Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The association between Castleman's disease (CD) and cardiomyopathy has been rarely reported and the optimal therapeutic approach remains unknown. We report a previously healthy 20-year-old African American female who presented with fever, dyspnea, anasarca, and generalized lymphadenopathy. Diagnostic workup, including an axillary lymph node biopsy, revealed that she had human immunodeficiency virus-negative and human herpes virus-8-negative multicentric CD. She had a non-anaphylactoid infusion reaction during her fourth rituximab infusion. A few weeks later, she developed new-onset severe cardiomyopathy requiring inotropic therapy, warranting consideration for left ventricular assist device. Several clinical clues indicated her new-onset heart failure was a manifestation of her CD. Interestingly, a trial of tocilizumab (an anti-interleukin-6 receptor monoclonal antibody) resulted in complete resolution of her cardiomyopathy and other manifestations of CD. Tocilizumab received orphan drug approval for the treatment of CD in Japan, but is not yet approved for this indication in the United States. Clinicians should be aware of its potential clinical utility in CD.
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PMID:Reversal of cardiomyopathy with tocilizumab in a case of HIV-negative Castleman's disease. 2378 62

This study presents effects of intravenous laser blood irradiation (ILBI) in a transient immunodeficiency patient with juvenile idiopathic arthritis (JIA) treated with an interleukin-6 receptor inhibitor (Tocilizumab). Biological agents induce JIA remission, but some patients do not respond favorably to this final therapeutic line of defense. ILBI was performed in a 16-year-old male patient, with JIA and transient immunodeficiency. When ILBI was introduced, the patient was receiving disease-modifying drugs, steroids, tocilizumab, and physical therapy. Because the disease was not well controlled, ILBI was applied in addition to other ongoing therapies. The patient underwent 1 session daily, and 10 successive sessions per month, repeated every 3 months, for 7 months. Patient evaluation was performed before ILBI was started and at 3, 6, 9, and 12 months after ILBI initiation, using the ACR Pediatric response. The outcome was evaluated using Pediatric 50, 70, and 90 responses and compared to initial status, after 3, 6, 9, and 12 months. At the end of study, the titre of IgA and IgG levels returned to normal. Synergistic anti-inflammatory effect of ILBI was evident, if applied additionally in combination with tocilizumab, in a patient with a therapy-resistant severe form of JIA and related subacute transient immunodeficiency.
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PMID:Intravenous laser blood irradiation and tocilizumab in a patient with juvenile arthritis. 2471 26

Thrombocytopenia (T), anasarca (A), myelofibrosis (F), renal dysfunction (R), and organomegaly (O) (TAFRO) syndrome is a variant of multicentric Castleman's disease. We describe here a 57-year-old man who presented with persistent fever, pleural effusion, and ascites. He was negative for human immunodeficiency virus and human herpes virus-8. A computed tomography scan showed an anterior mediastinal mass and small inguinal lymphadenopathy. Although a biopsy of the anterior mediastinum showed fatty tissue infiltrated with CD20 (+) and CD45RO (+) lymphocytes, a biopsy of the left inguinal lymph node revealed a hyaline vascular type of Castleman's disease. He subsequently developed severe thrombocytopenia and renal dysfunction. In addition, his bone marrow biopsy showed myelofibrosis. TAFRO syndrome was diagnosed based on the lymph node pathology and the characteristic manifestations of the syndrome. Tocilizumab and glucocorticoid therapy achieved complete remission and regression of the mediastinal mass. To our knowledge, this is the first report of TAFRO syndrome accompanied by an anterior mediastinal mass, which responded very well to therapy.
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PMID:An anterior mediastinal lesion in TAFRO syndrome showing complete remission after glucocorticoid and tocilizumab therapy. 2751 89

While cytokine storm develops in a minority of patients with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, novel treatment approaches are desperately needed for those in whom it does. Tocilizumab, an interleukin-6 receptor antibody, has been utilized for the treatment of cytokine storm in a number of severe inflammatory conditions, including in patients with severe coronavirus disease 2019 (COVID-19). Here, we present the first published case utilizing this therapy in a patient with underlying immunodeficiency. Our patient with aplastic anemia developed cytokine storm due to COVID-19 manifested by fever, severe hypoxia, pulmonary infiltrates, and elevated inflammatory markers. Following treatment with tocilizumab, cytokine storm resolved, and the patient was ultimately safely discharged from the hospital.
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PMID:Case Report: Tocilizumab for the Treatment of SARS-CoV-2 Infection in a Patient With Aplastic Anemia. 3307 89