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Objective To explore the clinical manifestation,diagnosis and treatment of IgG4-related retroperitoneal fibrosis (IgG4-RRPF) and IgG4-related disease (IgG4-RD).Methods The clinical data of a case of IgG4-RRPF was analyzed retrospectively,the related literatures were reviewed,and a novel clinical entity IgG4-RD was introduced.Results The patient was a 52-year-old man with the complaint of left lower abdominal pain due to left hydronephrosis.CT and MRI revealed a soft tissue mass at the left psoas major muscle.PET-CT showed increased FDG uptake.After biopsy,the pathological diagnosis was consistent with IgG4-RD.The patient was diagnosed as IgG4-RRPF and treated with prednisone.The symptoms improved and retroperitonea] mass reduced.According to review the latest literatures,it is found that IgG4-RRPF was socalled idiopathic retroperitoneal fibrosis (IRF) formerly.Conclusion IgG4-RRPF is a rare disease in IgG4-RD spectrum,characterised by the presence of a retroperitoneal tissue,consisting of chronic inflammation and marked fibrosis,which often entraps the ureters or other abdominal organs.Hematological examination shows elevated serum IgG4 concentration (≥ 1.35 g/L).Histopathological examination shows marked tissue infiltration by lymphocytes and IgG4-plasma cells with fibrosis.Glucocorticoids are the first line of therapy.
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Purpose:To report three cases of myelodysplastic syndrome(MDS) transformed to atypical chronic myeloid leukemia(aCML).Methods:Compared the clinical findings,hemograms and bone marrow features in two different courses of the disease,discussed with review of related literatures.Results:The three patients were all old age.They onset with chronic anemia,the results of BM smear were MDS-RAS and MDS-RAEB;Then leukocytosis occurred in 6-65 months.The aCML were diagnosed according to the BM smear and normal chromosome.Conclusions:The MDS/MPD is a new category of the WHO classification that have both dysplastic and proliferative features.Atypical CML lacks the Ph chromosome and BCR/ABL fusion gene that are the hallmarks of classic CML.This kind of case is seldom seen.
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Purpose:To evaluate the effect of Auto-PBSCT combined with long-time low-dose interleukin-2 (after transplant) for increasing the rate of 5-year disease-free survival of lymphoid malignancies (ALL and NHL).Methods:U- sing chemotherapy combined with low dose G-CSF to mobilize peripheral blood stem cells,we carried out auto-PBSCT in lymphoid malignancies (7 cases CR_1 ALL、1 case CR_2 ALL、3 cases CR_1 NHL、5 cases refractory NHL).After transplant, 15 cases used low dose interleukin-2 at 5.10~5U/d for one year or longer.Results:7 cases CR_1 ALL are still alive,the aver- age disease-free survival is 73 months.Median disease-free survival is 51.5 months.2 of 3 cases CR_1 NHL are still alive. The 6 years disease-free survival in 10 cases CR_1 lymphoid malignancies(CR_1 NHL and CR_1 ALL) is 0.90?0.11,the aver- age disease-free survival is 66.8 months.In another PR (refractory) group of NHL and ALL (CR_2),3 years disease-free survival was 0.33?0.18,and average survival time was 20.66 months,but a chemotherapy-sensitive NHL is still alive (63 months),a case of lymphoblast cell NHL also survived for 46 months and died after relapse.Conclusions:Auto-PBSCT combined with long-turm low dose IL-2(after transplant) could effectively increase the rate of 5-year disease-free survival in CR_1 lymphoid malignancies(ALL and NHL).