1/5. Shwachman-diamond syndrome in a Mexican family.Shwachman-diamond syndrome (SDS) is an inherited condition with multisystemic abnormalities including pancreatic exocrine dysfunction, neutropenia, short stature, and skeletal abnormalities. In this report, we describe the case of a 14-year-old female with a history of neutropenia, pancreatic exocrine insufficiency and pancreatic endocrine sufficiency, pancreatic lipomatosis (10), and the development of myeloid leukemia. Postmortem examination revealed a high probability of SDS. We also describe the clinical findings in the patient's six siblings, suggesting this as a familial form of SDS. Because the gene(s) responsible for this syndrome have not yet been identified, genetic confirmation is not yet possible. This is the first report in the literature of a Mexican family with probable SDS.- - - - - - - - - - ranking = 1keywords = probability (Clic here for more details about this article) |
2/5. valproic acid-induced neutropenia.OBJECTIVE: To report a case of severe neutropenia caused by valproic acid (VPA). CASE SUMMARY: A 56-year-old white woman with an infectious brain abscess causing tonic-clonic seizure activity was treated with VPA. She developed severe neutropenia after 2 days of VPA therapy. The absolute neutrophil count reached a nadir of 47 cells/mm(3) during VPA use and returned to normal upon its discontinuation. DISCUSSION: VPA is considered to be a well-tolerated antiepileptic drug. While neutropenia has been reported, it has been mild and transient. This patient developed severe neutropenia during effective treatment with VPA, making her significantly susceptible to infection. The Naranjo probability scale indicates VPA as the probable cause of neutropenia in this case. CONCLUSIONS: This report of severe neutropenia caused by VPA emphasizes the importance of monitoring complete blood cell counts during therapy with this agent.- - - - - - - - - - ranking = 1keywords = probability (Clic here for more details about this article) |
3/5. Probable vancomycin-induced neutropenia.OBJECTIVE: To report a case of vancomycin-induced neutropenia and provide a review of the literature. CASE SUMMARY: A 64-year-old white man was treated with intravenous vancomycin 1.5 g/day for finger osteomyelitis. He developed neutropenia after 21 days of vancomycin therapy. The absolute neutrophil count reached a nadir of 418 cells/mm(3) during vancomycin use and returned to normal 7 days after its discontinuation. The eosinophil count was also elevated during the neutropenic episode and probably related to vancomycin. Based on the Naranjo probability scale, the reaction was probably related to vancomycin use. DISCUSSION: Articles describing cases of vancomycin-induced neutropenia were identified. All patients developed neutropenia as a result of vancomycin therapy >/=12 days. Neutrophil counts generally increased following discontinuation of vancomycin. One article reported successful resolution of neutropenia and infection by switching the patient's therapy to the structurally related antibiotic agent teicoplanin. Other patients were continued on vancomycin therapy, and neutropenia was treated with moderate to good success with filgrastim. Rechallenge was not generally attempted. The mechanism of neutropenia caused by vancomycin is unclear, but appears to be immune-mediated. CONCLUSIONS: vancomycin therapy should not be prolonged unless absolutely necessary, and therapy should be reserved for patients with clear indications for the drug, such as infections due to gram-positive organisms resistant to other therapies. patients should have periodic assessment of white blood cell and neutrophil counts with consideration to discontinue vancomycin if neutropenia develops.- - - - - - - - - - ranking = 1keywords = probability (Clic here for more details about this article) |
4/5. intestinal pseudo-obstruction caused by diltiazem in a neutropenic patient.OBJECTIVE: To describe a case of diltiazem-induced intestinal pseudo-obstruction in a neutropenic patient. CASE SUMMARY: A 74-year-old male with newly diagnosed acute myelogenous leukemia developed atrial fibrillation on day 12 of induction chemotherapy. He was initially treated with diltiazem 5 mg intravenously every 5-10 minutes for 5 doses and an amiodarone 150-mg loading dose intravenously. diltiazem 30 mg orally 4 times daily and amiodarone continuous infusion were started thereafter. amiodarone therapy was discontinued after one day due to an untoward adverse effect. The diltiazem dose was then escalated. By day 14, the patient was receiving diltiazem 120 mg orally 4 times daily. On day 15, he developed increasing abdominal distention with hyperactive bowel sounds. On day 16, a radiographic examination showed multiple dilated loops of both the small and large bowel representing possible intestinal pseudo-obstruction; diltiazem was discontinued that day. Starting on day 18, the patient showed recovery of intestinal pseudo-obstruction without intervention. No further GI complications developed during his remaining hospital course. DISCUSSION: intestinal pseudo-obstruction is usually associated with underlying medical conditions such as trauma, infection, cardiac disease, and after surgery. Medications rarely cause such a condition. Detailed examination of the patient's record indicated neither infection nor other medications contributed to the development of intestinal pseudo-obstruction. Additionally, a radiographic examination ruled out neutropenic enterocolitis, a common gastrointestinal complication in neutropenic patients. Use of the Naranjo probability scale indicated a probable relationship between pseudo-obstruction and diltiazem in this patient. CONCLUSIONS: Although calcium-channel blockers rarely cause intestinal pseudo-obstruction, clinicians must be aware of this serious but reversible adverse effect.- - - - - - - - - - ranking = 1keywords = probability (Clic here for more details about this article) |
5/5. thalidomide-associated thrombocytopenia.OBJECTIVE: To report thrombocytopenia in a patient prescribed thalidomide for multiple myeloma (MM). CASE SUMMARY: A 70-year-old woman was diagnosed in 2003 with MM. At diagnosis, melphalan 0.25 mg/kg/day and prednisolone 2 mg/kg/day were started; however, the patient became refractory to therapy. melphalan and prednisolone were discontinued, and monotherapy with dexamethasone 40 mg for 12 days per month was started. The patient's hematologic condition deteriorated again after about one year; dexamethasone was discontinued, and treatment with oral thalidomide 200 mg/day was initiated. About 2 weeks after thalidomide administration, the woman developed disabling adverse effects (flu-like symptoms, swollen fingers, rash and hematoma on her legs, shortness of breath, dry mouth, multiple petechiae). Laboratory testing showed neutropenia (neutrophils 0.4 x 10(9)/L) and thrombocytopenia (platelets 58 x 10(9)/L). thalidomide was promptly discontinued; within 3 weeks, the laboratory values returned to pretreatment levels (1.3 x 10(9)/L and 267 x 10(9)/L, respectively) and her symptoms disappeared. DISCUSSION: thrombocytopenia is a rarely reported hematologic adverse consequence of thalidomide therapy. A recent report identified 5 patients who developed thrombocytopenia while undergoing monotherapy with thalidomide for MM. According to the Naranjo probability scale, thalidomide was classified as the probable cause of thrombocytopenia in our patient. CONCLUSIONS: Unlike other antineoplastic drugs, thalidomide is rarely reported to cause severe hematologic toxicity. We present this case to increase clinicians' awareness for the potential of thalidomide to adversely affect platelet counts, particularly because its effectiveness in MM will likely result in expansion of its clinical use.- - - - - - - - - - ranking = 1keywords = probability (Clic here for more details about this article) |