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ABSTRACT: We report a 41 year old male with sickle cell disease who developed a myelodysplastic syndrome and acute myeloid leukemia with complex karyotype involving chromosomes 5, 7 and 17 after 15 years of hydroxyurea treatment. He responded poorly to induction chemotherapy with cytarabine/idarubicin followed by high dose cytarabine and succumbed to neutropenic sepsis. Multiple systematic reviews, observational studies and clinical trials were conducted to identify the toxicity profile of hydroxurea. Only six cases of leukemia/myelodysplastic syndrome were identified in patients with sickle cell anemia treated with hydroxyurea. Subsequently, it was concluded that hydroxyurea is not leukemogenic. However, it was noted that most of the published studies had only up to 9 years of follow-up. Our patient was started on hydroxyurea in 1990, before the widespread use of the drug and took hydroxyurea for 15 years. His presentation may reflect an outcome otherwise not yet observed because of the short follow-up of prior studies. We believe that the leukemogenic risk of hydroxyurea should be discussed with the patients and their families. Studies evaluating the adverse effects of hydroxyurea should have longer follow-up before definitive conclusions are drawn.
Clinical Medicine Insights: Oncology 01/2012; 6:149-52.
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ABSTRACT: Tumors of salivary glands are uncommon and comprise of about 2%-4% of all head and neck tumors. About 75%-80% of these tumors are benign and include pleomorphic adenoma, monomorphic adenoma, oncocytoma, and papillary cystadenoma lymphomatosum. Mucoepidermoid carcinoma is the most common malignant tumor of salivary glands, representing 5-10% of all salivary gland tumors. Although known to be metastatic to local lymph nodes, distant metastases are rare (especially, with low and intermediate grade tumors). Histologic grade and the expression of various mucin glycoproteins are useful prognostic indicators. We present a case of mucoepidermoid carcinoma of parotid gland origin with distant metastases which is an uncommon occurrence with intermediate grade tumors. Also, this is the first reported case of humoral hypercalcemia of malignancy secondary to mucoepidermoid carcinoma.
Clinical Medicine Insights: Oncology 01/2011; 5:83-7.
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ABSTRACT: Mixed warm and cold autoimmune hemolytic anemia runs a chronic course with severe intermittent exacerbations. Therapeutic options for the treatment of hemolysis associated with autoimmune hemolytic anemia are limited. There have been only two reported cases of the effective use of rituximab in the treatment of patients with mixed autoimmune hemolytic anemia. We report a case of severe mixed autoimmune hemolytic anemia that did not respond to steroids and responded to four weekly doses of rituximab (one cycle).
A 62-year-old Caucasian man presented with dyspnea, jaundice and splenomegaly. His blood work revealed severe anemia (hemoglobin, 4.9 g/dL) with biochemical evidence of hemolysis. Exposure to cold led to worsening of the patient's hemolysis and hemoglobinuria. A direct antiglobulin test was positive for immunoglobulin G and complement C3d, and cold agglutinins of immunoglobulin M type were detected. A bone marrow biopsy revealed erythroid hyperplasia. A positron emission tomographic scan showed no sites of pathologic uptake. There was no other evidence of a lymphoid or myeloid disorder. Initial therapy consisted of avoidance of cold, intravenous methylprednisolone and a trial of plasmapheresis. However, there was no clinically significant response, and the patient continued to be transfusion-dependent. He was then started on 375 mg/m2/week intravenous rituximab therapy. After two treatments, his hemoglobin stabilized and the transfusion requirement diminished. Rituximab was continued for a total of four weeks and led to the complete resolution of his hemolytic anemia and associated symptoms. At the patient's last visit, about two years after the initial rituximab treatment, he continued to be in complete remission.
To the best of our knowledge, this is the first reported case of mixed-type autoimmune hemolytic anemia that did not respond to steroid therapy but responded completely to only one cycle of rituximab. The previous two reports of rituximab use in mixed autoimmune hemolytic anemia described an initial brief response to steroids and the use of rituximab at the time of relapse. In both of these case reports, the response to one cycle of rituximab was short-lived and a second cycle of rituximab was required. Our case report demonstrates that severe hemolysis associated with mixed autoimmune hemolytic anemia can be unresponsive to steroid therapy and that a single cycle of rituximab may lead to prompt and durable complete remission.
Journal of Medical Case Reports 01/2011; 5:156.
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ABSTRACT: Introduction. Haptoglobin binds the globin portion of free hemoglobin. Serum haptoglobin measurement is used as a laboratory marker for the diagnosis of hemolytic anemia. Since stored blood contains free hemoglobin, transfusion may affect haptoglobin levels. Study Objectives. The aim of the study was to evaluate whether serum haptoglobin could be measured to assess hemolysis in recently transfused patients. Patients and Methods. Twenty-one patients, receiving more than one unit of packed red blood cells (PRBCs) for presumed nonhemolytic indications, were enrolled. Serum haptoglobin levels were recorded before, immediately after, and 24 and 48 hours after transfusion. Observations and Results. Analysis of variance with a repeated measures was used to examine the serum haptoglobin levels at different time periods and no significant difference was found (P = .28). Conclusion. The results suggest that serum haptoglobin can be used in the diagnosis of hemolysis in patients receiving multiple units of PRBC.
Advances in Hematology 01/2011; 2011:389854.
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ABSTRACT: Renal angiomyolipomas are recognized as clonal neoplasms with clonal chromosomal aberrations and a common progenitor cell, the perivascular epithelial cell (PEC). The epithelioid variant is a recently identified entity, characterized by predominance of PEC and a unique morphologic and immunohistochemical profile. There is accumulating evidence suggesting that renal epithelioid angiomyolipoma (REA) is a malignant disease.
We searched the literature for descriptions of clinical behavior of REA. A Pubmed search was performed using the following key words: angiomyolipoma, epithelioid, perivascular epithelial cell or/and renal tumors. We reviewed a case of fatal REA at our institution. A pathologist reviewed slides to confirm the diagnosis.
Upon review of 140 articles, 37 eligible articles were found including 10 articles describing the clinical course of REA. Almost all of the patients described, for whom there was a follow-up available, died of neoplastic progression of the disease, with liver, lung and bone metastases. Four cases were reclassified after retrospective pathology review, and they were fatal. Three of these had been misdiagnosed as renal cell carcinoma (RCC), while 1 was diagnosed as classic angiomyolipoma.
Unlike commonly benign classic angiomyolipoma, REA behaves aggressively. It is crucial for the clinician to be aware of and identify this epithelioid variant as a malignant disease. It should be carefully differentiated from RCC. Resection alone may not be curative, and adjuvant therapy should be considered. A multimodality treatment approach needs to be explored for this newly recognized malignant variant renal angiomyolipoma.
Journal of nephrology 03/2010; 24(1):18-22. · 1.65 Impact Factor
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ABSTRACT: This phase I/II study evaluated the safety of the combination of irinotecan, docetaxel, and estramustine for selected advanced solid tumors and also obtained initial efficacy data. Twenty-two patients were enrolled in the study. The regimen consisted of docetaxel 30 mg/m(2) and irinotecan 60 mg/m(2) both given intravenously on days 1 and 8 every 21 days in combination with escalating doses of estramustine (500 mg/m(2)/day escalated to 750 mg/m(2)/day on days 0, 1, 2, 7, 8, and 9 given every 21 days) during phase I. Dose escalation was continued until the maximum planned dose level of estramustine (750 mg/m(2)/day) was reached. After the appropriate phase II dose of estramustine was found additional patients were enrolled. Twenty-one of the 22 patients were evaluable for toxicity and 17 for tumor response. The recommended phase II dose of estramustine was found to be 750 mg/m(2)/day orally on days 0, 1, 2, 7, 8, and 9 given every 21 days. Hematologic toxicity was fairly mild, with only one episode of grade 3 neutropenia. Diarrhea was the most common nonhematologic toxicity with grade 3 toxicity occurring in five of 21 patients. Only one episode of venous thrombosis was observed. Objective response rate was 15.8%, overall clinical benefit rate was 63%, and median time to progression was 15 weeks. Estramustine in combination with the doublet of docetaxel and irinotecan is a well-tolerated regimen with minimal hematologic toxicity, mild to moderate nonhematologic toxicity, and promising initial antitumor activity in previously treated patients with advanced solid tumors.
Anti-cancer drugs 04/2009; 20(6):508-12. · 2.23 Impact Factor
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ABSTRACT: Malabsorptive bariatric procedures such as Roux-en-Y gastric bypass and biliopancreatic diversion/duodenal switch expose the patient to malnutrition and commonly cause iron deficiency. The optimal replacement and monitoring method remain undetermined. To identify high-risk patients who might need intravenous iron supplementation after bariatric surgery, we evaluated bariatric surgery patients who received parenteral iron at a university hospital-affiliated hematology center.
We performed a retrospective analysis and reviewed the records of 165 patients who had received parenteral iron from May 2004 to June 2007. Of the 165 patients, 42 bariatric surgery patients were identified. The type of bariatric procedure and menstrual status of the patients were compared.
The average patient age was 40 years. Of the 42 patients, 2 were men and 40 were women. Of the 40 women, 32 were premenopausal and 8 were postmenopausal. The patients in the biliopancreatic diversion/duodenal switch group had a significantly lower hemoglobin at presentation (P = .02), relatively lower ferritin levels, and required more additional parenteral iron treatment after the initial resolution of anemia (P = .001). The premenopausal women required earlier parenteral replacement (P = .008) and were at a greater risk of anemia-related hospitalization (P = .00033).
The available published studies lack any data regarding parenteral iron replacement needs after bariatric surgery. Our results have identified the need for long-term parenteral iron replacement therapy after malabsorptive bariatric procedures, especially in premenopausal women. Patients who do not respond to oral iron therapy should be referred early for parenteral iron replacement therapy to prevent anemia-related complications and to maintain patients' quality of life. Iron monitoring should continue indefinitely even after the initial repletion of iron stores and the resolution of anemia.
Surgery for Obesity and Related Diseases 07/2008; 4(6):715-9. · 3.93 Impact Factor
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ABSTRACT: Patients with chronic kidney disease (CKD) or end-stage renal disease (ESRD) are known to develop metastatic soft-tissue calcification, secondary to hyperparathyroidism, in tissues including the breast. Such calcifications in women could pose a problem for interpretation of mammograms, since they are thought to mimic malignant lesions and interfere with differentiation of benign from malignant disease. Investigation of this issue is important to provide high-quality, accurate breast care to women with CKD or ESRD, but little evidence is so far available. In a systematic review of the literature on the types and patterns of breast calcifications, we found only three studies that examined metastatic soft-tissue calcifications of the breast. The studies did, however, confirm that women with CKD or ESRD have a higher frequency of breast calcification than women with normal kidney function. The two older studies reported that these breast calcifications are not associated with malignancy, but the later study reported a raised rate of suspicious breast calcification among women with ESRD receiving hemodialysis, leading to an increased biopsy referral rate. In this Review we discuss the strengths and limitations of the available data and whether mammography is recommended in women with CKD or ESRD.
Nature Clinical Practice Nephrology 07/2008; 4(6):337-41. · 6.08 Impact Factor
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ABSTRACT: This article reports a hospital's experience confronting a community crisis, stemming from local and national breast health access issues, and evaluates the subsequent effectiveness of the initiative to improve breast care service. An interdisciplinary Breast Care Facility was developed adjacent to a Community Hospital. Patients receiving breast cancer screening during the year prior to the Breast Center opening (2002) were compared with patients in subsequent years (2003-2005). Program effectiveness was evaluated by examining screening mammography volume, wait times and cancer detection rates. Screening volume increased by 29.6%. Wait times declined from 30 weeks to 3.5 weeks. Initially, patients with a suspicious screening mammography had a 2-3 week delay for diagnostic mammography and the subsequent evaluation took another 3-4 weeks. Both times improved to an average of 2-5 days. Screening cancer detection rates increased from 3.2 per 1,000, to 6.3 per 1,000. In addition, the number of cancers identified by screening increased from 40% to 58%, p = 0.002. Patient satisfaction measured by survey was over 95%, in areas of courtesy, counseling, and overall care. Our study demonstrates that a comprehensive breast center model can increase access to breast care services, improve patient satisfaction and address focal areas of shortage. Furthermore, in the years after the opening of the breast center the cancer detection rate during screening increased, an important observation that needs to be investigated with future studies.
The Breast Journal 14(4):353-6. · 1.64 Impact Factor
