Relapsing or refractory idiopathic thrombotic thrombocytopenic purpura‐hemolytic uremic syndrome: the role of rituximab

Caramazza, Domenica; Quintini, Gerlando; Abbene, Ignazio; Malato, Alessandra; Saccullo, Giorgia; Coco, Lucio Lo; Trapani, Rosa Di; Palazzolo, Roberto; Barone, Rita; Mazzola, Giuseppina; Rizzo, Sérgio; Ragonese, Paolo; Aridon, Paolo; Abbadessa, Vincenzo; Siragusa, Sergio

doi:10.1111/j.1537-2995.2010.02763.x

Cited by 66 publications

(54 citation statements)

References 68 publications

(194 reference statements)

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“…[34][35][36][37] Most of the data are based on case reports and case series where rituximab was used concomitantly with other immunosuppressive agents. 23,34,[36][37][38][39][40][41][42] A recent thoughtful review on this topic adds some clarity to its use in this clinical situation.…”

Section: Rituximabmentioning

confidence: 99%

How I treat refractory thrombotic thrombocytopenic purpura

Sayani¹,

Abrams

2015

Blood

143

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Acquired thrombotic thrombocytopenic purpura (TTP) is characterized by thrombocytopenia and microangiopathic hemolytic anemia (MAHA) without an obvious cause, and may include fever, mild renal failure, and neurologic deficits. It is characterized by a deficiency of the von Willebrand factor (VWF) cleaving enzyme, ADAMTS13 (a disintegrin and metalloproteinase, with a thrombospondin type 1 motif, member 13), resulting in formation of microthrombi in the high sheer environment of the microvasculature. This causes microvascular occlusion, MAHA, and organ ischemia. Diagnosis is based on the presence of clinical symptoms, laboratory aberrations consistent with MAHA, decreased ADAMTS13 activity, and possibly presence of anti-ADAMTS13 autoantibodies. Upfront treatment of acute TTP includes plasma exchange and corticosteroids. A significant number of patients are refractory to this treatment and will require further interventions. There are limited data and consensus on the management of the refractory TTP patient. Management involves simultaneously ruling out other causes of thrombocytopenia and MAHA, while also considering other treatments. In this article, we describe our management of the patient with refractory TTP, and discuss use of rituximab, increased plasma exchange, splenectomy, and immunosuppressive options, including cyclophosphamide, vincristine, and cyclosporine. We also review recent evidence for the potential roles of bortezomib and N-acetylcysteine, and explore new therapeutic approaches, including recombinant ADAMTS13 and anti-VWF therapy. (Blood. 2015;125(25):3860-3867) Case A 25-year-old previously healthy woman presented with a 3-day history of fatigue, nausea, abdominal pain, and easy bruising. She was alert, oriented, afebrile, had mild abdominal tenderness, and purpura on her extremities. Her hemoglobin was 8.4 g/dL; platelets, 15 3 10 9 /L; creatinine, 1.1 mg/dL; and her lactate dehydrogenase and reticulocytes were increased. Schistocytes and nucleated red blood cells were easily seen on her peripheral blood smear. In the absence of other obvious precipitators of thrombocytopenia and microangiopathic hemolytic anemia (MAHA), she was diagnosed with acquired thrombotic thrombocytopenic purpura (TTP). Blood samples were sent for ADAMTS13 (a disintegrin and metalloproteinase, with a thrombospondin type 1 motif, member 13) activity and inhibitor levels, and the patient was immediately started on plasma exchange (PEX) and prednisone 1 mg/kg per day. She responded well initially, and her platelets rose to 105 000/mL on day 4. However, on day 5, she was febrile and her platelets dropped to 40 000/mL. BackgroundAcquired TTP was initially characterized by thrombocytopenia, MAHA, renal failure, neurologic deficits, and fever. However, it is now well accepted that neither renal failure nor high fevers are key diagnostic features. Thrombocytopenia and MAHA are required for diagnosis when TTP is suspected. TTP is a hematologic emergency, with a mortality of 90% if untreated. Treatment with PEX and cortico...

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Section: Rituximabmentioning

confidence: 99%

How I treat refractory thrombotic thrombocytopenic purpura

Sayani¹,

Abrams

2015

Blood

143

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“…The diagnosis of PRES includes the presence of neurological symptoms such as seizure, headache, encephalopathy, and visual disturbances, in addition to radiologic findings on brain MRI of focal vasogenic edema [1][2][3][4][5][6][7]. The syndrome was described in association with acute hypertension, preeclampsia or eclampsia, renal disease, sepsis, and exposure to immunosuppressive agents [8][9][10][11][12][13][14].…”

Section: Discussionmentioning

confidence: 99%

Rituximab-related posterior reversible encephalopathy syndrome in HUS patient post kidney transplant

Habli¹,

Elyoussef²,

Khoury³

et al. 2016

IJCRI

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International Journal of Case Reports and Images (IJCRI) is an international, peer reviewed, monthly, open access, online journal, publishing high-quality, articles in all areas of basic medical sciences and clinical specialties.Aim of IJCRI is to encourage the publication of new information by providing a platform for reporting of unique, unusual and rare cases which enhance understanding of disease process, its diagnosis, management and clinico-pathologic correlations. IJCRI publishes Review

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“…It has also been reported that, in patients with primary refractory or relapsing de novo TTP-HUS, administration of intravenous immunoglobulin, anti-CD20 antibody, rituximab, and belatacept in combination with plasma exchange may also had some benefits in terms of improvment (16)(17)(18)(19). These drugs were not available in our center and we do not have access to them.…”

Section: Discussionmentioning

confidence: 93%