Síndrome de Good, una causa infrecuente de diarrea crónica / Good Syndrome, a rare cause of chronic diarrhea

Chronic diarrhea is a frequent cause of consultation in daily clinical practice. There are multiple diagnostic algorithms that allow a staggered approach to the most frequent pathologies, leaving out some lesser-known ones. This article reports the case of a 66-year-old female patient with a history of arterial hypertension, dyslipidemia and resected AB thymoma and a history of chronic diarrhea of 8 weeks of evolution. The etiological study ruled out infectious causes, celiac disease and negative viral serology. Due to a history of thymoma, immunoglobulin count was performed, showing severe pan-hypogammaglobulinemia. Good's Syndrome is the combination of thymoma and hypogammaglobulinemia, where patients may present with diarrhea secondary to immunodeficiency. Hypogammaglobulinemia associated with the presence of a thymoma is a rare cause but widely described in the literature as Good's Syndrome. Therefore, it seems relevant to describe a case, its approach and subsequent management.

La diarrea crónica constituye una causa frecuente de consulta en la práctica clínica diaria. Existen múltiples algoritmos diagnósticos que permiten realizar un abordaje escalonado de las patologías más frecuentes y permiten descartar algunas menos conocidas. En el presente artículo se reporta el caso de una paciente de género femenino de 66 años, antecedentes de hipertensión arterial, dislipidemia y timoma AB resecado con historia de diarrea crónica de 8 semanas de evolución. Dentro del estudio etiológico se descartan las causas infecciosas, enfermedad celíaca y serologías virales negativas. Por antecedente de timoma, se realizó recuento de inmunoglobulinas, evidenciando una severa pan-hipogammaglobulinemia. El Síndrome de Good es la combinación de timoma e hipogammaglobulinemia, donde los pacientes podrían presentar diarreas secundarias a inmunodeficiencia. La hipogammaglobulinemia asociada a la presencia de un timoma es una causa poco frecuente pero ampliamente descrita en la literatura como Síndrome de Good. Por lo antes señalado, nos parece relevante describir un caso, su abordaje y manejo posterior.

Good / 中南大学学报(医学版)

A patient with thymoma associated immunodeficiency syndrome (Good's syndrome) and bronchiectasis was retrospectively analyzed. Good's syndrome is a rare condition of immunodeficiency that is characterized by thymoma and hypogammaglobulinemia. It is important to bear in mind that Good's syndrome should be included in the differential diagnosis When patients repeatedly visited for bronchiectasis or infection, we should alert to their immune state and history of thymoma. Early screening of immunological status and aggressive correction of immune deficiency are beneficial to improving the prognosis to patients with Good's syndrome.

Retinite por citomegalovírus em paciente com Síndrome de Good / Cytomegalovirus retinitis in Good Syndrome

Resumo A Síndrome de Good é uma síndrome paraneoplásica caracterizada pela associação de timoma e hipogamaglobulinemia, cursando com imunossupressão. Relatamos um caso raro de retinite por citomegalovírus em paciente com esta síndrome.

Abstract Good syndrome is a paraneoplastic syndrome characterized by the association of thymoma and hypogammaglobulinemia, with immunosuppression. We report a rare case of cytomegalovirus retinitis in a patient with this syndrome.

Myasthenia gravis and thymus: long-term follow-up screening of thymectomized and non-thymectomized patients / Miastenia gravis e timo: investigacao no seguimento de longo prazo em pacientes timectomizados e nao timectomizados

Thymoma screening is recommended at the onset of myasthenia gravis (MG) or when patients with MG present with clinical deterioration or a progressive increase of anti-acetylcholine receptor antibody. However, it is unknown if it is necessary to repeat the screening of thymoma at fixed intervals, even in the absence of MG deterioration, when the initial screening is negative. We analyzed the recurrence rate and incidence of new thymoma in a series of patients with well-controlled MG. The sample consisted of 53 patients, aged 17 to 72 years, and the follow-up varied between 75 and 472 months. The chest computerized tomography detected thymus abnormalities in eight patients at the initial screening and no abnormalities in all patients at a second screening after five years. The findings of this study support the classical opinion that screening for thymoma should be recommended only if there is clinical deterioration due to the disease.

A investigação de timoma é recomendada em pacientes com miastenia gravis (MG) no início da doença, em caso de haver piora clínica ou aumento dos níveis do anticorpo antirreceptor de acetilcolina. Contudo, não foi estabelecido se é necessário repetir a investigação de timoma em intervalos fixos, na ausência de piora clínica, quando a investigação inicial foi negativa. A taxa de recorrência e a incidência de novo timoma foram analisadas em uma série de pacientes com MG bem controlada. A amostra consiste de 53 pacientes, idade entre 17 e 72 anos, com tempo de acompanhamento variando entre 75 e 472 meses. A primeira tomografia computadorizada de tórax detectou anormalidades no timo em oito pacientes durante a investigação inicial da doença e nenhuma anormalidade no segundo exame, após cinco anos de doença, em todos os pacientes. Os achados desse estudo corroboram a clássica opinião de que a investigação de timoma deveria ser recomendada somente se houver piora clínica da doença.

Thymoma associated with myasthenia gravis and Sjögren syndrome / Timoma asociado con miastenia grave y síndrome de Sjögren

Thymoma is the most common neoplasm of the anterior mediastinum but thymoma with Sjögren syndrome (SS) is rare. Sjögren syndrome is a systemic autoimmune inflammatory disorder. It is characterized by lymphocytemediated destruction of exocrine glands, which leads to absent glandular secretion. Here, we present the case of a 63yearold man with thymoma and concurrent myasthenia gravis and SS, who achieved remission after thymectomy.

El timoma es la neoplasia más frecuente del mediastino anterior, pero un timoma acompañado del síndrome de Sjögren (SS) constituye una ocurrencia rara. El síndrome de Sjögren es un trastorno inflamatorio autoinmune sistémico. Se caracteriza por la destrucción - mediada por linfocitos - de las glándulas exocrinas, lo cual conduce a la ausencia de secreción glandular. Aquí presentamos el caso de un hombre de 63 años de edad con timoma, y miastenia gravis y SS concurrentes, que logró la remisión después de una timectomía.

Síndrome de Good y aplasia pura de la serie roja, reporte de un caso y revisión de la literatura: Report of one case / Association of Good syndrome with pure red cell aplasia

Background: The association between Good's syndrome (hypogammaglobulinemia and thymoma) with pure red aplasia is very uncommon. We report a 70-year-old male, who had a thymoma excised nine years before. Afterwards, he suffered frequent respiratory infections, which were attributed to a humoral immunodeficiency. Nine years later, he developed a pure red cell aplasia. He received prednisone and cyclosporine, resulting in a progressive rise of hemoglobin level, after one month of treatment. The patient died shortly thereafter due to infection, complicating a domestic accident.

Isaac's syndrome associated with myasthenia gravis and thymoma.

A 65-year-old male developed fatigable weakness of ocular and bulbar muscle and positive anti-acetyl cholinesterase antibodies suggesting the diagnosis of myasthenia gravis. His condition responded to anticholinesterase and immunotherapy. However, 18 months later, he developed painful paresthesiae, muscle cramps with hyperhiderosis, and was diagnosed as having Isaac's syndrome (neuromyotonia, continuous muscle fibre activity). Computed tomography of the chest revealed a thymic mass, which was confirmed after surgery and histopathology as thymic cell carcinoma. The co-occurrence of myasthenia gravis and continuous muscle fiber activity should prompt the consideration of the occurrence of these disorders as one of the paraneoplastic manifestations, most often due to a thymic neoplasm. Both these conditions respond to treatment of underlying thymoma. This case is a very rare presentation worth reporting.

Tratamento cirúrgico da síndrome da veia cava superior causado por timoma invasivo / Surgical treatment of superior vena cava syndrome caused by invasive thymoma

Paciente do sexo masculino, branco, 57 anos, portador de síndrome da veia cava superior havia 3 meses, devido a timoma invasivo em mediastino médio e anterior, levando a comprometimento da veia cava superior intrínseca e extrinsecamente. Após avaliação por tomografia computadorizada e angiorressonância magnética de tórax, o paciente foi submetido à ressecção radical do timoma - derivação venosa da veia subclávia esquerda para átrio direito, com tubo de PTFE (politetrafluoroetileno). Relevante caso de timoma invasivo ocasionando a oclusão da veia cava superior. A evolução clínica, após 7 meses, foi considerada satisfatória.

We report on a case of a 57 years-old white male, patient, who presented superior vena cava syndrome (SVC) for 3 months, derived from an invasive thymoma in the medium and anterior mediastinum, compromising intrinsic and extrinsic to the SVC. After evaluation by computed tomography and magnetic angioresonance of the thorax, the patient underwent radical resection of the thymoma - bypass from left subclavian vein to right atrium, using polytetrafluoroethylene tube. Relevant case of invasive thymoma causing the occlusion of SVC. The clinic evolution of the patient after 7 months was considered satisfactory.

Acquired Amegakaryocytic Thrombocytopenia after Thymectomy in a Case of Pure Red Cell Aplasia Associated with Thymoma / 대한진단검사의학회지

The association of thymoma with pure red cell aplasia has been well documented, but amegakaryocytic thrombocytopenia is not a recognized paraneoplastic syndrome complicating thymoma. We report a case of thymoma-complicated pure red cell aplasia and amegakaryocytic thrombocytopenia in a 73-yr-old woman. Pure red cell aplasia was diagnosed seven months after the detection of thymoma. One year after the diagnosis of pure red cell aplasia and seven months after thymectomy, bone marrow aspiration and biopsy showed an absence of megakaryocytes, marked erythroid hypoplasia with normal myeloid series. A diagnosis of amegakaryocytic thrombocytopenia and pure red cell aplasia was made. Oral steroid maintenance therapy resulted in recovery of platelet count. She has still transfusion-dependant anemia but platelet and neutrophil counts had been maintained in normal range for more than five months, until the last follow-up. We think that autoreactive T cells may induce a clinical autoimmune response even after eradication of thymoma, and aplastic anemia as a late complication following thymectomy was described in previous cases. This patient also has to be under a close observation because of the possibility to evolve into aplastic anemia.

Thymoma Associated with Myasthenia Gravis in Infancy.

Thymomas are associated with various autoimmune disorders in adults, most commonly with myasthenia gravis (MG). In MG antibodies developed against acetylcholine (ACh) receptors, resulting weakness of muscle after repeated use and recovery following a period of rest. However, MG due to thymoma is extremely rare in pediatric age group.1We report an infant with MG due to invasive thymoma.

Timoma, síndrome de Good y carcinoide intestinal: caso clínico y revision de la literatura / Case report: thymoma Good's syndrome and intestinal carcinoid

The association of thymoma, with myasthenia gravis, pure red cell aplasia and hypogammaglobulinemia (Good's syndrome), has been described. We report a case of a 51 years old woman who after a surgieal resection of a thymoma had several Symptoms and study showed hypogammaglobulinemia. During the study of a persitent diarrhea distal ileum carcinoid tumor was diagnosed. The forgotten association of thymoma and malignancies is of a great clinical significance. Evidence linking thymoma with other neoplasms is provided by the clinical literature.

La asociación de timoma con miastenia gravis, aplasia eritrocítica pura e hipogamaglobulinemia (Síndrome de Good) ha sido descrita. Presentamos el caso de una mujer de 51 años que después de la operación de un timoma presentó varios síntomas confirmándose una hipogammaglobulinemia. En el estudio de una diarrea persistente se encontró un tumor carcinoide del íleon distal. La asociación olvidada entre timoma y cáncer es de gran importancia clínica.

Case of generalised myasthenia gravis with membranous nephropathy.

We report a 40-year old woman with bilateral partial ptosis, complete external ophthalmoplegia, and weakness and fatiguability of upper limbs. She was on treatment for hypertension for 5 months at the time of admission. She was found to have generalised myasthenia gravis and membranous nephropathy with end-stage renal disease. Her symptoms and signs improved within 2 months on treatment with neostigmine and prednisolone. It is postulated that either thymic hyperplasia or the subclinical stage of a thymoma may be the underlying aetiological factor in this patient.

A case of granulomatous lung disease in a patient with Good's syndrome

Good's syndrome is extremely rare. This adult-onset condition is characterized by a thymoma with immunodeficiency, low B- and T-cell counts, and hypo-gammaglobulinemia. The initial clinical presentation is either a mass-lesion thymoma or a recurrent infection. Patients with Good's syndrome are very susceptible to infections; common respiratory and opportunistic infections can be life-threatening. There are no reports of granulomatous lung disease in patients with Good's syndrome, although it has been observed in patients with common variable immunodeficiency, of which Good's syndrome is a subset. We describe a 53-year-old male thymoma patient who presented with respiratory symptoms caused by granulomatous lung disease and an opportunistic infection. He died of uncontrolled fungal infection despite repeated intravenous immunoglobulin and supportive care. Clinicians should look for evidence of immunologic dysfunction in thymoma patients presenting with severe recurrent infections, especially opportunistic infections.

[Guillain-barre syndrome following surgery]

Thymoma is the most common neoplasm of anterior mediastinum with incidence peaks during the fourth, fifth and sixth decades of life. Two-thirds of patients are asymptomatic and identified accidentally by chest x-rays Of the total, 40-70% of patients have one of more parathymic syndromes of which Myasthenia Gravis [MG] is the most common and reported in 10-50% Gullain-Barre Syndrome [CBS] has not been reporterd as a parathymic presentation in present literature. We report a 37 years old woman who presented with CBS after thymoma resection. There is one question: Is there any association between thymoma and CBS or is it a complication of surgery [thymectomy]?

Pure red cell aplasia with malignant thymoma: a rarity.

Pure red cell aplasia sometimes accompanies thymoma. Herein we report a PRCA patient with malignant thymoma. Only two Indian cases of PRCA with malignant thymoma have been reported so far and six cases in the world literature, to the best of our knowledge. This paper describes a malignant thymoma in a 40 year old male who later on developed pure red cell aplasia 7 months after thymectomy.

Intestinal pseudo-obstruction as initial presentation of thymoma.

A 45-year-old-man presented with severe vomiting, constipation, abdominal distention and bilateral ocular abductor palsy. Evaluation revealed diffuse autonomic dysfunction characterized by intestinal pseudo-obstruction, xerophthalmia, xerostomia, postural hypotension, erectile dysfunction and loss of sinus arrhythmia. Paraneoplastic work-up revealed thymoma. Most symptoms resolved after surgical removal of the thymoma. Six weeks later he developed worsening of external ophthalmoparesis with ptosis, responding to acetylcholinesterase inhibitor, confirming myasthenia gravis.

Bócio de tireóide intratorácico e timoma invasivo: apresentação simultânea incomum / Intrathoracic goiter and invasive thymoma: rare concomitant presentation

Apresentamos uma rara situação de ocorrência simultânea de dois tumores de mediastino com diferentes topografias e histologias, encontrados durante a ressecção de volumosa massa mediastinal em paciente assintomático. A possibilidade de diferentes tumores contidos numa mesma massa tumoral está relatada; entretanto, não encontramos na literatura médica relato de diferentes tumores em localizações distintas. Os bócios de tiróide intratorácicos e os timomas representam uma grande parcela dos tumores encontrados no mediastino. O tratamento cirúrgico, sempre que possível, desempenha papel fundamental na perspectiva de cura. A exploração cirúrgica minuciosa é fundamental para uma ressecção completa e possível achado de lesões concomitantes.

We present a rare situation in which two mediastinal tumors of different topology and histology were found during the resection of an extensive mediastinal tumor in an asymptomatic patient. Different histologies within the same mass have been reported, although, to our knowledge, there have been no reports of different tumors at distinct locations. Thymomas and intrathoracic goiters account for a large proportion of the tumors found in the mediastinum. When feasible, surgical resection plays a fundamental role in effecting a cure. In order to identify concomitant lesions and perform a complete resection, detailed surgical exploration is required.