Genu Valgum Deformity in Hurler Syndrome After Hematopoietic Stem Cell Transplantation: Correction by Surgical Intervention

1999 ◽  
Vol 19 (2) ◽  
pp. 270-274 ◽  
Author(s):  
Ebenezer Odunusi ◽  
Charles Peters ◽  
William Krivit ◽  
James Ogilvie
Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 1283-1283 ◽  
Author(s):  
Antonio M. Jimenez ◽  
George Behrens ◽  
John Maciejewski ◽  
Norman Wool ◽  
Elizabeth Shima Rich ◽  
...  

Abstract Abstract 1283 Background: Neutropenic enterocolitis is an acute, life-threatening condition characterized by transmural inflammation of the cecum, often with involvement of the ascending colon and ileum, in patients who are severely myelosuppressed. Mortality rates are high, and treatment is controversial, with options varying from conservative medical management to surgical intervention (Ullery BW, et al. Surg Infect 2009;10(3):307-14). Although initially described in pediatric leukemia patients (pts) it has increasingly been reported in adults with a variety of myeloproliferative disorders, solid tumors, and in the setting of immunosuppression with hematopoietic stem cell transplantation (HSCT). We present a retrospective review of our clinical experience with neutropenic enterocolitis in HSCT recipients. Patients and Methods: Data from 264 consecutive HSCT pts over a 5 year period were reviewed. 24 pts with a clinical diagnosis of neutropenic enterocolitis based on the clinical triad of fever, abdominal pain and neutropenia with radiographic evidence of cecum wall thickening were identified. Demographics, indication for transplantation, clinical presentation and outcomes were analyzed. Results: 264 pts underwent a HSCT (autologous and allogeneic), at our institution from 2004 to 2010. A total of 24 (9.1%) pts (average age 52.67 yrs, range 23 – 70yrs) were diagnosed with neutropenic enterocolitis. 9 (37.5%) pts were female and 15 (62.5%) pts were males. 9 (37.5%) pts had ≤ 1 comorbidities and 15 (62.5%) pts had ≥ 2 comorbidities. 14 (58.3%)pts underwent an autologous HSCT and 10 (41.67%) an allogeneic HSCT. Indications for transplant included relapsed/refractory non-Hodgkin lymphoma, multiple myeloma, acute myelogenous leukemia, acute lymphocytic leukemia, chronic lymphocytic leukemia and myelodysplastic syndrome. 21(87.5%) pts received a Melphalan-based conditioning regimen. Clinical findings were identified at a median day of 4.4 (range 2 – 8 days) and radiographic evidence was noted on a median day of 9.28 (range 5–21days) after stem cell infusion. Median colonic thickness was 7.91mm per CT criteria (range 2 –16mm) and in 4 (33.33%) pts this was ≥ 10 mm. All patients were treated conservatively with IV fluids, bowel rest, parenteral broad spectrum antibiotic therapy and no patients underwent surgical intervention. 5 (20.8%) pts required admission to MICU and 3 (12.5%) pts died. Conclusion: We conclude that neutropenic enterocolitis is a severe and not uncommon complication in Hematopoietic Stem Cell Transplantation recipients, with an incidence of 9% and mortality of 12.5% in this retrospective study. The outcomes of neutropenic enterocolitis for Hematopoietic Stem Cell Transplantation recipients compares favorably to patients with acute leukemia reported in the literature. Broad spectrum antibiotic and bowel rest appear to be an effective therapy for most patients. Prospective trials comparing conservative medical therapy with surgical intervention are required to validate this observation. Future studies will be needed to assess prognostic factors in our patient population. Disclosures: No relevant conflicts of interest to declare.


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