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PubMed1999

Improved survival with plasma exchange in patients with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome.

PURPOSE: Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome are uncommon disorders that are generally fatal if left untreated. Plasma exchange therapy is associated with high response rates and improved short-term survival, but most previous studies have been limited by small numbers of patients or short duration of follow-up. METHODS: We performed a retrospective cohort analysis in 126 consecutive patients with thrombotic thrombocytopenic purpura/hemolytic uremic syndrome, most of whom were treated principally with plasma exchange at the Sacramento Medical Foundation Blood (Center and the University of California Davis Medical Center between 1978 and 1998. We measured the effect of therapeutic plasma exchange on 30-day mortality, response rate, and overall survival, and determined which factors were associated with 30-day mortality and relapse. RESULTS: The overall 30-day mortality was 10% of the 122 patients who received plasma exchange as their principal treatment (a median of 9 exchanges and a mean cumulative infused volume of 43 +/- 77 L fresh frozen plasma); 56% were complete responders and 21% were partial responders. The relapse rate was 13%. The estimated 2-year survival was about 60%; among patients without serious underlying comorbid conditions, the estimated 2-year survival was about 80%. Each unit increase in clinical severity score (on a 0 to 8 scale) was associated with a 2.2-fold (95% confidence interval [CI]: 1.3 to 3.9) increase in the odds of 30-day mortality. Patients who were febrile at presentation were substantially less likely to suffer a relapse (odds ratio = 0.2; 95% CI: 0.03 to 0.9). CONCLUSION: Plasma exchange therapy produced high response and survival rates in this large cohort of patients with thrombotic thrombocytopenic purpura/hemolytic uremic syndrome. The Clinical Severity Score may be useful in predicting 30-day mortality, whereas fever at onset was associated with a lesser risk of relapse. Prospective studies should stratify patients according to these prognostic factors.

Adult↗
PubMed2005

[A case of Wegener's granulomatosis without PR3-ANCA at relapse].

A 49-year-old man was admitted to our hospital with fever. His chest radiograph showed some nodules in the right upper and lower lung fields. The cytoplasmic-antineutrophil cytoplasmic antibody test was positive, and histopathologic biopsy of a small nasal polyps yielded a diagnosis of Wegener's granulomatosis. He was started on prednisolone and cyclophosphamide. The findings on his chest radiograph and his symptoms improved rapidly, and we stopped these drugs after one year. Two years after cessation of treatment, his chest radiograph showed two nodules with cavities. Relapse of Wegener's granulomatosis was diagnosed. The proteinase 3-antineutrophil cytoplasmic antibody test was negative. He was started on prednisolone and cyclophosphamide, and the findings on his chest radiograph improved rapidly. Chest radiographs are useful for follow-up observation of patients with Wegener's granulomatosis after treatment.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed2004

Results of a phase II multicenter trial of single-agent gemcitabine in patients with relapsed or chemotherapy-refractory Hodgkin's lymphoma.

This study's objective was to determine the efficacy and safety of gemcitabine in patients with relapsed or chemotherapy-refractory Hodgkin's lymphoma (HL). Twenty-nine patients were enrolled. Eight of the first 10 patients received intravenous gemcitabine (1250 mg/m2) days 1, 8, and 15 every 4 weeks. Two patients withdrew consent before treatment. Because of toxicity, the remaining 19 patients received 1000 mg/m2 on days 1 and 8 every 3 weeks. Of the 29 treated patients, 16 (55.2%) were male, the median age was 43 years (range, 20.9-77.3), and 89.7% of them were white. Twelve patients (41.4%) had an Eastern Cooperative Oncology Group performance status (PS) of 0, 14 (48.3%) had a PS of 1, and 3 (10.3%) had a PS of 2. All patients had >/= 2 prior chemotherapy regimens. Eighteen patients (62%) had a relapse following bone marrow transplantation. Of 27 evaluable patients, 6 (22%) had partial response, 14 (52%) had stable disease, and 7 (26%) had progressive disease. The median time to progression for all patients was 6.4 months (range, 1.1-21.9). The median survival for all patients was 26.9 months (range, < 1-28.4). All patients have discontinued treatment because of disease progression or relapse. Grade >/= 3 toxicity occurred in 14 patients (48.3%): thrombocytopenia (33.3%), neutropenia (29.6%), anemia (7.4%), increased alanine aminotransferase, reduced cardiac function, and fever (3.7% for each event). This study confirms the activity of gemcitabine in relapsed and highly refractory HL. Dose and schedule may be modified in the future to optimize responses. As gemcitabine is active in highly refractory/relapsed HL, future studies should consider incorporating gemcitabine in combination regimens as first-line therapy for patients with high-risk HL.

Adult↗
PubMed2001

Successful use of recombinant tissue plasminogen activator in a patient with relapsing peritonitis.

Intraperitoneal (IP) administration of either streptokinase (SK) or urokinase (UK) has assumed an adjunctive role to antibiotic therapy in selected patients with relapsing peritonitis. In these circumstances, bacteria may be protected from antibiotics through sequestration in either fibrinous structures or biofilms within the lumen of the peritoneal dialysis (PD) catheter or the peritoneal cavity. In some cases, it appears that disruption of these sheltered microenvironments by thrombolytic agents facilitated eradication of the offending organism and obviated the need for catheter removal, replacement, or interim hemodialysis. Although IP SK has been generally well tolerated as additive therapy in relapsing peritonitis, sporadic reports of significant complications, such as abdominal pain, fever, and severe hypotension, have precluded its more widespread acceptance. The only other thrombolytic agent used in this setting, UK, is presently unavailable because of a manufacturing shortfall. Therefore, adjunctive thrombolytic therapy for relapsing peritonitis is currently restricted. To circumvent these limitations, we devised an IP tissue plasminogen activator (tPA) protocol to eliminate recurring infection in a patient undergoing chronic ambulatory PD. After a third episode of peritonitis caused by Enterobacter cloacae, treated twice previously with an adequate antibiotic regimen, we instilled 6 mL of tPA (1 mg/mL) into the PD catheter for a 2-hour dwell time. The treatment was well tolerated and, in conjunction with a third course of antibiotic therapy, has produced an infection-free interval of 8 months.

Adult↗
PubMed1999

[Adjuvant therapy of colon cancer stage C MAC. Adverse effects and efficacy in the Department of Oncology, Soroka Medical Center in the years 1991-1994].

The National Institutes of Health (NIH) held a consensus conference which recommended 5-FU and levamisole as adjuvant chemotherapy for colon cancer MAC (Modified Astler Coller) stage C. From 1991-1994, 37 such patients diagnosed here were treated with 5-FU (intravenous dose of 450/mg/m2/d for 5 days and from day 29, once a week for 48 weeks) and oral levamisole (50 mg 3 times/d. for 3 days, every 2 weeks for a year), as suggested by NIH guidelines. 16 patients were males and 21 were females, mean age was 62 years and median 64. Cancer locations were: right colon (in 16, 43%), left colon (19, 51%), multiple colon primaries (2, 1%). 25 (68%) had 1-3 positive lymph nodes and 12 (32%) had 4 or more positive lymph nodes. Only 20 (54%) finished treatment as prescribed. In the others, 1 or both drugs caused side-effects for which the drugs had to be stopped. 6 patients relapsed while on treatment. The most common side-effects were diarrhea, stomatitis and bone marrow suppression. 3 were hospitalized due to neutropenic fever. 5-year actuarial survival of all patients was 61%; 5-year relapse-free survival was 61%; 5-year relapse-free survival of right versus left colon was 41% and 82%, respectively (p < 0.01). There was no significant difference in 5-year survival of those with 1-3 positive lymph nodes as compared to those with 4 or more (62% and 56%, respectively). 5-year survival in those who finished or did not finish treatment (excluding those who stopped treatment because of progressive disease) was 83% and 70%, respectively (NS). The 5-year survival of our series was similar to that of patients treated similarly elsewhere. The 5-FU and levamisole treatment was not tolerated well by our study population. It has recently been replaced in our service by a 5-FU and leucovorin regimen given for 6 months.

Adenocarcinoma↗
PubMed2002

Irinotecan in relapsed or refractory non-Hodgkin's lymphomas. Indications of activity in a phase II trial.

Because irinotecan (CPT-11, Camptosar) is a topoisomerase I inhibitor with a broad spectrum of antitumor clinical activity, we investigated its activity in relapsed or refractory non-Hodgkin's lymphomas (NHLs). Irinotecan at 300 mg/m2 i.v. was administered every 21 days with intensive loperamide management of diarrhea. Responders received up to six treatment cycles. Of 44 registered patients, 32 are evaluable for response. Seventeen patients had received one previous regimen, and 15 patients had received two. Disease was refractory to the regimen preceding irinotecan in 12 patients. At baseline, serum lactate dehydrogenase levels were high in 47% (14/30), and beta-2-microglobulin levels were higher than 3.0 mg/L in 29% (8/28) of patients. Responses were seen in 12 of 32 (38%) patients (95% confidence interval [CI] = 21%-56%). Response rates were 43% for seven indolent (95% CI = 10%-82%), 0% for three mantle cell (95% CI = 0%-71%), 44% for 18 relapsed aggressive (95% CI = 22%-69%), and 20% for five refractory aggressive NHLs (95% CI = 1%-72%). Grade 3/4 toxicities included myelosuppression, neutropenic fever, and diarrhea. Irinotecan appears active and relatively well tolerated in patients with relapsed aggressive or indolent NHL. Accrual to this study is continuing for better determination of response rates in all histologic subtypes of NHL.

Adult↗
PubMed1977

Neutropenia, fever, and infection in children with acute lymphocytic leukemia.

In an attempt to determine the relationship between neutropenia (absolute granulocyte count less than 1,000/cu mm), infection, and disease status, 20 patients with acute lymphoblastic leukemia were observed for a total of 34 patient-years. Febrile episodes occurred with much greater frequency in patients during the course of treatment induction (0.9/mo), or while in relapse (2.46/mo) than while in remission (0.19/mo). A cause for fever was identified much more frequently in patients in remission, both when neutropenic and nonneutropenic. When absolute granulocyte counts fell below 200/cu mm, a cause for fever was generally identified regardless of disease status. We propose that the majority of febrile episodes in patients at the time of induction of treatment or in relapse with neutrophil counts of more than 200/cu mm are caused by the disease process rather than secondary to a diagnosable infection.

Agranulocytosis↗
PubMed2001

L-asparaginase induced durable remission of relapsed nasal NK/T-cell lymphoma after autologous peripheral blood stem cell transplantation.

A 60-year-old Japanese woman who presented with right nasal congestion and high fever was admitted to our hospital in March 1999. She was diagnosed with nasal NK/T-cell lymphoma clinical stage IVB. Because her NK/T-cell lymphoma was highly aggressive and chemo-resistant, she underwent autologous peripheral blood stem cell transplantation (PBSCT). The patient received a pretransplantation conditioning regimen of ranimustine, etoposide, carboplatin, and cyclophosphamide. On July 29, 1999, 1.0 x 10(6)/kg CD34+ cells were infused. The patient achieved first complete remission. In January 2000, NK/T-cell lymphoma relapsed in the skin and fever developed. CHOP (cyclophosphamide, vincristine, doxorubicin, and prednisolone) was administered, resulting in partial regression of the skin lesions, but fever persisted. L-asparaginase (L-Asp) at a dose of 6,000 U/m2 per day was administered for 7 days, resulting in the complete disappearance of the skin lesions and resolution of the fever. The patient has been in second complete remission for more than 18 months since the completion of L-Asp treatment (as of July 2001). The effect of L-Asp in this patient was dramatic. Several cases have been reported describing the effectiveness of L-Asp in patients with nasal lymphoma and cutaneous T-cell lymphoma. A front-line chemotherapy regimen containing L-Asp for NK/T-cell lymphoma may warrant further evaluation.

Antineoplastic Agents↗
PubMed2000

Surgical aspects of endocarditis.

Endocarditis represents a difficult medical problem that must occasionally be treated surgically. Constitutional symptoms of infection are important and are usually the reason that the patient seeks medical attention. Fever is the most common sign of infective endocarditis and a heart murmur, which changes in character or is new, is a significant hallmark. The diagnosis for infective endocarditis is made by high index of suspicion in a patient with valvular heart disease or a prosthetic heart valve and in the presence of fever and a cardiac murmur. A positive blood culture is the hallmark of the diagnosis. The absolute indications for operative intervention are congestive heart failure, unstable prosthetic valve, uncontrolled infection, and relapse after optimal therapy (prosthetic valve). Relative indications for operative intervention are perivalvular extension of the infection, staphylococcal infection of a prosthesis, persistent fever (culture negative), large vegetation, or relapse after optimal therapy (native valve). The principles of surgical management are to remove all infected tissue by thorough debridement back to normal tissue. This is combined with replacement of damaged valves and repair of associated defects. The mortality after operation for infective endocarditis is 15-20%. Late survival after operation for infective endocarditis on a native heart valve is 70-80% at 5 years. Survival falls to 50-80% at 5 years for surgery on an infected prosthetic heart valve.

Journal Article↗
PubMed1985

Treatment of four patients with erythrophagocytic lymphohistiocytosis by a combination of epipodophyllotoxin, steroids, intrathecal methotrexate, and cranial irradiation.

Familial erythrophagocytic lymphohistiocytosis, a rare disorder affecting infants, is characterized by a visceral infiltration of histiocytes and lymphocytes resulting in rapid death. It has recently been reported that use of epipodophyllotoxin, VP 16-213, could induce a complete remission of the disease. Such treatment does not, however, prevent fatal CNS relapse. Four patients with the characteristic features of the disease--fever, hepatosplenomegaly, pancytopenia, low plasmatic fibrinogen level, hyperlipidemia, and histiocytic meningitis--are described. These patients were treated with a combination therapy including systemic administration of VP 16-213, steroids, and intrathecal methotrexate followed by cranial irradiation after the age of 12 months. The four patients achieved complete remission of the disease after clearing of the CNS localization. Two patients had secondary relapses, but all four patients have had a disease-free survival exceeding 12 months. All patients have been in remission of the disease for 27, 20, 16, and 13 months, respectively, after disease onset without major setbacks from the treatment. This combination therapy appears to be a promising approach toward long-term remission of the disease.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1984

Schistosoma intercalatum and relapses of Salmonella infection in children.

Twenty-five African children from Libreville had concomitant typhoid or paratyphoid fever (Salmonella typhi, 4 children; Salmonella paratyphi A, 1; S. paratyphi B, 5; S. paratyphi C, 15) and Schistosoma intercalatum infection. In 19 children treated for both infections, no relapse occurred. In the six others, antibiotics alone were given and the Salmonella infection relapsed after 1 month. No relapse occurred after a second course of antibiotics together with treatment for the S. intercalatum infection. This observation suggests that S. intercalatum prolongs Salmonella infection, as do other species of schistosomes. The concomitant treatment of both infections is recommended.

Adolescent↗
PubMed2004

Sweet's syndrome revealing relapsing polychondritis.

Sweet's syndrome is extremely rare in relapsing polychondritis. We report a rare case of Sweet's syndrome revealing relapsing polychondritis. A 77-year-old man presented with fever, associated with respiratory symptoms and non pruritic, painful, erythematous, papules and plaques mainly on the arms and legs. Skin biopsy of the arm found a neutrophilic dermal infiltrate consistent with Sweet's syndrome. A month and a half after admission, the patient presented with ear chondritis and dysphonia. Biopsy of the ear cartilage found a significant perichondral inflammatory cells infiltrate. The diagnosis of relapsing polychondritis was established and a steroid treatment was instituted with a complete regression of the cutaneous signs and chondritis.

Aged↗
PubMed1991

[Successful reinduction of complete remission in a patient with ALL associated with multiple liver abscesses who relapsed after 6 years].

We reported on a 35 year-old patient suffering from ALL with multiple liver abscesses (Journal of Kyusyu Hematological Society, Vol. 31, No. 3 & 4, Dec., 1983). He experienced six years of complete hematological remission from 1983 to 1989, with low fever, positive CRP, polyclonal gamma-globulinemia and elevated alkaline phosphatase. A relapse occurred in June of 1989. A subsequent biopsy revealed fibrosis of the liver attributed to inflammation. At present, he has returned to the complete remission stage with no exacerbation of the liver abscesses.

Adult↗
PubMedDate not supplied

Murine typhus and spotted fever in Israel in the eighties: retrospective analysis.

One hundred and twenty-one cases of murine typhus and spotted fever in Israel between 1976 and 1985 in the Chaim Sheba Medical Center are reviewed. Clinical manifestations of murine typhus were similar to those described previously, but those of spotted fever were different from classical Mediterranean spotted fever: rash was present in 87% but eschar was noted in only two out of 38 patients. The occurrence of relapse in eight of the 16 patients treated with chloramphenicol (seven cases of murine typhus and one case of spotted fever) was striking, particularly since none occurred in 86 patients treated with tetracycline hydrochloride (p less than 0.01). Relapses were milder than the primary illness, except for one which did not receive specific therapy and was fatal.

Chloramphenicol↗
PubMed1993

[Incidence of rheumatic fever in Slovakia during the last 20 years].

The incidence of rheumatic fever has a declining trend in Slovakia. In 1972 there were 26.7 primary attacks and 8.7 relapses per 100,000 children aged 0-14 years, in 1990 there are 0.5 primary attacks and 0.4 relapses and in 1991 0.4 primary attacks and no relapses. The interregional (former regions) distribution was to the disadvantage of eastern parts but gradually this was evened out. Rheumatic fever occurs in girls and boys, in the majority mild arthritis forms are involved with unequivocal regression of carditic-arthritic forms. The decline can be ascribed to the rising socio-economic standard and health care.

Adolescent↗
PubMed1995

Poststreptococcal reactive arthritis: a form of acute rheumatic fever in adulthood.

We report the case of a middle-aged woman suffering from a relapsing sterile inflammatory arthritis preceded by a streptococcal infection. This observation suggests that streptococcal infection may need to be included in the list of infections known to precipitate reactive arthritis. The clinical presentation of poststreptococcal arthritis in adulthood and its relationship to acute rheumatic fever is discussed.

Acute Disease↗
PubMed1978

Late prosthetic valve endocarditis: clinical features influencing therapy.

To assess the clinical features which might influence therapy, we studied 43 patients with late prosthetic valve endocarditis (LPVE). Twenty patients (47 per cent) survived. Of patients with streptococcal LPVE 61 per cent (11 of 18) survived compared to 36 per cent (nine of 25) of the patients with nonstreptococcal LPVE (p less than 0.10). Among patients with new regurgitant murmurs 33 per cent (nine of 27) survived versus 69 per cent (11 of 16) with such murmurs (p less than 0.03). Of patients with moderate to severe congestive heart failure (CHF) 16 per cent (three of 19) survived compared to 71 per cent (17 of 24) with mild or no CHF (p less than 0.001). The concurrence of two of these three features, i.e., nonstreptococcal etiology, a new regurgitant murmur or moderate to severe CHF, was associated with a mortality rate of 50 to 90 per cent. Persistent fever during therapy, a regurgitant murmur, atrioventricular conduction disturbances and relapse frequently reflected myocardial invasion. In view of the poor outcome with medical therapy and late reoperation, early surgical intervention should be considered when two of the three features noted are present or when myocardial invasion is suspected.

Adult↗