Sjogren's Induced CD4+ Lymphocytopenia Causing Pulmonary MAC Infection (original) (raw)
INTRODUCTION: Sjogren's Syndrome (SS) is known to cause lymphocytopenia, and there have been cases of opportunistic infections described in patients with Sjogren's disease without evidence of AIDS(1) .To our knowledge, there has been no report describing MAC infection due to chronic lymphocytopenia induced by Sjogren's syndrome. CASE PRESENTATION: A 38F with insignificant medical history presented to ER with complaints of fever, dyspnea, and productive cough for 6 months. She was initially hospitalized at another facility 3 months prior with same complaints and discharged on azithromycin. She was later notified that she had grown mycobacterium avium complex from respiratory cultures. She was offered treatment but was unable to afford. HIV testing done at outside hospital as well as repeat testing at our institution were negative. During hospitalization at our institute, she had sputum that was positive for AFB however, TB PCR returned negative. CT chest showed severe bilateral upper cavitary lung disease (Fig;1). She was started on ethambutol, rifabutin and clarithromycin. Autoimmune testing revealed positive SSA (RO) antibody. Blood flow cytometric analysis did not reveal evidence of myeloproliferative disorder however, absolute CD4 helper T cells was 95/ul. DISCUSSION: US Centers for Disease Control (CDC) defines idiopathic CD4+ T lymphocytopenia (ICL) as: prolonged (at least 2) low counts of <300/mm3 or <20% CD4+ without HIV infection or other known causes of immunosuppression. The association between SS and ICL has already been observed previously. Pulmonary MAC is an AIDS defining illness and commonly presents in HIV positive patients with low CD4 counts. In our patient, susceptibility was due to a profound and persistent depletion of CD4+ T cells. No infection, therapy, malnutrition or defined immunodeficiency could be implicated. Opportunistic infections
Related papers
CD-4/CD-8 lymphocytopenia in HIV negative patients with severe, chronic granulomatous infections
The Southwest Respiratory and Critical Care Chronicles, 2016
Background: CD-4 lymphocytopenia can occur in acquired immunodeficiency syndrome (AIDS), in severe combined immunodeficiency, with the use of corticosteroids and/or immunosuppressive drugs, and in patients with idiopathic CD-4 lymphocytopenia. The mechanism for the lymphocytopenia is different in each of these illnesses. Objective: Description of HIV-negative patients with severe disseminated tuberculosis or coccidioidomycosis and lymphocytopenia. Settings and patients: All patients were referred to a University Medical Center in Northwest Texas, USA. Four had disseminated tuberculosis, and three had disseminated coccidioidomycosis. Main outcome measures: Follow-up of lymphocyte subset counts and clinical improvement with the treatment of the underlying granulomatous infection. Results: Five patients had an increase in both CD-4 and CD-8 lymphocyte subset counts with treatment of the underlying granulomatous infection. All patients had clinical improvement with initial therapy of the granulomatous infection. One patient succumbed to disseminated tuberculosis (meningitis) and two to disseminated coccidioidomycosis. One patient was lost to follow up. Conclusions: We report a group of HIV-negative patients who had CD-4 lymphocytopenia in response to severe, disseminated, chronic granulomatous infections. With the treatment of the granulomatous infection the lymphocytopenia improved. This finding, coupled with preserved CD-4/CD-8 ratios, can help to differentiate these patients from those with other causes of lymphocytopenia or AIDS.
Journal of Evidence Based Medicine and Healthcare, 2017
BACKGROUND Since 1989, several investigators have reported unusual cases of severe opportunistic infections associated with CD4 lymphocytopenia in the absence of human immunodeficiency virus infection. The cause of this condition is unknown. The Centres for Disease Control and Prevention (CDC) defines Idiopathic CD4 T Lymphocytopenia (ICL) as a clinical condition in which patients with depressed numbers of circulating CD4+ T-cell lymphocytes (<300 cells/μL or <20% of total T cells) at a minimum of two separate time points at least 6 weeks apart, have no laboratory evidence of infection with human HIV-1 or HIV-2, or any defined immunodeficiency or therapy associated with depressed levels of CD4 T cells. The aim of the study is to analyse the clinical profile, opportunistic infections, laboratory parameters and outcome in terms of survival of patients diagnosed with ICL. MATERIALS AND METHODS Eight HIV negative patients who presented with opportunistic infections and who were diagnosed with ICL from 2007 to 2015 were included in the study. A detailed history was taken; physical examination was performed and the nature of illness with which they presented was documented. Then, CD4 and CD8 counts were done and CD4 count was repeated after a 6-week interval. The patients were followed up until discharge or death. RESULTS The mean age was 37.50±9.55 years. There were six males (75%) and two females (25%). Fever was a presenting symptom among six (75%) of them. Two were diagnosed to have cutaneous cryptococcosis (25%), two with invasive aspergillosis (25%) and four with tuberculosis (50%). Absolute lymphocyte count was less than 1200 in seven patients (87.5%), which roughly correlates with a CD4 count of less than 200 cells/μL, among PLWHIV. The mean CD4 count was 183.63±63.74 cells/μL during the first measurement and 214.43±103.98 cells/μL during the second one. Two patients died (37.5%). None of the patients were recorded to have any form of malignancy.
A Case of Isolated Pulmonary MAC Being the First Presentation of a Newly Diagnosed HIV/AIDS
2020
39y old HIV positive female with isolated manifestations of pulmonary Mycobacterium Avium Complex infection being initial presentation.Pulmonary MAC infection is extremely rare, with an incidence of 1 case per 100,000 per year. we have not found any cases of newly diagnosed HIV/AIDS patients with pulmonary MAC being the initial symptom Key Clinical Message Pulmonary MAC infection in a patient without structural lung disease is extremely rare. There is no case report of HIV/AIDS patient in known their initially presenting primary diagnosis is pulmonary MAC infection.
Idiopathic CD4 lymphocytopenia: a case of missing, wandering or ineffective T cells
Arthritis Research & Therapy, 2012
Idiopathic CD4 lymphocytopenia (ICL) was described in 1992 as an immunodefi ciency syndrome characterized by opportunistic infections and low CD4 T-cell counts in the absence of HIV infection. Despite the 20 years that have elapsed, the clinical spectrum, pathogenesis, and possible treatment for ICL remain obscure. Here, we attempt to summarize the salient features of this condition on the basis of the available literature to date.
Idiopathic CD4+ lymphocytopenia in Hispanic male: case report and literature review
International Medical Case Reports Journal, 2014
Idiopathic cluster of differentiation 4 (CD4+) T-cell lymphocytopenia (ICL) is a rare non human immunodeficiency virus (HIV)-related syndrome with unclear natural history and prognosis that was first reported and defined in 1992. ICL has been observed in patients after the onset of an opportunistic infection without known immunosuppression. Case presentation: A 20-year-old Hispanic male patient without significant past medical history presented with progressive shortness of breath and cough for 3 weeks. Chest computed tomography showed bilateral cavitary lesions in the upper lung lobes. The HIV rapid screening test as well as the sputum acid-fast bacilli test were both positive. The patient was started on antituberculosis therapy. The CD4 count was noticed to be low. However, the HIV Western blot test was negative, and the HIV viral load was within normal limit. Further radiologic studies, hemato-oncologic, and autoimmune workups were normal. The patient was discharged on the treatment for tuberculosis. Follow-up after 8 weeks revealed a persistent low CD4+ count, and the repeated HIV tests were negative. Conclusion: The clinical features of ICL range from an asymptomatic condition to life-threatening complications that imitate the clinical course of HIV-infected patients. The differential diagnosis in adults comprises primarily HIV infection and other diseases or drug side effects. ICL is very rare and should be considered in the absence of any defined immunodeficiency or therapy associated with depressed levels of CD4+ T-cells. Early detection and recognition of the disease allow purposeful and systemic treatment approach and screening for the affected patients.
Annals of Internal Medicine, 1991
respiratory problems continue to be a major burden of disease in hiVinfected patients, with more than 50% suffering at least one respiratory episode. in early hiV infection, respiratory infections are similar to those found in the general population, but more common. Progressive hiV-induced immunosuppression increases the risk of opportunistic infections and tumours. highly active antiretroviral therapy (haart) has reduced the incidence of hiV-associated opportunistic infections and tumours, but has had less impact on the incidence of bacterial pneumonia, tuberculosis and non-hodgkin lymphoma. haart may trigger immune phenomena including over-exuberant and uncontrolled immune response to exogenous antigen most commonly seen in Mycobacterium tuberculosis, where two 'syndromes' have been described: immune reconstitution inflammatory syndrome (iris) and 'unmasking' of asymptomatic, latent, indolent or incipient infection (ali3).
Loading Preview
Sorry, preview is currently unavailable. You can download the paper by clicking the button above.