Rhabdomyolysis(RM) is a syndrome characterized by injury to skeletal muscle fibers with disruption and release of toxic metabolites into circulation. It is characterized by triad of muscle weakness, myalgia and dark urine and is associated with increased creatine kinase (CK) and lactate dehydrogenase (LDH). A severely malnourished 10 year old girl with severe diabetic ketoacidosis (DKA) as hemr initial presentation of type 1 diabetes mellitus developed rhabdomyolysis (CK- 12,000 U/L) with nonoliguric renal failure during her initial course of hospital stay. The possible cause of her RM was attributed to severe hypophosphatemia (minimum serum phosphate, 0.8 mg/dL). Management of DKA, phosphate supplementation and urinary alkalinization with diuresis improved her clinical course. She was discharged on Day 9 with Insulin. We recommend frequent monitoring of serum phosphate during early period of DKA, particularly in malnourished children, and its normalization in case of severe hypophosphatemia. Keywords: diabetic ketoacidosis; hypophosphatemia; malnutrition; renal failure; rhabdomyolysis.
Introduction: Systemic mastocytosis (SM) is a rare hematological disorder characterized by clonal proliferation and activation of abnormal mast cells. It can vary from an indolent form to an aggressive form including progression to leukemia. There is limited data on epidemiology, clinical characteristics and outcome of this disease in population based setting. Till date, a retrospective study by Lim, Ken-Hong, et.al which included 342 patients diagnosed with systemic mastocytosis in Mayo clinic, is the largest series of patient published so far. This study reports the clinical presentation and outcome of patients with SM but there are no population based study in United States so far. We therefore conducted this population based study to determine epidemiology, survival pattern and incidence of second primary malignancy among patients with SM. Methods: We used SEER 18 database (2000-2014) to select all adult patients with age 20 or above with SM. Patient population was divided into various cohorts based on age (20-59, 60-79 and 80+ years), sex, race (Caucasians, African American and Others), area of residence (rural, urban and metropolitan) and annual household income (<$25000, $25000-<$50000 and ≥$50000). Age adjusted incidence rate was calculated using 2000 US standard population using SEER stat rate session. 5-year relative survival (RS) rate was calculated using SEER stat and compared using Z test. Cox proportional hazard model was used for multivariate analysis of factors associated with relative survival using Cansurv software. MP-SIR session in SEER stat was used to calculate the risk of second primary malignancy. Result: The incidence was found to be 0.046 per 10000 among general population. Incidence was found to be higher among Caucasians compared to African American (0.056 vs. 0.018 per 100000). Median age at diagnosis was 55 years. Of the total 425 patients, majority were Caucasians (92.5%), age <60 years (59.3%) and from metropolitan area (84.7%) but there was equal distribution among male and females. 10 year overall survival was found to be 61.5±3.1% by KM curve. 5-year RS was found to be 74.0±2.7% for the whole population. Females had significantly better survival compared to males, 5-year RS of 84.7±3.1% vs. 62.3±4.3%, P <0.0001. Survival trended to be better for Caucasians compared to African American but didn't reach clinical significance, 74.6±2.8 vs. 50±14.8, p 0.08. Patients <60 years had significant better survival compared to 60 to 79 and 80+ years ( 5-year RS of 88.6±2.4 vs. 58.4±5.2 and 16.0±11.2 respectively with P <0.0001 for both comparison). In multivariate analysis, younger age group, female sex and Caucasian race were found to be independent predictor of better 5 year relative survival with P<0.0001 compared to their counterparts. Patients with systemic mastocytosis were found to have higher risk of developing both solid organ as well as hematological malignancy within 5 years of diagnosis (as shown in table 1). After that the risk decreased and was comparable to general population. Conclusion: Our study shows that systemic mastocytosis is rare disease in general population and survival is better among specific subgroup of patients including females, younger patient and Caucasians. As the majority deaths occurred in first 5 years after diagnosis and as incidence of second primary malignancy is higher in the first 5 years, we recommend close follow up for first five years after diagnosis. Disclosures No relevant conflicts of interest to declare.
e18090 Background: There has been significant improvement in survival among patients with primary Chronic Lymphocytic Leukemia (CLL) in recent years but, not much is known about survival among CLL developed after a first primary malignancy. Methods: SEER 18 database, 2015 submission, was used to calculate 5 and 10 year relative survival (RS) by period survival modeling method for 1993 to 2012, with division of the period into 4 cohorts of 5 years each. SEER*Stat software from National Cancer Institute was used to calculate relative survival (RS) for 4 different periods of 5 years duration. The trend in survival for cases with CLL as second primary cancer (CLLSPC) was evaluated using COX proportional hazard method using Cansurv software and also compared with that of primary CLL cases. Results: A total of 8731 patients with CLLSPC were included in the study, which represents 14.5% of all cases of CLL. The median age at diagnosis was 75 years. Median time to diagnosis of CLL was 50 months after diagnosis of first primary malignancy. Prostate cancer, breast cancer and colorectal cancer were 3most common primary cancers. 5-year and 10-year RS improved significantly in the year 2008-2012 compared to 1993-1997 (59.3% to 70.2%,P 0.044 and 40.1% to 50.6%, p 0.045). In subgroup analysis, significant improvement in 5-year and 10-year RS was seen in females and ≥ 80 years age group but no significant improvement was observed in males and age group 60-79 years. Survival among CLLSPC was significant worse compared to first primary CLL in all periods, even after adjusting for age and sex. Conclusions: CLLSPC represents about 14.5% of all CLL cases. Relative survival among patients with CLLSPC is gradually improving but still lags behind that of CLL as first primary cancer.
Background: Primary cutaneous diffuse large B cell lymphoma-leg type (DLBCL-LT) is relatively uncommon lymphoma accounting for about 1 to 3 % of all cutaneous lymphoma and about 10-20% of primary cutaneous B cell lymphomas. There is data for survival outcomes in primary cutaneous B cell lymphoma in general population after introduction of rituximab but little is known about outcomes in DLBCL-LT patients in general population. The available data on survival in DLBCL-LT are from small case series and single institution studies. We conducted this study to evaluate the survival outcome in patients with PCDLBCL-LT after introduction of rituximab. Methods: We utilized SEER 18 database to select adult (18+ years) patients diagnosed from 2004 to 2014 with ICD-3-0 code of 9680/3 (diffuse large B cell lymphoma, not otherwise specified)with localized stage by summary stage 2000 with skin as the primary site of disease to identify patient with DLBCL-LT. Patients were divided into various cohorts based on age (<65 versus 65+ years), sex, race (Caucasians, African American and Others) and median household income of county of residence (< $50,00 versus ≥ $50,000). We utilized SEER*stat to calculate age adjusted incidence rate using 2000 US standard population. Kaplan Meyer curve was utilized to calculate 5-year overall survival. Cox proportional hazard model was used for multivariate analysis of factor associated with survival. Result: A total of 485 patients were identified with DLBCL-LT as the only or first primary cancer case. The median age at diagnosis was 70 years. Its incidence was found to be about 0.09 case per 100,000 population with incidence in males being about 1.6 times that of females. Majority of patient included in our study were males (52 %), older (59.4%), and Caucasians (85.6%). 5-year overall survival was found to be 59%. Older patients were found to have significantly worse 5-year overall survival compared to younger patients with HR of 7.9 with 95% CI of 4.9 to 12.7. There was no disparity in cancer outcome based on race, sex or median income of the county of residence. When we compared the outcome of patients diagnosed in earlier 5 years to later 6 years of the study period, the survival curves almost overlapped suggesting that there has been little if any improvement in outcome in recent years. Conclusion: Our study showed that about 60% of patients with DLBCL-LT live for 5 years in real world setting which is comparable to the outcome reported by other studies on outcome in primary cutaneous B cell lymphoma. Even though this is disease of elderly, as the median age at diagnosis is 70 years, the outcome in older population was found to be significantly worse compared to younger patients. Given this is relatively uncommon disease, we recommend large registry-based studies to try to improve outcome in these patient population. Disclosures No relevant conflicts of interest to declare.
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