RESUMEN
Disseminated Cryptococcosis infection typically occurs in immunocompromised patients, often manifested as pneumonia or meningoencephalitis. Cases with involvement of either prostate or adrenal glands are less frequent. We describe a case of an immunocompromised 62-year-old man with new-found Idiopathic CD4 + T lymphocytopenia who presented with urinary irritation symptoms followed by headache. The patient was finally diagnosed as disseminated cryptococcosis of prostate, adrenal gland involvement with the help of combining histopathology of formalin-fixed, paraffin-embedded tissue with metagenomic next-generation sequencing technique to identify C neoformans sensu stricto in prostate, adrenal gland tissues. Clinicians should be aware of atypical presentations of cryptococcal disease. In this case of cryptococcosis in immunocompromised patients, we find that cryptococcosis can affect varied organs simultaneously and should be considered in the differential of infectious diseases. And mNGS technology helps to confirm the diagnosis.
Asunto(s)
Criptococosis , Cryptococcus neoformans , Meningoencefalitis , Linfocitopenia-T Idiopática CD4-Positiva , Masculino , Humanos , Persona de Mediana Edad , Próstata , Criptococosis/complicaciones , Criptococosis/diagnóstico , Linfocitopenia-T Idiopática CD4-Positiva/complicaciones , Linfocitopenia-T Idiopática CD4-Positiva/diagnósticoRESUMEN
INTRODUCTION: Idiopathic CD4+ Lymphocytopenia (ICL) is a rare entity grouped in non- HIV-related syndromes. ICL is characterized by a marked low CD4 T cell count of <300 cells/mm3 with ambiguous natural history and prognosis. In addition, cryptococcal and nontuberculous mycobacterial infections are reported as known opportunistic infections. Therefore, management turns around vigilant follow-up and treatment of the current clinical scenario of these patients. CASE PRESENTATION: Here, a 55-year-old lady was referred with a history of diffuse headache and intermittent fever for two months, projectile vomiting, and altered mental status for five days. Nonpruritic maculopapular rashes and diffuse desquamation of the skin were noted. She had no significant previous medical history. Based on clinical findings and investigations, she was diagnosed with ICL having disseminated cryptococcosis. Unfortunately, the patient did not undergo specific treatment as she was recognized late, and unfortunately, she died. CONCLUSION: It is of paramount importance to recognize the clinical entity as early as possible to start appropriate treatment, which may positively impact the outcome. Therefore, the clinician must be aware of disseminated cryptococcosis associated with non-HIV states.
Asunto(s)
Criptococosis , Linfopenia , Linfocitopenia-T Idiopática CD4-Positiva , Femenino , Humanos , Persona de Mediana Edad , Linfocitopenia-T Idiopática CD4-Positiva/complicaciones , Linfocitopenia-T Idiopática CD4-Positiva/diagnóstico , Criptococosis/complicaciones , Criptococosis/diagnóstico , Criptococosis/tratamiento farmacológico , Linfopenia/complicaciones , Linfopenia/microbiología , Linfocitos T CD4-Positivos , Recuento de Linfocito CD4RESUMEN
BACKGROUND: Idiopathic CD4 + T lymphocytopenia (ICL) is a rare immunodeficiency syndrome, unaccompanied by various opportunistic infections. Cryptococcus and varicella-zoster viruse are the most common opportunistic infections. METHOD: We described a case of disseminated cryptococcosis with varicella-zoster virus coinfection in a patient with ICL and reviewed all published reports. A total of 26 cases with cryptococcal meningitis in ICL were enrolled. DISCUSSION: ICL remains poorly understood to clinicians. Patients with cryptococcal meningitis in ICL mostly suffered with headache and fever in a subacute or chronic period, while some patients might have atypical manifestations which makes a difficulty for early diagnosis. Some characteristics of cerebrospinal fluid can help to predict the prognosis of the disease. Cryptococcosis with varicella-zoster virus coinfection is rare but serious. CONCLUSION: We recommed CD4 + T cells should be assessed in patients with unusual or recurrent infections. As the underlying pathophysiology is poorly understood, there is no standard therapy for ICL. Increased awareness of the disease and early prevention for CD4 reduction are needed.
Asunto(s)
Coinfección , Criptococosis , Linfocitopenia-T Idiopática CD4-Positiva , Linfocitos T CD4-Positivos , Coinfección/complicaciones , Coinfección/diagnóstico , Criptococosis/complicaciones , Criptococosis/diagnóstico , Criptococosis/tratamiento farmacológico , Herpesvirus Humano 3 , Humanos , Linfocitopenia-T Idiopática CD4-Positiva/complicaciones , Linfocitopenia-T Idiopática CD4-Positiva/diagnósticoAsunto(s)
Enfermedades del Sistema Nervioso/etiología , Linfocitopenia-T Idiopática CD4-Positiva/complicaciones , Adulto , Biopsia , Encéfalo/diagnóstico por imagen , Recuento de Linfocito CD4 , Resultado Fatal , Femenino , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Mirtazapina/uso terapéutico , Enfermedades del Sistema Nervioso/diagnóstico , Enfermedades del Sistema Nervioso/tratamiento farmacológico , Enfermedades del Sistema Nervioso/psicología , Esteroides/uso terapéutico , Linfocitopenia-T Idiopática CD4-Positiva/diagnóstico , Linfocitopenia-T Idiopática CD4-Positiva/tratamiento farmacológicoRESUMEN
Idiopathic CD4+ T lymphocytopenia (ICL) is a very rare immunodeficiency syndrome with an unexplained depletion of CD4+ T lymphocytes and no evidence of Human Immunodeficiency Virus (HIV) infection. Here we report a 29-year-old male patient who had severe ulcerative colitis with low level CD4+ count of 254 cells/mm3, and had no evidence of HIV or Human T cell Lymphotrophic virus type I or II infections. He had recurrent Candidiasis infection and his CD4 count was just 53 cells/mm3 after 3 months. The cause for the decline of CD4 T lymphocytes was unknown.
Asunto(s)
Linfocitos T CD4-Positivos/patología , Candidiasis/epidemiología , Colitis Ulcerosa/complicaciones , Linfocitopenia-T Idiopática CD4-Positiva/diagnóstico , Adulto , Humanos , Masculino , Linfocitopenia-T Idiopática CD4-Positiva/sangreRESUMEN
Idiopathic CD4 lymphocytopenia (ICL) is a rare disease characterized by marked loss of CD4 T-cells without human immunodeficiency virus infection. CD4 T-cells play an important role in granuloma formation in cryptococcal infection. Thus far, among ICL patients, it has not been concluded definitely whether granuloma is formed or not. We report the case of a 39-year-old woman with ICL and disseminated cryptococcal infection with granuloma formation. She was referred to our department because of a lung mass, osteolytic lesion, and a subcutaneous mass identified on a computed tomography scan, and an elevated C-reactive protein level. Cryptococcus neoformans was isolated from the tissues. She also had marked CD4 lymphocytopenia (33 cells/µL), without human immunodeficiency virus infection. In a biopsy specimen of the lung mass, granulomas containing CD4 T-cells were observed. The cryptococcosis was treated with liposomal amphotericin B followed by fluconazole and she was found to be cured. The CD4 T-cell count was persistently low. This case showed that granulomas containing CD4 T-cells can be formed in ICL patients with cryptococcal infection despite very low CD4 T-cell counts.
Asunto(s)
Criptococosis/diagnóstico , Granuloma/diagnóstico , Linfocitopenia-T Idiopática CD4-Positiva/diagnóstico , Adulto , Anfotericina B/uso terapéutico , Antifúngicos/uso terapéutico , Biopsia/métodos , Recuento de Linfocito CD4 , Criptococosis/complicaciones , Criptococosis/tratamiento farmacológico , Criptococosis/inmunología , Cryptococcus neoformans/aislamiento & purificación , Femenino , Fluconazol/uso terapéutico , Granuloma/complicaciones , Granuloma/inmunología , Granuloma/patología , Humanos , Pulmón/diagnóstico por imagen , Síndrome de Sjögren/complicaciones , Síndrome de Sjögren/diagnóstico , Linfocitopenia-T Idiopática CD4-Positiva/complicaciones , Linfocitopenia-T Idiopática CD4-Positiva/inmunología , Tomografía Computarizada por Rayos X/métodos , Resultado del TratamientoRESUMEN
Miller-Dieker syndrome (MDS; OMIM 247200) is a rare contiguous gene deletion syndrome associated with lissencephaly and characteristic facial dysmorphism. T-cell lymphopenia is an immunodeficiency disorder which can be early detected by newborn blood screening, and all live vaccines should be avoided. We report a 2.32-Mb microdeletion at chromosome 17p13.3p13.2 and T-cell lymphopenia in a 6-month-old male infant with MDS. This is, to our knowledge, the first description of these 2 conditions co-occurring in the same patient.
Asunto(s)
Lisencefalias Clásicas y Heterotopias Subcorticales en Banda/diagnóstico , Linfocitopenia-T Idiopática CD4-Positiva/diagnóstico , Cromosomas Humanos Par 17/genética , Lisencefalias Clásicas y Heterotopias Subcorticales en Banda/genética , Comorbilidad , Humanos , Hibridación Fluorescente in Situ , Lactante , Masculino , Eliminación de Secuencia , Linfocitopenia-T Idiopática CD4-Positiva/genéticaRESUMEN
Cryptococcosis is an opportunistic fungal infection commonly seen in HIV cases. We present a case of disseminated cryptococcosis with multiple non-continuous infective foci in a non-HIV, non-transplant case.
Asunto(s)
Enfermedades de los Anexos , Criptococosis , Meningitis Criptocócica , Linfocitopenia-T Idiopática CD4-Positiva , Enfermedades de los Anexos/complicaciones , Enfermedades de los Anexos/diagnóstico , Enfermedades de los Anexos/patología , Criptococosis/complicaciones , Criptococosis/diagnóstico , Criptococosis/patología , Femenino , Humanos , Meningitis Criptocócica/complicaciones , Meningitis Criptocócica/diagnóstico , Meningitis Criptocócica/patología , Persona de Mediana Edad , Linfocitopenia-T Idiopática CD4-Positiva/complicaciones , Linfocitopenia-T Idiopática CD4-Positiva/diagnóstico , Linfocitopenia-T Idiopática CD4-Positiva/patologíaAsunto(s)
Pneumocystis carinii , Neumonía por Pneumocystis/etiología , Linfocitopenia-T Idiopática CD4-Positiva/complicaciones , Recuento de Linfocito CD4 , Humanos , Pulmón/diagnóstico por imagen , Pulmón/microbiología , Masculino , Persona de Mediana Edad , Neumonía por Pneumocystis/diagnóstico por imagen , Neumonía por Pneumocystis/microbiología , Radiografía Torácica , Linfocitopenia-T Idiopática CD4-Positiva/diagnóstico , Tomografía Computarizada por Rayos XAsunto(s)
Criptococosis/complicaciones , Criptococosis/diagnóstico , Cefalea/etiología , Letargia/etiología , Linfocitopenia-T Idiopática CD4-Positiva/complicaciones , Linfocitopenia-T Idiopática CD4-Positiva/diagnóstico , Encéfalo/diagnóstico por imagen , Edema Encefálico/complicaciones , Edema Encefálico/diagnóstico por imagen , Niño , Cryptococcus neoformans , Diagnóstico Diferencial , Femenino , Humanos , Imagen por Resonancia Magnética/métodosRESUMEN
A 67-year-old man was admitted to our hospital complaining of dry cough. Chest computed tomography showed diffuse infiltrates and ground-glass opacities in the bilateral lung fields. Transbronchial lung biopsy specimens showed alveoli filled with yeast-like fungi. With a diagnosis of pneumocystis pneumonia (PCP), he was given oral sulfamethoxazole/trimethoprim, to which he responded well. However, seven months later, PCP relapsed. Analyses revealed a low bronchoalveolar lavage fluid CD4/CD8 ratio of 0.04 and CD4+ lymphocytopenia (250/µL). Despite intensive work-up, we were unable to detect the underlying cause of CD4+ lymphocytopenia; therefore, a final diagnosis of idiopathic CD4+ T-lymphocytopenia was made.
Asunto(s)
Síndrome de Inmunodeficiencia Adquirida/complicaciones , Antibacterianos/uso terapéutico , Neumonía por Pneumocystis/diagnóstico , Neumonía por Pneumocystis/tratamiento farmacológico , Linfocitopenia-T Idiopática CD4-Positiva/diagnóstico , Linfocitopenia-T Idiopática CD4-Positiva/tratamiento farmacológico , Combinación Trimetoprim y Sulfametoxazol/uso terapéutico , Anciano , Líquido del Lavado Bronquioalveolar/microbiología , Humanos , Pulmón/patología , Masculino , Pneumocystis/aislamiento & purificación , Neumonía por Pneumocystis/etiología , Linfocitopenia-T Idiopática CD4-Positiva/etiología , Tomografía Computarizada por Rayos X , Resultado del TratamientoRESUMEN
BACKGROUND: Idiopathic CD4 lymphocytopenia (ICL) is a rare condition characterized by an unexplained deficit of circulating CD4 T cells leading to increased risk of serious opportunistic infections. The pathogenesis, etiology, clinical presentation, and best treatment options remain unclear. OBJECTIVE: To describe the clinical presentation, treatment strategies, and outcome of patients with ICL seen in a single referral center. METHODS: In a retrospective study, from January 1993 to January 2014, the demographic characteristics, clinical presentation, and treatments of patients diagnosed with ICL were reviewed. RESULTS: Twenty-four patients (14 female [58%] and 10 male [42%]) were evaluated. The mean age was 45 ± 17.6 years (range 7-76 years). Mean CD4 and CD8 T-cell counts at the time of diagnosis were 119 ± 84/mm3 (range 4-294/mm3) and 219 ± 258/mm3 (range 7-630/mm3), respectively. Seventeen patients (71%) had opportunistic infections, 4 (17%) had malignancies, and 3 (13%) had unexplained demyelinating disease and neurologic problems. Most patients had normal levels of immunoglobulins. Thirteen patients had abnormally low to absent response to phytohemagglutinin, concanavalin A, and antigens (candida and tetanus). Three patients had resolution of warts and 1 had mycobacterial lung infection on interleukin-2 with increases in CD4 count. The 11 patients on trimethoprim and sulfamethoxazole had no further hospital admissions for infections. CONCLUSION: The pathogenesis of ICL remains unclear. Although only some patients are healthy, most patients present with opportunistic infections. There is no known standard treatment aside from prophylactic antibiotics.
Asunto(s)
Antibacterianos/uso terapéutico , Enfermedades Desmielinizantes/tratamiento farmacológico , Neoplasias/tratamiento farmacológico , Infecciones Oportunistas/tratamiento farmacológico , Linfocitopenia-T Idiopática CD4-Positiva/tratamiento farmacológico , Adolescente , Adulto , Anciano , Recuento de Linfocito CD4 , Linfocitos T CD4-Positivos/efectos de los fármacos , Linfocitos T CD4-Positivos/inmunología , Linfocitos T CD4-Positivos/patología , Linfocitos T CD8-positivos/efectos de los fármacos , Linfocitos T CD8-positivos/inmunología , Linfocitos T CD8-positivos/patología , Niño , Enfermedades Desmielinizantes/complicaciones , Enfermedades Desmielinizantes/diagnóstico , Enfermedades Desmielinizantes/inmunología , Manejo de la Enfermedad , Femenino , Humanos , Masculino , Persona de Mediana Edad , Neoplasias/complicaciones , Neoplasias/diagnóstico , Neoplasias/inmunología , Infecciones Oportunistas/complicaciones , Infecciones Oportunistas/diagnóstico , Infecciones Oportunistas/inmunología , Estudios Retrospectivos , Sulfametoxazol/uso terapéutico , Linfocitopenia-T Idiopática CD4-Positiva/complicaciones , Linfocitopenia-T Idiopática CD4-Positiva/diagnóstico , Linfocitopenia-T Idiopática CD4-Positiva/inmunología , Trimetoprim/uso terapéuticoRESUMEN
We report a 42 year old male who was an interesting case of "idiopathic CD4 lymphocytopenia"(ICL) in a non-HIV adult with-extra pulmonary tuberculosis along with the diffuse splenic calcification and whose symptoms improved with Antitubercular treatment. He was found to have low CD4 counts on two occasions.
Asunto(s)
Ganglios Linfáticos/diagnóstico por imagen , Bazo/diagnóstico por imagen , Linfocitopenia-T Idiopática CD4-Positiva/diagnóstico , Tuberculosis Pulmonar/tratamiento farmacológico , Adulto , Recuento de Linfocito CD4 , Linfocitos T CD4-Positivos/patología , Humanos , Masculino , Infecciones Oportunistas/sangre , Infecciones Oportunistas/diagnóstico , Tomografía de Emisión de Positrones , Linfocitopenia-T Idiopática CD4-Positiva/sangre , Tomografía Computarizada por Rayos X , Resultado del Tratamiento , Tuberculosis Pulmonar/complicacionesRESUMEN
Herpes zoster (HZ) is of rare occurrence after interventional procedures with few events reported until now. A 74 year-old man with a past medical history of idiopathic thrombocytopenic purpura, splenectomy, autoimmune hemolytic anemia, and polymyalgia rheumatica developed HZ on the right median nerve 7 days after he underwent a coronariography for managing an acute coronary syndrome. He evolved with cutaneous dissemination and required intravenous acyclovir therapy. Laboratory evaluation disclosed a previously unknown idiophatic CD4 lymphocytopenia. HZ should be added to the list of complications after interventional cardiology and associated immunosuppressive factors ruled out.
Asunto(s)
Herpes Zóster/diagnóstico , Herpes Zóster/patología , Intervención Coronaria Percutánea/efectos adversos , Complicaciones Posoperatorias/diagnóstico , Complicaciones Posoperatorias/patología , Linfocitopenia-T Idiopática CD4-Positiva/complicaciones , Linfocitopenia-T Idiopática CD4-Positiva/diagnóstico , Anciano , Humanos , MasculinoRESUMEN
A case history is presented of a 35-year-old man admitted to the hospital with tuberculous meningitis complicated by caseous necrosis of cervical lymph nodes and thrombosis of the left jugular vein. Another complication, malignant brain edema, appeared more than one year after discharge from hospital and was managed at the neurosurgery department. The most probable cause was a post-inflammatory obstruction of the cerebrospinal fluid pathways. A challenging finding, observed repeatedly while in hospital and at follow ups after discharge, was medium significant CD4+ T cell lymphopenia, with the lowest CD4+ T cell count of 308 cells/µl of peripheral blood. For this reason, the patient was screened several times for anti-HIV antibodies, but always with a negative result. Active tuberculous infection was considered as another possible reason behind persistent CD4+ T cell lymphopenia. However, imaging and laboratory analyses were not suggestive of tuberculosis. The patient is currently in good condition and his CD4+ T lymphocyte counts returned to normal at seven years of follow-up. It is underlined that patients after tuberculous meningitis need a long-term follow-up.
Asunto(s)
Recuento de Linfocito CD4 , Linfocitos T CD4-Positivos/inmunología , Linfocitopenia-T Idiopática CD4-Positiva/diagnóstico , Tuberculosis Meníngea/diagnóstico , Adulto , Humanos , Ganglios Linfáticos , Linfopenia , Masculino , Linfocitopenia-T Idiopática CD4-Positiva/complicaciones , Tuberculosis Meníngea/inmunologíaAsunto(s)
Colangitis Esclerosante/genética , Mutación del Sistema de Lectura/genética , Síndromes de Inmunodeficiencia/genética , Subunidad alfa del Receptor de Interleucina-21/genética , Linfocitopenia-T Idiopática CD4-Positiva/genética , Niño , Colangitis Esclerosante/complicaciones , Colangitis Esclerosante/diagnóstico , Humanos , Síndromes de Inmunodeficiencia/complicaciones , Síndromes de Inmunodeficiencia/diagnóstico , Masculino , Linfocitopenia-T Idiopática CD4-Positiva/complicaciones , Linfocitopenia-T Idiopática CD4-Positiva/diagnósticoRESUMEN
Idiopathic CD4+ T-Lymphocytopenia is a rare immunodeficiency disorder characterised by significantly low absolute CD4 lymphocytes in absence of any viral infections. We present a case of Disseminated Cryptococcosis with Caverno- Oesophageal Fistula in a case of Idiopathic CD4+ T-Lymphocytopenia. 29 year old lady was referred to Institute in view of lung mass not responding to anti-TB treatment. Subsequently patient had developed headache. Radiological evaluation showed presence of ring enhancing lesion in the occipital region. On evaluation with Fibre-optic bronchoscopy, there was no evidence of malignancy or tuberculosis. Sputum showed presence and growth of Cryptococcus neoformans. Patient's investigations were negative for virus infection, with normal immunoglobulin levels. Her CD4 counts were 129 cells/mm3. Patient was treated with injectable antifungals. Patient developed a Caverno-oesophageal fistula which was confirmed on endoscopy and radiology. Patient was managed with percutaneous jejunal feeding (PEJ). Patient improved symptomatically with CD4 count of 475 cells/mm3.
Asunto(s)
Criptococosis/complicaciones , Criptococosis/epidemiología , Cryptococcus neoformans , Fístula Esofágica/diagnóstico por imagen , Fístula del Sistema Respiratorio/diagnóstico por imagen , Linfocitopenia-T Idiopática CD4-Positiva/epidemiología , Adulto , Antifúngicos/administración & dosificación , Fístula Esofágica/etiología , Femenino , Fluconazol/administración & dosificación , Humanos , Lóbulo Occipital/microbiología , Radiografía , Fístula del Sistema Respiratorio/etiología , Esputo/microbiología , Linfocitopenia-T Idiopática CD4-Positiva/diagnósticoRESUMEN
We report a case of an HIV-seronegative pregnant woman with disseminated cryptococcosis, poorly controlled during gestation. Immunological studies showed T-lymphocytopenia during gestation, but rapid recovery postpartum. T-lymphocytopenia may play a role in increased susceptibility to and severity of cryptococcal infection during pregnancy.