scholarly journals Stroke secondary to thrombotic microangiopathy

2021 ◽  
Author(s):  
João Vitor Ribeiro dos Santos ◽  
Mariana Spitz ◽  
Ana Carolina Andorinho

Introduction: Thrombotic thrombocytopenic purpura (TTP) is a hematological disease resulting from the ADAMTS 13 plasmatic protein deficit. It can be congenital or sporadic, and is usually autoimmune. Pathological platelet adhesion occurs, leading to microthrombi in capillary and arterial circulation, microangiopathic anemia and ischemia. The clinical picture includes thrombocytopenia, renal dysfunction, fluctuating neurological symptoms, microangiopathic hemolytic anemia, and fever. Methods: Case report of a 51-year-old male hypertensive patient, diagnosed with idiopathic thrombocytopenic purpura (ITP) 10 years ago and submitted to splenectomy 5 years ago, who developed acute cholecystitis. He underwent urgent colecistectomy, and on the fourth postoperative day presented sudden space and time disorientation, transcortical motor aphasia and right faciobrachial paresis, with ipsilateral Babinski and Hoffman signs. Results: Brain CT showed left frontoparietal hypodensity. During hospitalization, there was worsening of renal function, increased LDH, and thrombocytopenia. Hematoscopy identified signs of intravascular hemolysis (erythrocyte fragmentation, reticulocytosis, helmet erythrocytes). Direct Coombs was negative. There was no history of heparin use. TTP was diagnosed, and fresh frozen plasma and prednisone 1mg/kg were prescribed. There was resolution of thrombotic microangiopathy, with subsequent increase of platelet levels, decreased LDH and improved hematoscopy. Conclusions: This case illustrates a rare cause of stroke and an unusual association of two hematological conditions: ITP and TTP. The treatment of TTP consists of replacement of deficient ADAMTS13 protein through plasmapheresis or fresh frozen plasma. The use of immunosuppressants is also associated, initially with glucocorticoids, followed by rituximab or splenectomy in order to prevent recurrences.

Author(s):  
Hortensia De la Corte-Rodriguez ◽  
E. Carlos Rodriguez-Merchan ◽  
M. Teresa Alvarez-Roman ◽  
Monica Martin-Salces ◽  
Victor Jimenez-Yuste

Background: It is important to discard those practices that do not add value. As a result, several initiatives have emerged. All of them try to improve patient safety and the use of health resources. Purpose: To present a compendium of "do not do recommendations" in the context of hemophilia. Methods: A review of the literature and current clinical guidelines has been made, based on the best evidence available to date. Results: The following 13 recommendations stand out: 1) Do not delay the administration of factor after trauma; 2) do not use fresh frozen plasma or cryoprecipitate; 3) do not use desmopressin in case of hematuria; 4) do not change the product in the first 50 prophylaxis exposures; 5) do not interrupt immunotolerance; 6) do not administer aspirin or NSAIDs; 7) do not administer intramuscular injections; 8) do not do routine radiographs of the joint in case of acute hemarthrosis; 9) Do not apply closed casts for fractures; 10) do not discourage the performance of physical activities; 11) do not deny surgery to a patient with an inhibitor; 12) do not perform instrumental deliveries in fetuses with hemophilia; 13) do not use factor IX (FIX) in patients with hemophilia B with inhibitor and a history of anaphylaxis after administration of FIX. Conclusions: The information mentioned previously can be useful in the management of hemophilia, from different levels of care. As far as we know, this is the first initiative of this type regarding hemophilia.


Blood ◽  
1992 ◽  
Vol 79 (3) ◽  
pp. 826-831 ◽  
Author(s):  
B Horowitz ◽  
R Bonomo ◽  
AM Prince ◽  
SN Chin ◽  
B Brotman ◽  
...  

Abstract Fresh frozen plasma (FFP) is prepared in blood banks world-wide as a by- product of red blood cell concentrate preparation. Appropriate clinical use is for coagulation factor disorders where appropriate concentrates are unavailable and when multiple coagulation factor deficits occur such as in surgery. Viral safety depends on donor selection and screening; thus, there continues to be a small but defined risk of viral transmission comparable with that exhibited by whole blood. We have prepared a virus sterilized FFP (S/D-FFP) by treatment of FFP with 1% tri(n-butyl)phosphate (TNBP) and 1% Triton X-100 at 30 degrees C for 4 hours. Added reagents are removed by extraction with soybean oil and chromatography on insolubilized C18 resin. Treatment results in the rapid and complete inactivation of greater than or equal to 10(7.5) infectious doses (ID50) of vesicular stomatitis virus (VSV) and greater than or equal to 10(6.9) ID50 of sindbis virus (used as marker viruses), greater than or equal to 10(6.2) ID50 of human immunodeficiency virus (HIV), greater than or equal to 10(6) chimp infectious doses (CID50) of hepatitis B virus (HBV), and greater than or equal to 10(5) CID50 of hepatitis C virus (HCV). Immunization of rabbits with S/D-FFP and subsequent adsorption of elicited antibodies with untreated FFP confirmed the absence of neoimmungen formation. Coagulation factor content was comparable with that found in FFP. Based on these laboratory and animal studies, together with the extensive history of the successful use of S/D-treated coagulation factor concentrates, we conclude that replacement of FFP with S/D-FFP, prepared in a manufacturing facility, will result in improved virus safety and product uniformity with no loss of efficacy.


2013 ◽  
Vol 119 (4) ◽  
pp. 1050-1057 ◽  
Author(s):  
Marie Roguski ◽  
Kyle Wu ◽  
Ron I. Riesenburger ◽  
Julian K. Wu

Object A primary goal in the treatment of patients with warfarin-associated subdural hematoma (SDH) is reversal of coagulopathy with fresh-frozen plasma. Achieving the traditional target international normalized ratio (INR) of 1.3 is often difficult and may expose patients to risks of volume overload and of thromboembolic complications. This retrospective study evaluates the risk of mild elevations of INR from 1.31 to 1.69 at 24 hours after admission in patients presenting with warfarin-associated SDH. Methods Sixty-nine patients with warfarin-associated SDH and 197 patients with non–warfarin-associated SDH treated at a single institution between January 2005 and January 2012 were retrospectively identified. Charts were reviewed for patient age, history of trauma, associated injuries, neurological status at presentation, size and chronicity of SDH, associated midline shift, INR at admission and at hospital Day 1 (HD1), concomitant aspirin or Plavix use, platelet count, and medical comorbidities. Patients were stratified according to use of warfarin and by INR at HD1 (INR 0.8–1.3, 1.31–1.69, 1.7–1.99, and ≥ 2). The groups were evaluated for differences the in rate of radiographic expansion of SDH and in the rate of clinically significant SDH expansion resulting in death, unplanned procedure, and/or readmission. Results There was no difference in the rate of radiographic versus clinically significant expansion of SDH between patients not on warfarin and those on warfarin (no warfarin: 22.3% vs 20.3%, p = 0.866; warfarin: 10.7% vs 11.6%, p = 0.825), but the rate of medical complications was significantly higher in the warfarin subgroup (13.3% for patients who did not receive warfarin vs 26.1% for those who did; p = 0.023). For warfarin-associated SDH, there was no difference in the rate of radiographic versus clinically significant expansion between patients reversed to HD1 INRs of 0.8–1.3 and 1.31–1.69 (HD1 INR 0.8–1.3: 22.5% vs 20%, p = 1; HD1 INR 1.31–1.69: 15% vs 10%, p = 0.71). Conclusions Mild INR elevations of 1.31–1.69 in warfarin-associated SDH are not associated with a markedly increased risk of radiographic or clinically significant expansion of SDH. Larger prospective studies are needed to determine if subtherapeutic INR elevations at HD1 are associated with smaller increases in risk of SDH expansion.


2019 ◽  
Vol 2019 ◽  
pp. 1-3 ◽  
Author(s):  
Osamah Hasan ◽  
Ankit A. Patel ◽  
James J. Siegert

Recreational use of synthetic cannabinoids (SCs), also known as “K2” or “Spice,” is becoming a major public-health concern due to their potential for abuse and harmful consequences. New substances are constantly being added to the content of SCs. The dearth of information on these newly added contents as they are introduced into the black market hinders risk assessments of these compounds. We report a highly unusual case of gross hematuria in a 28-year-old male patient after SC use. He was found to have a supratherapeutic INR with no history of prior anticoagulation. His hematuria resolved after four units of fresh-frozen plasma were administered. We include a literature review of the clinical effects of SCs and their possible mechanism of gross hematuria and management.


2019 ◽  
Vol 12 (10) ◽  
pp. e229481
Author(s):  
Emma Tenison ◽  
Ashar Asif ◽  
Mathew Sheridan

Congenital thrombotic thrombocytopenic purpura (cTTP) is a rare, life-threatening disease, characterised by episodes of microangiopathic haemolytic anaemia (MAHA), thrombocytopenia and small vessel thrombosis. We describe a case of cTTP first diagnosed at age 70 years in a female presenting with an acute ischaemic stroke and thrombocytopenia, in whom A Disintegrin And Metalloproteinase with a Thrombospondin type 1 Motif, member 13 (ADAMTS13) levels were <10%, suggestive of thrombotic thrombocytopaenic purpura (TTP). The patient underwent plasma exchange and started rituximab for presumed immune TTP; however, anti-ADAMTS13 antibody titres were negative on two occasions. This, together with a history of pregnancies complicated by presumed disseminated intravascular coagulation, and two previous episodes of sepsis with MAHA, prompted investigation for cTTP, which was confirmed by genetic testing. Despite treatment with infusions of solvent/detergent-treated, virus-inactivated fresh frozen plasma, she has re-presented with further neurological deficit, associated with new infarcts on imaging. cTTP has a varied phenotype which, as demonstrated in this case, can include large vessel occlusion.


Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 4139-4139
Author(s):  
Marie Scully ◽  
Michael Flynn ◽  
Jenny Berryman ◽  
Samuel J. Machin

Abstract Thrombotic thrombocytopenic purpura (TTP) is an acute, life threatening disorder. The mainstay of treatment is plasma exchange (PEX) as a source of ADAMTS 13. In the UK, 20–25% of all plasma consumed is in patients with TTP. In our protocol (up until 31st December 2005) apheresis was initially with cryosupernatant (National Blood Service, UK) unless patients had a previous severe allergic reaction or refractory disease. Apheresis therefore continued with Solvent-Detergent Fresh Frozen Plasma (S/D FFP) Octaplas, (Octapharma, Vienna Austria) virally inactivated plasma, available throughout Europe. We reviewed 50 acute TTP episodes involving 32 patients. Thirteen episodes used cryosupernatant only and in 15 episodes, treatment started with cryosupernatant and changed to Octaplas. The reasons for changing were refractory disease in 2 episodes and major or recurrent allergic reactions to cryosupernatant in 13 cases. Once Octaplas had been used, it was continued on further admissions. In 22 episodes, Octaplas was used exclusively; in 4 cases as physicians choice and in the remaining due to previous reactions to cryosupernatant. The total volume of cryosupernatant used was 508250mls, 27.6% of all plasma; total volume of Octaplas was 1327600mls, 72.4% of all plasma. Citrate mediated reactions associated with symptomatic hypocalcaemia during apheresis were present in 11% of Octaplas and 20% of cryosupernatant. Acute or delayed urticarial or allergic reactions were noted in 5% of Octaplas and 10% cryosupernatant procedures. A particular complication of apheresis is central line infection. There were 21 line infections and in 43% of cases the infection was associated with a reduction in platelet count < 150 × 109/L. In all 50 episodes, the only documented thrombosis was a superficial non central vein in a patient who had received Octaplas. Prevention of venous thrombosis is by use of thromboembolic stockings, low dose aspirin and low molecular weight heparin in patients when platelet counts >50 × 109/L. In episodes receiving only cryosupernatant or Octaplas, there was no significant difference in the median number of PEX to remission, 7(3–14) and 8.5 (5–30) respectively. Baseline viral screen in all episodes was negative after discharge following an acute TTP episode. In conclusion: cryosupernatant and S/D FFP (Octaplas) appear equally efficacious. However, the risk of allergic/urticarial reactions was twice as common with cryosupernatant, as were citrate reactions. Milder allergic reactions to cryosupernatant are possibly higher, but may have been treated with antihistamines and data not recorded. There was no documented viral transmission with either product.


Sign in / Sign up

Export Citation Format

Share Document