, 2009.
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