ABSTRACT
Objective To strengthen the recognition of atypical POEMS syndrome in order to improve diagnosis rate of rare cases of POEMS syndrome. Methods The diagnosis and treatment of a rare case of POEMS syndrome coexisting with Castleman disease but without M protein in serum, urine and bone marrow who was admitted to Xuanwu Hospital, Capital Medical University in November 2017 were retrospectively analyzed with review of the literature. Results The elder male was suspected with a diagnosis of POMES syndrome, but absence of monoclonal plasma cell disease that made it difficult to diagnose. Systemic PET-CT found an active metabolic lesion in left iliac bone. Although the lymph node biopsy had been performed for a diagnosis of Castleman disease, a bone biopsy was also done for a definite diagnosis. Pathological result indicated a plasmacytoma which confirmed a diagnosis of POEMS syndrome without M protein in serum, urine and bone marrow. Literature review suggested that the application of immunofixation electrophoresis was helpful to improve the diagnostic rate of POEMS syndrome. For patients with a suspected diagnosis of POEMS syndrome, bone biopsy, flow cytometry and systemic PET-CT may assist in the search for monoclonal plasma cell. Periphery neuropathy, bone lesion and treatment response were helpful in distinguishing Castleman disease coexisting with POEMS syndrome from Castleman disease without POEMS syndrome. Conclusions When a mandatory major criterion of POEMS syndrome is not sufficient, it should be actively sought, especially for patient with a suspected diagnosis of POEMS syndrome. For patients with multiple lesions, multi-site biopsies are necessary to assist in diagnosis.
ABSTRACT
Objective To investigate the clinical value of low-dose decitabine (DAC) in elderly patients with acute myeloid leukemia (AML) and myelodysplastic syndromes (MDS) patients with intermediate-or high-risk. Methods Low-dose DAC (10 mg/d, 7 days) combined with CAG regimen were given to 19 elderly patients with AML and intermediate- or high-risk MDS patients. The efficacy and adverse reactions were evaluated after a course of treatment, and the patients were followed up for survival. Results After a course of treatment, 8 patients achieved complete remission (CR), 7 patients achieved partial remission (PR). After 4 courses of treatment, 68.4 % (13/19) of patients achieved CR, the overall response rate reached 78.9% (15/19). Fewer side effects were seen associated with chemotherapy. After 42 months of follow-up, there were 12 survival cases, the median survival time was 13.5 months (3-42 months). Conclusion Low-dose DAC combined with CAG regimen have a better efficacy, higher safety, and lower economic burden for elderly AML patients and intermediate- or high-risk MDS patients, which is beneficial to greatly improve patients' compliance.
ABSTRACT
Objective To explore the meaning of midkine (MK) levels in serum in different development stage of acute leukemia,and to explore the relationship between MK and WT1.Methods The levels of the MK in serum of 86 cases of acute leukemia and 30 cases of normal people were detected by ELISA.Real-time quantitative PCR (RQ-PCR) method was used to determine the expression of WT1 at mRNA level in 15 AML patients.Results The MK level in serum in the new diagnosed group was higher than that in the complete remission group and the normal control group [7.52 (5.44,10.55) ng/ml vs 3.52 (1.56,5.20) ng/ml vs 2.44 (1.89,3.12) ng/ml].There' s no statistical difference between midkine level in new diagnosed acute B cell leukemia (B-ALL) group and acute myeloid leukemia (AML) group [7.88 (5.78,15.78) ng/ml vs 6.25 (4.59,16.33) ng/ml].The clear correlation was found between the level of serum MK and quantities of marrow WT1 gene (r =0.529,P =0.043).Conclusions The level of MK in serum of acute leukemia patients is increased at the time of new diagnosis and decreased at complete remission.ELISA may be a way to measure the status of AL.The location of MK gene is adjacent to WT1 gene and MK' s clinical significance is similar to WT1' s,furthermore,there is a clear correlation between MK in serum and WT1 of marrow in quantities.
ABSTRACT
Recent studies show that the structure integrality and function are more important than the length of telomere in maintaining tumor cells reproduction and proliferation.Mammalian telomeres bound by a specialized six-protein(TRF1,TRF2,TIN2,RAP1,POT1 and TPP1 )complex known as telosome or shelterin,has fundamental role in the regulation of telomere activity,maintaining the length and protecting telomere chromosome terminal.Telosome is involved in the complex regulation of cell mitosis process.The study of the role of telomere binding protein complex in tumorigenesis is very important for searching new methods of tumor diagnosis and treatment.