ABSTRACT
Objective: To analyze the clinical features of patients with hemophagocytic lymphohistiocytosis (HLH) during perinatal stage. Methods: From January 2011 to October 2016, the obstetric materials, clinical features, associated disease/factors and therapeutic outcomes were studied in 11 patients with HLH in perinatal period. Results: In these 11 patients, 9 were primipara. As for the onset time of HLH, 5 were before delivery and 6 were after delivery. Six were complicated with other associated disease/factors, and infection was the commonest (5/6) . Treatment included glucocorticoid + fludarabine in 1 case, HLH-94/04 protocols in 9 cases, and monotherapy corticosteroids in 1 case. Two patients died eventually. Conclusions: Perinatal HLH is commonly observed in the pregnancy/postpartum related HLH (28th week of pregnancy to one week after delivery) . Infection is still the commonest associated factors. HLH-94/04 protocols after the cessation of pregnancy may be effective, but the cessation of pregnancy itself may not be enough for the perinatal stage related-HLH. The perinatal stage related-HLH still has a better outcome than the other subtype of secondary-HLH.
Subject(s)
Female , Humans , Pregnancy , Adrenal Cortex Hormones , Lymphohistiocytosis, HemophagocyticABSTRACT
<p><b>OBJECTIVE</b>To study the clinical outcome, adverse effect and treatment cost of homoharringtonine (HHT) in combination with all-trans retinoic acid (ATRA) and arsenic trioxide (AS2O3) for newly diagnosed with patients acute promyelocytic leukemia (APL).</p><p><b>METHODS</b>Clinical data of treatment of newly diagnosed patients with APL in experimental group (HHT + ATRA + AS2O3, n = 14) and control group \[Idarubicin (IDA) + ATRA + AS2O3, n = 21\] were analyzed retrospectively. The therapeutic effects, side effects and costs during induction therapy were compared between the two groups.</p><p><b>RESULTS</b>(1) The complete remission (CR) rate were 92.9% (13/14) and 95.2% (20/21) in experimental group and control group, respectively. The time to achieve CR were (28.1 ± 3.8) and (31.7 ± 4.2) days, respectively (P > 0.05). The negative rate of PML-RARα fusion gene at the time of CR were 76.9% (10/13) and 75.0% (15/20), respectively, and that in CR patient at the end of the first cycle treatment were 100.0% (13/13) and 95.0% (19/20), respectively (P > 0.05). (2) 5-year overall survival (OS) rate were (92.6 ± 0.6)% and (89.9 ± 0.5)%, respectively (P > 0.05), 5-year disease free survival (DFS) rate were 100.0% and (86.8 ± 0.6)%, respectively (P > 0.05). (3) During induction therapy, the incidence of infection in experimental and control group were 23.1% (3/13), 60.0% (12/20), respectively (P < 0.05). The amount of platelet transfusion were (54.7 ± 29.6) and (76.5 ± 25.6) units, respectively (P > 0.05), and that of fresh frozen plasma were (1157.1 ± 238.4) and (1423.5 ± 324.6) ml, respectively (P > 0.05). The total medical costs (excluding HHT and IDA) in experimental and control group were (36074.9 ± 1245.6) and (50564.5 ± 3658.4)CNY, respectively (P < 0.05).</p><p><b>CONCLUSION</b>HHT in combination with ATRA and AS2O3 regimen for newly diagnosed APL has a better efficacy, a higher long-term survival rate, and a lower costs, which is one of the reasonable choice.</p>