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Talaromyces Marneffei infection is rarely reported in patients with chronic active Epstein-Barr virus (EBV) infection. We reported an old man with chronic fever, pleomorphic rash, cough, EBV viraemia, and secondary hemophagocytic syndrome. Repeated histological biopsy and culture of skin lesions revealed Talaromyces Marneffei. This patient was diagnosed as chronic active EBV infection, and Talaromyces Marneffei infection. After treated with glucocorticoid steroids and anti-fungal therapy, the patient finally recovered. EBV infection is usually seen in immune compromised patients, who are susceptible to opportunistic pathogens rarely as Talaromyces Marneffei in this case.
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Objective@#To evaluate the response of oral melphalan plus high-dose dexamethasone (MDex) for patients with primary light chain amyloidosis (pAL).@*Methods@#Clinical data, hematological and organ responses, and survival of 76 patients with pAL who had received MDex from January 2009 to July 2017 were retrospectively analyzed.@*Results@#Of 76 patients (47 males and 29 females with the median age of 56 [range, 20-74] years old), 19.70% patients were defined as Mayo 2004 stage 3, involvement of more than or two organs was presented in 65 (85.53%) patients. Among 60 response evaluable patients, overall hematological response was 48.33% with complete response of 20.00% and very good partial response of 20.00%, respectively. The median time to the hematological response was 5 (range, 1-15) months. 36.67% patients achieved organ response. After the median follow up of 23(range, 1-113) months for surviving patients, median progression-free survival (PFS) and overall survival (OS) were 34 and 43 months, respectively. In a three months landmark analysis, the median rates of PFS and OS were 46 and 65 months, respectively. The median OS rates of patients with Mayo 2004 stage 3 and non Mayo 2004 stage 3 were 5 and 65 months (P=0.001), respectively.@*Conclusions@#MDex was an effective treatment for patients with early stage pAL, but was not suitable for those with severe cardiac involvement.
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A middle aged male presented with darkened skin and edema of lower extremities was reported. He was diagnosed as primary adrenocortical insufficiency ( Addison' s disease ) accompanied with hypergonadotropic hypogonadism, hypoparathyroidism, and subclinical hypothyroidism after endocrinological workup. In addition, the patient also had thickened skin, hirsutism, and polyserositis. The diagnosis of POEMS syndrome was confirmed by elevated M protein and VEGF level. The image of CT showed normal adrenals. Besides the autoimmune polyglandular syndrome ( APS), the possibility of POEMS syndrome in an adult patient with multiple endocrine dysfunction should be considered.
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Objective@#To evaluate the clinical characteristics and outcomes of very high risk patients with primary immunoglobulin light-chain amyloidosis (pAL) at a single center in China.@*Method@#Clinical data, treatment and outcome of 205 pAL patients in Peking Union Medical College Hospital from January 2009 to February 2016 were retrospectively analyzed. A 'very high risk’ group includes patients with Mayo 2004 stage Ⅲb and Mayo 2012 stage 4.@*Results@#Of 205 patients, 34 (16.6%) were defined as very high risk pAL patients. The median age at diagnosis was 57 (20-84) years, and 22 patients (64.7%) were male. All 34 patients were diagnosed with cardiac involvement, multi-organ involvement was observed in 15 patients (44.1%) , and 27 (81.8%) had New York Heart Association Class Ⅲ or Ⅳ. Median values of serum cTnI, NT-proBNP, and free light chains difference were 0.25 μg/L, 11 733 ng/L, and 403 mg/L, respectively. Eight (24.2%) had more than 10% plasma cell on the bone marrow aspirate. Sixteen (47.1%) patients received bortezomib based chemotherapy and overall hematologic response rate was 58.3%. Median overall survival (OS) was 4 months. The estimated OS at 3, 6, 12, and 24 months was 51.3%, 44.0%, 35.2%, and 29.6%, respectively. Fourteen (41.2%) patients died within 3 months after the diagnosis. The estimated 1-year survival rate for the patients who got hematologic response, without hematologic response, and palliative treatment was 90.9%, 11.1%, and 0, respectively (P<0.001) .@*Conclusion@#Patients with very high risk pAL had very poor prognosis and the early death rate remained high. Those patients who obtained hematologic remission would have significantly better outcomes.
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Monoclonal protein (M protein) is a serum surrogate used to conduct diagnostic, prognostic, and therapeutic evaluations of monoclonal plasma cell proliferative disorders. Two basic methods, namely, serum protein electrophoresis and immunofixation elec-trophoresis, are employed to detect and characterize M protein. Although these techniques have considerably improved, M protein quantification exhibits several drawbacks. In serum protein electrophoresis, M protein can migrate to various locations, and low M pro-tein levels cannot form a typical peak;as a consequence, additional problems in measurements arise. In 2009, a novel immunoassay in-volving a heavy/light chain (HLC) was developed. HLC recognizes immunoglobulins with specific heavy and light chain isotypes. The ra-tio between an involved monoclonal immunoglobulin and an uninvolved background polyclonal immunoglobulin can be calculated through immunoglobulin quantitation by using isotype-specific light chains. This review summarizes relevant parameters that provide diagnostic, prognostic, and therapeutic data regarding monoclonal plasma cell proliferative disorders.
ABSTRACT
<p><b>OBJECTIVE</b>To evaluate the usage of Mayo staging system in Chinese patients with primary light chain (LC) amyloidosis.</p><p><b>METHOD</b>Clinical data, treatment and outcome of 162 primary LC amyloidosis patients with Mayo Clinic staging in Peking Union Medical College Hospital from January 2009 to June 2015 were retrospectively analyzed.</p><p><b>RESULTS</b>The median age of 162 patients with Mayo Clinic 2004 stage was 57 (20-81) y, of them 62.3% were male. The number of patients with stage I to III were 44 (27.2%), 69 (42.6%), and 49 (30.2%), respectively. The median overall survival was not reached, 23 months and 12 months in patients with Mayo Clinic 2004 stage I, II, and III, respectively (P<0.001). Among 128 patients with Mayo Clinic 2012 stage, 48 patients (37.5%), 32 patients (25.0%), 32 patients (25.0%) and 16 patients (12.5%) were staged as Mayo Clinic 2012 stage 1 to 4, and the median OS was not reached, not reached, 13 months and 3 months, respectively (P<0.001).</p><p><b>CONCLUSION</b>Mayo Clinic staging systems had important prognostic value in patients with primary LC amyloidosis.</p>