piperidines and Lymphohistiocytosis--Hemophagocytic

Topics: Adenine; Aged; Antineoplastic Agents; Antineoplastic Combined Chemotherapy Protocols; Female; Humans; Leukemia, Lymphocytic, Chronic, B-Cell; Lymphohistiocytosis, Hemophagocytic; Male; Middle Aged; Piperidines; Pyrazoles; Pyrimidines; Salvage Therapy; Waldenstrom Macroglobulinemia

Anti-melanoma differentiation-associated gene 5 juvenile dermatomyositis (anti-MDA5 JDM) is associated with high risk of developing rapidly progressive interstitial lung disease (RP-ILD). Here we report an 11-year-old girl with anti-MDA5 JDM and RP-ILD which led to a fatal outcome, further aggravated by SARS-CoV-2 infection. She was referred to our hospital after being diagnosed with anti-MDA5 JDM and respiratory failure due to RP-ILD. On admission, fibrobronchoscopy with bronchoalveolar lavage (BAL) revealed

Topics: Adenosine Monophosphate; Alanine; Anti-Bacterial Agents; Antibodies, Monoclonal, Humanized; Antiviral Agents; Autoantibodies; Bronchoscopy; Child; COVID-19; COVID-19 Nucleic Acid Testing; Cyclophosphamide; Dermatomyositis; Disease Progression; Fatal Outcome; Female; Humans; Hydroxychloroquine; Immunocompromised Host; Immunoglobulins, Intravenous; Immunologic Factors; Immunosuppressive Agents; Interferon-Induced Helicase, IFIH1; Lung; Lung Diseases, Interstitial; Lymphohistiocytosis, Hemophagocytic; Mediastinal Emphysema; Methylprednisolone; Piperidines; Pneumonia, Pneumocystis; Pneumothorax; Pyrimidines; Respiratory Insufficiency; Shock, Septic; Subcutaneous Emphysema; Tomography, X-Ray Computed; Trimethoprim, Sulfamethoxazole Drug Combination

Topics: Aged; Fatal Outcome; Female; Humans; Janus Kinases; Lymphohistiocytosis, Hemophagocytic; Macrophage Activation; Piperidines; Protein Kinase Inhibitors; Pyrimidines; Pyrroles; Remission Induction; Still's Disease, Adult-Onset

Topics: Adenine; Antineoplastic Combined Chemotherapy Protocols; Autopsy; Biopsy; Chromosome Aberrations; Drug Resistance, Neoplasm; Fatal Outcome; Gene Rearrangement, B-Lymphocyte; Humans; Immunophenotyping; In Situ Hybridization, Fluorescence; Leukemia, Lymphocytic, Chronic, B-Cell; Lymphohistiocytosis, Hemophagocytic; Male; Middle Aged; Neoplasm Staging; Piperidines; Protein Kinase Inhibitors; Pyrazoles; Pyrimidines; Time Factors