RESUMO
Patients with thrombocytopenia (platelet count <150 × 103/µL) often develop pulmonary hemorrhage (PH) after Stenotrophomonas maltophilia (SM) respiratory infection, resulting in a high respiratory failure rate and increased mortality. Developing an efficient method for early prediction of PH in these patients may improve survival. This study aimed to evaluate risk factors in PH and to develop an index measuring serial platelet deficit to predict PH in patients with SM respiratory infection. Data of patients with SM respiratory infection and thrombocytopenia treated in a tertiary university hospital during 2018-2020 were retrospectively retrieved from electronic medical records and analyzed. SM respiratory infection was defined as SM isolated from sputum, endotracheal suction, or bronchial alveolar lavage plus acute respiratory symptoms. Between PH and non-PH groups, clinical characteristics and laboratory parameters were collected and compared. The newly developed platelet dissimilarity index (d-index) was calculated by accumulating differences between the actual and the lowest normal level of the platelet count in each patient at different time points. Within 1,039 patients with positive SM culture, 437 cases matched the criteria and were analyzed. A total of 125 (28.6%) patients developed PH and 312 (71.4%) did not. The patients with PH had increased prothrombin time/international normalized ratio (PT/INR), lower platelet count, and higher platelet d-index. Multivariate analysis revealed that extreme thrombocytopenia (platelet count <50 × 103/µL) is a common independent risk factor in PH and mortality. The performance of platelet deficit and d-index varied between patients with different comorbidities. Performance of platelet deficit to predict PH is better in patients with hematology/oncology or liver disease (area under curve, 0.705-0.757), while d-index is better in patients with sepsis/treatment and various other groups (0.711-0.816). Prolonged and extreme thrombocytopenia is a determinant risk factor in PH in patients with SM respiratory infection. Given the complexity of causes of thrombocytopenia and associated comorbidities, different strategies should be applied when assessing the risk for PH.
RESUMO
BACKGROUND: In patients with frequently relapsing nephrotic syndrome, immunosuppressive therapy such as cyclosporine are often required to maintain remission. Cyclosporine has been noted to have tumorgenesis effects. In this case report, we present a child with relapsing nephrotic syndrom developed a rhabdomyosarcoma on her tongue after adout 4 years of continual immunosuppressive therapy. CASE PRESENTATION: A 2-year-old female child had nephrotic syndrome (urine protein-creatinine ratio 749.1 mg/mg; blood urea nitrogen 11 mg/dL; serum creatinine 0.3 mg/dL; and serum albumin 1.8 g/dL.) Proteinuria resolved on treatment with daily prednisolone for 4 weeks at the dose of 45 mg (2.5 mg/kg/day) but recurred with taper from 25 mg/day to 10 mg/day. At least five more episodes of relapse occurred within about a 3-year period. After the third relapse, she was treated with prednisolone and cyclosporine (at initial dose of 50 mg/day [1.7 mg/kg/day]) for immunosuppression. About 4 years after the diagnosis of nephrotic syndrome had been made, an embryonal rhabdomyosarcoma developed on her tongue. The cancer was treated with TPOG-RMS-LR protocol, with vincristine, actinomycin, and cyclophosphamide. Magnetic resonance imaging scan, performed about 3 years after the start of TPOG-RMS-LR therapy, revealed complete remission of the cancer. CONCLUSIONS: Although treatment with cyclosporine cannot be conclusively implicated as the cause the rhabdomyosarcoma in this patient, the association should prompt consideration of its use in the treatment of frequently relapsing nephrotic syndrome in children.