Thrombotic thrombocytopenic purpura. Treatment with corticosteroids and splenectomy

Hill, John

doi:10.1001/archinte.122.4.353

Cited by 29 publications

(4 citation statements)

References 13 publications

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“…Understandably the TTP literature abounds with a multiplicity of therapeutic modalities almost always used in various combinations and permutations [1,11,52,76,78,80,137,140,171]. Under such conditions in the salvaged patient the identification of the particular therapeutic agent to which benefits may be allocated is forfeited.…”

Section: Therapy For the Ttp Paradigmmentioning

confidence: 99%

Thrombotic Thrombocytopenic Purpura: An Extended Editorial

Nalbandian¹,

Henry²,

Bick³

2008

Semin Thromb Hemost

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Mystery and perplexity have burdened the history of TTP. The etiology of this disease remains unknown. The pathogenesis has been an enigma. No specific diagnostic test has yet been devised. The diagnosis is made tentatively and with imprecision. The tissue pathologic features are not at all pathognomonic. The laboratory findings are interchangeable with numerous other entities. The treatment is in perpetual debate. This regrettable catalogue of flawless failure also records that a strong clue for life-saving treatment in this deadly disease has been overlooked for half a century, until recent times.We propose new discriminatory, diagnostic standards for the identification of thrombotic thrombocytopenic purpura (TTP). Only by the use of a stringently defined paradigm applied prospectively to TTP will it be possible to understand the pathogenesis, to improve diagnostic accuracy, and especially to evaluate reliably the efficacy of a growing list of therapies. The diagnosis of TTP is in confusion and must be put right.The bleak record of progress in TTP is best regarded as bristling with opportunities for discovery. Continuing investigations by Kwaan [93,96, this issue, and recent discoveries by Byrnes and co-workers [35-37,101, this issue, pages 199-215] have given us invaluable insights into pathogenesis and treatment respectively. Suddenly, we are hopeful that some of our ignorance about TTP will soon vanish.The Byrnes' group has reported that plasma transfusions constitute effective, life-saving treatment in TTP [this issue pages [204][205][206]. This boon, however, is coupled with a hazard. Since TTP is rare, no single group may acquire any substantial clinical experience with the disease. Furthermore, diagnostic criteria for TTP are so non-specific, that more than TTP is so labeled. Thus, a proposed therapy for authentic cases of TTP will appear to be ineffective when inappropriately applied also to cases simulating

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Section: Therapy For the Ttp Paradigmmentioning

confidence: 99%

Thrombotic Thrombocytopenic Purpura: An Extended Editorial

Nalbandian¹,

Henry²,

Bick³

2008

Semin Thromb Hemost

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“…109 TTP and HUS are relatively uncommon and a wide range of uncontrolled studies of antiplatelet agents, anticoagulants, steroids, splenectomy, exchange transfusion, plasma exchange, and plasma infusion have been used in their treatment. 3,5,6,32,76,90,92,97,111,125,196,200,201,215,220,225,226,254,257,261,269 All forms of therapy have anecdotal claims of success and failure but more recent interest has focused on the role of re placement of the patient's plasma with blood pro ducts. 38 ' 97 ' 132 ' 196 ' 216 ' 226 This current interest in blood products underscores Moschcowitz' original description 191 of TTP in which he mentions that a colleague reported seeing four similar cases that re sponded to blood transfusion.…”

Section: Natural Surfacementioning

confidence: 99%

Platelet Destruction in Renal Disease

Lindsay¹,

Clark²

1982

Semin Thromb Hemost

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“…Splenectomy, until recently a standard thera peutic modality, is presently controversial since there is little apparent rationale for its use in TTP [18,69,105]. Nonetheless, splenectomy has been as sociated with induction of TTP remission [9,25,40,66,73]. High doses of corticosteroids (up to 500 mg of prednisone or equivalent per day) are included in many treatment regimens.…”

Section: State Of the Art Of Ttp Therapymentioning

confidence: 99%