Immune dysregulation-associated lung disease / Family history of HLH
Hi everyone, I’m 25 years old. I suddenly developed dyspnea after inhaling high-concentration oxygen. I have complex pulmonary symptoms and a family history of HLH, and I’m seeking help. Currently, I cannot be diagnosed with any specific disease, but I have been suffering from dyspnea for a month; both inhalation and exhalation are extremely labored. At present, all indicators are generally normal, with a mild restrictive ventilatory defect, DLCO SB 80%, RV SB 160%, and resting blood oxygen at 98%. My CT scan shows increased lung markings and multiple disseminated nodular fibrotic lesions. Rheumatic indicators are all negative, IgG3 is low, and others are normal. My father once suffered from HLH; massive doses of hormones and chemotherapy were ineffective, but he was cured by immunoglobulin.
My lymphocyte subset analysis:
B lymphocytes (CD19%) 14.6% (8-15.4);
B-lymphocyte count 334 (180-324);
CD3+ T cells(%) 29.6% (30-46);
CD8+CD38+(Activation) 65.5 (45-65);
NK cell DR+ (activation) 55.1% (14.5-33.4);
NK cell PD-1+ (exhaustion) 4.6% (0.8-2);
CD8+CD28+ 462 (202-328);
CD8+CD28+/CD8+% 74.1 (27-72);
CD8+DR+/CD8+% 20 (25-46);
CD4+PD1+/CD4+ 14.2 (28-40);
CD19+CD38+/CD19+% 92.9 (77-88);
CD56+DR+/CD56+% 55.1 (14.5-33.4);
CD56+PD1+/CD56+% 4.6 (0.8-2).
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