Studies of a patient with recurring thrombotic thrombocytopenic purpura

Kitchens, Craig S.

doi:10.1002/ajh.2830130310

Cited by 30 publications

(15 citation statements)

References 23 publications

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“…Our experience with one patient (Case 4), as well as that of others [86,87,91,93], indicates that plasma infusion alone may be beneficial. The choice of treatment should, of course, be determined by the severity of the clinical manifestations, as PE is an invasive and not entirely benign procedure.…”

Section: Discussionmentioning

confidence: 72%

Thrombotic thrombocytopenic purpura: Report of fourteen cases—occurrence during pregnancy and response to plasma exchange

Caggiano

Fernando

Schneider

et al. 1983

J of Clinical Apheresis

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Thrombotic thrombocytopenic purpura (TTP), a syndrome of diverse etiology probably related to factors regulating platelet-vessel wall interaction, is predominantly a disorder of women. We report our experience with 14 patients in an 11-year period. Thirteen were female and aged between 25-69 years. Four were postmenopausal, and of the nine premenopausal women three were pregnant, one was immediately postpartum, and three were taking estrogen-containing oral contraceptives. A review of the literature confirms the two to one female/male preponderance and that TTP is reported in 56 women who are pregnant or recently postpartum. While this association with possible hormonal events has been noted, it has previously received little comment. We stress the similarity between TTP and some occurrences of preeclamptic toxemia, and that this may suggest not only a common etiology but that therapeutic attempts should be similar. While no single therapeutic modality is universally successful, our experience is that plasma exchange is the most effective, with five of seven patients so-treated obtaining prolonged remission; four of five patients responded to splenectomy and corticosteroids, but one died of infection postoperatively. Five patients, including two treated exclusively with antiplatelet aggregating agents, died without achieving remission. The frequency of successful therapy is not changed by the concurrent pregnancy, but the fetal loss is high. There does seem to be an increased risk of recurrence of TTP in a subsequent pregnancy, and this might be considered when counseling premenopausal patients who have achieved remission of TTP.

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Section: Discussionmentioning

confidence: 72%

Thrombotic thrombocytopenic purpura: Report of fourteen cases—occurrence during pregnancy and response to plasma exchange

Caggiano

Fernando

Schneider

et al. 1983

J of Clinical Apheresis

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“…Long-lasting remissions after splenectomy in patients with relapsing TTP or chronic TTP have been observed by others in small series and case reports (Table 3) [,-21, 24-26]. TTP is rare in patients in whom the spleen had been removed [7,17,35]. Relapses after splenectomy have been described, in most of the patients after long disease-free intervals [14,19,24].…”

Section: Splenectomy In Relapsing Ttpmentioning

confidence: 73%

The role of splenectomy in the treatment of relapsing thrombotic thrombocytopenic purpura

et al. 1995

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Thrombotic thrombocytopenic purpura (TTP) is a serious disorder of unknown etiology. Clinical findings are the result of vascular occlusions by platelet aggregates. Treatment with plasma exchange, often used in combination with corticosteroids, vincristine, aspirin, and dipyridamole, has reduced mortality to 20%. Relapses may occur even after long disease-free intervals. In this report we describe our experience with splenectomy in patients with relapsing TTP. Between July 1978 and March 1994, 16 patients with TTP were treated in our hospital. Five of the 13 patients surviving the first episode of TTP had relapses. Most relapses were treated as the first episode of TTP with plasma exchange with fresh-frozen plasma, followed by plasma infusions, corticosteroids, and vincristine. Sometimes aspirin and dipyridamole were added. Splenectomy was performed after five relapses in the first two patients and after two and three relapses in the other patients. Before splenectomy the disease-free interval varied from 3 weeks to 27 months and the incidence rate of relapses was 1.5 relapse/patient/year. None of the patients had relapses after splenectomy. The mean follow-up after splenectomy is 39 months with a range of 9-62 months. We conclude that patients with relapsing TTP can benefit from splenectomy, since it seems to increase disease-free intervals. Further investigation is necessary to understand the role of the spleen in the pathogenesis of TTP.

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“…Further, the use of a high-dose of IgG would be inappropriate to control TTP during pregnancy due to its high cost and the uncertainty of its effect. It also has been reported [15] that the cryosupernatant fraction of plasma is clinically effective against TTP. Moake et al [ 161 have reported that the cryosupernatant fraction is able to control the metabolism of the unusually large vWf multimers.…”

Section: Discussionmentioning

confidence: 94%

Efficacy of the high molecular weight fraction of plasma for the maintenance of pregnancy associated with thrombotic thrombocytopenic purpura

Koyama¹,

Suehiro²,

Kakishita³

et al. 1990

American J Hematol

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We have investigated the methods for the maintenance of a pregnancy in a patient with thrombotic thrombocytopenic purpura (TTP), said condition, since 1984, having been controlled by a plasma infusion every 3 to 4 weeks. In a preliminary trial it was confirmed that an infusion of the high molecular weight fraction (HMW-F) of plasma, separated by an Evaflux 2A fractionator, improved the patient's thrombocytopenia as the plasma infusion, and maintained its beneficial effect for about 2 weeks during early pregnancy. Though an occurrence of a toxemia-like syndrome responded to repeated plasma infusion, the dose of plasma required to improve the thrombocytopenia gradually increased and reached 5,040 ml by the 20th week of pregnancy. Thus, instead of periodic infusions of whole plasma, periodic infusions of the HMW-F of plasma were used. Under this regimen the platelet count remained above 10.0 x 10(4)/microliters during late pregnancy, and the total dose (2,600 ml) of HMW-F of plasma that was administered until delivery at full term was less than the dosage of whole plasma that was used during early pregnancy. In this manner we were able to obtain a healthy baby by controlling the patient's TTP during pregnancy. This method of preventing thrombocytopenia appears to be safer with respect to volume loading during pregnancy in the TTP patient.

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Studies of a patient with recurring thrombotic thrombocytopenic purpura

Cited by 30 publications

References 23 publications

Thrombotic thrombocytopenic purpura: Report of fourteen cases—occurrence during pregnancy and response to plasma exchange

Thrombotic thrombocytopenic purpura: Report of fourteen cases—occurrence during pregnancy and response to plasma exchange

The role of splenectomy in the treatment of relapsing thrombotic thrombocytopenic purpura

Efficacy of the high molecular weight fraction of plasma for the maintenance of pregnancy associated with thrombotic thrombocytopenic purpura

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