ABSTRACT
Due to its multisystem involvement, IgG4 -related disease should be considered in the differential diagnosis of medical conditions such as lymphadenopathies, aortitis, serositis and retroperitoneal fibrosis. It shares features with other entities historically described as "great mimickers" such as syphilis, tuberculosis, sarcoidosis, and systemic lupus erythematosus. We report a 40 year-old male with recurrent effusive - constrictive pericarditis, lymphadenopathy and aortitis. The study revealed an inactive tuberculosis with negative cultures for acid fast bacilli. The patient had high serum levels of IgG4 and a mediastinal lymph node biopsy was consistent with IgG4 -related disease. The patient was treated with prednisone 40 mg/day with an excellent response.
Subject(s)
Aortitis/diagnosis , Immunoglobulin G/blood , Lymphatic Diseases/diagnosis , Adult , Aortitis/blood , Biopsy , Diagnosis, Differential , Humans , Lymphatic Diseases/blood , Male , Tomography, X-Ray ComputedABSTRACT
Due to its multisystem involvement, IgG4 -related disease should be considered in the differential diagnosis of medical conditions such as lymphadenopathies, aortitis, serositis and retroperitoneal fibrosis. It shares features with other entities historically described as "great mimickers" such as syphilis, tuberculosis, sarcoidosis, and systemic lupus erythematosus. We report a 40 year-old male with recurrent effusive - constrictive pericarditis, lymphadenopathy and aortitis. The study revealed an inactive tuberculosis with negative cultures for acid fast bacilli. The patient had high serum levels of IgG4 and a mediastinal lymph node biopsy was consistent with IgG4 -related disease. The patient was treated with prednisone 40 mg/day with an excellent response.
Subject(s)
Adult , Humans , Male , Aortitis/diagnosis , Immunoglobulin G/blood , Lymphatic Diseases/diagnosis , Aortitis/blood , Biopsy , Diagnosis, Differential , Lymphatic Diseases/blood , Tomography, X-Ray ComputedABSTRACT
Reticular dysgenesis is a rare congenital disorder characterized by severe combined immunodeficiency and profound neutropenia, curable to date, only by bone marrow transplantation. This report describes the association of bilateral sensorineural deafness with this disease.
Subject(s)
Deafness/congenital , Deafness/diagnosis , Leukopenia/congenital , Leukopenia/diagnosis , Lymphatic Diseases/congenital , Lymphatic Diseases/diagnosis , Severe Combined Immunodeficiency/congenital , Severe Combined Immunodeficiency/diagnosis , Thymus Gland , Audiometry , Bone Marrow Transplantation , Evoked Potentials, Auditory, Brain Stem , Female , Histocompatibility Testing , Humans , Incidence , Infant , Infant, Newborn , Leukocyte Count , Leukopenia/blood , Leukopenia/genetics , Leukopenia/therapy , Lymphatic Diseases/blood , Lymphatic Diseases/genetics , Lymphatic Diseases/therapy , Male , Retrospective Studies , Severe Combined Immunodeficiency/blood , Severe Combined Immunodeficiency/genetics , Severe Combined Immunodeficiency/therapySubject(s)
Lymphatic Diseases/complications , Lymphatic Diseases/pathology , Lymphoma, Non-Hodgkin/complications , Lymphoma, Non-Hodgkin/pathology , Pulmonary Fibrosis/complications , Child , Cytokines/metabolism , Fatal Outcome , Female , Humans , Infant, Newborn , Lymph Nodes/pathology , Lymphatic Diseases/blood , Lymphatic Diseases/drug therapy , Lymphoma, Non-Hodgkin/blood , Pulmonary Fibrosis/blood , Pulmonary Fibrosis/pathology , Respiratory Distress Syndrome, Newborn/blood , Respiratory Distress Syndrome, Newborn/complications , Respiratory Distress Syndrome, Newborn/diagnosis , Transforming Growth Factor beta/bloodABSTRACT
The authors present the clinical history of the first case of benign hemophagocytic syndrome diagnosed in Panama. The patient, a 4 year old girl, presented with fever, anemia, cervical lymphadenitis, hepatomegaly, lymphocytosis and histophagocytosis of red cells, lymphocytes, neutrophils and platelets. Spontaneous remission of the fever occurred sixty days after the onset of the disease. Although it was not possible to demonstrate serologically that the syndrome was due to acute toxoplasmosis, she was treated with sulfadiazine and pyrimethamine for fifteen days, on the 37th hospital day, and with clindamycin for ten days, consecutively. Remission occurred seventy days after the onset of fever. A second serological examination for toxoplasmosis (immunofluorescent antibodies) was positive in a titer of 1:2048 again, nine months after the first serology.
Subject(s)
Lymphatic Diseases/physiopathology , Child, Preschool , Erythrocytes , Female , Humans , Lymphatic Diseases/blood , Lymphatic Diseases/drug therapy , Panama , Phagocytosis , SyndromeABSTRACT
Se estudia la história clínica del primer caso de Síndrome Hemofagocítico Benigno (SHB) diagnóstico en Panamá. La paciente presentó un cuadro clínico caracterizado por fiebre, anemia, linfadenitis cervical, hepatomegalia, linfocitosis e histofagocitosis de células rojas y linfocitos de neutrófilos y plaquetas. La remisión de la fiebre se produjó setenta días después del comienzo de la enfermedad. No obstante que no se pudo demostrar serológicamente que el síndrome hemofagocítico era debido a la Toxoplasmosis de tipo agudo se le prescribió Sulfadiazina y Pirimetamina, por quince días (a los 37 días de hospitalización) y Clindamicina, por 10 días (desde los 53 días) en forma consecutiva. La remisión se produjo 70 días después del comienzo de la fiebre. Un segundo examen serológico por toxoplasma (IFA) demostró que la reacción fue nuevamente de 1:2048, nueve meses después de la anterior