scholarly journals Hemophagocytic Syndrome Associated with Immune Reconstitution Inflammatory Syndrome in a Patient with AIDS Related Burkitt’s Leukemia/Lymphoma

2014 ◽  
Vol 2014 ◽  
pp. 1-3 ◽  
Author(s):  
Metin Kanıtez ◽  
Mahir Kapmaz ◽  
Nilufer Alpay ◽  
Fatih Selcukbiricik ◽  
Atahan Çağatay ◽  
...  

Highly active antiretroviral therapy (HAART) has markedly decreased human immunodeficiency virus- (HIV-) related mortality and the incidence of opportunistic infections. The dramatic reduction in HIV-1 RNA and increase in CD4 lymphocyte count mean a recovery in immune function. This restoration in immune function may be associated with paradoxical deterioration in subclinical opportunistic infections in some patients, a condition called immune reconstitution inflammatory syndrome (IRIS). IRIS, a “paradoxical” inflammatory response to either previously treated or subclinical infections or noninfectious diseases, can manifest during the restoration phase of immunity hemophagocytic syndrome (HS) which is a very rare complication in patients with acquired immune deficiency syndrome (AIDS). We describe a case of hemophagocytic syndrome associated with IRIS in a patient with AIDS related Burkitt’s leukemia/lymphoma (BL). IRIS was probably the cause of hemophagocytosis for our patient. Zoster infection may facilitate to IRIS. With the increasing number of people with HIV infection and the accompanying use of HAART, much more clinical manifestations of IRIS will be experienced especially in patients given high dose chemotherapy, just like in our case.

2016 ◽  
Vol 8 (4) ◽  
Author(s):  
Markela Pagonitsa Zorzou ◽  
Maria Chini ◽  
Athina Lioni ◽  
Georgios Tsekes ◽  
Thomas Nitsotolis ◽  
...  

Although the connection of [secondary hemophagocytic syndrome (sHS)] with HIV has been well documented, optimal treatment regimen is not well established. This is due not only to the rarity of the syndrome, but also to the heterogeneity of the involved population. Most cases are related to opportunistic infections or malignancies in advanced stage, but many cases are also related to seroconversion, in the primary infection setting. Moreover, in the [antiretroviral treatment (ART)] era, rare cases of ART-related sHS have been reported. In these, often fatal cases, an [immune reconstitution inflammatory syndrome (IRIS)] process is involved, posing a serious challenge to the treating physician. We hereby report a case of successful treatment of an HIV patient with primary effusion lymphoma who experienced sHS shortly after ART onset. Our patient, treated with high dose dexamethasone and gamma globulin, achieved complete remission. This case might hint possible therapeutic insights in the treatment of IRIS-related sHS.


2018 ◽  
Vol 8 (2) ◽  
pp. 139-149 ◽  
Author(s):  
E. V. Boeva ◽  
N. A. Belyakov

Antiretroviral therapy (ART) leads to suppression of HIV replication, contributes to increase in the number of CD4-lymphocytes count and  partial restoration or activation of the immune system. The  consequence is a reduction of incidence of opportunistic diseases,  increase in the duration and quality of life of people living with HIV  (PLHIV). However, in some patients with severe immunosuppression, this may be accompanied by a worsening of the condition and risks  of formation of the immune reconstitution inflammatory syndrome  (IRIS), which manifests itself in the development of new or  previously treated opportunistic, secondary and exacerbating non- infectious diseases against a virologically effective ART. The  frequency of the development of IRIS varies widely, in cases of  tuberculosis-associated manifestation it can reach 50%. Risk factors  for the development of IRIS are low initial CD4-lymphocyte count  and a high load of HIV RNA in the blood, the presence of  opportunistic infections during the initiation of ART. Discussed terminology issues, other possible risk factors for the  development of the syndrome, regularities of the pathological process are considered. Epidemiological statistics of  IRIS, pathogenetic bases, variants of clinical and laboratory  manifestations of complications are given. The criteria for diagnosis  of the syndrome, as well as the necessary conditions for its  occurrence, are considered. Particular attention is paid to the most  common opportunistic infections that cause the manifestation of  IRIS, the peculiarities and polymorphism of clinical manifestations  and the prevention of their occurrence. Currently, there is an  increasing incidence of HIV infection in the late stages. Laboratory  and clinical differences in the manifestations of acquired  immunodeficiency syndrome (AIDS) and IRIS have been sanctified. In view of the blurring of the diagnostic criteria, in the  Russian Federation specialists rarely expose IRIS to clinical or pathological diagnoses, therefore it is rather difficult to trace the  frequency of occurrence of this condition. Clinical and laboratory  manifestations are systematized, which allows to formulate this  diagnosis on the basis of their totality. Prevention of IRIS is the  prudent prescription of antiretroviral drugs. It is neces sary to  conduct a qualitative and timely diagnosis of concomitant diseases of infectious and non-infectious nature before the initiation of ART and  during treatment, the appointment of effective etiotropic therapy for  opportunistic and secon dary infections. In order to improve the  prognosis of HIV infection, preferably early onset of ART with stable  CD4-lym phocyte counts and low HIV RNA levels in the blood.


2021 ◽  
Vol 14 ◽  
pp. 175628642110355
Author(s):  
Maike F. Dohrn ◽  
Gisa Ellrichmann ◽  
Rastislav Pjontek ◽  
Carsten Lukas ◽  
Jens Panse ◽  
...  

Progressive multifocal leukoencephalopathy (PML) is a subacute brain infection by the opportunistic John Cunningham (JC) virus. Herein, we describe seven patients with PML, lymphopenia, and sarcoidosis, in three of whom PML was the first manifestation of sarcoidosis. At onset, the clinical picture comprised rapidly progressive spastic hemi- or limb pareses as well as disturbances of vision, speech, and orientation. Cerebral magnetic resonance imaging showed T2-hyperintense, confluent, mainly supratentorial lesions. Four patients developed punctate contrast enhancement as a radiological sign of an immune reconstitution inflammatory syndrome (IRIS), three of them having a fatal course. In the cerebrospinal fluid, the initial JC virus load (8–25,787 copies/ml) did not correlate with interindividual severity; however, virus load corresponded to clinical dynamics. Brain biopsies ( n = 2), performed 2 months after symptom onset, showed spotted demyelination and microglial activation. All patients had lymphopenia in the range of 270–1150/µl. To control JC virus, three patients received a combination of mirtazapine and mefloquine, another two patients additionally took cidofovir. One patient was treated with cidofovir only, and one patient had a combined regimen with mirtazapine, mefloquine, cidofovir, intravenous interleukin 2, and JC capsid vaccination. To treat sarcoidosis, the four previously untreated patients received prednisolone. Three patients had taken immunosuppressants prior to PML onset, which were subsequently stopped as a potential accelerator of opportunistic infections. After 6–54 months of follow up, three patients reached an incomplete recovery, one patient progressed, but survived so far, and two patients died. One further patient was additionally diagnosed with lung cancer, which he died from after 24 months. We conclude that the combination of PML and sarcoidosis is a diagnostic and therapeutic challenge. PML can occur as the first sign of sarcoidosis without preceding immunosuppressive treatment. The development of IRIS might be an indicator of poor outcome.


Author(s):  
Mio Sakai ◽  
Masahiro Higashi ◽  
Takuya Fujiwara ◽  
Tomoko Uehira ◽  
Takuma Shirasaka ◽  
...  

AbstractWith the advent of antiretroviral therapy (ART), the prognosis of people infected with human immunodeficiency virus (HIV) has improved, and the frequency of HIV-related central nervous system (CNS) diseases has decreased. Nevertheless, mortality from HIV-related CNS diseases, including those associated with ART (e.g., immune reconstitution inflammatory syndrome) remains significant. Magnetic resonance imaging (MRI) can improve the outlook for people with HIV through early diagnosis and prompt treatment. For example, HIV encephalopathy shows a diffuse bilateral pattern, whereas progressive multifocal leukoencephalopathy, HIV-related primary CNS lymphoma, and CNS toxoplasmosis show focal patterns on MRI. Among the other diseases caused by opportunistic infections, CNS cryptococcosis and CNS tuberculosis have extremely poor prognoses unless diagnosed early. Immune reconstitution inflammatory syndrome shows distinct MRI findings from the offending opportunistic infections. Although distinguishing between HIV-related CNS diseases based on imaging alone is difficult, in this review, we discuss how pattern recognition approaches can contribute to their early differentiation.


2012 ◽  
Vol 54 (4) ◽  
pp. 231-233 ◽  
Author(s):  
Walter de Araujo Eyer-Silva ◽  
Maria Cecília da Fonseca Salgado ◽  
Jorge Francisco da Cunha Pinto ◽  
Fernando Raphael de Almeida Ferry ◽  
Rogério Neves-Motta ◽  
...  

Immune reconstitution inflammatory syndrome (IRIS) in HIV-infected subjects initiating antiretroviral therapy most commonly involves new or worsening manifestations of previously subclinical or overt infectious diseases. Reports of non-infectious IRIS are much less common but represent important diagnostic and treatment challenges. We report on a 34-year-old HIV-infected male patient with no history of gout who developed acute gouty arthritis in a single joint one month after initiating highly active antiretroviral therapy.


2020 ◽  
pp. 112067212091933
Author(s):  
Jeremy Chiabo ◽  
Sacha Nahon-Esteve ◽  
Mohamed Alketbi ◽  
Sandra Lassalle ◽  
Nathalie Tieulie ◽  
...  

The immune reconstitution inflammatory syndrome is a phenomenon typically described in HIV patient during the restoration of CD4 count after highly active antiretroviral therapy. Non-HIV immune reconstitution inflammatory syndrome has also been described after organ transplantation or immune recovery in neutropenic patients. We report the case of a 50-year-old man who presented to our department with left painful proptosis and ophthalmoplegia 2 days after having performed cytapheresis for a mantel cell lymphoma. Systemic work up and biopsy were performed and symptoms were relieved with intravenous steroids therapy. To our knowledge, this is the first case of orbital non-HIV immune reconstitution inflammatory syndrome described in the literature.


2019 ◽  
Vol 89 (3) ◽  
Author(s):  
Federico Piffer ◽  
Guido Levi ◽  
Giampietro Marchetti ◽  
Chiara Barbieri

We present the case of a 46-year-old Caucasian male, affected by ulcerative colitis, who developed tuberculous pleurisy during immunosuppressive therapy; despite proper therapy, worsening of the radiological findings was observed. The case was discussed among an online group of Italian physicians and diagnosis of immune reconstitution inflammatory syndrome (IRIS) tuberculosis was established. Therapy was continued and full recovery was obtained. IRIS is a syndrome initially described during opportunistic infections in HIV infected after being placed in anti-retroviral therapy. It reveals itself through a wide variety of manifestations, including fever, lymphadenopathies, worsening of lung infiltrates, pleural or pericardial effusion, central nervous system involvement. Few data are available regarding the best therapeutic options. IRIS is an insidious and potentially serious complication of opportunistic infections in immunocompromised patients. The always wider diffusion of immunosuppressive therapies increases the number of patients at risk, therefore physicians need to be aware of the issue.


2019 ◽  
Author(s):  
Tim Sinnecker ◽  
Jeffrie Hadisurya ◽  
Tilman Schneider-Hohendorf ◽  
Nicholas Schwab ◽  
Karsten Wrede ◽  
...  

Abstract Background Progressive multifocal leukoencephalopathy (PML) is a rare complication of patients treated with fingolimod. Case presentation Routine MRI eventually led to diagnosis of asymptomatic early PML that remained stable after discontinuation of fingolimod. As blood lymphocyte counts normalized, signs of immune reconstitution inflammatory syndrome (IRIS) and renewed MS activity developed. Both, advanced laboratory and ultrahigh field MRI findings elucidated differences between PML and MS. Conclusions In our case, early discontinuation of fingolimod yielded a good outcome, lymphocyte counts reflected immune system activity, and paraclinical findings helped to differentiate between PML-IRIS and MS.


2021 ◽  
Vol 8 (Supplement_1) ◽  
pp. S785-S785
Author(s):  
Ahad Azeem ◽  
Faran Ahmad ◽  
Manasa Velagapudi

Abstract Background Tumor necrosis factor (TNF)-α inhibitors are known for the reactivation of latent tuberculosis (TB). As a paradox, it has been reported to have a role in the treatment of immune reconstitution inflammatory syndrome (IRIS) from anti-TB therapy. Methods We report a case of paradoxical worsening of central nervous system TB after initiation of anti-TB medications, which was treated successfully with infliximab (TNF-α inhibitor). Results A 34-year-old man from Nepal with a history of untreated latent TB presented with complaints of occipital headache, slurred speech, and witnessed seizure. His physical exam was consistent with hyperreflexia. MRI of the brain revealed multiple small contrast-enhancing lesions in cerebral hemispheres. CT Chest showed bilateral centrilobular nodules suggestive of miliary TB. Cerebrospinal fluid (CSF) analysis showed pleocytosis, high protein, and low glucose. He was started on isoniazid, rifampin, ethambutol, and pyrazinamide along with high-dose dexamethasone for TB meningitis. Later, MTB DNA probe from bronchioalveolar lavage and CSF detected Mycobacterium Tuberculosis which was pan-susceptible. Repeat MRI of the brain 6 months into therapy revealed worsening of brain lesions. Moxifloxacin and linezolid were added to the regimen given clinical progression on first-line therapy. 6-months into this enhanced regimen he started experiencing blurring of vision. Visual field mapping showed left homonymous hemianopia. Repeat MRI of the brain confirmed extensive changes of basilar meningitis completely enveloping the optic chiasm. IRIS from TB was suspected. His prednisone dose was increased, and 3-doses of infliximab infusion were, 2-weeks apart were administered which showed clinical and radiological improvement. MRI Brain MRI Brain (axial T2/flair sequence) shows hyperintensities in multiple locations including the involvement of the left optic nerve and the left occipital region. Conclusion Exacerbation of pre-existing clinical symptoms, formation of new lesions, or cavitation of prior pulmonary infiltrates is known as tuberculosis IRIS or paradoxical reaction. Despite the clinical and radiological exacerbation, mycobacterial cultures usually stay negative. Continuation of anti-TB medications and high-dose corticosteroids are the backbone of treatment but in refractory cases, immune modulation is needed with anti-TNF-α agents. Disclosures All Authors: No reported disclosures


Sign in / Sign up

Export Citation Format

Share Document