Membrane Plasma Exchange for the Treatment of Thrombotic Thrombocytopenic Purpura

Pernat, Andreja Marn; Buturović‐Ponikvar, Jadranka; Kovač, Janko; Knap, Bojan; Premru, Vladimir; Benedik, M; Varl, J; Škofic, N; Gubenšek, Jakob; Kersnič, Boštjan; Ponikvar, Rafael

doi:10.1111/j.1744-9987.2009.00732.x

Cited by 4 publications

(4 citation statements)

References 15 publications

(22 reference statements)

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“…Plasma exchange therapy with fresh-frozen plasma is the most effective treatment for TTP. 3,4 Cyclophosphamide is used in chemotherapeutic regimens in a variety of malignancies and also during initial treatment of certain types of vasculitis but is not a first-line treatment for TTP. The patient does not have any indication for hemodialysis at this juncture; however, he may require hemodialysis with progression of TTP.…”

Section: Which One Of the Following Is The Recommended Initial Treatmmentioning

confidence: 99%

46-Year-Old Man With Fatigue and Brown Urine

Mao

Hoyt

Nyman

2011

Mayo Clinic Proceedings

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A 46-year-old man presented for evaluation of a 3-day history of progressive fatigue, malaise, brown urine, and spontaneous ecchymosis of the right arm associated with anorexia and bilious emesis. He denied any traumatic injury, recent illness, fever, weight loss, night sweats, abdominal pain, or sick contacts.The patient was previously healthy, and his medical history was notable only for obesity and erectile dysfunction. Current medications included sildenafil and varenicline, and he denied any medication allergies. He was a current smoker with a 26 pack-year smoking history and occasionally used alcohol and marijuana (last use, approximately 3 weeks previously). He was married in a monogamous relationship and worked as a real estate broker. He had not traveled outside the United States recently.At presentation, his vital signs were as follows: temperature, 37.4˚C; heart rate, 85 beats/min; respiratory rate, 20 breaths/min; and blood pressure, 140/85 mm Hg. Physical examination was remarkable for a 5 × 5 cm dark ecchymosis on the right medial arm and scattered petechiae on the soft palate and lower extremities. His mucous membranes were dry, the jugular venous pressure was low, and slight scleral icterus was present. Findings on the remainder of the comprehensive physical examination, including mental status and neurologic examination, were within normal limits.Initial laboratory results were as follows (reference ranges provided parenthetically): hemoglobin, 15.4 g/dL (13.5-17.5 g/dL); leukocytes, 10.6 × 10 9 /L (3.5-10.5 × 10 9 /L) with a normal differential; platelets, 6 × 10 9 /L (150-450 × 10 9 /L); reticulocytes, 2.6% (0.60%-1.83%); international normalized ratio, 1.0; partial thromboplastin time, 25 s (21-33 s); blood urea nitrogen, 56 mg/dL (8-24 mg/ dL); creatinine, 3.1 mg/dL (0.8-1.2 mg/dL); alkaline phosphatase, 122 U/L (45-115 U/L); aspartate aminotransferase (AST), 95 U/L (8-48 U/L); alanine aminotransferase, 25 U/L (7-55 U/L); total bilirubin, 5.0 mg/dL (0.1-1.0 mg/ dL); and direct bilirubin, 0.7 mg/dL (0.0-0.3 mg/dL). The urine appeared brown, and urinalysis revealed +3 blood, +3 protein, 3 to 5 nondysmorphic red blood cells per highpowered field, and no leukocytes or casts. Which one of the following tests would be most appropriate at this time? a. b. c. d. e.Abdominal ultrasonography is a noninvasive study that can identify hepatobiliary obstruction and characterize liver parenchyma in a patient with elevated transaminase and bilirubin levels. However, this patient has an hyperbilirubinemia and not the hyperbilirubinemia characteristic of biliary obstruction. Rhabdomyolysis with accompanying elevated creatine kinase and urinary myoglobin levels can cause brown discoloration of the urine, but acute rhabdomyolysis 1 would likely be associated with a greater elevation in AST levels and would not explain the thrombocytopenia and hyperbilirubinemia. Cryoglobulins are immunoglobulins plus or minus complement components that can be associated with a small-to mediumvessel vasculitis caused by immune co...

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Section: Which One Of the Following Is The Recommended Initial Treatmmentioning

confidence: 99%

46-Year-Old Man With Fatigue and Brown Urine

Mao

Hoyt

Nyman

2011

Mayo Clinic Proceedings

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“…Plasma exchange and immunosuppression have been found to be effective treatment in patients with TTP. In a retrospective analysis of 11-year experience on TTP, 93% were reported to have excellent response to early initiation of plasma exchange while 86% reached complete remission [2]. Similarly, 55% of patients achieved remission with corticosteroids alone, in a study conducted prior to systematic use of plasma exchange in TTP [3].…”

Section: Introductionmentioning

confidence: 99%

Cytomegalovirus induced refractory TTP in an immunocompetent individual: a case report

et al. 2019

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Background Thrombotic thrombocytopenic purpura (TTP) is a rare, potentially fatal disease with multisystem involvement. Cytomegalovirus (CMV) infection as a cause of refractory TTP, has been reported only in immunocompromised individuals. We report a case of CMV-induced refractory TTP in an immunocompetent individual. Case presentation A 35-year-old, previously healthy Sri Lankan man, presented with fever for 3 days with gum bleeding and progressive drowsiness. His Glasgow coma scale score was 10/15. He did not have papilloedema or neck stiffness. Laboratory evaluation showed a severe thrombocytopenia with microangiopathic haemolytic anaemia. There was marginal renal impairment and normal coagulation profile. Non-contrast CT scan of brain was normal. A diagnosis of thrombotic thrombocytopenic purpura was made. Despite daily plasma exchanges and high-dose steroids, he failed to achieve the expected therapeutic response, thus demonstrating refractory TTP. On exploring for possible causes of refractoriness to treatment, a clinically significant PCR titre of CMV was detected. Treatment of CMV infection lead to complete recovery of TTP. His disease course was further complicated with spontaneous spinal haemorrhage leading to neurological sequelae. Discussion and conclusions This is the first report of CMV induced refractory TTP in an immunocompetent adult. It is also the first report of clinically significant spontaneous spinal haematoma in TTP. These two rare occurrences should be considered when patients with refractory TTP do not improve as expected.

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“…In patients refractory to plasma exchange, using cryopoor plasma (cryosupernatant) can sometimes lead to a response. This is FFP that has had the cryoprecipitate removed and is thus depleted of high-molecular weight von Willebrandmultimers, which have a pathogenic role in TTP (9). Daily PEX should continue for a minimum of 2 days after platelet count has been >150 x 10(9)/l and then stopped.…”

Section: Managementmentioning

confidence: 99%

Thrombotic Thrombocytopenic Purpura (Syndroma Moschkowitz), Severe Disease with Potential Fatal Outcome

2016

IJSR

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Thrombotic thrombocytopenic purpura (TTP) is a rare disease with an untreated mortality rate of 90%. The disease consists of the pentad of microangiopatic hemolytic anemia, thrombocytopenic purpura, neurologic abnormalities, fever and renal failure. TTP, inherited or autoimmune, is mainly caused by the plasma deficiency of the von Wilebrand factor cleaving protease, ADAMTS13, owing to gene mutations or autoantibodies. The prompt delivery of plasma exchange (PEX) has reduced the mortality rate to 10-20%. Daily PEX should continue for a minimum of 2 days after platelet count has been >150 x 10(9)/l. In refractory cases, the use of anti CD20 monoclonal antibody is recommended. Vincristine also has been shown to be useful in refractory patients.

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Membrane Plasma Exchange for the Treatment of Thrombotic Thrombocytopenic Purpura

Cited by 4 publications

References 15 publications

46-Year-Old Man With Fatigue and Brown Urine

46-Year-Old Man With Fatigue and Brown Urine

Cytomegalovirus induced refractory TTP in an immunocompetent individual: a case report

Thrombotic Thrombocytopenic Purpura (Syndroma Moschkowitz), Severe Disease with Potential Fatal Outcome

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