Survey of Practice Pattern in Patients With Heparin-Induced Thrombocytopenia Requiring Cardiopulmonary Bypass.

BACKGROUND: Heparin-induced thrombocytopenia (HIT) is an immune-mediated adverse reaction to heparin. Patients undergoing cardiac surgery with cardiopulmonary bypass (CPB) are routinely anticoagulated with heparin before the initiation of bypass. Heparin is contraindicated, however, in patients with acute HIT, and alternatives to routine practice are often used. While guidelines have recently been published addressing this topic 10, there remains variance between institutions in how these cases are treated. Our goal was to better delineate practice trends in the diagnosis and management of HIT patients requiring CPB. METHODS: We surveyed members of the Society of Cardiovascular Anesthesiologists (SCA) and the American Society for Extracorporeal Technology (AmSECT) using an online survey tool. RESULTS: We received 304 completed surveys (5.8% response rate), 75% completed by an anesthesiologist, and 24% by a perfusionist. The majority of respondents used clinical history and/or antibody testing (71% and 63%, respectively) to diagnose HIT. Seventy-five percent of respondents reported using an institutional protocol for HIT-CPB cases. Most respondents (89%) reported having at least 1 case in the last 3 years, with a total case experience of at least 785 cases (785 = the minimum number of cases in each case volume category × the number of respondents choosing that category). The strategy recommended in published guidelines, bivalirudin, was the most commonly reported alternative anticoagulation strategy (75%) used by respondents in HIT cases, with most (83%) using the activated clotting time (ACT) to monitor anticoagulation. CONCLUSIONS: Most responding SCA and AmSECT members reported that their institution used a protocol or guideline for HIT/CPB cases, and most guidelines directed the use of bivalirudin as an alternative anticoagulant. Various other methods such as plasmapheresis are also being used with success in this patient population. Further research, including comparison studies of alternative anticoagulant strategies, is required to elucidate the best approach to these difficult cases.

Myasthenia gravis exacerbation in association with antibody overshoot following plasmapheresis.

INTRODUCTION/AIM: Antibody overshoot following therapeutic plasmapheresis (PLEX) is defined by subsequent increase in antibody to levels exceeding those prior to removal. It has been infrequently described in the past, and its influence on the clinical course of myasthenia gravis (MG) remains unclear. METHODS: This was a retrospective analysis of five patients with generalized MG treated with PLEX. RESULTS: All five patients possessed antibodies against acetylcholine receptor (AChR-Ab). After undergoing 3 to 12 PLEX treatment sessions, AChR-Ab titer increased to a median of 1292% of the baseline level. The median interval from the last PLEX session to peak AChR-Ab detection was 6 wk. In four patients, AChR-Ab overshoot was associated with a clinical deterioration. DISCUSSION: The AChR-Ab overshoot may occur following PLEX. In patients who deteriorate following PLEX treatment, the presence of antibody overshoot may serve as additional guidance for treatment adjustment.

Changing Trends in Therapeutic Plasmapheresis: An Indian Perspective.

Indications for therapeutic apheresis (TA) are dynamic; they keep changing and expanding because of introduction of newer treatment modalities and accumulating evidence in the form of case-reports, case-series and original articles. We evaluated our therapeutic plasmapheresis (TP) data and compared this data with an earlier published Indian report for indications, frequency, response rate and adverse reactions. We conducted a retrospective analysis of all TP procedures performed from January 2014 to June 2016 in department of Transfusion Medicine at a tertiary care hospital. All TP procedures performed for various clinical disorders including neurological, hematological, renal, hepatic and rheumatologic indications were included in the study. Analysis was performed with respect to demography, procedure details and response. 187 patients (118 Males and 69 females) underwent 683 TP procedures during study period. According to 2013 ASFA guidelines, 99, 59 and 29 patients belonged to category I, II and III respectively. In comparison with the earlier report, the number of patients and procedures have increased, indications have changed, response rate is comparable, and the frequency of adverse reactions have decreased. In the last decade there has been increase in number and spectrum of indications for therapeutic apheresis and frequency of procedures. The response rate and safety of these procedures have also improved.

Lambert-Eaton myasthenic syndrome: Epidemiology and therapeutic response in the national veterans affairs population.

INTRODUCTION: One nationwide study (The Netherlands) of Lambert-Eaton myasthenic syndrome (LEMS) has been published. We report LEMS epidemiology and its therapeutic response in the United States Veterans Affairs (VA) population. METHODS: Medical records for all active patients (12.5 million) in the VA health system were queried for relevant ICD-9 codes for the period October 1, 1999 to September 30, 2013. Clinical, electrophysiologic, and serologic features were evaluated to confirm diagnosis; epidemiologic and treatment data were collected. RESULTS: Point prevalence was estimated at 2.6 per 1,000,000 (confirmed cases) and 3.3 per 1,000,000 (combined confirmed and probable cases). Crude prevalence was similarly estimated at 9.2 and 10.9 per 1,000,000 respectively. A total of 18 of 48 (38%) patients received 3,4-diaminopyridine (3,4-DAP); 14 of 18 (78%) improved. CONCLUSIONS: This investigation was a large North American epidemiologic study of LEMS. LEMS prevalence in the national VA population was found to be similar to previously published rates in other large international populations. Most patients experienced improvement with therapy, including a majority with 3,4-DAP. Muscle Nerve 56: 421-426, 2017.

Myasthenia gravis: newer therapies offer sustained improvement.

Myasthenia gravis is a prototypical antibody-mediated autoimmune neuromuscular disorder. Treatments have improved over the past 30 years, leading to significantly fewer deaths and better quality of life. Future research should further elucidate its pathogenesis, reveal better ways to diagnose it, and yield new treatments.

The lipid- and lipoprotein- [LDL-Lp(a)] apheresis techniques. Updating.

Therapeutic plasmapheresis allows the extracorporeal removal of plasmatic lipoproteins (Lipid-apheresis) (LA). It can be non selective (non specific), semi - selective or selective low density lipoprotein-lipoprotein(a) (specific [LDL- Lp(a)] apheresis) (Lipoprotein apheresis, LDLa). The LDL removal rate is a perfect parameter to assess the system efficiency. Plasma-Exchange (PEX) cannot be considered either specific nor, selective. In PEX the whole blood is separated into plasma and its corpuscular components usually through centrifugation or rather filtration. The corpuscular components mixed with albumin solution plus saline (NaCl 0.9%) solution at 20%-25%, are then reinfused to the patient, to substitute the plasma formerly removed. PEX eliminates atherogenic lipoproteins, but also other essential plasma proteins, such as albumin, immunoglobulins, and hemocoagulatory mediators. Cascade filtration (CF) is a method based on plasma separation and removal of plasma proteins through double filtration. During the CF two hollow-fiber filters with pores of different diameter are used to eliminate the plasma components of different weight and molecular diameter. A CF system uses a first polypropylene filter with 0.55 µm diameter pores and a second one of diacetate of cellulose with 0.02 µm pores. The first filter separates the whole blood, and the plasma is then perfused through a second filter which allows the recovery of molecules with a diameter lower than 0.02 µm, and the removal of molecules larger in diameter as apoB100-containing lipoproteins. Since both albumin and immunoglobulins are not removed, or to a negligible extent, plasma-expanders, substitution fluids, and in particular albumin, as occurs in PEX are not needed. CF however, is characterized by lower selectivity since removes also high density lipoprotein (HDL) particles which have an antiatherogenic activity. In the 80's, a variation of Lipid-apheresis has been developed which allows the LDL-cholesterol (LDLC) (-61%) and Lp(a) (-60%) removal from plasma through processing 3 liters of filtered plasma by means of lipid-specific thermofiltration, LDL immunoadsorption, heparin-induced LDL precipitation, LDL adsorption through dextran sulphate. More recently (90's) the DALI®, and the Liposorber D® hemoperfusion systems, effective for apoB100- containing lipoproteins removal have been developed. All the above mentioned systems are established LDL-apheresis techniques referable to the generic definition of LDLa. However, this last definition cannot describe in an appropriate manner the removal of another highly atherogenic lipoprotein particle: the Lp(a). Thus it would be better to refer the above mentioned techniques to the wider scientific and technical concept of lipoprotein apheresis.

[Demography and donation frequencies of blood and plasma donor populations in Germany. Update 2010 and 5-year comparison]. / Demografie und Spendeaktivität von Blut- und Plasmaspendern in Deutschland. Update 2010 und 5-Jahres-Vergleich.

The Robert Koch Institute collects and evaluates nationwide data on the incidence and prevalence of transfusion-relevant infections among blood and plasma donors in Germany. Since 2006 data not only on the number of donations tested but also on the number of the respective donors have become available. The demographic profile and donation frequencies of German whole blood, plasma and platelet donors in 2010 and the percentages among the general population are described and compared to data from 2006. Although the general population eligible to donate blood is on the decline since 2003, with a loss of 2% between 2006 and 2010, this has not led to a decrease in the number of blood donors and donations. Instead, the number of new and repeat whole blood donors increased by 8% and 7%, respectively. At the same time, the number of new plasma donors grew by 23%, that of repeat plasma donors by 41%. In 2010 more than 4.3% of the population aged 18-68 years was active as repeat whole blood donors; 0.4% repeatedly donated plasma or platelets. Since 2006 the percentage of donors among the general population increased significantly, especially among the youngest age group (18-24 years). Donation frequency varied depending on donor age and sex, with an average of 1.9 per year for whole blood donations, 12.5 for plasmapheresis and 5.0 for plateletpheresis. While the donation frequency for whole blood remained unchanged since 2006, the frequency of apheresis donations increased, especially among older donors. By recruiting more new donors and retaining and reactivating existing ones more effectively, the number of whole blood and apheresis donations was augmented.

Recent developments in desensitization of crossmatch-positive kidney transplant recipients.

Currently, there are two major options for the successful and timely transplantation of sensitized kidney transplant recipients: (1) avoidance of the sensitization barrier using special allocation programs, or (2) desensitization. In the case of broadly sensitized kidney patients, a combination of both options might be necessary. This review focuses on new advances in desensitization of crossmatch-positive kidney transplant recipients which include immunoadsorption and the administration of new substances such as the complement C5 inhibitor eculizumab. Finally, integrated algorithms that combine different measures are acknowledged.

Crioglobulinemia con acronecrosis no asociada a infección por hepatitis C: presentación de un caso / Cryoglobulinemia with acronecrosis not associated with hepatitis C infection: a case report

La crioglobulinemia es una enfermedad rara en la cual se producen inmunoglobulinas monoclonales y/o policlonales que precipitan con el frío. Si bien este fenómeno puede observarse en una gran cantidad de trastornos, se ha asociado más frecuentemente a la infección por el virus de la hepatitis C en más del 90%. El porcentaje restante, llamado crioglobulinemia esencial, se ha caracterizado por curso más severo y falta de respuesta al tratamiento convencional. El presente artículo describe el caso de un paciente con crioglobulinemia esencial que se presenta con acronecrosis, en la que su mala evolución, a pesar del tratamiento, la lleva a la amputación (AU)

Cryoglobulinemia is a rare disease characterized by the production of monoclonal or polyclonal immunoglobulins that precipitate in cold temperature. While this phenomenon can be observed in a large number of disorders, it has been associated with hepatitis C virus infection in more than 90% of cases. The remaining 10%, called essential cryoglobulinemia, has been characterized by a more severe course and a failure to respond to conventional treatment. This article describes the case of a patient with essential cryoglobulinemia presenting with acronecrosis with a poor outcome, despite treatment, leading to amputation (AU)

Treatment of acute immune-mediated neuropathies: Guillain-Barré syndrome and clinical variants.

The author reviews current treatment approaches for the management of acute immune-mediated peripheral neuropathies, including the Guillain-Barré syndrome together with the clinical variants, which are acute inflammatory demyelinating polyradiculoneuropathy, acute motor axonal neuropathy, acute motor and sensory axonal neuropathy, and the Fisher syndrome. A summary of clinical evidence for drug therapies is provided, with additional recommendations for commonly used treatment modalities including a focus on the approach to using intravenous immune globulin and plasma exchange therapy.

Update on investigation and management of postinfectious encephalitis.

PURPOSE OF REVIEW: Encephalitis is a complex syndrome associated with significant morbidity and mortality. Despite biological and neuroimaging investigations, the cause of encephalitis remains undetermined in more than half of the cases. The aim of this review was to describe available data concerning diagnosis and treatment of postinfectious encephalitis, focusing on acute disseminated encephalomyelitis (ADEM) and acute hemorrhagic leukoencephalitis (AHLE). RECENT FINDINGS: The increasing availability of brain MRI studies has allowed a better delineation of diagnosis and prognosis of postinfectious central nervous system disorders. Beneficial effects of steroids and plasma exchange have been described in the most severe forms of postinfectious encephalitis, including ADEM and AHLE, but randomized controlled studies are lacking. Intravenous immunoglobulins may be of value in ADEM with peripheral nerve involvement and for patients in whom corticosteroid therapy is contraindicated. SUMMARY: Postinfectious encephalitis needs to be identified early in the management of patients with unexplained encephalitis as it represents a treatable disease. Randomized studies are needed in order to assess the potential benefit of early combined immunotherapy in ADEM.

Hematuria como forma de presentación del síndrome hemolítico urémico / Hemolytic uremic syndrome presenting with blood in urine

No disponible

Neuromielitis óptica. Principales diferencias con la esclerosis múltiple / Optic neuromyelitis. Main differences with multiple sclerosis

La neuromielitis optica o síndrome de Devic es una enfermedad inflamatoria, desmielinizante y autoinmune del sistema nervioso central. Se caracteriza por ataques de neuritis óptica y mielitis, pudiendo producir ceguera, gran invalidez neurológica e incluso la muerte a corto plazo. Hasta el momento no existe un tratamiento efectivo, la terapia se centra en el tratamiento de los ataques agudos, la prevención médica de las complicaciones y la rehabilitación. Se realiza una revisión de esta enfermedad poco común, considerando que su prevalencia en nuestro país ha ido en aumento y se establece una comparación entre la neuromiel itisóptica y la esclerosis múltiple, basándose en las principales características clínico-epidemiológicas, que distinguen estas dos patologías, consideradas por muchos variantes clínicas de una misma enfermedad

The optic neuromyelitis or syndrome of Devic is an inflammatory and autoimmune illness of the central nervous system. It is characterized by attacks of optic neuritis and myelitis, being able to produce blindness, great neurological disability and even the short term death. Until the moment an effective treatment doesn't exist, the therapy is centred in the treatment of the acute attacks, the medical prevention of the complications and the rehabilitation. This article is a revision of this not very common illness, considering that its prevalence in our country has gone in increase. We compare between the optic neuromyelitis and the multiple sclerosis, being based on the main ones characteristic clinical-epidemic that distinguishes these two pathologies, considered by many clinical variants of oneself illness

Treatment and treatment trials in multiple sclerosis.

PURPOSE OF REVIEW: This review focuses on advances in current and novel treatment approaches in multiple sclerosis. RECENT FINDINGS: New therapeutic approaches in multiple sclerosis are emerging. Orally available treatment strategies are more acceptable for patients and may improve adherence to therapy. An oral formulation of glatiramer acetate failed to demonstrate efficacy in a clinical trial, but other promising compounds are on the horizon, such as FTY720. Advances are currently being made in use of therapeutic monoclonal antibodies that specifically target key molecules involved in the immunopathogenesis of multiple sclerosis. Natalizumab directed against the adhesion molecule very late antigen-4 represents the first specific antibody to be added to our therapeutic armamentarium for multiple sclerosis. Further evidence that immunomodulation should be initiated as early as possible has been reported. SUMMARY: Treatment of multiple sclerosis has changed dramatically over the past decade. Enhanced understanding of the immunopathological processes that underlie the disease, advances in biotechnology and development of powerful magnetic resonance imaging technologies, together with improvements in clinical trial design have led to a variety of valuable therapeutic approaches, which are currently being studied in detail.

Síndrome de Stevens-Johnson y necrólisis epidérmica tóxica: experiencia clínica y revisión de la literatura especializada / Stevens-Johnson syndrome and toxic epidermal necrolysis

Introducción. El objetivo de este trabajo es reflejar la experiencia clínica del Servicio de Dermatología del Hospital General de Valencia en el síndrome de Stevens-Johnson (SSJ) y necrólisis epidérmica tóxica (NET) o síndrome de Lyell durante los últimos 15 años. Métodos. A través de un estudio retrospectivo se recogieron datos sobre epidemiología, agentes causales probables, pruebas complementarias, patologías concomitantes, tratamiento, evolución y complicaciones. Resultados. Se incluyeron 13 pacientes con edad media de 53 años. Los fármacos más frecuentemente implicados fueron los antibióticos (50 %) seguidos de los anticomiciales (16,6 %). Las mucosas se afectaron en el 84,6 % de los casos. El 61,5 % de los pacientes presentó clínica sistémica. El hallazgo de laboratorio más frecuente fue la hipoproteinemia. Los corticoides se emplearon en el 69 % de casos y las inmunoglobulinas intravenosas, en el 15 %. Dos pacientes oncológicos con diagnóstico de NET fallecieron (15 % de mortalidad global). Conclusiones. El SSJ y la NET son reacciones mucocutáneas poco frecuentes a menudo inducidas por fármacos que asocian una importante morbimortalidad. La patogenia permanece en parte desconocida y ningún tratamiento específico se ha demostrado claramente beneficioso, por lo que la mejor terapéutica consiste en el diagnóstico precoz, la retirada del fármaco sospechoso y la terapia de soporte

Introduction. The aim of this work is to reflect the clinical experience of the Dermatology Department of Hospital General in Valencia with Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) or Lyell's syndrome over the last 15 years. Methods. Data on epidemiology, likely causal agents, complementary tests, concomitant pathologies, management, evolution and complications was gathered through a retrospective study. Results. Thirteen patients were included, with a mean age of 53 years. The most frequently involved drugs were antibiotics (50 %), followed by anti-convulsants (16.6 %). The mucous membranes were involved in 84.6 % of the cases. 61.5 % of the patients presented with systemic symptoms. The most frequent laboratory finding was hypoproteinemia. Corticosteroids were used in 69 % of the cases, and intravenous immunoglobulins in 15 %. Two oncological patients with a diagnosis of TEN died (15 % overall mortality). Conclusions. SJS and TEN are infrequent mucocutaneous reactions, often drug induced, with significant associated morbidity and mortality. Their pathogenesis is still partially unknown, and no specific treatment has been proven to be clearly beneficial; therefore, the best treatment consists of early diagnosis, the withdrawal of the suspect drug and support therapy

Quo vadis haemapheresis. Current developments in haemapheresis.

The techniques of haemapheresis originated in the development of centrifugal devices separating cells from plasma and later on plasma from cells. Subsequently membrane filtration was developed allowing for plasma-cell separation. The unspecificity of therapeutic plasma exchange led to the development of secondary plasma separation technologies being specific, semi-selective or selective such as adsorption, filtration or precipitation. In contrast on-line differential separation of cells is still under development. Whereas erythrocytapheresis, granulocytapheresis, lymphocytapheresis and stem cell apheresis are technically advanced, monocytapheresis may need further improvement. Also, indications such as erythrocytapheresis for the treatment of polycythaemia vera or photopheresis though being clinically effective and of considerable importance for an appropriate disease control are to some extent under debate as being either too costly or without sufficient understanding of the mechanism. Other forms of cell therapy are under development. Rheohaemapheresis as the most advanced technology of extracorporeal haemorheotherapy is a rapidly developing approach contributing to the treatment of microcirculatory diseases and tissue repair. Whereas the control of a considerable number of (auto-) antibody mediated diseases is beyond discussion, the indication of apheresis therapy for immune complex mediated diseases is quite often still under debate. Detoxification for artificial liver support advanced considerably during the last years, whereas conclusions on the efficacy of septicaemia treatment are debatable indeed. LDL-apheresis initiated in 1981 as immune apheresis is well established since 24 years, other semi-selective or unspecific procedures, allowing for the elimination of LDL-cholesterol among other plasma components are also being used. Correspondingly Lp(a) apheresis is available as a specific, highly efficient elimination procedure superior to techniques which also eliminate Lp(a). Quality control systems, more economical technologies as for instance by increasing automation, influencing the over-interpretation of evidence based medicine especially in patients with rare diseases without treatment alternative, more insight into the need of controlled clinical trials or alternatively improved diagnostic procedures are among others tools ways to expand the application of haemapheresis so far applied in cardiology, dermatology, haematology, immunology, nephrology, neurology, ophthalmology, otology, paediatrics, rheumatology, surgery and transfusion medicine.

Treatment options for POEMS syndrome.

POEMS syndrome is a rare paraneoplastic syndrome secondary to a plasma cell dyscrasia. Recognition of the complex of a combination of peripheral neuropathy, organomegaly, endocrinopathy, monoclonal plasmaproliferative disorder, skin changes, sclerotic bone lesions, Castleman's disease, thrombocytosis, papilledema, peripheral oedema, pleural effusions, ascites, fingernail clubbing and white nails, is the first step in effectively managing the disease. More than 95% of patients will have monoclonal lambda sclerotic plasmacytoma(s) or bone marrow infiltration. In patients with a dominant sclerotic plasmacytoma, first-line therapy should include radiation to the lesion. Retrospective analysis and personal experience would dictate that systemic therapy be considered for patients with diffuse sclerotic lesions or absence of any bone lesion, and for those who have not demonstrated stabilisation of their disease 3-6 months after completing radiation therapy. For those patients with diffuse disease, systemic therapy is indicated. Useful approaches include therapy with corticosteroids, low dose alkylator therapy and high dose chemotherapy with peripheral blood stem cell transplant. Until the pathogenesis is fully understood, these are the mainstays of treatment for patients with POEMS syndrome.