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Query: UMLS:C0027947 (neutropenia)
 17,527 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a patient with the syndrome of large granular lymphocytes in whom the initial clinical features were polyarthritis, hepatosplenomegaly and neutropenia. Relative lymphocytosis was also demonstrated at the expense of a subpopulation with morphology and surface markers characteristic of large granular lymphocytes (CD2+, CD8+, CD16+ and HNK-1+). After 6 months of asymptomatic course, without changes in clinical or laboratory data, the patient died from an acute abdomen with mesenteric ischemia of different likely causes as suggested by necropsy data (multivisceral diffuse infiltrate by large granular lymphocytes, systemic vasculitis and Clostridium sepsis). The association between this syndrome and systemic vasculitis is discussed.
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PMID:[Vasculitis associated with proliferation of large granular lymphocytes]. 225 May 16

In a series of 320 patients with lymphoid neoplasms treated with polychemotherapy, three patients with non-Hodgkin's lymphoma and one with myeloma were diagnosed as having neutropenic enterocolitis (NEC). All patients were adult, all had received multiple chemotherapeutic drugs and, during neutropenia, they had clinically presented with fever and abdominal pain, generally in the right lower quadrant. The diagnosis was clinical in all cases, and the imaging techniques provided only the suspicion of retro-cecal abscess in one of them. Two patients were operated on because of the development of features of peritoneal involvement, another because of septic shock and another because of retro-cecal abscess. Surgery and pathological study confirmed the diagnosis. The fundamental findings were ileocecal wall edema, mucosa ulceration, local necrosis, hemorrhage and thrombosis, and clusters of bacterial colonies without evidence of granulocytic or tumoral infiltration. NEC can develop with varying types of morphological involvement resulting in a highly variable clinical severity spectrum ranging from nonspecific abdominal symptoms to acute abdomen. Thus, diagnosis is very difficult and is only possible with a high suspicion index. It should rely on clinical data, which are unique, to assess the evolution and to indicate medical or surgical therapy. These therapeutic modalities should be individualized in each patient. All physicians treating neutropenic patients should be familiar with this condition and consider it in the differential diagnosis of abdominal pain.
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PMID:[Neutropenic enterocolitis during treatment of lymphoproliferative neoplasms]. 261 46

A 57-year-old patient with chronic granulocytic leukemia in blast crisis and severe neutropenia is presented. This patient developed right sided peritonitis due to an isolated transmural granulocytic sarcoma of the terminal ileum. The affected segment was resected and the patient survived 4 more months. Thus, despite neutropenia, an aggressive surgical approach should be considered in a leukemic patient presenting with unexplained acute abdomen, since, as demonstrated here, a localized lesion which could not have otherwise been detected, was ultimately found and promptly resected.
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PMID:Acute abdomen due to granulocytic sarcoma of the terminal ileum. 318 48

The association between Clostridium septicum sepsis in patients with malignant disease has been frequently documented. A presentation with fever, neutropenia, vomiting, and an acute abdomen is characteristic of this anaerobic infection which has been uniformly fatal in children with acute leukemia. We report the unusual course and the successful treatment of an adolescent with an abdominal Burkitt's lymphoma with leukemic transformation and clostridium septicum sepsis and cellulitis.
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PMID:Successful therapy of Clostridium septicum sepsis in a child with Burkitt's lymphoma. 678 26

Recombinant human (rh) granulocyte-macrophage colony-stimulating factor (GM-SCF) is currently being tested in clinical trials for the treatment of acute myeloid leukemias with two main intentions: reduction of neutropenia and recruitment of leukemic blasts into cell cycle to enhance cytarabine (ara-C) mediated cytotoxicity. We report a case of a fatal spleen rupture in a patient with acute monocytic leukemia (AML M5b) who was treated according to a clinical phase I/II protocol with rh GM-CSF priming and standard induction chemotherapy TAD 9 (thioguanine/ara-C/daunorubicin). During treatment we observed rapidly rising peripheral blast counts and the development of an acute abdomen. Ultrasound examination revealed splenomegaly due to diffuse cellular infiltration and spleen rupture. The patient died 17 days later due to pneumonia and renewed spleen hemorrhage. Bone marrow progenitor assays before treatment showed exclusive growth of monocytoid blast cell colonies (CFU-L). Colony growth could be stimulated with rh GM-CSF and blocked dose-dependently by a monoclonal anti-GM-CSF antibody. CFU-L proliferation also increased after stimulation with rh interleukin-3 (rh IL-3) and supra-additively with rh granulocyte colony-stimulating factor (rh G-CSF) combined with rh GM-CSF. Furthermore, rh GM-CSF induced surface marker expression of CDw 65 and CD 11b on isolated CFU-L blasts. After short-term suspension culture, rh GM-CSF enhanced the expression of CD 29- and CD 11b-adhesion molecules on peripheral blast cells. In summary, this case represents a fatal spleen rupture occurring during rh GM-CSF priming and induction chemotherapy for acute monocytic leukemia. Although the etiology of this spleen rupture remains uncertain, in view of our data we suggest special caution, when further testing this therapy protocol in acute leukemias with monocytic subtype and high peripheral blast cell counts.
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PMID:Fatal spleen rupture during induction chemotherapy with rh GM-CSF priming for acute monocytic leukemia. Clinical case report and in vitro studies. 845 Jun 76

The clinical course of patients with hematological disease, especially after treatment, is often complicated by gastrointestinal infections. Between 1986 and 1990 a total of 18 patients affected with hematologic disease and presenting with an acute abdomen were admitted to the surgery department at the University of Rome "La Sapienza". Most patients were affected with acute or chronic myeloid leukemia (61%) and lymphoma. Five patients with acute appendicitis, three with necrotizing enterocolitis, three with spontaneous hemoperitoneum, three with cholecystitis, two splenic infarctions and two intestinal occlusions were diagnosed. Symptoms were often vague and non specific and blood counts revealed neutropenia in all but two patients, while anemia was characteristic in spontaneous hemoperitoneum and in neutropenic enterocolitis. Fungemia occurred in only two cases while bacteremia was present in seven. The most critical patients were those affected by neutropenic enterocolitis and acute cholecystitis. Sonography was meaningful in the diagnosis of hemoperitoneum, splenic infarct and acute cholecystitis. All patients underwent surgical procedures within 48 hours of admission to the department. In all cases peritoneal washing was performed and at least one peritoneal drainage was left. In all cases of necrotizing enterocolitis, intestinal resections, either ileal or colonic, were followed by an immediate anastomosis in two layers. Intensive hematological and antibiotic post surgical care was performed in all patients. Seven patients presented minor complications (38.8%), and only one died (5.5%). Emergency surgical treatment may be safely carried out in patients with hematological diseases presenting with an acute abdomen. Intensive postsurgical care is mandatory for the recovery of patients and the patient's critical condition should not be a deterrent to surgical intervention.
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PMID:The surgical choice in neutropenic patients with hematological disorders and acute abdominal complications. 847 83

Cases of neutropenic enterocolitis associated with Clostridium septicum infection have been reported with increasing frequency in the past decade. We report two such cases involving unusual hosts and briefly discuss possible pathogenetic mechanisms such as ischemia, mucosal damage related to chemotherapy and neutropenia, and immunosuppression. One case involved a young man with chronic Epstein-Barr infection who developed extensive gas gangrene of the right side of his trunk and thigh and who died within 12 hours of presentation to the emergency department. Diagnosis was only made at postmortem examination. The second, middle-aged patient was admitted with an acute abdomen shortly after he completed chemotherapy for pleural mesothelioma. A right hemicolectomy was performed, but the patient developed antibiotic-associated pseudomembranous colitis and died. These cases indicate that neutropenic enterocolitis may arise in a variety of underlying conditions and that prompt diagnosis and therapy will be required to salvage more patients with this disorder.
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PMID:Neutropenic enterocolitis. Two unusual cases with review of the literature. 848 43

A multicentre phase II trial was undertaken to evaluate the activity and toxicity of docetaxel plus cisplatin as first-line chemotherapy in patients with urothelial cancer. Thirty-eight patients with locally advanced or metastatic transitional-cell carcinoma of the bladder, renal pelvis or ureter received the combination of docetaxel 75 mg m(-2) and cisplatin 75 mg m(-2) on day 1 and repeated every 21 days, to a maximum of six cycles. The median delivered dose-intensity was 98% (range 79-102%) of the planned dose for both drugs. There were seven complete responses and 15 partial responses, for and overall response rate of 58% (95% CI, 41-74%). Responses were even seen in three patients with hepatic metastases. The median time to progression was 6.9 months, and the median overall survival was 10.4 months. Two patients who achieved CR status remain free of disease at 4 and 3 years respectively. Grade 3-4 granulocytopenia occurred in 27 patients, resulting in five episodes of febrile neutropenia. There was one toxic death in a patient with grade 4 granulocytopenia who developed acute abdomen. Grade 3-4 thrombocytopenia was rare (one patient). Other grade 3-4 toxicities observed were anaemia (three patients), vomiting (five patients), diarrhoea (four patients), peripheral neuropathy (two patients) and non-neutropenic infections (seven patients). Docetaxel plus cisplatin is an effective and well-tolerated regimen for the treatment of advanced urothelial cancer, and warrants further investigation.
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PMID:Phase II multicentre study of docetaxel plus cisplatin in patients with advanced urothelial cancer. 1187 92

Clostridium myonecrosis is a rare and deadly infection that progresses very rapidly; thus, prompt diagnosis and treatment is vital. In adults, clostridial myonecrosis used to be a well-known complication of war wounds. Today, it is usually seen in settings of trauma, surgery, malignancy, skin infections/burns, and septic abortions. More recently, cases of nontraumatic or spontaneous clostridial myonecrosis have been reported in both adults and children. Clostridium perfringens and Clostridium septicum are responsible for the majority of the clinically relevant infections. Higher mortality rates are seen when C septicum is the causative agent. Here we present a child who survived a severe case of C septicum myonecrosis involving both abdominal and thoracic cavities. This rare infection has a high mortality rate and might be easily misdiagnosed in children, even by experienced clinicians, because of its nonspecific presentation. We also review all reported pediatric cases of C septicum infection and myonecrosis and discuss the surgical and medical interventions associated with improved survival. We identified a total of 47 cases of C septicum infection; of these, 22 (47%) were cases of C septicum associated with myonecrosis. Several factors, if available, were analyzed for each case: age, gender, infection location, previous diagnoses, presenting signs and symptoms, neutropenia, gross pathology of the colon, antibiotic use, surgical intervention, and final outcome. We found that conditions related with C septicum infection in children can be grouped into 3 major categories: patients with neutrophil dysfunction; patients with associated bowel ischemia; and patients with a history of trauma. Malignancies were found in 49% of the cases, cyclic or congenital neutropenia in 21%, hemolytic-uremic syndrome in 11%, structural bowel ischemia in 4%, and local extremity trauma in 6%. In addition, 6% of the cases had no known underlying disorder. Abdominal symptoms including vomiting, diarrhea, blood per rectum, abdominal pain, anorexia, and/or acute abdomen, were reported in 85% of the children. Fever was also a common finding. The mainstay of treatment for C septicum infection was parenteral antibiotics and/or surgical intervention. The mortality rate for children with C septicum infection and myonecrosis was 57% and 59%, respectively. Although 82% of all cases received antibiotics, only 43% underwent therapeutic surgical intervention. Several clinical factors were found to be associated with improved survival. Only 35% of the children with gastrointestinal tract involvement survived, compared with 86% of the children without gastrointestinal tract involvement. The survival rates for other conditions ranged from 0% to 50%. One hundred percent survival was reported in patients with no previously diagnosed conditions and those with infections resulting from trauma to the extremities. All survivors received antibiotic treatment, compared with only 68% of the nonsurvivors. Most survivors (84%) underwent therapeutic surgical intervention, compared with only 12% of nonsurvivors. Other treatments were used adjunctively, including hyperbaric oxygen, granulocyte colony-stimulating factor, granulocyte transfusions, and intravenous immunoglobulin. C septicum infections in children are often fatal; thus, one needs to have a high index of suspicion in at-risk patients. This review describes who these patients are, their clinical presentation, and the therapeutic strategies associated with improved survival.
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PMID:Clostridium septicum infections in children: a case report and review of the literature. 1656 92

Omental infarction is a rare cause of acute abdomen in childhood. We describe a case of omental infarction mimicking acute appendicitis occurring in a child with cyclical neutropenia. Neutropenic enterocolitis, a serious cause of the acute abdomen, has been linked with cyclical neutropenia. In neutropenic patients, omental infarction when diagnosed pre-operatively can be managed conservatively with the focus on improving the neutrophil count. If after imaging the diagnosis is in doubt, there should be a low threshold for laparoscopy. The low incidence of omental infarction will continue to mean that it is a diagnosis made at operation for suspected appendicitis. In these cases, the infarcted tissue may be removed by the laparoscopic or open technique.
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PMID:Unusual cause of acute abdomen--omental infarction occurring in a child with cyclical neutropenia. 2035 33


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