Tumor de timo asociado a inmunodeficiencia primaria o síndrome de Good / Thymus tumor associated with primary immunodeficiency or Good syndrome i: en

Se describe el caso clínico de un paciente de 54 años de edad, quien fue ingresado en el Hospital Docente Clínico-Quirúrgico Hermanos Ameijeiras de La Habana por presentar fiebre, hipotensión y diarrea, a lo que se añadió dificultad respiratoria complicada durante su estancia en la institución y requirió apoyo ventilatorio mecánico en la Unidad de Terapia Intensiva. Como hallazgo radiográfico se observó una sombra en el pulmón izquierdo que resultó ser un timoma; mientras que en el examen sanguíneo se comprobó hipogammaglobulinemia. Se decidió realizar timectomía convencional mediante esternotomía media, luego de lo cual evolucionó satisfactoriamente y se le dio el alta hospitalaria 10 días después.

The case report of a 54 years patient is described, who was admitted to Hermanos Ameijeiras Clinical-Surgical Teaching Hospital in Havana city due to fever, hypotension and diarrhea, to which complicated respiratory difficulty was added during his hospitalization and he required mechanical ventilator support in the Intensive Care Unit. As a radiographic finding, a shade in the left lung was observed that turned out to be a thymoma; while the blood test revealed hipogammaglobulinemia. It was decided to carry out conventional thymectomy by means of midline sternotomy, after which the patient had a favorable clinical course and was discharged from the hospital 10 days later.

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.

Opportunistic lung infections as a manifestation of Good's syndrome

Good's syndrome is an acquired immunodeficiency state associated with thymoma. It is characterized by recurrent infection, autoimmune disease, and immunologic abnormality. The insufficient immunity can be managed by intravenous immunoglobulin (IVIG) replacement therapy. We describe 2 patients who presented with Pneumocystis jiroveci pneumonia and Cytomegalovirus pneumonia after thymectomy for a thymoma. Immunologic study revealed hypogammaglobulinemia with very low B-cell count, consistent with Good's syndrome. They were successfully treated with trimethoprim/sulfamethoxazole and gancyclovir respectively, and they are all well without additional infections, receiving regular IVIG replacement.

Good's syndrome with pulmonary lesions: 3 case reports with literature review / 中华全科医师杂志

Three cases of Good's syndrome with pulmonary lesions in our hospital from June 1,2010 to June 1,2013 were retrospective analyzed and relevant literatures were reviewed.Clinical manifestation,characteristics of pulmonary lesions,diagnosis and treatment were summarized.Abnormality of lymphocyte subpopulation to varying degrees in peripheral blood was detected in all 3 cases.However none showed hypogammaglobulinemia.If thymoma patients developed recurrent respiratory infections,Good's syndrome should be considered.Pulmonary manifestations of Good's syndrome have lesions similar to those of diffuse panbronchiolitis or interstitial lung disease.Early screening of immune competency and treatment for immunodeficiency may improve prognosis.

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.

A Case of Good's Syndrome with Weak ABO Reverse Type / 대한수혈학회지

Good's syndrome (thymoma with immunodeficiency) is a rare cause of combined B-cell and T-cell immunodeficiency in adults. We present here a case of Good's syndrome involving a 52 year-old man with an ABO blood group abnormality. He had undergone surgery for thymoma with myasthenia gravis 27 years ago. He also had a history of pulmonary tuberculosis, herpes zoster and pure red cell aplasia. On admission, he was suspected of having pneumonia, and S. pneumoniae was isolated from blood culture. The immunoglobulin levels were markedly decreased. Lymphocyte subset analysis revealed the absence of CD19+ B cells. The result of ABO typing showed a normal strong reaction on the cell typing, but a relatively weak reaction on the serum typing. Therefore, we performed ABO genotyping to confirm his ABO type, which was revealed to be B/O1 . This case suggests that ABO typing should be performed when the diagnosis of Good's syndrome is made. Moreover, Good's syndrome (thymoma with hypogammaglobulinemia) should be considered and evaluated for in patients with a weak ABO reverse type.

A Case of Good's Syndrome

Good's syndrome is the association of thymoma with immunodeficiency, characterized by hypogammaglobulinemia, B-cell lymphopenia and variably defects in cellular immunity with CD4+ T-cell lymphopenia and an inverted CD4+:CD8+ T-cell ratio. We report a 43-year-old male patient who presented with a 18-month history of productive cough and postnasal drip. One year ago, he underwent the operation for resection of a thymoma. Despite of appropriate management, sinusitis relapsed multiple times. He was found to have hypogammaglobulinemia with nearly absent B cells(4/microliter). The CD4+ T-cell count was 554/microliter with an inverted CD4+:CD8+ T-cell ratio of 0.6. His symptoms and signs improved with antibiotic treatment and monthly administration of intravenous immunoglobulin (IVIG, 400 mg/kg).

A Case of Good's Syndrome

Good's syndrome is the association of thymoma with immunodeficiency, characterized by hypogammaglobulinemia, B-cell lymphopenia and variably defects in cellular immunity with CD4+ T-cell lymphopenia and an inverted CD4+:CD8+ T-cell ratio. We report a 43-year-old male patient who presented with a 18-month history of productive cough and postnasal drip. One year ago, he underwent the operation for resection of a thymoma. Despite of appropriate management, sinusitis relapsed multiple times. He was found to have hypogammaglobulinemia with nearly absent B cells(4/microliter). The CD4+ T-cell count was 554/microliter with an inverted CD4+:CD8+ T-cell ratio of 0.6. His symptoms and signs improved with antibiotic treatment and monthly administration of intravenous immunoglobulin (IVIG, 400 mg/kg).

A case of Good's syndrome / 천식및알레르기

A 52-year-old woman suffering from recurrent orogenital ulcerations with superficial candidiasis and chronic mucocutaneous herpes simplex infection was admitted for headache and productive cough. She had undergone a thymectomy due to thymoma of an epithelial cell type several years ago. Radiologic and immunologic examinations revealed bronchiectasis in the left lower lobe, severe deficiency in all isotypes of immunoglobulin and an abnormal delayed hypersensitivity to ubiquitous antigens on skin test. Analysis of lymphocyte subsets in peripheral blood and bone marrow showed marked decreases in the proportion of cells bearing B cell markers. Her symptoms and signs were improved with antibiotics, antifungal and antiviral agents, and monthly administration of high dose intravenous immunoglobulin (IVIG, 400mg/kg). This is a rare case of Good's syndrome presenting, a humoral and cellular immune deficiency syndrome related with thymoma. The pathogenic mechanism of hypogammaglobulinemia in this case might be caused by a block in the early stage of B cell differentiation.