PMC10154546_01
Blue rubber bleb on the back and eyes before and after low-dose sirolimus combined with glucocorticoids treatment. Blue rubber bleb on the back before treatment (A) and after treatment. (C) Blue rubber bleb on the eyes before treatment (B) and after treatment (D).
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F1
There were multiple tumors of different sizes on the head and neck, jaw, eyes, tongue, back (Figure 1A), abdomen, limbs, perineum and other parts, some of which were blue, soft and elastic, and compressible by palpation. Right eyelid swelling, limited upward lift, and difficulty in opening eyes were observed (Figure 1B). The cutaneous damage was less severe than before (Figure 1C). The swelling of the right eyelid disappeared without significant limitation in lifting (Figure 1D).
Comparison of lesions on head magnetic resonance imaging (MRI) before and after treatment. Lesions on head MRI before treatment (A) and after treatment (B).
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F2
Magnetic Resonance Imaging (MRI): examination revealed multiple systemic abnormal signals in skin (maxillofacial region, shoulder, abdomen, etc.), brain (cerebral hemisphere, thalamus, infratentorial of cerebellum, etc.) (Figure 2A), viscera (liver, lung, kidney, etc.), muscles (psoas major, iliopsoas muscle, gluteal muscle, etc.), and bone (hip, jaw, cone, etc.), which conformed to the imaging manifestations of BRBNS. MRI showed that the lesions were significantly smaller than before (Figure 2B), and no significant adverse drug reactions were observed.
Time course of the main results of laboratory examinations and treatment. Thrombocytopenia, anemia and coagulopathy improved with treatment (arrow, transfusion). Plt, Platelets; Hb, Hemoglobin; Fib, fibrinogen concentration; PT, prothrombin time; APTT, activated partial thrombin time; FDP, fibrin degradation products.
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F3
She was given oral low-dose sirolimus (0.8 mg/m2/day) on day 1 and was transfused with cryoprecipitate 1 U, plasma 40 ml and fibrinogen 0.5 g/25 ml on day 2 due to obvious abnormalities in coagulation function (Figure 3).
Timeline.
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F4
On day 11, the dose of prednisone tablets was adjusted to 1 mg/kg/day, which was slowly reduced to drug withdrawal after two months (Figure 4).