Your browser doesn't support javascript.
loading
Show: 20 | 50 | 100
Results 1 - 20 de 40
Filter
1.
Medicine (Baltimore) ; 103(19): e38103, 2024 May 10.
Article in English | MEDLINE | ID: mdl-38728448

ABSTRACT

RATIONALE: Thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy caused by reduced activity of the von Willebrand factor-cleaving protease (ADAMTS13), which can be life-threatening. The patient reported in this case study also had concurrent Sjögren syndrome and renal impairment, presenting multiple symptoms and posing a great challenge in treatment. PATIENT CONCERNS: A 25-year-old woman in the postpartum period visited the hospital due to indifference in consciousness for more than 1 day following cesarean section 8 days prior. DIAGNOSIS: Notable decreases were observed in platelets, hemoglobin, creatinine, and ADAMTS13 levels. After a consultative examination by an ophthalmologist, she was diagnosed with retinal hemorrhage in the right eye and dry eye syndrome in both eyes. INTERVENTIONS: Having been diagnosed with TTP with Sjögren syndrome and renal impairment, she received repeated treatments with plasmapheresis combined with rituximab. OUTCOMES: Following treatment and during the follow-up period, the patient's platelet counts and bleeding symptoms significantly improved. LESSONS: TTP has a high mortality rate, and when combined with Sjögren syndrome and renal impairment, it poses an even greater challenge in treatment. However, after administering standard plasmapheresis combined with rituximab treatment, the treatment outcome is favorable.


Subject(s)
Plasmapheresis , Purpura, Thrombotic Thrombocytopenic , Rituximab , Sjogren's Syndrome , Humans , Female , Sjogren's Syndrome/complications , Sjogren's Syndrome/therapy , Plasmapheresis/methods , Adult , Purpura, Thrombotic Thrombocytopenic/therapy , Purpura, Thrombotic Thrombocytopenic/complications , Purpura, Thrombotic Thrombocytopenic/drug therapy , Rituximab/therapeutic use , Rituximab/administration & dosage , Combined Modality Therapy , Renal Insufficiency/therapy , Renal Insufficiency/etiology , Immunologic Factors/therapeutic use , Immunologic Factors/administration & dosage
2.
Medicine (Baltimore) ; 103(20): e38112, 2024 May 17.
Article in English | MEDLINE | ID: mdl-38758904

ABSTRACT

RATIONALE: Thrombotic thrombocytopenic purpura (TTP) is a syndrome characterized by widespread blood vessel clotting and bleeding. It can affect individuals of any age but is more commonly observed in females, particularly during pregnancy. Pregnancy combined with TTP is a critical and rapidly progressing condition that is often misdiagnosed as an obstetric disorder like severe preeclampsia or HELLP syndrome. To deepen the understanding of TTP during pregnancy with the help of a clinical case. PATIENT CONCERNS: A 20-year-old patient, is pregnancy 1 birth 0, 32 weeks dated by her last menstrual period, presented chest tightness, and shortness of breath after physical activity for 3 days. DIAGNOSES: TTP. INTERVENTIONS: At present, there are no preventive measures. Timely diagnosis and treatment are useful. Plasma exchange and treat to the patient hinder autoantibodies, such as gamma globulin, methylprednisolone, rituximab, and cyclosporine were effective. OUTCOMES: The patient exhibited stable vital signs, normal examination results, and experienced no complications. We continued to monitor her progress after she was discharged. LESSONS SUBSECTIONS: The acute onset of TTP is often associated with pregnancy, as it is a triggering factor. Timely identification, accurate diagnosis, and a comprehensive treatment approach involving plasma exchange, immunosuppressants, and the termination of pregnancy can lead to remission and a favorable outlook for the majority of patients.


Subject(s)
Plasma Exchange , Pregnancy Complications, Hematologic , Purpura, Thrombotic Thrombocytopenic , Humans , Female , Pregnancy , Purpura, Thrombotic Thrombocytopenic/diagnosis , Purpura, Thrombotic Thrombocytopenic/therapy , Purpura, Thrombotic Thrombocytopenic/complications , Plasma Exchange/methods , Young Adult , Pregnancy Complications, Hematologic/diagnosis , Pregnancy Complications, Hematologic/therapy
3.
Clin Lab ; 70(4)2024 Apr 01.
Article in English | MEDLINE | ID: mdl-38623670

ABSTRACT

BACKGROUND: The aim was to explore the treatment of a case of congenital thrombotic thrombocytopenic purpura induced by pregnancy complicated with cerebral vasospasm. METHODS: We present a case study of congenital TTP where disease onset occurred during two separate pregnancies. Interestingly, the disease course exhibited distinct differences on each occasion. Additionally, following plasma transfusion therapy, there was a transient occurrence of cerebral vasospasm. RESULTS: In this case, ADAMTS13 levels reached their lowest point three days after delivery during the first pregnancy, triggering morbidity. Remarkably, a single plasma transfusion of 400 mL sufficed for the patient's recovery. Nonetheless, a recurrence of symptoms transpired during her second pregnancy at 24 weeks of gestation. Plasma transfusions were administered during and after delivery. Sudden convulsions developed. ADAMTS13 ac-tivity returned to normal, but cranial MRA revealed constrictions in the intracranial segments of both vertebral arteries, the basilar artery, and the lumen of the anterior, middle, and posterior cerebral arteries. A subsequent cranial MRA conducted a month later showed no lumen stenosis, indicating spontaneous recovery. CONCLUSIONS: These findings highlight the importance of careful consideration when administering plasma transfusions in congenital TTP during pregnancy. Moreover, the development of novel therapeutic approaches such as recombinant ADAMTS13 is crucial for minimizing complications and optimizing patient care.


Subject(s)
Pregnancy Complications, Hematologic , Purpura, Thrombotic Thrombocytopenic , Vasospasm, Intracranial , Humans , Pregnancy , Female , Purpura, Thrombotic Thrombocytopenic/complications , Purpura, Thrombotic Thrombocytopenic/diagnosis , Purpura, Thrombotic Thrombocytopenic/therapy , Pregnancy Complications, Hematologic/diagnosis , Pregnancy Complications, Hematologic/therapy , Blood Component Transfusion/adverse effects , Vasospasm, Intracranial/complications , Vasospasm, Intracranial/therapy , Plasma
4.
Hematology ; 29(1): 2335420, 2024 Dec.
Article in English | MEDLINE | ID: mdl-38546338

ABSTRACT

OBJECTIVES: Thrombotic thrombocytopenic purpura (TTP) is a rare but life-threatening hematological disorder. Early differentiation between TTP and primary immune thrombocytopenia (ITP) accompanied by anemia is crucial to initiate an appropriate therapeutic strategy. The objective of this study was to evaluate the predictive value of red blood cell lifespan (RBCLS), determined using the carbon monoxide breath test, in the differential diagnosis of these two diseases. METHODS: We conducted a retrospective analysis of 23 patients with TTP and 32 patients with ITP accompanied by anemia. RBCLS measurements were compared and evaluated between these two patient groups. RESULTS: TTP patients had a significantly shorter mean RBCLS (20 ± 8 days) than patients with ITP accompanied by anemia (77 ± 22 days, P < 0.001) and healthy controls (114 ± 25 days, P < 0.001). In TTP patients, RBCLS showed a significant negative correlation with reticulocyte percentage and lactic dehydrogenase levels (P < 0.001). When using a standard baseline of 75 days, RBCLS demonstrated a sensitivity of 100% and specificity of 53.1% in identifying TTP. The diagnostic accuracy could reach 93% by excluding the impact of gastrointestinal bleeding. By employing the Receiver Operator Characteristics (ROC) curve, the area under the curve for RBCLS was 0.985 (95% CI: 0-1, P < 0.01) in predicting TTP, with an optimal cut-off value of 32 days, and sensitivity and specificity of 95.7% and 96.9%, respectively. CONCLUSIONS: Our study proposes a simple and accessible method for evaluating RBCLS to differentiate between TTP and ITP accompanied by anemia.


Subject(s)
Anemia , Purpura, Thrombocytopenic, Idiopathic , Purpura, Thrombotic Thrombocytopenic , Humans , Carbon Monoxide , Purpura, Thrombocytopenic, Idiopathic/complications , Purpura, Thrombocytopenic, Idiopathic/diagnosis , Purpura, Thrombotic Thrombocytopenic/complications , Purpura, Thrombotic Thrombocytopenic/diagnosis , Retrospective Studies , Breath Tests
5.
Cancer Rep (Hoboken) ; 7(3): e2036, 2024 Mar.
Article in English | MEDLINE | ID: mdl-38517451

ABSTRACT

BACKGROUND: Cancer-related thrombotic microangiopathy (CR-TMA) is a rare type of Coombs-negative hemolytic anemia, which is caused by malignancy and has a poor prognosis. CASE: A 76-year-old female was referred to our hospital due to Coombs-negative hemolytic anemia, which was causing fatigue and dyspnea on exertion, accompanied by schistocytosis. A bone marrow examination demonstrated bone marrow carcinomatosis, and the tumor cells were morphologically suspected to be signet-ring cell carcinoma cells. As we failed to find the primary tumor site before the patient died, she was diagnosed with CR-TMA due to bone marrow carcinomatosis of unknown primary origin. Thrombotic thrombocytopenic purpura (TTP) was rapidly ruled out based on her PLASMIC score. In addition, immunohistochemical staining of a clot section of the bone marrow and tumor marker data were useful for narrowing down the likely primary tumor site. CONCLUSION: Although CR-TMA is an extremely rare phenomenon, clinicians who suspect CR-TMA should quickly rule out TTP and decide whether to provide appropriate chemotherapy or plan for palliative care.


Subject(s)
Anemia, Hemolytic , Carcinoma , Disseminated Intravascular Coagulation , Neoplasms, Unknown Primary , Peritoneal Neoplasms , Purpura, Thrombotic Thrombocytopenic , Thrombotic Microangiopathies , Female , Humans , Aged , Disseminated Intravascular Coagulation/etiology , Disseminated Intravascular Coagulation/complications , Purpura, Thrombotic Thrombocytopenic/complications , Purpura, Thrombotic Thrombocytopenic/diagnosis , Bone Marrow , Neoplasms, Unknown Primary/complications , Neoplasms, Unknown Primary/diagnosis , Thrombotic Microangiopathies/etiology , Thrombotic Microangiopathies/complications , Anemia, Hemolytic/complications
6.
Turk J Pediatr ; 66(1): 1-16, 2024.
Article in English | MEDLINE | ID: mdl-38523374

ABSTRACT

Classical clinical triad of hemolytic uremic syndrome (HUS) is microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury associated with endothelial cell injury. Several situations, including infections, medications, malignancies, and transplantation can trigger endothelial damage. On the HUS spectrum, atypical hemolytic uremic syndrome (aHUS) deserves special attention in pediatric patients, as it can cause endstage kidney disease and mortality. A dysfunction in the alternative complement pathway, either acquired or genetic, has been shown to be the main underlying cause. In the last decades, breathtaking advances have been made in understanding the pathophysiology of this rare disease, which has led to more efficient treatment. Recent studies have implicated genes in pathways beyond the alternative complement system, such as DGKE, TSEN2, and INF2 highlighting the importance of personalized management. Eculizumab has brought about dramatic improvements in the treatment of aHUS. Beyond eculizumab, there are many alternative therapeutics in the pipeline that target the complement system. Because of the rarity of aHUS, data from multiple patient registries are very important. The present report aimed to summarize the most important aspects of diagnosing and treating aHUS based on the Turkish national registry and the literature so as to improve clinical practice.


Subject(s)
Acute Kidney Injury , Anemia, Hemolytic , Atypical Hemolytic Uremic Syndrome , Kidney Failure, Chronic , Purpura, Thrombotic Thrombocytopenic , Humans , Child , Atypical Hemolytic Uremic Syndrome/diagnosis , Atypical Hemolytic Uremic Syndrome/genetics , Atypical Hemolytic Uremic Syndrome/therapy , Purpura, Thrombotic Thrombocytopenic/complications , Acute Kidney Injury/etiology
8.
Clin Appl Thromb Hemost ; 29: 10760296231219252, 2023.
Article in English | MEDLINE | ID: mdl-38099718

ABSTRACT

Utilizing the comprehensive Nationwide Inpatient Sample (NIS) database, we examined the impact of thrombotic thrombocytopenic purpura (TTP) on the outcomes of patients with coronavirus disease-19 (COVID-19), emphasizing the potential role of the ADAMTS13 enzyme in disease pathogenesis and evolution. We analyzed extensive data from the NIS database using STATA v.14.2 and accounted for potential confounders using multivariate regression analysis to uphold the validity and reliability of the study. Among 1 050 045 adult patients hospitalized with COVID-19, only 300 (0.03%) developed TTP. These patients were younger (mean age 57.47 vs 64.74, P < .01) and exhibited a higher prevalence of preexisting conditions, such as congestive heart failure (13.33% vs 16.82%, P value not provided) and end-stage renal disease (3.33% vs 3.69%, P value not provided). On multivariate regression analysis, COVID-19 patients with concomitant TTP demonstrated a significant increase in mortality (adjusted odds ratio [AOR] 3.99, P < .01), venous thromboembolism (AOR 3.33, P < .01), acute kidney injury (AOR 7.36, P < .01), gastrointestinal bleeding (AOR 10.75, P < .01), intensive care unit admission (AOR 14.42, P < .01), length of hospital stay (17.42 days, P < .01), and total hospitalization charges ($298 476, P < .01). Thrombotic thrombocytopenic purpura in COVID-19 patients elevates the risk of mortality and complications, likely driven by the thrombotic nature of TTP. Our data underline the potential significance of ADAMTS13 in COVID-19 and TTP pathophysiology, suggesting its possible role as a therapeutic target.


Subject(s)
COVID-19 , Purpura, Thrombotic Thrombocytopenic , Adult , Humans , Middle Aged , Purpura, Thrombotic Thrombocytopenic/complications , Purpura, Thrombotic Thrombocytopenic/epidemiology , Inpatients , Reproducibility of Results , COVID-19/complications , Prevalence , ADAMTS13 Protein
9.
Arch. argent. pediatr ; 121(5): e202202758, oct. 2023. ilus
Article in English, Spanish | LILACS, BINACIS | ID: biblio-1509952

ABSTRACT

La púrpura trombótica trombocitopénica es una entidad poco frecuente en pediatría, pero de alta mortalidad sin tratamiento adecuado y oportuno. Se caracteriza por presentar anemia hemolítica microangiopática asociada a signos y síntomas neurológicos, cardíacos, abdominales y menos frecuentemente renales; puede estar acompañada de fiebre. En niños, el diagnóstico se basa en los hallazgos clínicos y de laboratorio. La actividad de ADAMTS13 <10 % apoya, pero no confirma el diagnóstico y, dada la gravedad de la patología, el resultado no debe retrasar el inicio del tratamiento. Se presenta una paciente de 15 años, previamente sana, con signos neurológicos asociados a anemia hemolítica y trombocitopenia. Durante su internación, se arribó al diagnóstico de púrpura trombótica trombocitopénica adquirida.


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.


Subject(s)
Humans , Female , Adolescent , Purpura, Thrombotic Thrombocytopenic/complications , Purpura, Thrombotic Thrombocytopenic/diagnosis , Purpura, Thrombotic Thrombocytopenic/therapy , Anemia, Hemolytic/diagnosis , Pediatrics
11.
São Paulo med. j ; 135(5): 491-496, Sept.-Oct. 2017. tab
Article in English | LILACS | ID: biblio-904110

ABSTRACT

ABSTRACT CONTEXT: Thrombotic microangiopathy syndrome or thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) describes distinct diseases sharing common pathological features: microangiopathic hemolytic anemia and thrombocytopenia, without any other apparent cause. CASE REPORT: An 18-year-old second-trimester primigravida presented with a history of fifteen days of intense weakness, followed by diarrhea over the past six days. She reported having had low platelets since childhood, but said that she had never had bleeding or menstrual abnormalities. Laboratory investigation showed anemia with schistocytes, thrombocytopenia and hypohaptoglobulinemia. Red blood cell concentrate and platelet transfusions were performed. The hypothesis of TTP or HUS was put forward and ADAMTS13 enzyme activity was investigated. The patient evolved with increasing platelet counts, even without specific treatment, and she was discharged. One month afterwards, she returned presenting weakness and swollen face and legs, which had developed one day earlier. The ADAMTS13 activity was less than 5%, without presence of autoantibodies. Regarding the two previous admissions (at 9 and 16 years of age), with similar clinical features, there was spontaneous remission on the first occasion and, on the second, the diagnosis of TTP was suspected and plasmapheresis was performed, but ADAMTS13 activity was not investigated. CONCLUSION: To date, this is the only report of congenital TTP with two spontaneous remissions in the literature This report reveals the importance of suspicion of this condition in the presence of microangiopathic hemolytic anemia and thrombocytopenia without any other apparent cause.


RESUMO CONTEXTO: A síndrome de microangiopatia trombótica, ou púrpura trombocitopênica trombótica-síndrome hemolítico urêmica (PTT-SHU), descreve doenças diversas com clínica e achados patológicos comuns: anemia hemolítica microangiopática e trombocitopenia, na ausência de outra causa aparente. RELATO DO CASO: Primigesta de 18 anos no segundo trimestre apresenta-se com quadro de 15 dias de fraqueza intensa seguida por diarreia há seis dias. Relata ter plaquetas baixas desde a infância e nega sangramentos e anormalidades menstruais. Investigação laboratorial identificou anemia com esquizócitos, plaquetopenia e hipo-haptoglobulinemia. Foi realizada transfusão de plaquetas e concentrado de hemácias. A hipótese de PTT ou SHU foi aventada e realizou-se pesquisa da atividade da enzima ADAMTS13. A paciente evoluiu com elevação das plaquetas, mesmo sem tratamento específico, tendo alta. Retornou após um mês da alta com queixa de fraqueza há um dia e inchaço de face e pernas. A atividade da ADAMTS13 foi menor que 5%, sem autoanticorpos. Nas duas internações anteriores (aos 9 e 16 anos), com quadros similares, houve remissão espontânea na primeira internação e, na segunda, o diagnóstico de PTT foi suspeitado e foi realizada plasmaférese, porém sem a pesquisa da atividade da ADAMTS13. CONCLUSÃO: Até esta data, este é único relato de TTP congênita com duas remissões espontâneas na literatura. Este relato revela a importância da suspeição desta patologia na presença de anemia hemolítica microangiopática e trombocitopenia sem outra causa aparente.


Subject(s)
Humans , Female , Pregnancy , Pregnancy Complications, Hematologic , Purpura, Thrombotic Thrombocytopenic/congenital , Abortion, Spontaneous/etiology , Pregnancy Trimester, Second , Purpura, Thrombotic Thrombocytopenic/complications , Recurrence , Remission, Spontaneous , Biomarkers/analysis , ADAMTS13 Protein/analysis
13.
Inmunología (1987) ; 34(3): 26-28, jul.-sept. 2015. tab, ilus
Article in Spanish | IBECS | ID: ibc-143024

ABSTRACT

Presentamos el caso clínico de una paciente con púrpura trombocitopénica refractaria al tratamiento y defectos en la inmunidad humoral, que, tras el tratamiento con rituximab, presentó marcada hipogammaglobulinemia y clínica infecciosa (AU)


No disponible


Subject(s)
Adolescent , Female , Humans , Purpura, Thrombotic Thrombocytopenic/complications , Purpura, Thrombotic Thrombocytopenic/drug therapy , Antibodies, Monoclonal/therapeutic use , Immunologic Deficiency Syndromes/immunology , Antibodies/immunology , Diagnosis, Differential , Evaluation of Results of Therapeutic Interventions/trends , Reproducibility of Results
14.
Medicina (B.Aires) ; 75(4): 221-224, Aug. 2015. ilus
Article in Spanish | LILACS | ID: biblio-841499

ABSTRACT

La púrpura trombótica trombocitopénica (PTT) es una microangiopatía trombótica asociada a cuadros de trasplante de células madre hematopoyéticas, embarazo y posparto, al uso de ciertas drogas, diarrea sanguinolenta, a un grupo heterogéneo de trastornos como enfermedades autoinmunes, hipertensión maligna, enfermedades neoplásicas, diversas infecciones y un grupo llamado idiopático, donde se incluyen aquellos en quienes no se cumplieron los criterios de las categorías clínicas anteriores. La tuberculosis (TBC) es una enfermedad de incidencia intermedia en Argentina, más frecuente en determinados grupos poblacionales. Presentamos el caso de un varón de 60 años que consulta por dolor abdominal, tos y sudoración nocturna; se detectó primero trombocitopenia y luego de algunos días anemia microangiopática, lo que llevó al diagnóstico de PTT. Posteriormente, al continuar con la evaluación, se diagnosticó TBC por: 1) baciloscopia positiva para bacilos ácido-alcohol resistentes (+++) por técnica de Ziehl Neelsen en lavado broncoalveolar, 2) lesiones en laringe y 3) lesiones en válvula ileocecal. La TBC ha sido pocas veces asociada con la PTT en la literatura, razón por la cual presentamos este caso.


Thrombocytopenic thrombotic purpura (TTP) is a thrombotic microangiopathy associated with hematopoietic stem cells transplantation, pregnancy, puerperium, drugs, bloody diarrhea, a heterogeneous group of illnesses, such as malignant hypertension, autoimmune and neoplastic diseases, infections, and an idiopathic group that includes the pathologies that don’t apply for the preceding clinical categories. Tuberculosis is an illness of intermediate incidence in Argentina, with increased frequency in high-risk groups. In this article, we present the case of a 60 years old male patient, with abdominal pain, cough and nocturnal sweating. He was diagnosed as having thrombocytopenia, and a few days later, microangiopathic anemia and TTP. Further examinations led TBC diagnosis based on: 1) acid fast bacillus (+++) for Ziehl Neelsen stain in bronco alveolar lavage, 2) laryngeal lesion and 3) ileocecal valve lesion. We report this case, because TBC has rarely been associated with TTP in medical literature.


Subject(s)
Humans , Male , Middle Aged , Purpura, Thrombotic Thrombocytopenic/complications , Purpura, Thrombotic Thrombocytopenic/diagnosis , Tuberculosis/diagnosis , Tuberculosis/complications
18.
Rev. esp. cardiol. (Ed. impr.) ; 64(7): 606-613, jul. 2011. tab, ilus
Article in Spanish | IBECS | ID: ibc-89707

ABSTRACT

La relación fundamental entre los trastornos sanguíneos y el sistema cardiovascular tiene su origen en múltiples puntos de contacto, que van del corazón y sus componentes estructurales, como las cámaras cardiacas, las válvulas, las arterias coronarias y las venas coronarias, a los vasos sanguíneos cerebrovasculares y periféricos. Mientras que los componentes celulares de la sangre circulante proceden inicialmente de células progenitoras pluripotenciales, los componentes plasmáticos, que incluyen las proteínas de la coagulación, se originan principalmente en la síntesis hepática y las células endoteliales. Presentamos aquí una revisión centrada en los trastornos hematológicos no oncológicos y sus posibles repercusiones en el sistema circulatorio arterial en forma de fenotipos frecuentes, como el infarto de miocardio, el ictus isquémico y los episodios de oclusión arterial periférica. En nuestro análisis utilizamos la tromboembolia venosa como patrón de referencia clínico. Presentamos también unos pasos prácticos y una guía para las pruebas diagnósticas y el manejo en la práctica clínica habitual (AU)


The fundamental relationship between blood disorders and the cardiovascular system originates within multiple points of interface, ranging from the heart and its structural constituents to include heart chambers, valves, coronary arteries, coronary veins, and the cerebrovascular and peripheral vasculature. While the cellular components of circulating blood derive their primary origin from multipotent progenitor cells, plasma-based components, which include coagulation proteins, are mostly born of hepatic synthesis and endothelial cells. Here we provide a focused overview of nononcological blood disorders and their potential impact on the arterial circulatory system as common phenotypes, including myocardial infarction, ischemic stroke and peripheral arterial occlusive events. Venous thromboembolism is employed in our discussion as a clinical template. We also provide practical steps and guidance for diagnostic testing and management in routine clinical practice (AU)


Subject(s)
Humans , Male , Female , Hemoglobinopathies/complications , Hemoglobinopathies/epidemiology , Thrombocytopenia/complications , Thrombocytopenia/epidemiology , Thrombophilia/complications , Thrombophilia/epidemiology , Thrombosis/complications , Thrombosis/epidemiology , Protein C Deficiency/complications , Blood Coagulation Disorders, Inherited/complications , Heart/physiopathology , Purpura, Thrombotic Thrombocytopenic/complications , Protein S Deficiency/complications , Antithrombin III Deficiency/complications , Antiphospholipid Syndrome/complications , Erythrocytes, Abnormal/pathology , Anemia, Sickle Cell/complications
19.
An. med. interna (Madr., 1983) ; 24(12): 591-594, dic. 2007.
Article in Es | IBECS | ID: ibc-62378

ABSTRACT

Publicamos un caso de un paciente de 30 años que acude al servicio de Urgencias con dolor abdominal, fiebre, inestabilidad hemodinámica, hepatoesplenomegalia, fracaso renal agudo anúrico, linfadenopatías latero-cervicales, anemia y trombocitopenia. El paciente fue tratado con antibióticos empíricos, esteroides, gammaglobulinas, noradrenalina y hemodiálisis intermitente diaria con buena respuesta. La biopsia renal mostró datos de microangiopatía trombótica y tras la biopsia de una adenopatía se diagnosticó de enfermedad de Castleman. La enfermedad de Castleman, también conocida como hiperplasia nodular linfática gigante o angiofolicular, es una entidad clínico-patológica de etiología desconocida. La afectación renal és heterogénea y frecuente (AU)


We report a case of a 30-year-old man presenting with abdominal pain, fever, homodynamic instability, hepatosplenomegaly, acute renal failure, cervical lymph nodes, anaemia and thrombocytopenia. The patient was treated with empiric antibiotics, high dose corticosteroids, gammaglobulins, noradrenalin and diary intermittent haemodialysis, with an excellent response. The renal biopsy showed a thrombotic microangiopathy, the lymph node biopsy showed a Castleman’s disease.Castleman’s disease (also known as giant lymph node hyperplasia or angiofollicular lymph node hyperplasia) is a clinicopathological entity of unknown aetiology. A number of renal alterations have been described in association with the Castleman’s disease (AU)


Subject(s)
Humans , Male , Adult , Castleman Disease/complications , Purpura, Thrombotic Thrombocytopenic/complications , Castleman Disease/diagnosis , Thrombocytopenia/etiology , Hemodynamics , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Purpura, Thrombotic Thrombocytopenic/diagnosis
20.
Arq. neuropsiquiatr ; 65(4a): 1010-1011, dez. 2007. ilus
Article in English | LILACS | ID: lil-470133

ABSTRACT

Cerebellar disorders associated with HIV infection are usually caused by opportunistic infections, central nervous system lymphoma, and toxic effects of medicines, nutritional and metabolic disorders, and cerebrovascular disease. We present an unusual association of cerebellar degeneration and immune thrombocytopenic purpura in a 28-years-old woman HIV infected. An autoimmune aetiology is likely.


Transtornos cerebelares associados a infecção pelo HIV são comumente causados por infecções oportunistas, linfoma do sistema nervoso central, efeitos tóxicos de medicamentos anti-retrovirais, alterações metabólicas e nutricionais, e doença cerebrovascular. Apresentamos um caso incomum de associação de degeneração cerebelar e púrpura trombocitopênica imunológica em um mulher de 28 anos infectada pelo HIV. Discutimos uma possível etiologia autoimune para justificar o quadro.


Subject(s)
Adult , Female , Humans , Cerebellar Diseases/complications , HIV Infections/complications , HIV-1 , Neurodegenerative Diseases/complications , Purpura, Thrombotic Thrombocytopenic/complications , Cerebellar Diseases/diagnosis , HIV Infections/diagnosis , HIV Infections/drug therapy , Magnetic Resonance Imaging , Neurodegenerative Diseases/diagnosis , Neurodegenerative Diseases/drug therapy , Purpura, Thrombotic Thrombocytopenic/diagnosis , Purpura, Thrombotic Thrombocytopenic/drug therapy
SELECTION OF CITATIONS
SEARCH DETAIL