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Severe pancytopenia and intrahepatic cholestasis in a patient with a history of Hodgkin's disease. Responsiveness to high-dose pulse steroid therapy and diagnosis of a late relapse.

A 47-year-old male, treated 7 years earlier for Hodgkin's disease (HD), was admitted with persistent fever, liver enlargement, and increased cholestasis parameters. He developed acute bone marrow failure and progressive worsening of his clinical condition and cholestasis markers without showing evidence of HD recurrence or second malignancy. High-dose intravenous pulse methylprednisolone therapy was given, after which resolution of pyrexia and progressive improvement in performance status and in hematological counts and cholestasis parameters were observed. During this phase, a bone biopsy showed HD marrow infiltration. This clinical course may reflect tumor cytokine-induced phenomena, significantly affected by high-dose steroids.

Journal Article↗

Melioidosis: insights into the pathogenicity of Burkholderia pseudomallei.

Burkholderia pseudomallei is a potential bioterror agent and the causative agent of melioidosis, a severe disease that is endemic in areas of Southeast Asia and Northern Australia. Infection is often associated with bacterial dissemination to distant sites, and there are many possible disease manifestations, with melioidosis septic shock being the most severe. Eradication of the organism following infection is difficult, with a slow fever-clearance time, the need for prolonged antibiotic therapy and a high rate of relapse if therapy is not completed. Mortality from melioidosis septic shock remains high despite appropriate antimicrobial therapy. Prevention of disease and a reduction in mortality and the rate of relapse are priority areas for future research efforts. Studying how the disease is acquired and the host-pathogen interactions involved will underpin these efforts; this review presents an overview of current knowledge in these areas, highlighting key topics for evaluation.

Bacterial Vaccines↗

Indomethacin treatment of infantile cortical periostosis in twins.

Twin girls presented with infantile cortical periostosis (Caffey's disease) at 2 and 3 weeks of age, respectively. This disorder initially involved their upper and lower limbs and resulted in fever, irritability and tenderness. X-rays showed extensive periosteal new bone formation. Multiple relapses occurred in the first year of life and during some of these relapses mandibular and clavicular involvement was noted. Prednisolone, 1 mg/kg per day, was used to treat relapses until 9 months of age. Indomethacin therapy at this age at a dose of 3 mg/kg per day allowed the cessation of prednisolone therapy and disease flares were thereafter infrequent and responsive to indomethacin.

Cyclooxygenase Inhibitors↗

Clinical efficacy of amoxycillin/clavulanate in laparoscopically confirmed salpingitis.

To test the efficacy and safety of amoxycillin/clavulanate (Augmentin), 102 hospital patients with laparoscopically confirmed acute salpingitis were treated with parenteral amoxycillin/clavulanate (1.2 g qid for three days) followed by oral amoxycillin/clavulanate (two tablets of 625 mg tid for a further six days). Bacteriological samples were obtained from the cervix uteri and the pouch of Douglas. One hundred patients were assessable for clinical outcome using several variables including pain scores. Amoxycillin/clavulanate alone was effective in 95 patients (95%). Three patients (3%) responded to amoxycillin/clavulanate with marked improvement but another antibiotic was subsequently added to their treatment regimen. Treatment failed in two patients. At follow-up two weeks after hospital discharge, three patients (3.8%) had relapsed or were re-infected. Adverse drug events included one case of drug fever, one case of rash and one case of severe diarrhoea. Treatment was stopped in all three cases. Gastrointestinal reactions, mainly mild diarrhoea, were seen in 31 patients. No clinically relevant changes in haematological or clinical chemical indices were attributable to the amoxycillin/clavulanate treatment. We conclude that amoxycillin/clavulanate is a clinically effective and safe treatment for acute salpingitis.

Acute Disease↗

Analysis of lupus activity in end-stage renal disease treated by hemodialysis.

OBJECTIVE: The activity of systemic lupus erythematosus (SLE) has been reported to decrease in patients who have developed end-stage renal disease (ESRD). However, extrarenal symptoms attributable to the disease activity are noted, especially during the first year of dialysis. We studied the clinical course and evaluate the disease activity of SLE in patients with ESRD on hemodialysis for more than 6 months. SUBJECT AND METHODS: Fourteen patients with SLE who had been initiated on maintenance dialysis at our center between 1982 and 1999 were examined retrospectively. Their clinical details, organ system manifestations, serologic profiles and immunosuppressive treatment regimens were reviewed. Patients with and without postdialysis flaras of SLE were compared statistically. RESULTS: Five patients exhibited 6 SLE flares under treatment with corticosteroids. Two flares occurred within the first year of the initiation of dialysis, and in 1 patient, aggravation of the disease activity was noted 98 months after the initiation of dialysis. Polyarthritis was noted in 5 cases and fever in 4 cases. The serum complement levels decreased in all 6 cases with relapse of SLE activity. Compared with the other 9 patients who did not exhibit SLE relapse, no significant differences were found in 5 patients who did with respect to the demographic and serologic features at the initiation of dialysis. CONCLUSION: We conclude that the disease activity does not always burn out in patients of SLE who show progression to ESRD. SLE flares can sometimes occur even after one year of the initiation of dialysis. SLE patients on dialysis should be carefully followed up by clinical and serological monitoring, and treated by appropriate immunosuppressive therapy.

Adolescent↗

Concordance of ulcerative colitis in monozygotic twin sisters.

The etiology of inflammatory bowel disease is multifactorial and appears to combine both genetic and environmental factors. We experienced here a rare occurrence of woman monozygotic twins with ulcerative colitis (UC). A 45-year-old woman (the elder monozygotic twin) was admitted to our hospital because of bloody diarrhea occurring over 10 times per day, abdominal pain and fever. She was diagnosed as UC at the age of 22, and repeated the relapse and remission. She was diagnosed as relapse of UC and total colitis type. Her younger monozygotic twin sister also suffered from UC at the age of 22. Human leukocyte antigen was examined serologically with DNA type in both patients. DRB1*1502, which was previously shown to be dominant in Japanese patients with UC, was not observed in this case. Although the concordance in monozygotic twin in UC is reported to be 6.3-18.8%, the concordant case like this is relatively rare. We report this rare case of UC and the previously reported cases are also discussed.

Colitis, Ulcerative↗

[The efficacy of G-CSF and GM-CSF in the adjunctive treatment of infections complicating chemotherapy of acute leukemia in children].

Growth factors (G-CSF Neupogen Roche i GM-CSF Leucomax Sandoz) have been applied in 133 therapeutic and prophylactic cycles in 88 children with acute leukaemias. GM-CSF and G-CSF were administered subcutaneously or intravenously at a dose of 2 to 8 micrograms/kg for 2 to 28 days. 45 prophylactic cycles had been administered in children with acute lymphoblastic leukaemia in high risk group and in relapses, which caused significant reduction in the number of infections, time of neutropenia and fever. Therapeutic cytokines cycles were applied when the absolute neutrophil count have fallen below 0.5 x 109/l. We observed significant reduction in duration of neutropenia in these cycles. Tolerance of GM-CSF and G-CSF was good. Side effects were not observed.

Adjuvants, Immunologic↗

[Specific gastric localization of Wegener's disease].

We report an unusual gastric ulcer disease in a 52 year old white man which relapsed despite antacids and anti-H2 treatment. During the course of the disease, fever, weight loss, and ocular, oropharyngeal as well as systemic manifestations occurred. Gastric biopsies were normal until macrobiopsy was performed 5 months later demonstrating necrotizing granulomatous vasculitis. Further identical oropharyngeal mucosal lesions and a pulmonary infiltrate occurred supporting the diagnosis of Wegener's disease. The subsequent course was complicated by a massive hemorrhage which required total gastrectomy. Despite specific treatment with cyclophosphamide and steroids, the patient died. Most of the gastrointestinal lesions consistent with Wegener's disease are usually found at necropsy. Clinical gastrointestinal manifestations are scarce and rarely due to the underlying disease. Prognosis depends upon the renal lesions. However, as well, involvement of the digestive tract seems to be a factor of poor evolution of the disease.

Biopsy↗

Hodgkin's and non-Hodgkin's lymphomas masquerading as "idiopathic" liver granulomas.

Three patients presented with liver granulomas and fever. An intensive investigation was initially negative. Corticosteroids were administered with dramatic clinical improvement. Ten months to 3 years later, upon tapering the corticosteroid dose, the initial manifestations reappeared. In two patients Hodgkin's lymphoma was discovered and in a third histiocytic lymphoma. Thus, the diagnosis of lymphoma should be considered in patients with idiopathic liver granulomas, fever, and hepatosplenomegaly without lymph node enlargement, who respond initially to steroid administration but relapse upon tapering the dose.

Adult↗

[Giant cell pneumonia. Complication in one case of acute lymphoblastic leukemia (author's transl)].

A patient suffering from acute lymphoblastic leukemia, in complete remission for two years, is treated for haematologic relapse with V.P.D. and C.O.A.P. consolidation. After this treatment, develops tiredness, sleepiness, a slight fever and cough, dying some days after, of interstitial pneumonia. Post-mortem anatomic-pathological studies, show giant cell multinucleated pneumopathia, with intranuclear inclusions bodies, that in ultrastructural level resembles paramyxovirus. When this complication took place, the patient had a brother with measles, but he hasn't, the typical symptomatology of said virus disease. According to Siegel, authors point out the frequency of death due to interstitial pneumonia as a complication caused by measles in immunodeficient patients, remarking the importance of an immediate diagnosis and its' prophylaxis.

Antineoplastic Agents↗

Typhoid and paratyphoid fever: a 10-year retrospective study of 41 cases in a Parisian hospital.

BACKGROUND: Enteric fever remains a major cause of fever in travelers. We evaluated new trends in enteric fever. METHODS: We reviewed the epidemiological, clinical, biological, bacteriological data, and outcome of all cases of typhoid and paratyphoid fever seen in our department over the last decade. The inclusion criteria were the presence of signs compatible with enteric fever and isolation of Salmonella typhi or Salmonella paratyphi A, B, or C from blood or stool cultures or any other site. RESULTS: Among the 41 patients, 38 (93%) had travel-associated enteric fever. The main geographic source of contamination was the Indian subcontinent. One patient had been vaccinated with parenteral Vi vaccine 1 year previously. Fever and headaches were the only signs which were present in more than 80% of patients. The Widal test at inclusion was positive in 27%, and a second serological test was found to be positive in 50% of evaluated cases. Blood cultures and stool cultures were positive in 34 cases and 10 cases, respectively. Salmonellae spp were isolated in both hemocultures and stool cultures in 4 cases and in urine in 1 case. Two strains of S. typhi were resistant to ampicillin, chloramphenicol, and trimethoprim-sulfamethoxazole. One strain of S. typhi and one of S. paratyphi B were nalidixic acid resistant. All evaluable patients were cured with the exception of 2 patients (1 failure, 1 relapse). We observed 3 toxic reactions. No patients died. CONCLUSION: The diagnosis and outcome of enteric fever are hampered by the lack of specificity of clinical and biological signs, the increasing rates of antimicrobial resistance, and the occurrence of toxic reactions during treatment.

Adolescent↗

[High-dose chemoradiotherapy combined with recombinant human granulocyte-macrophage colony-stimulating factor (rhGM-CSF) followed by autologous peripheral blood stem cell transplantation (PBSCT) in a case of refractory acute myelogenous leukemia (AML)].

Recombinant human granulocyte-macrophage colony-stimulating factor (rhGM-CSF) stimulates the growth of myeloid leukemic cells and increases their susceptibility to cell-cycle specific agents. We treated a patient with acute myelogenous leukemia (AML) in a state of second resistant relapse, with high-dose chemoradiotherapy combined with rhGM-CSF (total body irradiation: TBI 3Gy x 4, on days -8 & -7; cytosine arabinoside: Ara-C 3g/m2, iv, q12h, on days -5-2; rhGM-CSF 250 micrograms/m2/day, cont.iv, on days -5-2) followed by autologous peripheral blood stem cell transplantation (PBSCT). In this case, rhGM-CSF enhanced the proliferation of leukemic cells in vitro. The test dose of rhGM-CSF (84 micrograms/m2 over 8 hours) also promoted leukemic cell proliferation in vivo, resulting in an increase in the percentage of leukemic cells in the peripheral blood and reappearance of chromosomal aberrations in the bone marrow. The toxicity of rhGM-CSF-combined conditioning regimen included fever and mild liver damage. The patient achieved a complete remission lasting for 2 months, then relapsed. The rhGM-CSF-combined conditioning regimen was tolerated by this patient, but further studies will be required to confirm not only its safety but also its effectiveness in the treatment of refractory AML.

Adult↗

[The treatment of relapsing polychondritis using sulfones. One case (author's transl)].

A case of relapsing polychondritis was successfully treated with a daily dose of 120 mg of diaminodiphenylsulfone. Clinical signs (fever, fatigue, conjunctivitis, chondritis and hoarseness of the voice) rapidly regressed and remain so 8 months later, despite a decrease in the dose. Biological parameters returned to normal (sedimentation rate, plasma perchlorosoluble seromucoids, urinary acid mucopolysaccharides), and histological and ultrastructural lesions of the auricular cartilage disappeared. The pathogenic role of a herpes infection at the beginning of the disease is discussed.

Aged↗

[Relapsing polychondritis developing in a patient with Graves' disease: a possible association with propylthiouracil].

A 31-year-old woman presented 1993 with fever, painful swelling of cartilaginous portions of the ears and the bridge of nose, polyarthralgia including costochondroral pains, and episcleritis. She has been taking propylthiouracil since 1991 when she was diagnosed as Graves' disease. Laboratory evaluations revealed an elevated erythrocyte sedimentation rate (ESR) of 133 mm/h, a high CRP level of 13.2 mg/dl and positive antinuclear antibodies and anti-type II collagen antibodies. Histopathological findings of the biopsy specimen from the auricular cartilage included chondrocyte degeneration, matrix destruction and inflammatory cell infiltration. She was diagnosed as RP and treatment with 30 mg/day of prednisolone dramatically improved all symptoms and signs, accompanied by a fall in ESR, CRP and autoantibodies. When prednisolone was tapered to 5 mg/day, a clinical relapse occurred. After discontinuation of propylthiouracil, she has been well without prednisolone. Propylthiouracil-induced SLE-like syndrome or antineutrophil cytoplasmic antibodies (ANCA) related angitis has been reported previously. In addition, recent studies demonstrated that about 20% of sera from patients with relapsing polychondritis are P-ANCA positive. This is the first report suggesting a possible association between the development of relapsing polychondritis and propylthiouracil.

Adult↗

Bacillus cereus causing fulminant sepsis and hemolysis in two patients with acute leukemia.

PURPOSE: Hemolysis is so rarely associated with Bacillus cereus sepsis that only two very well documented cases have been reported. This article reports two unusual cases of Bacillus cereus sepsis with massive intravascular hemolysis in patients who had acute lymphoblastic leukemia (ALL). PATIENTS AND METHODS: A 20-year-old woman who was 9 weeks pregnant experienced a relapse of ALL. A therapeutic abortion was performed. During week 4 of reinduction the patient had abdominal pain, nausea, and vomiting, with severe neutropenia but no fever. Her condition deteriorated rapidly with cardiovascular collapse, acute massive intravascular hemolysis, and death within hours of the onset of symptoms. Blood cultures were positive for Bacillus cereus. Postmortem histologic examination and cultures revealed Bacillus cereus and Candida albicans in multiple organs. The second patient, a 10-year-old girl, presented with relapsed T-cell ALL. In the second week of reinduction, she had abdominal pain followed by hypotension. Again, no fever was noted. Laboratory studies showed intravascular hemolysis 12 hours after admission. Aggressive support was promptly initiated. Despite disseminated intravascular coagulation; cardiovascular, hepatic, and renal failure; and multiple intracerebral hypodense lesions believed to be infarcts, the patient recovered fully and resumed reinduction therapy. CONCLUSIONS: Bacillus cereus infection can have a fulminant clinical course that may be complicated by massive intravascular hemolysis. This pathogen should be suspected in immunosuppressed patients who experience gastrointestinal symptoms and should not be precluded by the absence of fever, especially if steroids such as dexamethasone are being given. Exchange transfusion may be lifesaving in Bacillus cereus septicemia associated with massive hemolysis.

Adult↗