Malignancy and thrombotic microangiopathy or atypical haemolytic and uraemic syndrome?

Favrè, Guillaume; Touzot, Maxime; Frémeaux‐Bacchi, Véronique; Hyvernat, H.; Rohrlich, Pierre‐Simon; Queyrel, V.; Esnault, Vincent; Fakhouri, Fádi

doi:10.1111/bjh.12907

Cited by 11 publications

(4 citation statements)

References 10 publications

(9 reference statements)

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“…A growing number of case reports suggest potential benefit of eculizumab but carry the bias of preferential publication of positive results. [21][22][23][24][25][26] A recent retrospective study reviewed 29 cases of secondary HUS treated with eculizumab in 11 Spanish nephrology centers. 12 HUS was mainly drug-induced (n ¼ 15) or related to systemic diseases (n ¼ 8) and was severe, as 14 patients (52%) required dialysis and 11 (38%) presented with extrarenal manifestations (mainly neurologic).…”

Section: Discussionmentioning

confidence: 99%

Atypical and secondary hemolytic uremic syndromes have a distinct presentation and no common genetic risk factors

Clech

Simon-Tillaux

François

et al. 2019

Kidney International

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Section: Discussionmentioning

confidence: 99%

Atypical and secondary hemolytic uremic syndromes have a distinct presentation and no common genetic risk factors

Clech

Simon-Tillaux

François

et al. 2019

Kidney International

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“…Nonetheless, in our case study above, the patient had normal ADAMTS13 level and did appear to have partial response from therapeutic plasma exchange with marked decline in both LDH and bilirubin while on exchange therapy along with decrease in transfusion requirements indicating improvement in the underlying hemolytic process. While there is no clear role for eculizumab in hemolysis of malignancy due to lack of demonstrated association between CA-MAHA and complement antibody treatment in two patients for whom hemolytic anemia was diagnosed prior to diagnosis of malignancy [12]. Management for microangiopathic hemolytic anemia of malignancy primary involves control of the underlying malignant process.…”

Section: Discussionmentioning

confidence: 99%

“…Hemolytic anemia of malignancy has a very poor prognosis, with a nearly 50% mortality rate within 1 month of diagnosis [3, 12, 15]. Response to chemotherapy may induce a complete remission after just one cycle, but relapse is frequent due to the presence of metastatic disease.…”

Section: Discussionmentioning

confidence: 99%

Hemolytic Anemia of Malignancy: A Case Study Involving Signet Ring Cell Metastatic Breast Cancer with Severe Microangiopathic Hemolytic Anemia

et al. 2019

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Hemolytic anemia in the setting of malignancy is a rare manifestation of paraneoplastic syndrome with significant morbidity. Here we discuss a case involving metastatic breast cancer presenting with severe hemolytic anemia and renal failure secondary to thrombotic microangiopathy of malignancy. This case discusses the workup for secondary hemolytic anemia, a possible role for therapeutic plasma exchange in this setting, as well the current understanding of the management of microangiopathic hemolytic anemia of malignancy.

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“…33 Of note, mutations in factor H have also been identified, similar to the findings in TMA-hematopoietic stem cell transplantation associated and TMA-complement associated; these findings indicate that underlying abnormalities in the regulation of the alternate complement pathway may be involved. 34 The usual course of therapy again matches that for TTP as described in Table 2. Evidence supporting efficacy of TPE in this setting is lacking, and the use of TPE may result in a delay in treating the underlying malignancy.…”

Section: Tma-malignancy Associatedmentioning

confidence: 99%

Plasma exchange in thrombotic microangiopathies (TMAs) other than thrombotic thrombocytopenic purpura (TTP)

Winters

2017

Hematology

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Thrombotic microangiopathies (TMAs) are a diverse group of disorders that are characterized by common clinical and laboratory features. The most commonly thought-of TMA is thrombotic thrombocytopenic purpura (TTP). Because of the marked improvement in patient mortality associated with the use of therapeutic plasma exchange (TPE) in TTP, this therapy has been applied to all of the TMAs. The issue, however, is that the pathophysiology varies and in many instances may represent a disorder of the endothelium and not the blood; in some cases, the pathophysiology is unknown. The use of TPE is further obscured by a lack of strong supporting literature on its use, with most consisting of case series and case reports; controlled or randomized controlled trials are lacking. Evidence supporting the use of TPE in the treatment of TMAs (other than TTP and TMA–complement mediated) is lacking, and therefore its role is uncertain. With the greater availability of genetic testing for mutations involving complement regulatory genes and complement pathway components, there seems to be a percentage of TMA cases, other than TMA–complement mediated, in which complement pathway mutations are involved in some patients. The ability of TPE to remove abnormal complement pathway components and replace them with normal components may support its use in some patients with TMAs other than TTP and TMA–complement mediated.

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Malignancy and thrombotic microangiopathy or atypical haemolytic and uraemic syndrome?

Cited by 11 publications

References 10 publications

Atypical and secondary hemolytic uremic syndromes have a distinct presentation and no common genetic risk factors

Atypical and secondary hemolytic uremic syndromes have a distinct presentation and no common genetic risk factors

Hemolytic Anemia of Malignancy: A Case Study Involving Signet Ring Cell Metastatic Breast Cancer with Severe Microangiopathic Hemolytic Anemia

Plasma exchange in thrombotic microangiopathies (TMAs) other than thrombotic thrombocytopenic purpura (TTP)

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