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Thrombotic thrombocytopenic purpura is a rare disease in pediatrics, but it has a high mortality if not managed in an adequate and timely manner. It is characterized by microangiopathic hemolytic anemia associated with neurological, cardiac, abdominal, and less frequently, renal signs and symptoms; it may be accompanied by fever. In children, diagnosis is based on clinical and laboratory findings. ADAMTS13 activity < 10% supports the diagnosis but does not confirm it and, given its severity, the result should not delay treatment initiation. Here we describe the case of a previously healthy 15-year-old female patient with neurological signs associated with hemolytic anemia and thrombocytopenia. During hospitalization, she was diagnosed with acquired thrombotic thrombocytopenic purpura., (Sociedad Argentina de Pediatría.)

Text: Acquired or immune thrombotic thrombocytopenic purpura (TTP) are thrombotic microangiopathies associated with high mortality if treatment is not started early. Onset is usually sudden, meaning that the condition is often diagnosed in hospital emergency departments, where TTP must be suspected as early as possible. These guidelines were drafted by specialists in emergency medicine and hematology to cover the diagnosis, referral, and treatment of patients suspected of immune-mediated TTP who require emergency care. Immune TTP should be suspected whenever a patient presents with hemolytic microangiopathy and has a negative Coombs test, and thrombocytopenia, possibly in conjunction with fever and neurologic and cardiac alterations. If one of the existing diagnostic algorithms indicates there is a high probability that the patient has immune TTP, plasma exchange therapy should be started along with immunosuppressants. Treatment with caplacizumab should also be considered. The patient should be referred immediately to the hematology department within the same hospital or a referral hospital.

Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy (TMA) characterized by the development of microangiopathic haemolytic anaemia, thrombocytopenia, and ischaemic organ dysfunction associated with ADAMTS13 levels lower than 10% in most cases. Recently there have been numerous advances in the field of PTT, new, rapid and accessible techniques capable of quantifying ADAMTS13 activity and inhibitors. The massive sequencing systems facilitate the identification of polymorphisms in the ADAMTS13 gene. In addition, new drugs such as caplacizumab have appeared and relapse prevention strategies are being proposed with the use of rituximab. The existence of TTP patient registries allow a deeper understanding of this disease but the great variability in the diagnosis and treatment makes it necessary to elaborate guidelines that homogenize terminology and clinical practice. The recommendations set out in this document were prepared following the AGREE methodology. The research questions were formulated according to the PICO format. A search of the literature published during the last 10 years was carried out. The recommendations were established by consensus among the entire group, specifying the existing strengths and limitations according to the level of evidence obtained. In conclusion, this document contains recommendations on the management, diagnosis, and treatment of TTP with the ultimate objective of developing guidelines based on the evidence published to date that allow healthcare professionals to optimize TTP treatment., (Copyright © 2021 Elsevier España, S.L.U. All rights reserved.)

Hematological manifestations for human immunodeficiency virus (HIV) infection are frequent and diverse due to its ability to affect almost all cell lines. Among these, thrombotic thrombocytopenic purpura (TTP) is one of the thrombotic microangiopathies syndromes, characterized by the presence of thrombocytopenia and microangiopathic hemolytic anemia with impaired renal function. Nowadays, the relationship between these two entities is rare given the current highly active antiretroviral therapy (HAART). We report the case of a 28-year-old patient, who presented with fever associated with gingival bleeding, generalized mucocutaneous pallor and progressive weakness. Routine investigations showed anemia and severe thrombocytopenia, schistocytes and micro spherocytes in peripheral blood smear. Required blood transfusion, with decreased ADAMTS 13 enzyme activity (6.8%). With these findings,TTP was diagnosed as the initial manifestation of the HIV infection. The patient received management with five sessions of plasmapheresis and HAART with subsequent improvement.

Tjalma syndrome or pseudo-pseudo Meigs' syndrome is a clinical condition characterized by pleural effusion, ascites and elevated CA-125 with no associated benign or malignant ovarian tumor in a patient with systemic lupus erythematosus (SLE). Tjalma described the first case of a patient with SLE, pleural effusion, ascites and elevated CA-125. We report the first case in a 14-year old patient who presented with ascites and pleural effusion refractory to treatment and elevated CA-125, in the absence of an ovarian tumor, that warranted aggressive management., (Copyright © 2017 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.)

Background and Aim: The term thrombotic microangiopathy (TMA) involves a heterogeneous group of diseases that can be overwhelming or invalidating, with an acute development, characterised by microangiopathic haemolytic anaemia and thrombocytopaenia. Its management during its initial hours is essential to improving the prognostic of these patients. The aim of this review is to give recommendations about the optimisation of TMA initial treatment and to accelerate the aetiological diagnosis., Patients and Methods: We provide a practice guideline based on four steps for the initial management of TMA: diagnosis of suspicion, syndromic confirmation, emergent treatment and complementary tests., Results: The detection of microangiopathic haemolytic anaemia (characterised by elevated reticulocytes, LDH and indirect bilirubin, negative direct Coombs test and schistocytes in peripheral blood), and thrombocytopaenia not explained by other secondary aetiologies confirm the syndromic diagnosis of microangiopathic haemolytic anaemia and thrombocytopaenia (MAHAT). These patients require admission to an Intensive Care Unit to initiate plasma exchange therapy as soon as possible, ideally within the first 4-8hours. Prior to this, samples for ADAMTS13 and complement study should be obtained. Finally, it is important to request the complementary tests necessary to have a correct aetiological diagnosis., Conclusions: Adherence to the agreed recommendations in this guideline will improve therapeutic results by facilitating cooperation between different specialists involved in TMA management., (Copyright © 2018 Elsevier España, S.L.U. All rights reserved.)

Thrombotic thrombocytopenic purpura (TTP) is the most extensive and dangerous intravascular platelet clumping disorder. For more than a half-century after its initial recognition, mortality was near 100% and the etiology totally obscure. Then, in the late 1970s to early 1980s, empiric, but successful, therapy with plasma exchange was followed by sudden laboratory insight into pathophysiology. The most important finding was the identification of a novel metalloprotease, named ADAMTS13, which is involved in the regulation of the size of von Willebrand factor. Inherited or acquired deficiencies of ADAMTS13 impair von Willebrand factor cleavage, leading to the disseminated formation of platelet-rich thrombi in the microcirculation and to symptoms of end-organ ischemia. Treatment with plasma exchange, available for more than 20 years, has dramatically altered the course of disease in adults with TTP. Long term follow-up has revealed increasing frequencies of relapse that require new therapeutic alternatives for these patients., (Copyright © 2009 Elsevier España, S.L. All rights reserved.)

We discuss the plasmapheresis (PE) carried out in an 18-bed polyvalent intensive care unit between the years 2003-2007. This article aims to report our experience in plasmapheresis performed with specific monitors for continuous renal replacement therapy (CRRT) that shows the versatility of the use of these procedures in intensive care. The utility of these procedures include many different critical disease settings in our units (neurology, hematology, and rheumatology patients). In short, our experience in PE has led us to the conclusion that plasmapheresis is a simple extracorporeal depuration treatment that can be performed by staff trained in intensive care at any moment within a wide spectrum of clinical indications, with CRRT monitors and with minimum adverse effects., (Copyright 2009 Elsevier España, S.L. y SEMICYUC. All rights reserved.)

Background: Thrombotic thrombocytopenic purpura (TTP) is characterized by anemia, thrombocytopenia, neurological and renal involvement of variable severity and it has a dismal prognosis. Platelet-derived von Willebrand Factor-cleaving metalloprotease ADAMTS-13 activity may orient the diagnosis, but normal levels do not discard it. The most effective therapy thus known is plasmapheresis., Aim: To report the experience in 18 patients with TTP., Material and Methods: Retrospective assessment of 11 patients and prospective assessment of seven subjects with TTP, aged 15 to 81 years., Results: All presented with anemia, thrombocytopenia and LDH elevation. Sixteen had neurological symptoms, five had fever, four had macroscopic urinary excretion of pigments, four had petechiae, and two had nosebleeds. Haptoglobin was low in 10 of 11 patients in whom it was measured. ADAMTS-13 had low activity in 15 of 17 patients (in 11, the inhibitor was found). Seventeen patients were treated with plasmapheresis and nine received steroids also. Seven patients died due to shock with respiratory involvement or multiple organic failure., Conclusions: TTP has heterogeneous modes of presentation. If the diagnosis is strongly suspected, plasmapheresis can be started without laboratory confirmation. An ADAMTS-13 activity below 6% is almost exclusive of TTP .

Neurological manifestations, secondary to perfusion problems, vasogenic edema or small infarcts, are common in thrombotic purpura. Moreover, they are the first symptoms of the disease in 50% of patients. We report a 50 year-old woman who presented with focal intermittent neurological signs with aphasia and right hemiparesis, who then developed progressive impairment of consciousness with stupor and generalized tonic-clonic seizures. Despite the severe neurological impairment, first neuroimaging studies were normal. A second magnetic resonance imaging showed small cortical infarcts, that were visible only with the technique of diffusion weighted imaging. The standard electroencephalograms showed focal left temporal slowing and low voltage first and then diffuse slowing accordind to the clinical condition of the patients. She was treated with plasmapheresis and had a partial neurological recovery at the fifth day, but died at the twelfth day of therapy.

Thrombotic microangiopathic hemolytic anemias include thrombotic thrombocytopenic purpura (TTP), hemolytic uremic syndrome (HUS) and pregnancy associated thrombotic microangiopathy (TMA). Eight adult patients (four males and four females) with TMA who were treated between 2003 and 2004 at the Hospital Italiano de Buenos Aires were reviewed. The average age was 40. Clinical diagnosis of TMA was made on admission in four patients. During their stay in hospital, 4 patients developed HUS characteristics, three as TTP and one presented pregnancy associated TMA. All of them revealed thrombocytopenia and microangiophatic hemolytic anemia. Renal impairment was the third most frequent characteristic at presentation. The patients with TTP revealed the most severe condition. All patients received daily plasma exchange. Immunosuppressants were also used. Four patients recovered completely, 2 passed away, one remains with renal impairment and requires hemodialysis, and a colectomy was performed on one of them. The TMA syndromes are occlusive disorders associated to platelet microvascular thrombi. Systemic and renal circulations are primarily affected. TTP/HUS might represent an overlapping spectrum of idiopathic or secondary disease. Prompt recognition and treatment are vital, because high mortality occurs due to these disorders. Among the differential diagnosis of TMA we can refer to sepsis, neoplasms, systemic vasculitis, eclampsia and others. The mainstay treatments are daily plasma exchange and infusion with fresh frozen plasma. Improving the management of these diseases is required considering their high morbidity and mortality.

Background: Plasmapheresis is a therapeutic alternative for diseases in which a "humoral factor" has pathogenetic relevance. However it is not devoid of adverse effects., Aim: To review the indications, number of procedures, morbidity and clinical evolution of plasmapheresis in critical patients., Patients and Methods: A retrospective and descriptive study in four intensive care units of an University hospital. The severity of patients was evaluated with APACHE II and SOFA scores., Results: Twenty patients were studied. The most common indications of plasmapheresis were thrombotic thrombocytopenic purpura (TTP) in 50% of subjects and small vessel vasculitides in 30%. The number of procedures per patient oscillated between 2 and 14 (mean: 7.1+/-3.3). The registered adverse effects were hypocalcemia in 50% of patients, hypotension in 42.1%, coagulopathy in 35%, hypokalemia in 29%, rash in 20%, procedure related infections in 18% and fever in 10%. There was a significant decrease of 17+/-28% in prothrombin time, after the procedures. Seventy five percent of patients had a favorable evolution. Global mortality rate was 15%. All deaths occurred in patients with TTP and were attributed to the progression of the disease. No death was attributed to the procedure. The initial APACHE II and SOFA scores were 12.4+/-8.4 and 5.3+/-2.9, respectively. Both scores decreased after the procedure. Among other therapeutic measures, 15% of the patients received immunosuppressant treatment, 27% were dialyzed and 32% were mechanically ventilated., Conclusions: The most common indication of plasmapheresis was TTP. Adverse effects were frequent, however there was no procedure related mortality. The global mortality rate was 15% and all deaths occurred in patients with TTP.

Objective: Thrombotic thrombocytopenic purpura (TTP) or Moschovitz' syndrome is rare and is even rarer in childhood. Clinically, it is characterized by microangiopathic hemolytic anemia, thrombocytopenia, neurologic abnormalities, fever and renal dysfunction. The etiology is still unknown, although different factors such as large von Willebrand factor multimers and prostacyclin have been implicated. The acute form is more frequent, and in most cases the course is fulminant if treatment is not initiated. Laboratory data typically reveal hemolytic anemia, with schistocytes on the peripheral smear, diminished serum haptoglobin, and thrombocytopenia., Material and Methods: We present the clinical cases of two children, aged 4 and 7 respectively, with TTP, but with different evolution and treatment. Evolution was favorable in both patients. The first child recovered spontaneously. In the second plasmapheresis was required and produced remission of all the symptomatology. Normality has been maintained for 36 and 24 months respectively, and the children have presented no clinico-biological alterations.

A 64-year-old woman came to the emergency room complaining of vomiting and abdominal pain; appendicitis was suspected and surgery ordered. A blood work up showed a significantly low platelet count (39,000/microliter) and 6 units were transfused before surgery. The only observations during surgery were ileitis and Meckel's diverticulum. Thrombocytopenia persisted over the first 48 hours after surgery in spite of another transfusion of platelets, with worsening awareness and acute renal failure. After diagnosis of thrombotic thrombocytopenic purpura (TTP), the patient was admitted to the intensive care unit and treatment with fresh plasma and corticoids was started. Two weeks later, after complex evolution and ten sessions of plasmapheresis, the patient was transferred to the hematology ward. TTP must be considered a medical emergency. Platelet transfusions are contraindicated, as they can cause serious clinical deterioration. A low platelet count before surgery should lead to differential diagnosis to determine the cause, with the aim of judging whether platelet transfusion is warranted or not. In some etiological processes, such as in the case we report, platelet transfusion may be life-threatening; corrective measures must be taken early in the process and such transfusion avoided.

The present article describes the case of a woman, aged 25 years, who the non-specific and variable neurologic symptomatology and the confusional episodes observed at admission required psychiatric care until the diagnosis, and subsequent treatment, of thrombotic thrombocytopenic purpura (TTP) was done. The variable and unstable manifestations induce "to interpret the disease psychologically", we must not forget the importance of the somatic assessment in psychiatry to prevent premature and mistaken conclusions that delay a correct diagnosis and, therefore, an adequate treatment.

Objective: To assess the fresh-frozen plasma (FFP) transfusion indications in a Mexican hospital., Setting: A general hospital for federal government employees located in the city of Morelia, State of Michoacan, Mexico., Material and Methods: We evaluated all FFP transfusions between February and August, 1998 classifying them as appropriate or inappropriate according to the recent FFP-transfusion guidelines: correction of known coagulation factor deficiencies with bleeding and correction of microvascular bleeding when prothrombin and activated partial thromboplastin time are > 1.6 times normal (coagulation index o CI), urgent reversal of warfarin therapy, antithrombin III deficiency, thrombotic thrombocytopenic purpura treatment, liver transplant and acute disseminated intravascular coagulation., Results: 78 patients with 292 FFP units transfused were analyzed: in 20 patients the indication was clotting support, 16 with CI < 1.5 and four with CI > 1.6, one with blood loss and one with surgical procedure; hypoalbuminemia in 10; hypovolemia in eight; unidentified reason in 33 and others in seven patients. Eleven units (3.76%) were considered properly transfused whereas 281 (96.23%) were inadequately indicated.

Thrombotic thrombocytopenic purpura (TTP) is a clinical syndrome characterized by microangiopathic hemolytic anemia, thrombocytopenia, fever, neurological symptoms, and renal involvement. The relationship of TTP to systemic lupus erythematosus (SLE) has been recognized in the medical literature since 1939. The differential diagnosis is difficult because both diseases have similar clinical features. The mainstay for recognizing TTP in the context of active SLE is the presence of helmet red cells, marked reticulocytosis, and negative direct Coomb's test. We report three female patients with simultaneous presentation of TTP and SLE. We suggest combined treatment with immunosuppressive therapy and plasma exchange using fresh frozen plasma.

Background: Thrombotic Thrombocytopenic Purpura (TTP) is an uncommon clinical syndrome with high mortality rate in the absence of treatment. Despite the therapeutic efficacy of plasma exchange, patients often relapse even after long periods of time in remission. Over the last few years, late relapses in previously diagnosed patients have been seen in our hospital., Patients and Methods: We analyzed retrospectively 22 episodes of TTP in 16 patients diagnosed during a four-year period. We reviewed the clinical features at diagnosis as well as the therapeutic results. In all but one, the treatment included daily plasma exchange. Other treatments, including vincristine, were also used in addition to plasma exchange, in 18 of 21 episodes., Results: A complete remission was obtained in eighty-two percent of the episodes treated by plasma exchange. The median number of plasma exchange to achieve a complete remission was 6. In 4 episodes, 20 or more plasma exchange were required before achieving a satisfactory response. A complete remission was obtained in 78% of episodes where vincristine was used, versus 84% response rate in episodes where vincristine was not used. In four patients the cause death was directly related to TTP, while a fifth patients died of progressive lymphoma without evidence of TTP. Five of the eleven surviving patients relapsed with a median time to relapse of 24.6 months. Relapsing episodes presented with a less severe clinical picture including less clear signs of microangiopathic hemolytic anemia (MAHA), when compared with the initial ones. All patients in relapse responded promptly to treatment. The variables analyzed failed to predict either the response to treatment or the probability of relapse., Conclusions: The therapeutic efficacy of plasma exchange in the treatment of TTP has been demonstrated in our series as previously observed by many other groups. We have observed some slow responders where the prolongation of treatment by plasma exchange succeeded in achieving a complete remission. The use of vincristine did not show any therapeutical advantage in our experience.

In the past years, more than thirty cases of thrombotic thrombocytopenic purpura (TTP) had been described associated to infection by the human immunodeficiency virus. Some authors have suggested the presence of a causal relationship between both entities, although the common nexus is still unknown. It usually has a fulminant onset, affecting all the risk groups and in any stage of the disease. The clinical manifestations are similar to the classical forms, as well as the evolution and response to treatment. We present a new clinical case, typical in its presentation and its good response to treatment with plasmatic spares associated to PFC and steroid infusion. We believe that the presence of clinical signs suggesting TTP in a patient would necessarily discard the presence of HIV infection and, the other way round, the presence of clinical signs suggesting TTP in a patient with HIV infection would determine the onset of an early and aggressive treatment based on plasmatic spares, given that the prognosis is linked to an early onset of the treatment.

Thrombotic thrombocytopenic purpura (TTP) is a syndrome characterized by fever, microangiopathic hemolytic anemia, thrombocytopenia, renal disease and neurologic changes. The development of little thrombi in the systemic microcirculation is thought to play an important role, however the etiology is unknown. We present two cases in which pancreatic disease was the first manifestation of TTP.

Two women are presented who were diagnosed of thrombotic thrombocytopenic purpura (TTP) during their first pregnancies, leading in both cases to spontaneous abortion. Both patients improved after plasma exchange or fresh plasma infusion. Two new pregnancies on each patient, closely watched on gynaecological and haematological grounds, led to TTP reactivation. Obstetrical and haematological management was similar for the two women (fresh-frozen plasma administration) but it yielded different results, since, although the risks for the mothers were controlled, one of the patients gave birth successfully on two occasions while the other developed stillborns on two occasions as well. These observations suggest the inter-relationship between TTP and pregnancy, and point to some pathophysiological aspects of the foetal deth repeteadly presented by one of the patients. A review of the literature is presented on the basis of these cases of PTT associated to pregnancy.

Two patients affected by thrombotic thrombocytopenic purpura (TTP) are treated with plasmapheresis, fresh frozen plasma infusion, steroid and inhibitors of platelet aggregation with an impaired final stage: the first patient died during a plasmapheresis session and the other had a good evolution, obtaining complete recovery, maintained a year later. We discuss the clinical features and highlight the needs for early treatment with all the latest therapies.

A combination of antiplatelet agents, corticosteroids, plasmapheresis and fresh plasma achieved complete remission in a 35-yr old man diagnosed of thrombotic thrombocytopenic purpura who presented with hemolytic anemia, renal and neurologic damage. No symptoms 9 months after finishing treatment have been observed. Platelet and lactate dehydrogenase levels were the most useful analytical parameters in the follow-up.